МИНИСТЕРСТВО ОБРАЗОВАНИЯ И НАУКИ РОССИЙСКОЙ ФЕДЕРАЦИИ
Федеральное государственное бюджетное образовательное учреждение высшего профессионального образования

«Новгородский государственный университет им. Ярослава Мудрого»
(НовГУ)

Е.Р. Барсегян

СБОРНИК
УЧЕБНО-МЕТОДИЧЕСКИХ ПОСОБИЙ 
ПО ДЕТСКОЙ ХИРУРГИИ

Дисциплина «Хирургические болезни»

Великий Новгород
2011
МИНИСТЕРСТВО ОБРАЗОВАНИЯ И НАУКИ РОССИЙСКОЙ ФЕДЕРАЦИИ
Федеральное государственное бюджетное образовательное учреждение высшего профессионального образования

«Новгородский государственный университет им. Ярослава Мудрого»
(НовГУ)

Е.Р. Барсегян

СБОРНИК
УЧЕБНО-МЕТОДИЧЕСКИХ ПОСОБИЙ 
ПО ДЕТСКОЙ ХИРУРГИИ

Дисциплина «Хирургические болезни»

Великий Новгород
2011

УДК 617-089-053.2                                                                               Печатается по решению
ББК – 54.5                                                                                                РИС ИМО НовГУ
С 23

Рецензент:
Доктор медицинских наук, профессор, заведующая кафедрой акушерства, гинекологии и педиатрии Института медицинского образования Новгородского государственного университета им. Ярослава Мудрого Е.И. Ройтман

С 23.   Сборник учебно-методических пособий по детской хирургии / Изд. перераб. и доп. / Е.Р. Барсегян – ассистент кафедры госпитальной хирургии / Великий Новгород, 2011. – 93с.

Аннотация
Учебно-методическое пособие подготовлено заведующей курсом детской хирургии кафедры госпитальной хирургии ИМО НовГУ, урологом-андрологом Детской областной клинической больницы. Автор делится большим научным и практическим опытом врача-профессионала.
Пособие предназначено для студентов 6 курса лечебного факультета медицинских вузов и медицинских факультетов университетов.

УДК 617-089-053.2
ББК – 54.5

© Новгородский государственный
университет, 2011
©Барсегян Е.Р., 2011

Оглавление

Введение	5	
Врожденные пороки желудочно-кишечного тракта	6	
Атрезия пищевода	6	
Врожденный трахеопищеводный свищ 	8	
Врожденная непроходимость кишечника.	9	
Аноректальные пороки развития	14	
Диафрагмальные грыжи	18	
Пороки развития передней брюшной стенки	23	
Грыжа пупочного канатика.	23	
Гастрошизис.	25	
Врожденный пилоростеноз.	26	
Болевой абдоминальный синдром.	28	
Приобретенная кишечная непроходимость.	33	
Острый аппендицит.	38	
Перитонит.	46	
Гнойные заболевания у детей.	53	
Флегмона новорожденных.	56	
Лимфаденит.	59	
Острый гематогенный остеомиелит.	61	
Патология влагалищного отростка брюшины. Паховые грыжи и водянки оболочек яичка у детей	68	
Острые заболевания яичек.	73	
Пороки развития полового члена.	75	
Опухоли.	80	
Доброкачественные опухоли мягких тканей.	82	
Гемангиома.	82	
Лимфангиома.	86	
Остеогенная саркома.	88	
Нефробластома.	90	
Нейрогенные опухоли.	93	
Литература.	93	

ЦЕЛЬ РАБОТЫ: 
Приобретение знаний по хирургическим болезням детского возраста.  Ознакомления с основными принципами работы детского хирурга, тактикой поведения врача общей практики при оказании помощи детям с хирургическими болезнями на первоначальном этапе. Овладение принципами хирургической тактики и выбора метода хирургического  лечения. Данный курс направлен на овладение студентами важными методами и особенностями диагностики и лечения детей любого возраста, начиная с момента рождения ребенка. 

В результате подготовки к занятию студент должен знать:
1.	Эмбриогенез внутренних органов,
2.	Топографо-анатомическое строение и особенности функционирования внутренних органов детей разного возраста,
3.	Пропедевтику педиатрических болезней,
4.	Особенности диагностики хирургических болезней детского возраста,
5.	Основными проблемами  в хирургии детского возраста.

ОСОБЕННОСТИ РАБОТЫ ДЕТСКОГО ХИРУРГА
Особенности детского контингента больных накладывают определенный отпечаток на деятельность персонала детских хирургических учреждений. Это касается общепедиатрической подготовки персонала, взаимоотношения с больным ребенком, деонтологических и некоторых других вопросов.
Детскому хирургу часто приходится дифференцировать хирургические болезни от различных инфекционных заболеваний, наблюдать детей с пороками развития, лечить новорожденных и даже недоношенных детей, поэтому он должен хорошо разбираться в инфекционных болезнях, знать основы генетики, эмбриологии и акушерства. Трудность диагностики некоторых хирургических заболеваний и их вариабельность в значительной степени зависят от фона, на котором протекает заболевание – возраста, развития, общего состояния ребенка, поэтому детскому хирургу совершенно необходимы фундаментальные знания по педиатрии.
Большого внимания заслуживают вопросы взаимоотношения персонала с больными детьми. Учитывая лабильность психики и отсутствие волевых качеств, негативизм ребенка, боязнь остаться в непривычных условиях без родителей, персонал детского хирургического отделения должен проявлять по отношению к своим больным максимальную чуткость и внимание. Детский врач, в частности хирург, является одновременно и воспитателем, поэтому тон и манера разговора с маленьким пациентом имеют огромное значение. Важно заручиться доверием ребенка. Не следует говорить о том, что ему не будет больно, если предстоит заведомо болезненная и неприятная процедура. Правильнее предупредить, что может быть немного больно, но другие дети это легко перенесли. Вообще полезно ссылаться на других детей, которым уже выполнили ту или иную процедуру, и тогда они сами объяснят своему соседу или соседке по палате, "что это не очень страшно". Однако, наряду с внимательным и чутким отношением к ребенку врачу не следует идти на поводу у своего иногда капризного пациента и твердо проводить нужные обследования и лечение.
Большим тактом и чуткостью должен обладать хирург при общении с родителями своих пациентов. Родителям, которые очень волнуются за судьбу ребенка, трудно выслушивать недостаточно серьезные и аргументированные заключения о его состоянии и лечении. Недопустим свободный и небрежный тон врача во время беседы, так как у родителей может сложиться впечатление, что их ребенок попал к несерьезным, легкомысленным врачам. Нужно следить за тем, чтобы не было разноречивых сведений о состоянии ребенка и его дальнейшем лечении, передаваемых различными врачами и сестрами.

ВРОЖДЕННЫЕ ПОРОКИ ЖЕЛУДОЧНО-КИШЕЧНОГО ТРАКТА

АТРЕЗИЯ ПИЩЕВОДА

Основные  вопросы темы:
1.	Понятие атрезии пищевода
2.	Клиника и диагностика
3.	Лечение
4.	
Вопросы контроля исходного уровня:
1.	Строение пищевода
2.	Определение «атрезия»

Атрезия пищевода - тяжелый порок развития, при котором верхний отрезок пищевода заканчивается слепо, нижний - чаше всего сообщается с трахеей. Варианты атрезии пищевода представлены на рисунке 1.

Рис. 1.
Часто атрезия пищевода сочетается с другими пороками развития - врожденными пороками сердца, желудочно-кишечного тракта, мочеполовой системы и др. В 5% случаев атрезия пищевода встречается при хромосомных болезнях. Популяционная частота - 0,3:1000. Сочетание мужского и женского пола 1:1. Развитие порока связано с нарушениями в ранних стадиях эмбриогенеза. Известно, что трахея и пищевод возникают из одного зачатка - головного конца передней кишки. На самых ранних стадиях трахея широко сообщается с пищеводом. Их разделение происходит на 4-5-й неделе эмбриогенеза. При несоответствии направления и скорости роста трахеи и пищевода, а также процессов вакуолизации в солидной ткани, которую пищевод проходит вместе с другими образованиями кишечной трубки в сроки от 20-го до 40-го дня, возможно развитие атрезии пищевода. Из анамнеза беременности типичными являются многоводие и угроза выкидыша в 1 триместре.
Клиника и диагностика. Признаки атрезии пищевода отчетливо проявляются в первые часы после рождения ребенка. Верхний слепой отрезок пищевода и носоглотка переполняются слизью, у ребенка появляются обильные пенистые выделения изо рта. Часть слизи новорожденный аспирирует, возникают приступы цианоза. После отсасывания содержимого носоглотки оно вскоре появляется вновь. Очень быстро в легких начинают прослушиваться хрипы, нарастает одышка.
Диагноз уточняют путем катетеризации пищевода тонким уретральным катетером с закругленным концом.  Катетер вводят через нос; пройдя на глубину 6-8 см, катетер упирается в слепой конец пищевода либо, заворачиваясь,  выходит через  нос  ребенка.  Производят отсасывание слизи. Воздух, введенный в слепой конец пищевода, с шумом выделяется из носоглотки (положительный симптом Эдефанта).
Учитывая огромную важность ранней диагностики атрезии - до возникновения аспирационной пневмонии, зондирование пищевода целесообразно проводить всем младенцам с синдромом дыхательных расстройств сразу после рождения.
Клиническая картина может иметь некоторые особенности в зависимости от формы атрезии. При наиболее частой форме атрезии с дистальным трахеопищеводным свищом выявляется вздутие живота, особенно в эпигастральной области. Выраженность аспирационного синдрома зависит от диаметра трахеопищеводного соустья.
Окончательный диагноз ставят после рентгенологического исследования. Вводят катетер в пищевод до упора, после чего выполняют обзорную рентгенограмму грудной клетки и органов брюшной полости. При атрезии рентгеноконтрастный катетер отчетливо виден в слепом отрезке пищевода. Обнаружение воздуха в желудке и кишечнике указывает на наличие свища между трахеей и абдоминальным отрезком пищевода.
При бессвищевых формах на фоне запавшего живота отмечается полное затемнение брюшной полости.
У детей со свищевыми формами атрезии о длине диастаза между концами
пищевода можно судить в какой-то степени по боковой рентгенограмме. Использование для диагностики рентгеноконтрастных растворов, особенно бариевой взвеси, крайне нежелательно из-за риска аспирационной пневмонии.
Лечение. Только раннее оперативное вмешательство может спасти жизнь ребенку с атрезией пищевода. Уже в родильном доме должна быть начата предоперационная подготовка, включающая аспирацию содержимого рото- и носоглотки каждые 15-20 мин, дачу кислорода, полное исключение кормления через рот. Транспортировка должна быть выполнена специализированной бригадой в максимально короткий срок. Общая длительность предоперационной подготовки определяется, тяжестью нарушений гомеостаза и гемодинамики, дыхательной недостаточностью, степенью дегидратации. При явных признаках аспирации, нарушения дыхания, а тем более при пневмонии или ателектазе необходимо как можно раньше прибегать прямой ларингоскопии с катетеризацией трахеи и аспирации. При неэффективности последней под наркозом проводят бронхоскопию или интубацию трахеи с тщательной аспирацией содержимого. Больного помешают в кувез, обеспечивая непрерывную подачу кислорода, аспирацию содержимого ротоглотки, согревание. Назначают инфузиоиную, антибактериальную, симптоматическую терапию.
Выбор метода оперативного вмешательства определяется формой атрезии и состоянием больного. При наиболее частой форме атрезии с дистальным трахеопищеводным свищом у больных с малым операционным риском (доношенных, без сочетанных пороков жизненно важных органов и симптомов внутричерепной родовой травмы) целесообразно начинать с торакотомии, разделения трахеопищеводного свища. Если диастаз между концами пищевода не превышает 1.5-2 см. накладывают прямой анастомоз. При большом диастазе отрезков пищевода накладывают шейную эзофагостому и гастростому по Кадеру. При несвищевых формах из-за значительного диастаза выполняют операцию гастростомии и эзофагостомии. У больных с высоким операционным риском оперативное вмешательство чаще начинают с наложения двойной гастростомии (первая - для кормления через зонд, введенный в двенадцатиперстную кишку, вторая - для декомпрессии желудка и уменьшения аспирации). Второй этап операции выполняют после улучшения- состояния спустя 2-4 дня.
В послеоперационном периоде продолжают начатую интенсивную терапию. Кормление ребенка осуществляют через зонд, введенный интраоперационно через анастомоз либо в гастростому после восстановления пассажа по кишечнику. На 6-7-е сутки исследуют состоятельность анастомоза. Под контролем рентгеновского экрана через рот ребенка вводят 1-2 мл водорастворимого контрастного вещества. Оценивают проходимость зоны анастомоза, исключают затеки контрастного вещества. При отсутствии осложнений ребенка начинают кормить через рот. Через 2-3 недели после операции проводят контрольную фиброэзофагогастроскопию с оценкой степени проходимости зоны анастомоза, состояния кардии, симптомов эзофагита. Сужение анастомоза, встречающееся в 30-40% случаев, требует бужирования (бужи № 22-24). Длительность бужирования контролируется эзофагоскопией.
Ребенка в послеоперационом периоде в течение первого года жизни необходимо постоянное диспансерное наблюдение. Возможны явления дисфагии, осложняющиеся непроходимостью в области анастомоза, что требует срочной эзофагоскопии. В связи с этим детям на первом году жизни рекомендуется давать гомогенизированную пищевую массу. Недостаточность кардии и желудочно-пищеводный рефлюкс, нередко осложняющий послеоперационный период, клинически проявляются ночными регургитациями, повторными пневмониями, срыгиваниями и требуют своевременной диагностики. В связи с операционной травмой возвратного нерва у детей в ближайшие 6-12 месяцев возможна осиплость голоса. У детей с эзофаго- и гастростомией в возрасте от 2-3 мес. до 3 лет выполняют второй этап операции - пластику пищевода толстокишечным трансплантатом.
ВРОЖДЕННЫЙ ТРАХЕОПИЩЕВОДНЫЙ СВИЩ

Основные  вопросы темы:
1.	Классификация трахеопищеводных свищей
2.	Клиника и диагностика
3.	Дифференциальная диагностика
4.	Лечение
5.	Вопросы контроля исходного уровня:
6.	Строение пищевода и трахеи

Изолированный врожденный трахеопищеводный свищ относится к редким порокам развития: частота его составляет 3-4% среди всех аномалий пищевода. Соустье, как правило, располагается высоко, па уровне VII шейного или I грудного позвонка.
Выделяют три вида трахеопищеводных свищей: узкий и длинный, короткий и широкий (встречается наиболее часто), с отсутствием разделения между пищеводом и трахеей на большом протяжении.
Клиника и диагностика. Выраженность симптомов зависит от диаметра свища и угла впадения  в трахею. Характерные симптомы: приступы кашля и  цианоза, возникающие во время кормления, более выраженные в  горизонтальном  положении  ребенка. При  узких и длинных свищах может отмечаться лишь покашливание ребенка при кормлении. При изменении положения симптоматика уменьшается. У ребенка часто возникает пневмония. Наиболее яркая клиническая картина наблюдается при широких соустьях, когда уже в начале кормления молоко легко проникает в трахеобронхиальное дерево. Быстро развивается острый воспалительный процесс в легких с ателектазами.
Диагностика трахеопищеводного свища трудна, особенно при узких свищах. Комплекс исследований включает рентгенологические и инструментальные методы. Рентгенографию выполняют в горизонтальном положении ребенка. Через зонд, введенный в начальный отдел пищевода, под контролем экрана вводят раствор водорастворимого контрастного вещества. Затекание его в трахею указывает на наличие свища. Информативность этого метода невелика. Большее диагностическое значение имеет трахеоскопия. Трахею осматривают на всем протяжении от голосовой щели до бифуркации. Свищ щелевидной формы располагается по задней поверхности трахеи. Прямой признак свища - появление пузырьков воздуха со слизью.
Дифференциальный диагноз проводят с атрезией пищевода, ахалазией кардии, желудочно-пищеводным рефлюксом, сужением пищевода, дисфагией, связанной с травмой голосовых связок при проведении реанимации в родах, дисфагией центрального генеза.
Лечение только оперативное. Предоперационная подготовка заключается в санации трахеобронхиального дерева, лечении аспирационной пневмонии. С этой целью выполняют санационную бронхоскопию, назначают УВЧ, антибактериальную, инфузионную терапию. Полностью исключают кормление через рот.
Операцию мобилизации, перевязки и пересечения соустья выполняют с использованием правостороннего шейного доступа, реже производят заднебоковую торакотомию. При своевременной диагностике прогноз благоприятный.

ВРОЖДЕННАЯ НЕПРОХОДИМОСТЬ КИШЕЧНИКА

Основные вопросы темы:
1.	Причины непроходимости кишечника
2.	Классификация врожденной кишечной непроходимости
3.	Клиника и диагностика
4.	Лечение и прогноз

Вопросы контроля исходного уровня:
1.	Этапы эмбрионального  развития кишечника
2.	Понятия обтурационной и странгуляционной кишечной непроходимости
Врожденная кишечная непроходимость - одно из наиболее частых страданий, требующих неотложного оперативного лечения. Встречается у детей любого возраста, однако чаще всего в периоде новорожденности. Причинами ее являются различные пороки развития, которые условно могут быть объединены в следующие группы:
•	пороки развития кишечной трубки (атрезии, стенозы);
•	пороки развития, вызывающие сдавление кишечной трубки извне (кольцевидная поджелудочная железа, аберрантный сосуд, энтерокистомы);
•	пороки развития кишечной стенки (аганглиоз, нейрональная дисплазия, гипоганглиоз - болезнь Гиршпрунга);
•	пороки, приводящие к обтурации просвета кишки вязким меконием (мекониевый илеус - муковисцидоз);
•	пороки поворота и фиксации брыжейки (синдром Ледда, изолированный заворот средней кишки, заворот изолированной кишечной петли).
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fffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffffff00040000002701ffff030000000000
Время возникновения этих пороков относится к периоду органогенеза (первые 3-4 нед внутриутробного развития), когда нарушается один из процессов формирования кишечной стенки, просвета кишки, рост или процесс его "вращения".
Пищеварительная трубка в процессе развития проходит "солидную" стадию, когда пролиферирующий  эпителий  полностью  закрывает  просвет кишечника. Возникающий вслед за этим процесс вакуолизации заканчивается восстановлением просвета кишечной трубки, однако в определенных условиях последняя фаза нарушается и кишечный просвет остается закрытым.
Если процесс реканализации нарушен на небольшом участке, то кишечный просвет закрыт тонкой перепонкой - перепончатая атрезия. В тех случаях, когда процесс реканализации уже начался, в перепонке образуются отверстия различной величины - перепончатый стеноз. При закрытии просвета на большом протяжении атрезия имеет характер фиброзного тяжа. Причиной этой формы атрезии может быть недоразвитие соответствующей ветви мезентериального сосуда. Атрезия может быть множественной ("сосисочная форма").
Одновременно с процессами, происходящими внутри кишечной трубки, и ее быстрым ростом в длину происходит внутриутробный нормальный поворот "средней" кишки (от двенадцатиперстной до середины поперечной ободочной). Процесс вращения начинается с 5-й недели внутриутробной жизни и проходит 3 периода.
Первый период вращения продолжается до 10-й недели. В это время кишечная трубка растет быстрее, чем брюшная полость, и часть "средней" кишки выступает в основание пуповины, образуя так называемую физиологическую эмбриональную грыжу.
Часть кишечной трубки вращается вокруг верхней брыжеечной артерии, как вокруг оси, в направлении против часовой стрелки на 90° и 180°. При нарушении вращения на первой стадии кишечная трубка возвращается в брюшную полость общим конгломератом, в результате чего остается общая брыжейка, которая является предрасполагающим моментом к возникновению изолированного заворота. Невозвращение кишечной трубки в брюшную полость формирует такие пороки, как гастрошизис (внутриутробная эвентрания кишечника) и эмбриональная грыжа.
Второй период продолжается от 10-й до 12-й недели эмбриогенеза и заключается в возвращении "средней" кишки в достаточно выросшую брюшную полость, кишка продолжает вращение против часовой стрелки еще на 90°. В случае задержки вращения на этом этапе ребенок рождается с незавершенным поворотом кишечника. При этом "средняя" кишка остается фиксированной в одной точке у места отхождения верхней брыжеечной артерии. Петли тонкой кишки располагаются в правой половине брюшной полости, слепая - в эпигастральной области, а толстая кишка - слева. При такой фиксации имеются условия для развития заворота вокруг корня брыжейки и для развития острой странгуляционной кишечной непроходимости. Слепая кишка, располагаясь в эпигастральной области, фиксируется эмбриональными тяжами, сдавливающими двенадцатиперстную: кишку и вызывающими ее непроходимость. Сочетание сдавления двенадцатиперстной кишки с заворотом "средней кишки" расценивается как синдром Ледда.

Принято различать высокую и низкую врожденную кишечную непроходимость. Уровнем деления служит двенадцатиперстная кишка. 

Таблица 1.
Классификация врожденной кишечной непроходимости

ВЫСОКАЯ	НИЗКАЯ	
ПОЛНАЯ

странгуляционная	ЧАСТИЧНАЯ

обтурационная	ПОЛНАЯ

странгуляционная	ЧАСТИЧНАЯ

обтурационная	
Заворот средней кишки

Синдром Ледда	Атрезия,
Перепончатый стеноз двенадцатиперстной кишки,

Кольцевидная поджелудочная железа,

Аберрантный сосуд	Заворот изолированной кишечной петли,

Ущемленные внутренние и наружные грыжи	Атрезия, стеноз тонкой, толстой, прямой кишок  и ануса,

Болезнь Гиршпрунга,

Энтерокистома,

Меконеальный илеус.	

Таблица 2.
Клиническая характеристика различных видов врожденной кишечной непроходимости

Порок развития	Начало заболевания	Симптомы	Рентгенологи-ческие данные	
		рвота	стул	живот при осмотре	поведение, состояние		
Атрезия двенадцатиперстной кишки
Ниже большого сосочка  двенадцатиперстной кишки	С 1-х суток жизни	Обильная с зеленью, усиливается после кормления	Отсутсву-ет, слизь в прямой кишке	Вздута эпигастральная область, нижняя половина запавшая, живот мягкий безболезненный	Спокойное, эксикоз, токсикоз появляются со 2-х суток жизни	Наличие 2-х газовых пузырей
и двух уровней жидкости, затемнение нижней половины брюшной полости	
Атрезия двенадцатиперстной кишки выше большого сосочка двенадцатиперстной кишки	То же	Срыгивание и рвота молоком	Слизь зеленого цвета, стул скудный	Вздута эпигастральная область. После рвоты вздутие уменьшает-ся, живот мягкий, безболезненный	Спокойное, эксикоз со вторых суток жизни	Растянутый желудок. Отсутствует газ в петлях кишечника	
Стеноз двенадцатиперстной кишки	Постепенное, в течение первой недели зижни	Срыгивание, рвота разной интенсивности с примесью желчи	Склонность к запору, скудный стул, обычной консистенции	Вздутие в эпигастральной области	Спокойное, выражена гипотрофия	Синдром «мегадуаденум» растянутая двенадцатиперстная кишка с уровнем жидкости, замедленная эвакуация контрастного вещества	
Заворот «средней кишки»

Синдром Ледда	 Острое обычно на
 3-5–й день жизни
Подострое, с 4-5 –х суток жизни	Сначала срыгивание, затем рвота с желчью и зеленью. Частая с примесью желчи	Слизь с примесью крови.

Скудный затем отсутствует	Живот запавший	Приступы беспокойства, состояние тяжелое, эксикоз. Токсикоз, шок. Беспокойство, прогрессирует эксикоз, токсикоз	Дуаденостаз, расширение двенадцатипер-стной кишки	
Атрезия тонкой кишки	Со 2-х суток жизни	Обильная кишечным содер-  
жимым	Отсутству-ет	Равномерн-ое вздутие живота,  видимая перистальтика кишечных петель, пальпация безболез-неная	Спокойное нарастание токсикоза и эксикоза, вялость	Множественные уровни жидкости
в верхнем этаже брюшной полости	
Меконеаль-ный илеус	Со 2-х суток жизни	Срыгива-ние, рвота с  незначительной примесью желчи	Отсутству-ет	Вздутие живота, пальпируются заполнен-ные меконием петли тонкой кишки	Беспокой-ное, стонет, тяжелое, токсикоз	Затемнение брюшной полости, уровни жидкости в верхних отделах	

Клиника и диагностика. Основными симптомами врожденной кишечной непроходимости являются отсутствие мекониального стула и рвота с патологическими примесями. Для различных пороков развития характерны свои особенности клинических проявлений, позволяющие не только поставить диагноз непроходимости, но и уточнить ее вид.
Существенную помощь в диагностике оказывает зондирование желудка с оценкой количества и качества желудочного содержимого: большое количество желчи и зелени характерно для высокой непроходимости, кишечное содержимое - для низкой; при непроходимости выше большого сосочка двенадцатиперстной кишки и зоны привратника застойное содержимое не содержит желчи. Обязательно выполняют обзорные рентгеновские снимки брюшной полости в двух проекциях в вертикальном положении ребенка. Два уровня жидкости в верхнем этаже брюшной полости при затемнении нижних отделов свидетельствуют о высокой полной кишечной непроходимости, множественные уровни жидкости в верхнем этаже брюшной полости - о низкой непроходимости.
Высокую кишечную непроходимость дифференцируют от дискинезии желудочно-кишечного тракта, начальных проявлений эзофагогастроэнтероколита, низкую кишечную непроходимость от динамической непроходимости.
Лечение. При врожденной кишечной непроходимости показано оперативное вмешательство. Важным моментом является предоперационная подготовка, специфика которой зависит от вида непроходимости, длительности заболевания и тяжести нарушений гомеостаза, возраста ребенка.
Характер оперативного вмешательства определяется анатомическим вариантом порока развития (расправление заворота, разделение спаек, резекция кишки, дуоденоеюностомия и другие операции).
Прогноз зависит от тяжести порока и сочетанных аномалий, своевременной диагностики, эффективности предоперационной подготовки и интенсивной послеоперационной терапии.
Все дети, оперированные по поводу врожденной кишечной непроходимости, требуют диспансерного наблюдения с коррекцией вскармливания и лечением дисбактериоза, анемии, гипотрофии, ферментной недостаточности.
АНОРЕКТАЛЬНЫЕ ПОРОКИ РАЗВИТИЯ

Основные  вопросы темы:
1.	Классификация аноректальных пороков развития
2.	Клиника различных форм  аноректальных пороков
3.	Лечение и прогноз

Вопросы контроля исходного уровня:
1.	Анатомия аноректальной области.
Пороки развития аноректальной области встречаются с частотой 0,25 -0,66:1000. 
Сочетанные пороки развития (сердце, мочевыделительная система, желудочно-кишечный тракт, опорно-двигательная система, ЦНС) встречаются почти в 30% случаев. Аноректальные пороки часто наблюдаются при следующих синдромах: каудальной регрессии, VACTER, Опица. Опица-Фриса, при хромосомных аномалиях 4р, 13q и т. л. Встречаются в соотношении мужского и женского полов 1:2.
В первые недели внутриутробной жизни у эмбриона заднепроходная (конечная) кишка открывается вместе с каналом первичной почки в одну общую полость - клоаку, которая закрыта клоачной мембраной. На 4-й неделе клоака делится спускающейся вниз перегородкой из мезобласта на две трубки. Передняя образует мочевой пузырь и мочеточники, а из задней, которая составляет продолжение конечной кишки, формируется прямая кишка с задним проходом. С 5-й недели эктодерма приближается к наружной поверхности анальной мембраны и образуется анальная ямка, углубляющаяся навстречу кишке. Процесс перфорации анальной мембраны заканчивается к S-й неделе эмбриогенеза.
Возникновение аноректальных пороков развития зависит от стадии, в которой произошло нарушение нормального эмбриогенеза. Нарушения формирования и разделения внутренней клоаки обусловливают следующие виды пороков: а) сохранение клоаки; б) ректовезикальный свищ; в) пектовагинальный свищ; г) ректовестибулярный свищ; д) атрезию заднего прохода без свища: е) ректоуретральный свищ.
Остановка развития на следующем этапе приводит к рождению ребенка с неперфорированной анальной мембраной или стенозом анальной мембраны. Недоразвитие промежности обусловливает эктопию заднего прохода и прикрытого заднепроходного отверстия с промежностным свищом.
В эмбриогенезе наружный сфинктер заднего прохода развивается самостоятельно. Однако если учесть, что ко времени образования уроректальной перегородки волокна сфинктера клоаки перекрещиваются на высоте центрального ядра промежности, то при отсутствии или резкой недостаточности зачатка первичной промежности происходит неправильная закладка пучков наружного сфинктера. При наиболее выраженных аноректальных пороках развития отмечается отсутствие либо резкое недоразвитие наружного сфинктера.
В 1970 г. на Международном конгрессе детских хирургов в Мельбурне была принята классификация, в основу которой положено отношение прямой кишки к мышцам тазового дна, в частности лонопрямокишечной мышце. Выделены три группы аномалий: высокие, средние, низкие. В первом случае подразумеваются агенезия и атрезия прямой кишки со свищом или без него; слепой конец кишки располагается над мышцами тазового дна. Ко второй группе относятся пороки, при которых слепой конец кишки располагается на уровне тазового дна. Третью группу составляют варианты, когда кишка располагается в центре петли лобково-прямокишечной мышцы. 
Каждой анатомической форме присущи свои особенности. Атрезию одного отверстия распознают без труда при первичном осмотре: заднепроходное отверстие отсутствует.

Мельбурнская классификация аноректальных аномалий

Во всех случаях возникает необходимость определить
высоту атрезии, т. е. отношение кишки к леваторным мышцам, которые у новорожденных залегают на глубине 2 см от кожи анальной области. Под низкими атрезиями подразумевают варианты, когда слепой конец кишки располагается на глубине до 2 см от кожи, а под средними и высокими атрезиями - когда слепой конец расположен на большой глубине. Клинически со стороны промежности можно отметить некоторые особенности, позволяющие оценить высоту атрезии. При высоких атрезиях промежность уменьшена в размерах, недоразвита, седалищные бугры сближены, нередко отсутствует копчик. На месте анального отверстия кожа чаще всего гладкая. Симптом "толчка" отрицательный (указательным пальцем наносят толчкообразное движение в проекции наружного сфинктера; если кишка, заполненная меконием, располагается вблизи промежности, то палец исследователя ощущает противоудар, при этом симптом считается положительным).
Если осмотр ребенка после рождения по каким-либо причинам не был проведен, то к концу суток новорожденный начинает беспокоиться, появляются обильные срыгивания и рвота желудочным содержимым, затем желчью и кишечным содержимым. Живот становится резко вздутым, видны растянутые петли кишечника. Меконий и газы не отходят. Развивается картина низкой кишечной непроходимости.

С целью определения высоты атрезии выполняют инвертограмму по Вангенстину.
На область проекции заднепроходного отверстия наклеивают рентгеноконтрастный предмет (например, монетку), после чего выполняют обзорный снимок в боковой проекции в положении ребенка вниз головой. По расстоянию между газовым пузырем в атрезированной кишке и меткой на промежности судят о высоте атрезии. Это исследование должно выполняться спустя 12-15ч после рождения, в противном случае газ не успевает достигнуть атрезированной кишки и можно получить ложный результат.
В сомнительных случаях применяют рентгеноконтрастные исследования. В проекции заднепроходного отверстия осуществляют пункцию промежности, погружая иглу на глубину 2 см, и под контролем рентгеновского экрана вводят 10-15% раствор верографина. Если игла попала в просвет кишки, то последняя отчетливо контрастируется.
При высоких атрезиях контрастное вещество инфильтрирует клетчатку.
В настоящее время с целью определения уровня атрезии широкое применение нашло ультразвуковое сканирование промежности.
Наличие, расположение и полноценность наружного сфинктера определяют с помощью электромиографии, выполняемой игольчатыми электродами с четырех точек.
Высокая частота сочетанных пороков развития требует выполнения ультразвукового исследования почек и сердца, проверки проходимости пищевода и желудка.
Атрезия заднепроходного отверстия и прямой кишки со свищом в мочевую систему - наиболее тяжелая форма порока. Встречается почти исключительно у мальчиков и, как правило, при высоких формах атрезий. В первые сутки жизни порок по клиническому течению не отличается от бессвищевых форм. К концу 2-х суток у ребенка развивается картина низкой кишечной непроходимости, так как свищи с мочевым. пузырем и уретрой чаще бывают узкими и малопроходимыми для мекония.
При осмотре ребенка в ряде случаев можно обнаружить выделение мекония из наружного отверстия уретры. Судить о локализации свища сложно. Наиболее часто, почти в 94% случаев, отмечается ректоуретральный свищ с мембранозной или простатической частью уретры. В этих случаях выделения мекония из уретры незначительны и могут быть не связаны с мочеиспусканием. Меконий выбрасывается в начале акта мочеиспускания почти неизменным, а последние порции мочи, как правило, прозрачные. Отхождение газов через уретру наблюдается вне мочеиспускания.
В тех случаях, когда свищ открывается в мочевой пузырь, меконий постоянно смешивается с мочой и окрашивает ее в зеленый цвет. При мочеиспускании моча интенсивно окрашена в зеленый цвет, особенно последние ее порции.
Перечисленные признаки могут быть выражены в различной степени и даже отсутствовать, так как диаметр свищевого отверстия варьирует. По данным Б. В. Парина, широкие свищи с выраженной симптоматикой составляют 18%, свищи средней ширины с непостоянными клиническими проявлениями - 41%, узкие, "бессимптомные" свищи - 41%.
Диагноз уточняют рентгенологически с помощью уретроцистографии. Под контролем экрана в начальный отдел уретры на глубину 1-2 см погружают катетер, через который вводят 10-15% раствор верографина. На снимке в боковой проекции видно затекание контрастного вещества в прямую кишку.
Свищ в половую систему характерен преимущественно для девочек. Обычно он открывается в преддверие влагалища в области задней спайки, реже во влагалище.
Клиническая картина при атрезии со свищом в половую систему во многом зависит от локализации и диаметра свища. Основным признаком соустья является выделение мекония, а затем кала и газов через половую щель с первых дней жизни, анальное отверстие отсутствует. Если свищ короткий и достаточно широкий, отмечается более или менее регулярный самостоятельный стул в первые месяцы жизни. При переходе на искусственное вскармливание стул становится реже, нарастает запор.
При атрезии со свищом во влагалище свищевое отверстие, как правило. узкое, расположено выше девственной плевы. У девочек с влагалищным свищом через отверстие постоянно выделяется кишечное содержимое, что создает условия для восходящей инфекции. Недостаточное самостоятельное опорожнение кишки и невозможность проведения клизм из-за высокого расположения свища приводят к раннему появлению калового завала, хронической интоксикации с прогрессирующим ухудшением состояния.
При ректовестибулярных свищах атрезия относится, как правило, к категории низких, в случаях ректовагинального соустья атрезия всегда высокая и обычно сопровождается инфантилизмом наружных половых органов.
Свищ промежности наблюдается у мальчиков несколько чаще, чем у девочек. У девочек промежностный свищ бывает коротким и широким. У мальчиков протяженность и ширина свища сильно варьируют; наружное отверстие может открываться в непосредственной близости от заднего прохода, в передней порции наружного сфинктера, у корня мошонки и даже в области полового члена. В зависимости от анатомического варианта возможна клиническая картина полной либо частичной кишечной непроходимости.
Под эктопией заднепроходного отверстия подразумевают состояние, когда заднепроходное отверстие, имеющее все признаки нормального (хорошо открывается, сжимается и нормально функционирует), расположено на необычном месте - близко к половым органам.
Различают промежностную и вестибулярную эктопию. Истинную эктопию необходимо дифференцировать от свищевых форм атрезии, так как последние не в состоянии обеспечить полноценной функции и требуют хирургической коррекции. Различия заключаются в визуальном выявлении сокращений наружного сфинктера или с помощью электромиографии. При эктопии он сокращается вокруг анального отверстия, а при свищах сокращение волокон наружного сфинктера наблюдается вне его. При эктопии заднепроходного отверстия функциональных отклонений не выявляется.
Врожденные сужения заднепроходного отверстия и прямой кишки локализуются чаще всего на месте перехода эндодермальной части кишки в эктодермальную, т.е. в области гребешковой линии анального кольца. Форма и протяженность стриктуры вариабельны. Иногда это тонкая мембрана, в других случаях - плотное фиброзное кольцо. Протяженность сужения составляет от нескольких миллиметров до 2-4 см.
В периоде новорожденности и первые месяцы жизни врожденные стриктуры клинически обычно не проявляются, так как жидкий кал свободно проходит через сужение. При резких степенях стеноза у ребенка с первых дней наблюдается запор, кал выделяется в виде узкой ленты. С введением прикорма запор становится более выраженным. Ребенок беспокоен, прогрессируют вздутие живота, плохой аппетит, отставание в массе тела; формируется вторичный мегаколон.
Диагностика врожденного сужения анального канала не вызывает затруднений. При ректальном пальцевом исследовании в зоне сужения определяется податливое эластическое кольцо; иногда при резком стенозе не удается провести кончик пальца. Уточняют диагноз с помощью рентгеноконтрастного исследования прямой кишки, колоноскопии.
Лечение. Все виды атрезии заднепроходного отверстия и прямой кишки подлежат хирургическому лечению в условиях специализированного отделения. Экстренной операции в первые 2 сут жизни требуют все виды полных атрезий, а также свищевые формы: ректоуретральная, ректовезикальная и при малых диаметрах - ректовагинальная, ректопромежностная. В возрасте от 1-3 мес до 1 - 3 лет проводят коррекцию пороков со свищами в половую систему и на промежность, не вызывающих симптомов кишечной непроходимости. Последнее время отмечается стремление к более ранней коррекции врожденных пороков развития, с тем, чтобы к моменту формирования и закрепления нервно-рефлекторных кортико-висцеральных связей добиться максимально приближенного к норме анатомического варианта.
При низких формах атрезии заднепроходного отверстия и прямой кишки выполняют одномоментную промежностную проктопластику.
В случаях средних форм атрезии (высота 1,5-2 см от кожи промежности) в настоящее время хорошо зарекомендовала себя сакропромежностная проктопластика, позволяющая хорошо мобилизовать, провести через пубококцигеальную связку и наружный сфинктер кишку.
Высокие формы атрезии требуют значительной мобилизации кишки, выполнить которую возможно лишь брюшно-сакропромежностным способом. Обширность и травматичность такой операции у новорожденного требуют ее разделения на два этапа. В первые дни жизни в левой подвздошной области накладывают противоестественный задний проход по Микуличу на сигмовидную кишку максимально близко к зоне атрезии для ликвидации кишечной непроходимости. В возрасте 2 мес. - 1 год выполняют второй этап радикальной операции. Такая тактика позволяет не только добиться выздоровления детей, но и получить лучшие функциональные результаты. После операции, начиная с 10-14-го дня, проводят профилактическое бужирование вновь созданного анального канала бужами Гегара, постепенно увеличивая от № 8 до № 11-12. Профилактическое бужирование в течение 2-2,5 мес позволяет создать нежный рубчик в месте перехода кишки в кожу и избежать стеноза прямой кишки. Бужирование проводят ежедневно в течение первых 1-2 нед - в стационаре, а затем дома родителями под еженедельным диспансерным контролем.
Всем детям с множественными пороками развития необходимы медико-генетическая консультация и постоянное диспансерное наблюдение. Поэтапно проводят коррекцию других сочетанных пороков - почек, половых органов, сердца, опорно-двигательной системы).
Прогноз заболевания, функциональный результат зависят как от тяжести порока и сочетанных аномалий, так и от правильности выбранной тактики оперативного вмешательства.

ДИАФРАГМАЛЬНЫЕ ГРЫЖИ

Основные вопросы темы:
1.	Классификация грыж
2.	Клиника и диагностика
3.	Дифференциальная диагностика
4.	Лечение

Вопросы контроля исходного уровня:
1.	Понятие грыжи
2.	Что такое «ложные и истинные» грыжи?

Под диафрагмальными грыжами понимают перемещение органов брюшной полости в грудную клетку через дефект в грудобрюшной преграде (диафрагме). В отличие от других грыж они не всегда имеют грыжевой мешок.

Рис. 2

У детей в основном отмечаются врожденные грыжи, являющиеся пороком развития диафрагмы.  Частота возникновения диафрагмальной грыжи колеблется, по данным разных авторов, в больших пределах - от 1:2000 до 1:4000 новорожденных, при этом не учитывается большая группа мертворожденных с пороками развития диафрагмы.
Порок начинает формироваться у эмбриона на 4-й неделе беременности, когда образуется закладка перегородки между перикардиальной полостью и туловищем зародыша.

Рис. 2

Недоразвитие мышц в отдельных  участках грудобрюшной преграды приводит к возникновению грыж с мешком, стенки которого состоят из серозных покровов - брюшного и плевральных листков. Такие грыжи являются истинными. При ложных грыжах имеется сквозное отверстие в диафрагме, которое образуется или в результате недоразвития плевроперитонеальной перепонки, или из-за разрыва ее вследствие перерастяжения.
По происхождению и локализации диафрагмальные грыжи следует разделять следующим образом.
I. Врожденные диафрагмальные грыжи:
•	диафрагмально-плевральные (ложные и истинные); 
•	парастернальные (истинные); 
•	френоперикардиальные (истинные);
•	 грыжи пищеводного отверстия (истинные).
II. Приобретенные грыжи - травматические (ложные).

Наиболее часто у детей встречаются диафрагмально-плевральные грыжи, грыжи пищеводного отверстия диафрагмы. Парастернальные грыжи встречаются значительно реже, френокардиальные в сущности являются казуистикой.
Парезы диафрагмы представляют собой отдельную нозологическую форму заболевания и поэтому не входят в данную классификацию,
Диафрагмально - плевральные грыжи могут быть как истинными, так и ложными. Часто они бывают левосторонними. Ложные грыжи справа наблюдаются очень редко. Истинные грыжи могут занимать ограниченную часть диафрагмы, но бывают значительных размеров и полными. В последних случаях, когда наблюдается высокое стояние всего купола диафрагмы с отсутствием мышечного слоя, этот вид диафрагмальной грыжи называют релаксацией диафрагмы. При ложных грыжах дефект в диафрагме чаще всего щелевидный и располагастя в реберно-позвоночном отделе (грыжа Богдалека). Из-за отсутствия грыжевого мешка при этих видах диафрагмальных грыж органы брюшной полости перемещаются в грудную полость без ограничения, что чаще приводит к синдрому грудного  напряжения.  Подобная ситуация, естественно, наблюдается и при истинных грыжах, когда имеется полное выпячивание диафрагмы.
Парастернальные грыжи обычно имеют грыжевой мешок и разделяются на загрудинные и загрудинно-реберные. Эти грыжи проникают в грудную полость через истонченный в переднем отделе участок диафрагмы (шель Ларрея). Грыжу, располагающуюся больше справа от грудины, некоторые авторы называют грыжей Морганьи.
Френоперикардиальная грыжа является ложной, с дефектом, располагающимся в сухожильной части диафрагмы и прилегающим к нему перикардом. Через этот дефект петли кишок могут перемещаться в полость перикарда, иногда может быть обратный эффект - вывихивание сердца в брюшную полость.
Грыжи пищеводного отверстия диафрагмы всегда относятся к истинным и разделяются на две большие группы - параэзофагельные и эзофагельные. Для параэзофагеальных характерно смещение желудка вверх рядом с пищеводом. При эзофагеальных пищеводно-желудочный переход располагается выше уровня диафрагмы. При этом степень смещения желудка может быть разной и даже меняться в зависимости от положения ребенка и объема заполнения желудка.
Тяжесть состояния и выраженность клинических проявлений обусловлены не только степенью и объемом перемещенных органов в грудную полость, но и сочетанными пороками развития. При диафрагмально-плевральных грыжах часто отмечаются недоразвитие легких, пороки сердца, центральной нервной системы и желудочно-кишечного тракта.  Особая тяжесть определяется степенью недоразвития легких и теми морфофункциональными нарушениями в них. которые приводят к нарушению кровообращения в малом круге с развитием гипертензии и возникновением праволевого шунта со сбросом крови на уровне артериального протока или же внутрисердечно. Не исключено шунтирование крови в легких за счет функционирующих фетальных коммуникаций. Дети с подобными тяжелыми пороками развития нередко рождаются мертвыми или погибают вскоре после рождения. 
Клиника. Каждый вид диафрагмальных грыж имеет довольно специфическую симптоматологию, хотя можно выделить два ведущих симптомокомплекса: сердечно-легочные нарушения, отмечаемые при диафрагмально-плевральных грыжах, сопровождающихся внутригрудным напряжением, и желудочно-пищеводный рефлюкс - при грыжах пищеводного отверстия диафрагмы.
При ложных диафрагмально-плевральных грыжах или истинных со значительным выбуханием мешка в плевральную полость, когда туда перемешается почти весь кишечник, клинические признаки дыхательной недостаточности выявляются рано. Сразу после рождения или через несколько часов развиваются одышка, цианоз. Кожные покровы и слизистая оболочка темно-синего и даже чугунного цвета. Острая дыхательная недостаточность прогрессирует очень быстро. При осмотре, помимо цианоза, обращает на себя внимание асимметрия грудной клетки с выбуханием на стороне поражения (как правило, слева) и с отсутствием экскурсии этой половины грудной клетки. Очень характерный симптом - запавший ладьевидный живот. Перкуторно над соответствующей областью грудной клетки определяется тимпанит, при аускультации - резкое ослабление дыхания. Сердечные тоны (при левосторонней грыже) слева почти не определяются, справа громкие, что указывает на смещение сердца в здоровую сторону. Иногда через грудную стенку удается выслушать перистальтику перемещенных петель кишок и шум плеска.
При грыжах меньшего размера клинические проявления менее выражены, респираторные нарушения в виде цианоза и одышки чаше наблюдаются при беспокойстве, крике, кормлении или изменении положения ребенка. Иногда ухудшение в состоянии возникает у детей ясельного и даже школьного возраста среди кажущегося полного здоровья, когда происходит ущемление стенки желудка в грыжевых воротах или его заворот. При этом ребенок жалуется на неопределенную боль в животе, появляются тошнота, рвота, постепенно усиливается беспокойство.
При истинных малых диафрагмальных грыжах, особенно при выпячивании ограниченной части диафрагмы справа, когда содержимым является внедрившийся участок печени, клинические симптомы отсутствуют. Дети ничем не отличаются от здоровых, хорошо развиваются, не отставая от своих сверстников. При подобных грыжах, локализующихся слева, несмотря па отсутствие видимых клинических проявлений, существует некоторое смещение сердца с его ротацией, что может вызвать скрытые сердечно-сосудистые нарушения. Для их выявления следует проводить функциональные нагрузки и дополнительные методы исследования.
При парастернальных грыжах симптомы не выражены и непостоянны, чаще выявляются у детей ясельного и школьного возраста, когда они начинают жаловаться на болезненные, неприятные ощущения в эпигастрии. Иногда бывают тошнота и даже рвота. Респираторные и сердечно-сосудистые нарушения при этом виде грыж не характерны. Почти в половине всех случаев дети жалоб не предъявляют. Методом перкуссии и аускультации удается определить в этой зоне тимпанит и ослабление сердечных тонов.
При грыжах пищеводного отверстия диафрагмы, особенно при эзофагеальной форме, клинические проявления связаны с наличием желудочно-пищеводного рефлюкса, возникающего в результате нарушения функции кардиального отдела пищевода. Возникает синдром, называемый рефлюкс-эзофагитом. При параэзофагеальной форме симптомы заболевания часто связаны с наличием желудочно-пищеводного рефлюкса, а зависят от нарушения эвакуации пищи из желудка, его перегиба, заворот, травмы, возможны сердечно-сосудистые нарушения из-за смещения и сдавления сердца. Иногда параэзофагеальные грыжи выявляются случайно при рентгенологическом исследовании.
Диагностика диафрагмальных грыж не всегда проста. Ведущее значение следует придавать рентгенологическому исследоваиию. Для диафрагмально - плевральных грыж характерны кольцевидные просветления над всей левой половиной грудной клетки, которые обычно имеют пятнистый рисунок: прозрачность этих полостей более выражена к периферии. Характерно изменчивость положения и форм участков просветления и затемнения, что можно видеть при сравнении двух рентгенограмм, полученных в разное время.
Смещение органов средостения и сердца зависит от количества кишечных петель, пролабировавших в грудную полость. У новорожденных и детей первых месяцев жизни смещение бывает столь значительным, что при этом даже не удается определить тень коллабированного легкого.
Отличить ложную диафрагмальную грыжу от истинной трудно, особенно если плевральная полость заполнена внедрившимися петлями кишок. Обычно при истинных грыжах удается рентгенологически проследить верхний контур грыжевого мешка, отграничивающий пролабированные петли кишечника в грудной полости.
Если состояние больного позволяет и имеются трудности в дифференциальной диагностике с такими заболеваниями, как поликистоз легкого или ограниченный пневмоторакс, следует провести контрастирование желудочно-кишечного тракта бариевой взвесью. При этом четко устанавливают, какой отдел кишечника находится в грудной полости. Иногда бывает достаточно катетеризации желудка. Подобная манипуляция может в некоторой степени облегчить состояние больного, так как при этом происходит декомпрессия желудка.
При расположении истинной грыжи справа обычно ее содержимым является часть печени, поэтому рентгенологически тень грыжевого выпячивания будет иметь плотную интенсивность, сливающуюся в нижних отделах с основной тенью печени, а верхний контур грыжи будет сферическим, т.е. создается впечатление наличия плотной округлой опухоли легкого, примыкающей к диафрагме. Для дифференциальной диагностики могут быть использованы компьютерная томография и диагностический пневмоперитонеум, при котором воздух, скапливается в грыжевом мешке, что позволяет отличить грыжу от других образований.
При парастернальной грыже диафрагмы выявляется тень полуовальной или грушевидной формы с крупноячеистыми кольцевидными просветлениями, проецирующимися на тень сердца в прямой проекции. В боковой проекции тень грыжи как бы вклинивается между тенью сердца и передней грудной стенкой. Рентгенологически отличить парастернальную грыжу от френоперикардиальной не представляется возможным. Для установления содержимого парастернальных грыж проводят рентгеноконтрастное исследование желудочно-кишечного тракта с бариевой взвесью. Лучше начинать с ирригографии, так как чаще всего содержимым грыжи является поперечная ободочная кишка.
Рентгенографическая картина грыж пищеводного отверстия диафрагмы зависит от их формы. При параэзофагеальных грыжах в грудной полости справа пли слева от срединной линии выявляется полость с уровнем жидкости, при этом газовый пузырь желудка, находящийся в брюшной полости, уменьшен или отсутствует. Контрастное исследование с находящийся в брюшной полости, уменьшен или отсутствует. Контрастное исследование с бариевой взвесью выявляет желудок типа "песочных часов", верхний отдел которого располагается в грудной полости, а нижний - в брюшной, причем бариевая взвесь может переливаться из одного отдела желудка в другой. Эзофагеальную грыжу, как правило, удается обнаружить лишь при контрастировании желудочно-кишечного тракта.

Лечение врожденных диафрагмальных грыж оперативное. Исключение составляют бессимптомно протекающие небольшие грыжи, локализующиеся справа, когда содержимым является часть печени. Срочность лечения определяется выраженностью симптомов дыхательной недостаточности и сердечно-сосудистых расстройств.

Обычно при ложных диафрагмально-плевральных или истинных больших грыжах респираторные и сердечно-сосудистые нарушения настолько выражены (даже у новорожденных), что необходима довольно длительная предоперационная подготовка, заключающаяся в декомпрессии  желудка катетером, назофарингеальной интубации, переводе ребенка на искусственную вентиляцию легких с созданием положительного давления на выдохе, которое должно быть минимальным - не более 20 см вод. ст.; в противном случае может развиться пневмоторакс. Устраняют метаболические расстройства. Инфузионная и лекарственная терапия должна быть адекватной и направлена на улучшение реологических свойств крови и восстановление гомеостаза. При этом очень важно применять лекарства, снижающие давление в малом кругу кровообращения (галазолин, допамин). Следует подчеркнуть, что дети с подобными расстройствами очень плохо переносят транспортировку, поэтому указанную терапию следует начинать в родильном доме и продолжать в специальных машинах. Только после улучшения гомеостаза, устранения сердечно-сосудистых расстройств и гипоксии ребенок может быть оперирован.

Принцип оперативного вмешательства заключается в низведении органов в брюшную полость, ушивании дефекта диафрагмы при ложных диафрагмальных грыжах и пластике диафрагмы при истинных грыжах. Иногда при аплазии диафрагмальных мышц и наличии только плеврального брюшного листка используют пластический материал. Операция может быть выполнена как через живот, так и через грудную полость, но чрезвычайно важным обстоятельством является то, чтобы в послеоперационном периоде не было значительного интраабдоминального давления, поэтому во время операции проводят декомпрессию желудочно-кишечного тракта. В послеоперационном периоде осуществляют довольно длительную назофарингеальную интубацию с искусственной вентиляцией легких до улучшения показателей гемодинамики и гомеостаза.

Результаты операции связаны в основном с тяжестью состояния больного при поступлении и степенью недоразвития легкого. Кроме того, большое значение имеет качество транспортировки и подготовки новорожденного к оперативному вмешательству. Если из-за выраженности сердечно-сосудистых и респираторных нарушений детей вынуждены доставлять в клиники в первые сутки после рождения, то прогноз более чем в 50% случаев неблагоприятный.

ПОРОКИ РАЗВИТИЯ ПЕРЕДНЕЙ БРЮШНОЙ СТЕНКИ
ГРЫЖА ПУПОЧНОГО КАНАТИКА

Основные вопросы темы:
1.	Понятие грыжи пупочного канатика
2.	Клиника и диагностика
3.	Лечение

Вопросы контроля исходного уровня:
1.	Краткий эмбриогенез
2.	Понятия эмбриональные и фетальные грыжи.

Грыжей пупочного канатика, пуповинной грыжей или эмбриональной грыжей (omphacele) называют порок развития, при котором к моменту рождения ребенка часть органов брюшной полости располагается внебрюшинно - в пуповинных оболочках, состоящих из амниона, вартонова студня и первичной недифференцированной брюшины.
В раннем эмбриогенезе в результате несовпадения "критических периодов развития" кишечника в брюшной полости, последняя не может вместить быстро увеличивающиеся в объеме кишечные петли. Располагаясь внебрюшинно, в пуповинных оболочках, они проходят временную стадию "физиологической эмбриональной грыжи", а затем, выполнив "процесс вращения", возвращаются в увеличивающуюся брюшную полость. Если в результате нарушения процесса вращения кишечника, недоразвития брюшной полости или нарушения замыкания брюшной стенки часть органов остается в пуповинных оболочках, ребенок рождается с грыжей пупочного канатика.
В зависимости от времени остановки развития передней брюшной стенки различают два основных вида пуповинных грыж - эмбриональные и фетальные. При эмбриональных грыжах печень не имеет фиброзной оболочки (глиссоновой капсулы) и срастается с оболочками пуповины.
Популяционная частота - 1:6000 новорожденных. Тип наследования предположительно аутосомно-доминантный и сцепленный с Х-хромосомой. Примерно у 65% детей с эмбриональными грыжами встречаются сочетанные пороки развития сердца (тетрада Фалло), желудочно-кишечного тракта, мочеполовой системы, синдром Беквита-Видемана.
Клиника и диагностика. При осмотре ребенка обнаруживают, что часть органов брюшной полости находится в пуповинных оболочках. Размеры грыжевого мешка варьируют от небольшого (2-5 см) до гигантского - 15-20 см.
Грыжевыми воротами является расширенное пупочное кольцо, размеры дефекта которого колеблются от 1-2см до значительного. В зависимости от размеров дефекта пупочного кольца грыжа может быть удлиненной с узкими воротами либо полушаровидной. Пуповина переходит в верхушку грыжевого мешка, в котором проходят три пупочных сосуда до вхождения в брюшную полость. Содержимым грыжевого мешка может быть кишечник, желудок, печень. При дефекте диафрагмы наблюдается эктопия сердца.
В первые часы после рождения пуповинные оболочки, образующие грыжевой мешок, блестящие, прозрачные, белесоватого цвета. Однако уже к исходу первых суток они высыхают, мутнеют, затем инфицируются и покрываются фибринозными наложениями.
Если не проводятся мероприятия по профилактике и лечению инфицированных оболочек, могут развиться перитонит и сепсис. При истончении и разрыве оболочек наступает эвентрация  внутренних органов, развивается перитонит.
По классификации грыжи пупочного канатика подразделяются следующим образом: по размерам: небольшие (до 5 см); средние (до 10 см); большие (более 10 см); по состоянию грыжевых оболочек: неосложненные (неизмененные грыжевые оболочки); осложненные (разрыв оболочек, гнойное их расплавление, кишечные свищи).
Диагностика грыж пупочного канатика не представляет затруднений.
Лечение детей с грыжами пупочного канатика начинают сразу по установлении диагноза. Применяют два метода лечения: оперативный и консервативный. Радикальному оперативному вмешательству подлежат новорожденные с малыми и средними грыжами при хорошо сформированной полости и отсутствии отягчающих факторов (глубокая недоношенность, родовая травма, сепсис). Операция сводится к иссечению пуповинных оболочек, вправлению внутренностей и пластике передней брюшной стенки.
Консервативное лечение показано детям с большими размерами грыжи, недоразвитием брюшной полости, отягченным преморбидным фоном. Сразу после рождения обрабатывают оболочки 2% настойкой йода, спиртом, 5% раствором перманганата калия с последующим наложением стерильных повязок.
В результате ежедневных перевязок оболочки превращаются в струп черного цвета, непроницаемый для инфекции.

По мере появления грануляций отторгается коагуляционный струп и начинается краевая эпителизация.
В этот период показаны повязки с препаратами, стимулирующими регенерацию (ируксол, бальзам Шостаковского, косерил и др.). Грыжевой мешок постепенно покрывается эпителием, сморщивается, уменьшается, содержимое частично погружается в брюшную полость. Полная эпителизация наблюдается через 2-2,5 месяца.
Для сокращения сроков консервативного лечения в настоящее время широко используется метод постепенного погружения внутренних органов в брюшную полость с поэтапной перевязкой от дна грыжевого мешка.
После консервативного лечения формируется вентральная грыжа. Ее устраняют оперативным путем в возрасте 2-5 лет.
Предварительно выполняют функциональную пробу, позволяющую выяснить степень компенсации повышения внутрибрюшного давления при вправлении грыжи. Если частота пульса и дыхания, показатели газового состава крови остаются в пределах нормы возможно устранение грыжи. При учащении пульса и одышки, операцию откладывают до тех пор, пока брюшная полость не достигнет достаточного размера. В дооперационном периоде в обязательном порядке проводят массаж мышц брюшной стенки, лечебную гимнастику и применяют бандаж, препятствующий выпячиванию внутренних органов.

Прогноз при грыжах пупочного канатика всегда серьезный, особенно у незрелых детей с сочетанными пороками развития.
Множественные пороки развития: экстрофия клоаки, эмбриональная грыжа, экстрофия мочевого пузыря, атрезия ануса, эвагинация петли подвздошной кишки, спинно-мозговая грыжа, двусторонняя косолапость.
Дети, успешно оперированные  в периоде новорожденности,  в дальнейшем растут и развиваются нормально.

ГАСТРОШИЗИС

Основные  вопросы темы:
1.	Понятие гастрошизиса
2.	Клиника и диагностика
3.	Дифференциальная диагностика
4.	Лечение

Гастрошизис, или внутриутробная эвентрация внутренних органов, относится к тяжелым порокам развития, который характеризуется дефектом передней брюшной стенки не более 2-3 см в диаметре. Этот дефект почти всегда локализуется справа от пуповины, причем пуповина выходит нормально. Грыжевой мешок никогда не развивается. Через дефект в передней брюшной стенке выпадает короткий, не совершивший поворот отдел средней кишки, кровоснабжаемый от верхней брыжеечной артерии. Кишка короче обычной, эдематически пропитана, в три-четыре раза больше обычного по диаметру, с серозной оболочкой и признаками зародышевого 
перитонита. Кишечные петли  часто спаиваются друг с другом

Отеку кишечной стенки способствуют сдавление ее узкими краями дефекта брюшной стенки, нарушение венозного и лимфооттока. Просвет кишки заполнен густым и вязким меконием.

Клиника и диагностика. Диагноз обычно не вызывает затруднений. Важное значение имеют внутриутробная диагностика данного порока и планирование оперативного родоразрешения. В противном случае при прохождении плода по родовым путям эвентрированные внутренние органы получают дополнительную травму (кровоизлияния, разрывы, инфицирование). Дети чаше рождаются незрелыми и малокровными, тяжесть их состояния после рождения обусловлена болевым перинатальным шоком, анурией.
Гастрошизис необходимо дифференцировать от грыжи пупочного канатика при разрыве ее оболочек в момент родов. При гастрошизисе грыжевой мешок отсутствует.

Лечение только оперативное, после выведения ребенка из шока и восстановления диуреза (общее обезболивание, новокаиновая блокада брыжейки, иифузионная и антибактериальная терапия). Сложность хирургического лечения заключается в том, что резко недоразвитая брюшная полость не в состоянии вместить отечные и утолщенные кишечные петли. В связи с этим над внутренними органами ушивают отсепарированную кожу либо, синтетическую ткани, формируют "вентральную" грыжу. В послеоперационном периоде в связи с трофическими расстройствами и текущим перитонитом длительно не налаживается пассаж пищи по кишечнику, требуются рациональное парентеральное питание и интенсивная терапия.
Прогноз намного хуже, чем при эмбриональной грыже, хотя сочетанные пороки развития встречаются гораздо реже. Операцию по поводу сформированной вентральной грыжи выполняют у детей в возрасте 2-5 лет.

ВРОЖДЕННЫЙ ПИЛОРОСТЕНОЗ

Основные вопросы темы:
1.	Классификация грыж
2.	Клиника и диагностика
3.	Дифференциальная диагностика
4.	Лечение

Вопросы контроля исходного уровня:
1.	Понятие грыжи
2.	Что такое ложные и истинные грыжи

В основе заболевания лежит нарушение проходимости пилорического отдела желудка, обусловленное пороком развития пилорического жома в виде нарушения морфологических его структур (мышечных волокон на уровне кавеол и миофибрилл, их гистохимических расстройств и нервных элементов). Заболевание является генетически гетерогенным. Описано рецессивное сцепленное с полом и аутосомно-доминантное наследование. Популяционная частота 0,5-3: 1000. Соотношение мужского и женского полов 4:1.
Клиника и диагностика. Первые симптомы заболевания обычно появляются с конца 2-й - начала 3-й недели. Вначале замечают рвоту фонтаном, возникающую между кормлениями. Рвотные массы имеют застойный характер, объем их превышает дозу однократного кормления, они содержат створоженное молоко с кислым запахом. Ребенок начинает худеть, появляются признаки обезвоживания, сопровождающиеся урежением мочеиспускания и скудным стулом. При острой форме заболевания симптоматика развивается бурно - в течение недели. Преобладают симптомы острой дегидратации II - III степени и декомпенсированного метаболического алкалоза. При подострой форме симптомы развиваются постепенно: срыгивания, одно- или двукратная рвота, которая, учащаясь, приводит к гипотрофии. Эта форма не сопровождается грубыми водно-электролитными нарушениями.
Диагноз подтверждается клиническим, лабораторным, инструментальным и рентгенологическим исследованиями.
При осмотре больного обращают внимание на степень развития гипотрофии, эксикоза, при осмотре живота - на вздутие эпигастральной области, усиленную перистальтику желудка в виде песочных часов.
Пальпаторно в ряде случаев удается определить гипертрофированный привратник. Данные лабораторных исследований указывают на сгущение крови (снижение ОЦК ОЦП; повышение ОЦЭ, гемоглобина, гематокрита), гипохлоремию, гипокалиемию, металлический алкалоз.
В настоящее время наиболее современный, безопасный и достоверный метод является УЗИ пилорического отдела. При ультразвуковой сканировании лоцируется гипертрофированный пилорический отдел, суженный канал и отсутствие пассажа по пилорическому каналу.
При рентгенологическом исследовании обращают внимание на увеличение размеров желудка, наличие большого уровня жидкости натощак, сниженное газонаполнение кишечных петель (а - обзорная рентгенограмма).
Рентгеноконтрастное исследование выполняется в вертикальном положении через 30-40 мин после дачи контрастного вещества (5% водная взвесь бария в грудном молоке в объеме разового кормления). Видна сегментирующая перистальтика желудка и отсутствие первичной эвакуации в двенадцатиперстную кишку (б). 
На рентгенограмме в боковой проекции определяется суженный пилорический канал - симптом "клюва" (в).
Все рентгенограммы должны быть выполнены в вертикальном положении ребенка. Как правило, дальнейшего обследования не требуется.
В последнее время для диагностики пилоростеноза применяют фиброэзофагогастроскопию.
При этом виден расширенный складчатый антральный отдел желудка, просвет пилорического канала резко сужен до величины булавочной головки, не раскрывается при раздувании воздухом (в отличие от пилороспазма).
Кроме того, фиброскопия дает возможность осмотреть пищевод, определить степень выраженности рефлюкс-эзофагита, который часто сопровождает пилоростеноз.
Возможна и ультразвуковая диагностика.
Дифференциальный диагноз проводят с пилороспазмом, псевдопилоростенозом (адреногенитальный синдром, сольтеряющая форма - синдром Дебре-Фибигера), желудочно-пищеводным рефлюксом, стенозом двенадцатиперстной кишки выше большого сосочка двенадцатиперстной кишки. Дифференциальная диагностика основана на разнице во времени и характере клинических проявлений, данных лабораторных исследований, рентгенологической и эндоскопической картины.
При пилороспазме в результате вегетативной дистонии по симпатическому типу заболевание начинается с рождения; хороший эффект дают противоспазматическая терапия и лечение НМК. Эндоскопически привратник хорошо проходим. Для адреногенитального синдрома характерны примесь желчи в рвотных массах, полиурия, периодически разжиженный стул, гиперкалиемия, гипонатриемия, метаболический ацидоз. Эндоскопически и рентгенологически привратник хорошо проходим.
У детей с желудочно-пищеводным рефлюксом типичны начало заболевания с рождения, рвота и регургитация в горизонтальном положении. Эндоскопически определяются фибринозно-язвенный эзофагит, зияние кардии, рентгенологически - наличие желудочно-пищеводного рефлюкса.
Для высокой частичной кишечной непроходимости двенадцатиперстной кишки специфично появление симптомов с первых дней жизни; рентгенологически обнаруживается наличие двух уровней жидкости в желудке и двенадцатиперстной кишке, ее расширение.
Лечение. Пилоростеноз требует оперативного лечения. Вмешательству предшествуют предоперационная подготовка, направленная на коррекцию пиповолемии, алкалоза, гипокалиемии. Выполняют внеслизистую пилоромиотомию по Фреде - Рамштедту.
В ходе операции устраняется анатомическое препятствие, восстанавливается проходимость привратника.
Через 3 - 6 ч после операции ребенка начинают поить 5 % раствором глюкозы, затем молоком по 5 - 10 мл через 2 ч. В последующие сутки количество молока ежедневно увеличивают на 100 мл (10 мл на одно кормление). К 6-м суткам увеличивают объем кормления до 60 - 70 мл с интервалом 3 ч, после чего ребенка переводят на обычное кормление. В первые дни после операции дефицит жидкости, электролитов, белка и других ингредиентов восполняется за счет инфузионнои терапии и вспомогательного парентерального питания, а также назначением микроклизм (5 % раствор глюкозы и раствор Рингера - Локка в равных количествах по 30 мл, 4 раза в день в теплом виде).
Прогноз благоприятный. Дети требуют диспансерного наблюдения с целью дальнейшего лечения гипотрофии, анемии, гиповитаминоза.

БОЛЕВОЙ АБДОМИНАЛЬНЫЙ СИНДРОМ

В детском возрасте острые заболевания, обусловливающие клиническую картину острого живота, наблюдаются весьма часто. Чем младше ребенок, тем более несовершенны физиологические функции многих его органов и систем, особенно ЦНС. Поэтому при любом патологическом изменении состояния организма ребенка чаще всего появляются однотипные симптомы: беспокойство, отказ от еды, многократная рвота, гипертермия, нарушение функции кишечника. При этом вовсе не обязательно наличие заболевания органов брюшной полости, т.е. в такой ситуации может иметь место и псевдоабдоминальный синдром, в частности при пневмонии, отите, менингите, остеомиелите, инфекционных заболеваниях.
Острые хирургические заболевания в детском возрасте имеют специфические особенности. Такие распространенные заболевания у взрослых, как воспалительные процессы в желчном пузыре, поджелудочной железе, прободная язва желудка и двенадцатиперстной кишки, в детском возрасте почти не встречаются. Тем не менее, спектр острых хирургических заболеваний органов брюшной полости, определяющих клиническую картину острого живота у детей, довольно обширен и разнообразен. Чаще встречаются воспалительные заболевания, кишечная непроходимость, патология половых органов у девочек.
При всем многообразии клинических проявлений острой хирургической патологии органов брюшной полости главными из них являются боль, рвота, повышение температуры, нарушение функции кишечника. Однако характер этих симптомов и их сочетания могут быть различными в зависимости от заболевания и возраста ребенка.

Боль.
Боль в животе - основной и ведущий симптом всех острых хирургических заболеваний и травматических повреждений органов брюшной полости и забрюшинного пространства. У взрослого больного выявление характера боли в животе и установление локализации не представляют больших сложностей, чего нельзя сказать о детях, особенно в первые 1,5-2 года жизни. В этом возрасте трудно говорить о болевом абдоминальном синдроме как таковом, здесь существуют его эквиваленты: беспокойство, плач, отказ от еды, нарушение сна.
Причин для беспокойства грудного ребенка может быть множество: голод, физический дискомфорт, скопление газов в кишечнике. Обычно это и первые проявления катастрофы в брюшной полости. Самый частый ее признак - периодически возникающий плач и состояние ребенка, о котором говорят "сучит ножками". При кишечной инвагинации приступ болей в животе носит резкий, приступообразный характер, ребенок громче плачет, на лице появляется гримаса боли. Отказ ребенка от еды, являющийся также своеобразной реакцией на боль в животе, рассматривается как тревожный симптом, который необходимо учитывать в сочетании с другими проявлениями заболевания. Важно принимать во внимание и факт нарушения сна ребенка. При наличии боли в животе дети обычно плохо спят, часто просыпаются, плачут, бывают беспокойны. В более старшем возрасте, когда дети начинают говорить, они нередко сами жалуются на боль в животе и могут показать место наибольшей болезненности на передней брюшной стенке.
Характер боли в животе при острых хирургических заболеваниях бывает разнообразным. При воспалительных заболеваниях, как правило, отмечается постоянная, нерезко выраженная боль, в то время как все виды кишечной непроходимости сопровождаются выраженной приступообразной болью в животе.
В некоторых случаях боль в животе носит особенно острый характер, дети очень беспокойны, мечутся, не находят себе места, нередко принимая вынужденное положение в постели. Подобные ситуации могут наблюдаться при перекруте кисты яичника, почечнокаменной колике, завороте кишечника и др.
Напротив, тупые боли чаще всего наблюдаются при вяло текущих воспалительных заболеваниях, что может быть связано с реактивностью организма ребенка. Необходимо учитывать, что дети по-разному переносят боль: одни очень остро реагируют даже на слабую боль, другие же бывают спокойнее и при сильной боли. В старшем возрасте обычно не возникает сложностей с выяснением характера и локализации боли, однако нередко дети из-за страха  возможной госпитализации, а тем  более операции,  пытаются дезинформировать врача и скрыть факт наличия боли в животе.
Боль в животе может быть как в покое, так и при движении. Если у ребенка в состоянии покоя боли нет, а возникает она только при движении, можно предполагать стихание воспалительного процесса. Постоянная же боль свидетельствует об остроте процесса в брюшной полости.

Рвота.
Рвота - наиболее частый признак острого процесса в брюшной полости у детей, а также заболеваний других органов и систем. У детей грудного возраста, и, особенно, у новорожденных рвота возникает довольно легко вследствие перекармливания, а также у так называемых детей-аэрофагов. Почти всегда бывает рвота при диспепсиях, кишечных инфекциях, гепатите, менингите, панкреатите и других заболеваниях. Однако при появлении рвоты у ребенка прежде всего необходимо помнить об острых хирургических заболеваниях брюшной полости как наиболее опасных для здоровья.
Механизм возникновения рвоты при остром хирургическом заболевании может быть обусловлен механическими препятствиями в каком-либо отделе желудочно-кишечного тракта. Чем раньше возникает рвота, тем выше уровень непроходимости.
Рвота может иметь и рефлекторный характер. Так, при острой инвагинации кишечника вследствие внедрения одного отдела кишки в просвет другой с ущемлением брыжейки возникает выраженный болевой синдром, приводящий к рефлекторной рвоте, при которой рвотные массы имеют характер желудочного содержимого. Появление застойной рвоты темно-зеленого цвета свидетельствует о механической непроходимости.
Об уровне кишечной непроходимости можно судить по характеру рвотных масс. При высокой кишечной непроходимости (желудок, двенадцатиперстная кишка) рвотные массы имеют кислый запах. В запущенных случаях низкой кишечной непроходимости, особенно в области толстой кишки, возникает так называемая каловая рвота, при этом рвотные массы имеют специфический каловый запах.
Рвота очень часто бывает симптомом и острых воспалительных процессов брюшной полости. При этом вначале рвота бывает одно- или двукратная, но при переходе воспалительного процесса на брюшину становится более частой.
Наличие в рвотных массах примеси крови - тревожный симптом многих хирургических заболеваний: язвенной болезни желудка и двенадцатиперстной кишки, кровотечения из варикозно-расширенных вен пищевода при портальной гипертензии. эрозивного гастрита и др. Рвота с примесью крови возникает при некоторых заболеваниях, обусловленных нарушением свертывающей системы крови. В этих случаях даже незначительная травма приводит к кровотечению из верхних отделов желудочно-кишечного тракта. Иногда примесь крови в рвотных массах может быть не связана с острым хирургическим заболеванием. Чаще это обусловлено заглатыванием крови при носовом кровотечении.
Повышение температуры тела.
Все воспалительные заболевания брюшной полости (аппендицит, различные формы перитонита, мезаденит, холецистит, дивертикулит и др.), как правило, сопровождаются повышением температуры тела. Уровень увеличения температуры отражает степень выраженности основного воспалительного процесса или присоединение осложнения. Необходимо учитывать, что организм маленького ребенка, в основном первых 3 лет жизни, в ответ на любой воспалительный процесс отвечает значительным повышением температуры - до 38-39°С.
Температура может быть субфебрильной или нормальной даже при выраженном воспалительном процессе, если значительно снижена реактивность организма ребенка. Обычно это наблюдается у детей, незадолго до этого перенесших какое-либо другое заболевание или получавших антибиотики.
Применение антибактериальных препаратов значительно изменяет клиническую картину острого хирургического заболевания брюшной полости, поэтому недопустимо их самостоятельное применение родителями до первого осмотра ребенка врачом. Снижается температура тела и при применении обезболивающих средств (анальгин, амидопирин).
Практически все виды кишечной непроходимости в начальных фазах развития заболевании протекают, как правило, с нормальной температурой. Лишь при острой кишечной инвагинации иногда отмечается повышение температуры до субфебрильных цифр. Возможно незначительное повышение температуры (37,1- 37,4°C) даже при копростазе. В этих случаях назначение очистительной клизмы и опорожнение кишечника довольно быстро приводит к нормализации температуры тела ребенка.
У больных с клинической картиной острой кишечной непроходимости температура тела повышается при возникновении в стенке кишечника выраженных циркуляторных нарушений и развитии перитонита.

Нарушение функции кишечника.
Механизм нарушения функции кишечника при острых хирургических заболеваниях органов брюшной полости у детей различен в зависимости от вида хирургической патологии.
При полной кишечной непроходимости, которая возникает в проксимальных отделах тонкой кишки, в первые часы заболевания стул может быть самостоятельным нормальным, либо после клизмы. При острых воспалительных заболеваниях, в частности при остром аппендиците, функция кишечника в большинстве случаев не нарушена, и стул остается нормальным, однако нередко, особенно у детей первых лет жизни, отмечается жидкий стул. Его возникновение можно объяснить в этом возрасте быстрым  переходом воспалительного процесса с червеобразного отростка на свободную брюшную полость и развитием разлитого перитонита. Именно в этих случаях возможны диагностические ошибки: острое хирургическое заболевание принимают за кишечную инфекцию и необоснованно проводят консервативное лечение.
Наличие крови в стуле - тревожный синдром, наблюдаемый как при терапевтических (кишечные инфекции, дизентерия), так и при хирургических заболеваниях. Кровь в стуле появляется при язвенной болезни желудка и двенадцатиперстной кишки, портальной гипертензии, кровоточащем дивертикуле подвздошной кишки, кишечной инвагинации. По характеру крови в стуле при этих заболеваниях можно судить об уровне кишечного кровотечения.
Так, при кровотечении из верхних отделов пищеварительного тракта наблюдается черный стул или кровь темно-вишневого цвета, которая почти всегда перемешана с калом. При кровотечении из дивертикула подвздошной кишки кал также перемешан с кровью, но цвет его несколько ярче. Для кишечной инвагинации примесь крови в стуле является важнейшим симптомом заболевания, который возникает не ранее чем через 5-6 час от начала первых признаков болезни. При этом стул имеет особый характер: слизь, перемешанная с малоизмененной кровью ("малиновое желе"). Более яркая кровь бывает при трещине заднего прохода и кровоточащих полипах дистальных отделов толстой кишки.
При всей важности перечисленных симптомов в установлении диагноза острого хирургического заболевания брюшной полости ведущими являются данные объективного обследования ребенка.
Обследование ребенка.
Важную роль играет умение найти контакт с маленьким пациентом. Это касается и детей, которые уже начинают говорить. Обследованию ребенка предшествуют беседы, доступные его пониманию, в результате чего ребенок успокаивается, что дает возможность выявить важные детали. Осмотр необходимо производить в теплом, хорошо освещенном помещении. Прежде всего обращают внимание на общий вид больного: имеют ли место проявления токсикоза и эксикоза, каково выражение глаз, возбужден ли ребенок или, напротив, адинамичен. Тщательно осматривают кожные покровы и слизистые оболочки, так как распространенные детские инфекционные заболевания -корь, скарлатина, ветряная оспа, паротит, болезнь Боткина - часто протекают с болью в животе. Необходимо критически оценивать каждый элемент высыпания на коже: это может быть сыпь, обусловленная аллергической реакцией на пищевые продукты или лекарства, перегреванием ребенка, это может быть и первым проявлением перечисленных выше детских инфекционных заболеваний. Желтушное окрашивание склер зачастую является начальным признаком болезни Боткина.
Следующий этап исследования - тщательный осмотр живота. Уже его внешний вид часто дает ценную информацию для диагноза. Обращают внимание на вздутие или западение живота, асимметрию, участие в акте дыхания. При высокой кишечной непроходимости (желудок, двенадцатиперстная кишка и начальные отделы тощей кишки), как правило, отмечается выбухание в эпигастральной области, остальные же отделы выглядят запавшими. В этих случаях иногда бывает видна усиленная перистальтика кишечника, а при пилоростенозе - так называемый симптом песочных часов. Преимущественно верхние отделы живота увеличиваются в объеме при спленомегалии. Асимметрия живота наблюдается при новообразованиях брюшной полости (кисты, опухоли), особенно если они уже достигли значительных размеров. При различных видах перитонита всегда имеется вздутие живота за счет выраженного пареза кишечника. Низкая кишечная непроходимость также вызывает вздутие всего живота. В области пораженного органа нередко можно отметить локальное ограничение экскурсии передней брюшной стенки по сравнению с остальными отделами.
Необходимо также тщательно осмотреть паховые области, так как причиной болевого синдрома могут быть ущемленная паховая грыжа, острый лимфаденит, перекрут яичка и некроз гидатид у мальчиков и др.
Важное значение имеет и методика пальпации передней брюшной стенки. Приемы пальпации должны быть наиболее простыми и щадящими. Ощупывать живот ребенку нужно неспеша, мягкими нежными движениями теплой руки, вначале слегка касаясь брюшной стенки и постепенно увеличивая давление. Не следует торопиться осматривать у ребенка больное место. Вначале целесообразно обследовать заведомо интактные области (грудь, руки, ноги)- а затем уже проводить пальпацию брюшной полости. При жалобах ребенка на боль в правой подвздошной области необходимо начинать исследование с левой стороны, постепенно переходя к левому и правому подреберьям, а затем к эпигастральной и мезогастральной областям. И только в заключение пальпируют правую подвздошную область. Щадящая поверхностная пальпация позволяет выявить локализацию наибольшей болезненности, что имеет важное значение для установления источника болей. При воспалительных процессах в области печени и желчного пузыря боль локализуется в правом подреберье; острые патологические процессы в желудке, двенадцатиперстной кишке, поджелудочной железе сопровождаются, болью в эпигастральной зоне. При остром аппендиците, особенно в раннем возрасте, боль может локализоваться не в правой подвздошной области, а несколько выше, что обусловлено более высоким расположением слепой кишки. Гинекологические заболевания у девочек сопровождаются болью в нижних отделах живота. Однако следует помнить, что аналогичная клиническая картина наблюдается при так называемом тазовом расположении червеобразного отростка.
Для определения болезненной зоны (симптом локальной болезненности) у детей довольно широко применяют метод легкой перкуссии (поколачивания) по передней брюшной стенке (симптом Менделя). Пальпация живота у детей должна быть легкой, и ее также следует начинать с безболезненной зоны. Особенно ценные данные можно получить при воспроизведении этого симптома у детей первых лет жизни. При положительном симптоме Менделя на лице ребенка появляется гримаса боли, усиливаются плач и беспокойство.
Пальпация живота позволяет выявить защитное пассивное мышечное напряжение передней брюшной стенки - важнейший симптом острого воспалительного заболевания в брюшной полости, который возникает в области основного процесса.
У детей младшего возраста довольно часто бывает трудно отдифференцировать истинное (пассивное) мышечное напряжение от активного, которое возникает при плаче и беспокойстве ребенка. В этих случаях нужно терпеливо выждать момент вдоха и расслабления передней брюшной стенки. Если поверхностная пальпация не позволяет выявить локальную болезненность, необходимо провести глубокую пальпацию живота, исследуя кишечник, основание брыжейки, почки, органы малого таза, печень, селезенку. Поскольку болевой синдром у детей нередко связан с задержкой стула, целесообразно, если нет противопоказаний, обследовать живот до и после очистительной клизмы.
Во всех трудных для диагностики случаях проводят пальцевое ректальное бимануальное исследование. Это дает возможность уточнить диагноз, особенно в препубертатном и пубертатном периодах у девочек (фолликулярные и лютеиновые кисты, перекрут кисты яичника), а также выявить осложнение острого аппендицита (аппендикулярный инфильтрат).
Широкое распространение у хирургов получил осмотр ребенка во время сна. Однако для наступления физиологического сна ребенка часто требуется много времени. Учитывая быстроту динамики нарастания деструктивных изменений в брюшной полости у детей раннего возраста, столь длительное выжидание является особенно нежелательным. В этой ситуации у беспокойных детей оправдан метод осмотра в состоянии медикаментозного сна (А. Ф. Дронов). Метод сводится к следующему: после очистительной клизмы в прямую кишку с помощью катетера (примерно на расстояние 10-15 см) шприцем вводят 3 % раствор хлоралгидрата, подогретый до температуры тела, в следующих дозах: в возрасте до 1 года - 10-15 мл, от 1 года до 2 лет - 15-20 мл, от 2 до 3 лет - 20-25 мл. Через 15 -20 мин после введения хлоралгидрата наступает  сон,   и  можно приступать к обследованию живота. При этом сохраняются пассивное напряжение передней брюшной стенки и локальная болезненность; симптомы легко выявляются, поскольку исчезает двигательное возбуждение, снимаются психоэмоциональные реакции и активное напряжение мышц. Данный метод практически не имеет противопоказаний.
Из лабораторных методов исследования в ургентной абдоминальной хирургии чаще всего производят общий анализ крови, мочи, а по показаниям - биохимическое исследование крови.
В детской хирургии, помимо традиционных методов исследования (рентгенологические, инструментальные, функциональные и др.), все шире используют новые диагностические методы, отвечающие следующим необходимым в педиатрии требованиям: высокая информативность, объективность полученных данных, атравматичность, минимальные побочные воздействия на растущий организм. К таким методам относятся двухмерное ультразвуковое сканирование, компьютерная томография, радионуклидное сканирование, ангиография.

Лапароскопия. Среди эндоскопических методов исследования, кроме фиброгастроскопии и фиброколоноскопии, наибольшее распространение имеет лапароскопия. Современные педиатрические модели лапароскопов позволяют проводить лапароскопическое исследование у детей всех возрастных групп, включая новорожденных. Чаще всего показаниями к лапароскопии являются случаи затруднительной диагностики ряда острых хирургических заболеваний органов брюшной полости (острый аппендицит, заболевания половых органов у девочек, непроходимость кишечника и др.), закрытые повреждения живота, новообразования.

ПРИОБРЕТЕННАЯ КИШЕЧНАЯ НЕПРОХОДИМОСТЬ

Основные  вопросы темы:
1.	Причины приобретенной непроходимости кишечника
2.	Классификация приобретенной непроходимости кишечника
3.	Клиника и диагностика
4.	Лечение и прогноз

Вопросы контроля исходного уровня:
1.	Кровоснабжение кишечника
2.	Понятия механической и динамической кишечной непроходимости
У детей, как и у взрослых, приобретенную кишечную непроходимость разделяют на два основных вида - механическую и динамическую. В детском возрасте в группе механической непроходимости выделяют обтурационную, странгуляционную и инвагинацию кишечника. В свою очередь причиной обтурационной непроходимости нередко является копростаз при врожденном стенозе прямой кишки, болезни Гиршпрунга, мегаколоне или свищевой форме атрезии прямой кишки. Странгуляционная непроходимость иногда вызывается нарушением обратного развития желточного протока или следствием других пороков развития.
Тем не менее, в практике детской хирургии чаще всего приходится сталкиваться со спаечной кишечной непроходимостью, инвагинацией кишечника, динамической непроходимостью.
ИНВАГИНАЦИЯ
Инвагинация - внедрение одного отдела кишечника в просвет другого - наиболее частый вид приобретенной кишечной непроходимости. Этот своеобразный вариант кишечной непроходимости встречается преимущественно у детей грудного возраста (85 - 90%), особенно часто I период с 4 до 9 мес. Мальчики заболевают почти в 2 раза чаще девочек. У детей старше 1 года инвагинация наблюдается редко и в большинстве случаев бывает связана с органической природой (дивертикул подвздошной кишки, гиперплазия лимфоидной ткани, полип, злокачественное новообразование и др.).
Инвагинация относится к смешанному, или комбинированному, виду механической непроходимости, поскольку в ней сочетаются элементы странгуляции (ущемление брыжейки внедренной кишки) и обтурации (закрытие просвета кишки инвагинатом).
В зависимости от локализации различают илеоцекальную (более 95%) тонкокишечную и толстокишечную инвагинацию.
Термин "илеоцекальная инвагинация" является собирательным и применяется для обозначения всех видов инвагинации в илеоцекальном углу. Из всех форм инвагинации этой области чаще всего встречается подвздошно-ободочная, когда тонкая кишка внедряется через илеоцекальный клапан (баугиниеву заслонку) в восходящую кишку. Реже возникает слепоободочная инвагинация, при которой дно слепой кишки инвагинируется в восходящий отдел толстой кишки вместе с червеобразным отростком.
Изолированное внедрение тонкой кишки в тонкую (тонкокишечная инвагинация) и толстой в толстую (толстокишечная инвагинация) в общей сложности отмечается не более чем у 2 - 3 % всех больных с инвагинацией кишечника.
При инвагинации различают наружную трубку (влагалище) и внутреннюю (инвагинат). Начальный отдел внедрившейся кишки носит название головки инвагината.
Учитывая, что большинство инвагинаций наблюдается в илеоцекальном отделе кишечной трубки, причину ее возникновения связывают с функциональными и анатомическими особенностями строения этой области у детей раннего возраста (недостаточность илеоцекального клапана, высокая подвижность толстой кишки и др.). Имеет значение и расстройство правильного ритма перистальтики, заключающееся в нарушении координации сокращения продольных и круговых мышц с преобладанием сократительной способности последних. К некоординированному сокращению мышечных слоев могут привести изменения режима питания, введение прикорма, воспалительные заболевания кишечника, в том числе энтеровирусная инфекция.
Клиника и диагностика. Клинические проявления инвагинации зависят от ее вида и длительности. Типичными симптомами являются приступообразная боль в животе, двигательное беспокойство, одно- или двукратная рвота, задержка стула, кровянистые выделения из прямой кишки, пальпируемая "опухоль" в животе. В большинстве случаев заболевание начинается внезапно, среди полного здоровья и возникает, как правило, у хорошо упитанных детей. Ребенок становится резко беспокойным, плачет, отказывается от еды. Лицо приобретает страдальческое выражение. Приступ беспокойства заканчивается так же внезапно, как и начинается, но через короткий промежуток времени повторяется вновь. Обычно такие яркие клинические проявления наблюдаются у детей, страдающих подвздошно-ободочным внедрением.
Приступы боли в начале заболевания бывают частыми с небольшими интервалами затишья (3 - 5 мин). Это связано с волнами кишечной перистальтики и продвижением инвагината внутри кишки. В светлый промежуток ребенок обычно успокаивается на 5-10 мин, а затем возникает новый приступ боли.
Вскоре после начала заболевания появляется рвота, которая имеет рефлекторный характер и связана с ущемлением брыжейки инвагинированного участка кишки. В более поздние сроки развития инвагинации возникновение рвоты обусловлено полной непроходимостью кишечника.
Температура чаще всего остается нормальной. Лишь при запущенных формах инвагинации отмечается повышение температуры. В первые часы может быть нормальный стул за счет опорожнения дистального отдела кишечника. Спустя некоторое время из прямой кишки вместо каловых масс отходит кровь, перемешанная со слизью. Это объясняется выраженным нарушением кровообращения в инвагинированном участке кишки; чаще всего симптом появляется не менее чем через 5-6 ч от начала первого приступа боли в животе.
В ряде случаев выделение крови отсутствует на протяжении всего периода заболевания и в основном наблюдается при слепоободочной форме инвагинации. Это связано с тем, что у таких больных практически не возникает странгуляции, а преобладают явления обтурации. Соответственно клинические проявления при слепоободочной и толстокишечной формах инвагинации менее выражены: не отмечается резкого беспокойства ребенка, приступы боли в животе значительно реже и менее интенсивны. При этих формах инвагинации в начальных стадиях заболевания рвота наблюдается лишь у 20 - 25 % больных.
Обследование брюшной полости при подозрении на кишечную инвагинацию необходимо производить между приступами боли. В отличие от всех других форм непроходимости кишечника при инвагинации не наблюдается вздутия живота, особенно в первые 8 - 12 ч заболевания. Это объясняется, по-видимому, тем, что газы кишечника некоторое время проникают в просвет инвагината. В этот период живот бывает мягким, доступным глубокой пальпации во всех отделах. Справа от пупка, чаще к области правого подреберья, можно обнаружить опухолевидное образование мягкоэластической консистенции, малоболезненное при пальпации. Местоположение инвагината зависит от подвижности кишечника и сроков заболевания. Иногда при значительной длине брыжейки он достигает дистальных отделов толстой кишки; описаны случаи, когда инвагинат даже выпадает из заднего прохода.
При поздней диагностике заболевания, когда уже имеются выраженные циркуляторные нарушения в стенке кишки с развитием некроза и явлений перитонита, живот становится вздутым, напряженным, резко болезненным при пальпации во всех отделах.
При нечеткой клинической картине заболевания и недостаточно убедительных данных, полученных при обследовании живота, целесообразно провести пальцевое ректоабдоминальное исследование. Это помогает иногда бимануально обнаружить инвагинат. По извлечении пальца из прямой кишки вслед за ним довольно часто выделяется кровь со слизью.
Большое значение для ранней диагностики инвагинации имеет рентгенологическое исследование, которое проводят следующим образом. В прямую кишку под рентгенологическим контролем с помощью баллона Ричардсона осторожно нагнетают воздух и следят за постепенным его распространением по толстой кишке до выявления головки инвагината.
При этом инвагинат хорошо виден на фоне газа в виде округлой тени с четкими контурами, чаще в области печеночного утла толстой кишки.
Дифференциальный диагноз. Кишечную инвагинацию часто принимают за дизентерию. Тщательно собранный анамнез, характер выделений из прямой кишки, а также данные ректального исследования помогают вовремя поставить правильный диагноз и избежать диагностической ошибки.
У больных дизентерией в кале содержится значительное количество слизи, зелени и отмечаются прожилки алой крови. В противоположность этому при инвагинации, как правило, из заднего прохода выделяется кровь со слизью без примеси каловых масс. Своевременное рентгенологическое исследование толстой кишки воздухом помогает избежать диагностической ошибки.
Лечение. Инвагинацию можно устранить как консервативным, так и хирургическим лечением. Консервативное расправление показано при раннем поступлении ребенка в клинику (в первые 12 часов от начала заболевания). Во время диагностического рентгенологического исследования продолжают нагнетание воздуха с целью расправления инвагината, критерием чего является проникновение воздуха в дистальный отдел подвздошной кишки. По окончании исследования в прямую кишку вводят газоотводную трубку для удаления избыточного газа из толстой кишки.
После расправления инвагината ребенок обычно успокаивается и засыпает. Чтобы окончательно удостовериться в полном расправлении инвагината, ребенка обязательно госпитализируют для динамического наблюдения и исследования желудочно-кишечного тракта с бариевой взвесью, которую дают в киселе и следят за ее пассажем по кишечнику. Обычно при отсутствии тонкокишечной инвагинации контрастное вещество через 3 - 4 ч обнаруживается в начальных отделах толстой кишки, а спустя некоторое время бариевая взвесь появляется со стулом. Метод консервативного расправления инвагинации эффективен в среднем до 65 %.
В случаях поступления больного позже чем через 12 часов от начала заболевания резко возрастает вероятность расстройства кровообращения ущемленного отдела кишечника. Повышение внутрикишечного давления в этом случае опасно, а при расправлении инвагината невозможно оценить жизнеспособность пострадавших участков кишки. В подобных случаях, а также при неэффективности консервативного расправления ставят показания к оперативному лечению.
Оперативное лечение состоит в лапаротомии и ручной дезинвагинации, которую производят не вытягиванием внедренной кишки, а методом осторожного "выдавливания" инвагината, захваченного всей рукой или двумя пальцами.
Если не удалось осуществить дезинвагинацию или обнаружен некроз участка кишки, производят резекцию в пределах здоровых тканей с наложением анастомоза.
Такая тактика логична и оправданна, но несовершенна. Нередко выраженное ущемление и некроз инвагината развиваются через несколько часов от начала заболевания, а в сроки, превышающие 12 ч дезинвагинация во время операции не вызывает затруднений, кишечник минимально изменен.
Имеется еще одно несоответствие, связанное, по-видимому, с применением миорелаксантов: консервативное лечение безуспешно, а на операции инвагинат расправляется довольно легко. Число таких больных не так уж мало.
Включение лапароскопии в комплекс лечебно-диагностических мероприятий при кишечной инвагинации может существенно повысить процент больных, вылеченных консервативно. Цель лапароскопии визуальный контроль за расправлением инвагината и оценка жизнеспособности кишечника. Показаниями к этому методу являются: 1) неэффективность консервативного лечения при ранних сроках заболевания; 2) попытка консервативного расправления инвагината при позднем поступлении (исключая осложненные формы заболевания); 3) выяснение причины инвагинации у детей старше 1 года.
При лапароскопии визуально определяют место внедрения подвздошной кишки в толстую. Слепая кишка и червеобразный отросток чаще также вовлечены в инвагинат. При инструментальной пальпации определяется выраженное уплотнение толстой кишки на участке внедрения. После обнаружения инвагината производят его дезинвагинацию путем введения воздуха в толстую кишку через заднепроходное отверстие под давлением 100 -120 мм рт. ст. Дезинвагинация считается эффективной при обнаружении расправления купола слепой кишки и заполнении воздухом подвздошной кишки. При отсутствии резких циркуляторных изменений и объемных образований (нередкая причина инвагинации у детей старше 1 года) лапароскопию завершают. Такая тактика позволяет существенно снизить количество лапаротомий при кишечной инвагинации.
Прогноз зависит от сроков поступления в хирургический стационар. При ранней диагностике и своевременно произведенной операции летальных исходов от инвагинации, как правило, не наблюдается.

ДИНАМИЧЕСКАЯ НЕПРОХОДИМОСТЬ
Динамическая непроходимость - одна из самых частых форм кишечной непроходимости в детском возрасте. Различают паралитическую и спастическую формы. Преобладает первая.
У новорожденных и грудных детей динамическая непроходимость возникает как результат функциональной неполноценности пищеварительной системы на фоне родовой черепно-мозговой травмы, пневмонии, кишечных заболеваний и сепсиса, а также после операций на брюшной и грудной полостях. У старших детей динамическая непроходимость чаще развивается в послеоперационном периоде.
Явления паралитической непроходимости поддерживаются гипокалиемией, обусловленной большой потерей солей и жидкости с рвотными массами, а также недостаточным поступлением в организм калия при парентеральном питании.
Клиника и диагностика. Для динамической непроходимости характерны повторная рвота с примесью зелени, нарастающее вздутие живота, задержка стула и газов и выраженная интоксикация. В результате высокого стояния диафрагмы затрудняется дыхание. Живот мягкий, перистальтика не прослушивается.
При рентгенологическом исследовании выявляют множественные чаши Клойбера, однако диаметр их невелик и расширение равномерно, в то время как при механической непроходимости особенно резко расширены кишечные петли над препятствием.
Лечение. При динамической непроходимости прежде всего нужно установить ее причину. Одновременно проводят борьбу с парезом кишечника. В схему лечения пареза кишечника входят: 1) прямая стимуляция сократительной активности мускулатуры желудочно-кишечного тракта (очистительные, сифонные и гипертонические клизмы, внутривенное капельное введение растворов калия и натрия хлорида под контролем ЭКГ, электростимуляция); 2) блокада дуги рефлексов, определяющих торможение двигательной активности кишечника (применение прозерина, паранефральной новокаиновой блокады); 3) разгрузка желудочно-кишечного тракта (постоянный желудочный зонд, интубация кишечника).

ОБТУРАЦИОННАЯ НЕПРОХОДИМОСТЬ

Причинами обтурационной непроходимости у детей чаше всего являются копростаз, реже - опухоль, аскариды.
Копростаз - закупорка кишечника плотными каловыми массами. Встречается у детей в любом возрасте. Причиной ее могут быть вялая функция кишечника у ослабленных детей, а также порок развития мышц передней брюшной стенки, сопровождающийся атонией органов брюшной полости. Часто развитию копростаза способствуют аномалии и пороки развития толстой кишки (мегадолихоколон, болезнь Гиршпрунга, врожденное и рубцовое сужение прямой кишки).
Клиника и диагностика. В анамнезе всегда имеются указания на ранний запор. Стул, как правило, удается получить только после очистительной клизмы. Неправильный режим питания и недостаточный уход за ребенком приводят к каловым завалам, образованию каловых камней, которые в ряде случаев принимают за опухоль брюшной полости.
При полной обтурации кишечного просвета состояние ребенка ухудшается, нарастает вздутие живота, появляется рвота, развиваются явления интоксикации.
Провести дифференциальную диагностику между копростазом и опухолью кишечника помогает консистенция опухолевидного образования, которая при копростазе имеет тестоватый характер. Отмечается положительный симптом "ямки", остающейся при надавливании. В сомнительных случаях применяют контрастное рентгенологическое исследование, во время которого контрастная смесь обтекает каловый камень со всех сторон и тень его ясно контурируется.
Лечение. Необходимо настойчивое применение повторных клизм с 1%-ным раствором натрия хлорида комнатной температуры. Если обычные клизмы не помогают, делают повторно сифонные клизмы до полного размывания каловых масс и восстановления проходимости кишечника. Неправильная техника сифонных клизм может привести к тяжелым осложнениям, так как при размывании слежавшихся каловых масс теплым изотоническим раствором натрия хлорида начинается всасывание жидкости и развивается тяжелая каловая интоксикация, сопровождающаяся резким ухудшением состояния вплоть до отека мозга.
Помимо сифонных клизм, назначают диету, богатую клетчаткой, растительное или вазелиновое масло внутрь, легкие послабляющие средства, курс электростимуляции кишечника аппаратом "Амплипульс", всего 15-20 сеансов.
После ликвидации копростаза производят рентгеноконтрастное исследование кишечного тракта.
Глистная непроходимость в последние годы практически не встречается. Причиной закупорки в описанных наблюдениях являлся клубок аскарид, остановившихся у илеоцекального клапана. Если консервативными мероприятиями (сифонные клизмы) устранить непроходимость не удается, осуществляют оперативное вмешательство. После операции назначают противоглистное лечение.

ОСТРЫЙ АППЕНДИЦИТ

Основные вопросы темы:
1.	Этиология и патогенез острого аппендицита
2.	Клиника и диагностика
3.	Дифференциальная диагностика
4.	Лечение и прогноз

Вопросы контроля исходного уровня:
1.	Виды расположения червеобразного отростка
2.	Особенности строения илеоцекального сегмента у детей.
Воспаление червеобразного отростка (острый аппендицит) - наиболее распространенное хирургическое заболевание в детском возрасте. В отличие от взрослых острый аппендицит у детей клинически протекает более тяжело, а диагностика значительно сложнее. Эти закономерности наиболее выражены у детей первых лет жизни, что обусловлено анатомо-физиологическими особенностями детского организма.
Острым аппендицитом может заболеть ребенок любого возраста, даже грудной и новорожденный. Тем не менее, в первые 2 года жизни это заболевание встречается довольно редко. В последующие годы частота заболевания постепенно нарастает, достигая пика в возрасте 9-12 лет.
Общий уровень заболеваемости острым аппендицитом составляет 0,5 - 0,8 на 1000 детей.
Этиология и патогенез. Острый аппендицит у детей, как и у взрослых, имеет общие закономерности в основных этиологических и патогенетических звеньях. Однако анатомо-физиологические особенности илеоцекальной области и брюшной полости в целом обусловливают существенные отличия в частоте и развитии воспалительного процесса в червеобразном отростке у детей различных возрастных групп.
В развитии воспаления ведущая роль принадлежит собственной микрофлоре отростка или кишечника. Это положение подтверждают наблюдения, свидетельствующие, что у большинства больных не обнаруживается предшествующих или сопутствующих очагов инфекции в других органах. У небольшой группы детей нельзя исключить возможность гематогенного или лимфогенного инфицирования, так как прослеживается непосредственная связь с такими предшествующими заболеваниями, как фолликулярная ангина, скарлатина, отит и др.
Общеизвестно и влияние нервно-сосудистого фактора: чем младше ребенок, тем быстрее наступают деструктивно-некротические изменения в стенке отростка, что обусловлено незрелостью нервной системы аппендикса и илеоцекальной области. Определенную роль играют и местные иммунологические реакции, связанные с возрастными особенностями фолликулярного аппарата. В этом плане видят параллелизм между малым количеством фолликулов в отростке и редкостью острого аппендицита у детей первых лет жизни, особенно грудного возраста. Редкость аппендицита у детей до 1 года объясняется также особенностями анатомического строения червеобразного отростка (отсутствие застоя кишечного содержимого) и характером питания в этом возрасте (преимущественно жидкая молочная пища).
Ряд морфологических особенностей у детей раннего возраста предрасполагает к быстрому развитию сосудистых реакций и присоединению бактериального компонента. Слизистая оболочка кишечника более проницаема для микрофлоры и токсических веществ, чем у взрослых, поэтому при нарушении сосудистой трофики происходит быстрое инфицирование пораженного червеобразного отростка и ускоренное развитие выраженных деструктивных форм аппендицита.
Развитию воспалительного процесса способствуют перенесенные соматические и инфекционные заболевания, врожденные аномалии червеобразного отростка (перекруты, перегибы), попадание в отросток инородных тел или паразитов, образование камней.
Особенности строения червеобразного отростка, характер питания в грудном, ясельном возрасте и у более старших детей также влияют на частоту заболевания в определенных возрастных группах.
Предложено несколько клинико-морфологических классификаций острого аппендицита. В соответствии с этим принципом целесообразно выделение двух основных групп:
•	деструктивно-гнойные формы воспаления (флегмонозный, гангренозный, гангренозно-перфоративный аппендицит);
•	недеструктивные изменения в червеобразном отростке ("катаральные" формы, а также хронический аппендицит или обострение хронического аппендицита).
Особую сложность для клинициста и морфолога представляют недеструктивные формы, макроскопическая оценка которых не исключает субъективизма. К тому же у детей и при микроскопическом исследовании трудно установить, имеется ли в червеобразном отростке истинное катаральное воспаление или эти изменения вторичны и вызваны острыми респираторно-вирусными заболеваниями, мезаденитом, кишечной инфекцией и т.д. Вопрос о недеструктивных формах аппендицита имеет не только теоретическое, но и большое практическое значение. Чаще всего за этой формой скрываются другие заболевания, симулирующие острый аппендицит.
Клиника и диагностика. Клинические проявления острого аппендицита у детей вариабельны и во многом зависят от реактивности организма, анатомического положения червеобразного отростка и возраста ребенка. Общей характеристикой клинической картины заболевания является преобладание общих неспецифических симптомов над местными. В генезе подобных реакций имеет значение относительная незрелость отдельных органов и систем, в первую очередь центральной и вегетативной нервной системы. Эти явления выражены тем больше, чем младше ребенок.
Сложности диагностики усугубляются также трудностями обследования детей и выявления объективных местных признаков заболевания. Их достоверность зависит от ряда факторов (степень контакта с ребенком, владение методикой обследования, индивидуальный опыт врача и пр.). Влияние этих факторов особенно сказывается при распознавании острого аппендицита у детей раннего возраста.
В связи с этими обстоятельствами необходимо отдельно рассматривать вопросы клинической картины у детей старшей возрастной группы и первых 3 лет жизни.
Клиническая картина острого аппендицита у старших детей более отчетлива и складывается из следующих основных признаков, выявляемых из анамнеза: боль в животе, повышение температуры тела, рвота, иногда нарушение функции кишечника.
Заболевание начинается с того, что ребенок жалуется на не очень резкую, но постоянную боль в животе, в основном в правой подвздошной области. При этом родители отмечают, что дети становятся менее активными, отказываются от еды. Для аппендицита характерна непрерывная боль, которая не исчезает, а лишь несколько стихает. Непрерывность боли ведет к нарушению сна: сон поверхностный, дети часто просыпаются. Установлено, что заболевший ребенок хуже всего спит в первую ночь заболевания.
В некоторых случаях болевой синдром очень выражен, дети беспокойны, жалуются на резкую боль в животе, иногда принимают вынужденное положение. Такая болевая реакция обусловлена перерастяжением переполненного гноем аппендикса. Старшие дети почти всегда могут указать локализацию боли: чаще в правой подвздошной области.
Обычно вскоре после приступа спонтанной боли в животе отмечается повышение температуры тела до 37,5 - 38°С. Однако при тяжелых осложненных формах аппендицита (перитонит, периаппендикулярный абсцесс) температура тела может повышаться еще больше - до 39°С и выше. Иногда температура бывает нормальной на всех стадиях заболевания (почти в 15% случаев), хотя на операции у 1/3 больных обнаруживаются грубые деструктивные изменения в червеобразном отростке вплоть до перфорации и развития локального перитонита.
Одним из наиболее постоянных симптомов острого аппендицита у детей является рвота, которая наблюдается почти у 1/4 больных и носит рефлекторный характер. В последующие дни заболевания при развитии разлитого гнойного перитонита рвота становится многократной, нередко в рвотных массах имеется примесь желчи.
В начальных стадиях заболевания язык чистый, иногда слегка обложен. С развитием токсикоза и эксикоза язык становится сухим, шероховатым, на нем появляются наложения.
Симптом расхождения пульса и температуры наблюдается редко - при запоздалом диагнозе, когда имеются все признаки перитонита (5 - 7% случаев).
Довольно часто отмечается задержка стула (35% случаев). Жидкий стул, который появляется не ранее чем на 2-е сутки от начала заболевания, удается обнаружить почти у 7 % больных.
Такая клиническая картина бывает у большинства больных: как правило, особых трудностей при диагностике не возникает.
При атипичной локализации червеобразного отростка (15% больных) значительно меняются клинические проявления заболевания. Возникают дополнительные симптомы, обусловленные вовлечением в воспалительный процесс органов брюшной полости.
Так, при низком (тазовом) расположении отростка боль обычно локализуется над лобком или несколько правее, чаще носит схваткообразный характер и иногда сопровождается тенезмами. В таких случаях возможно появление жидкого кала. При выраженном вторичном проктите каловые массы могут быть в виде "плевков" слизи и даже с примесью небольшого количества крови. Возможно также частое болезненное мочеиспускание.
Если червеобразный отросток расположен ретроцекально, особенно при значительном подкожном жировом слое, боль в животе выражена умеренно. Это связано с более поздним вовлечением в воспалительный процесс париетальной брюшины передней брюшной стенки.
При медиальной локализации (верхушка отростка находится ближе к средней линии, у корня брыжейки) преобладают такие симптомы, как сильная приступообразная боль в животе, многократная рвота, жидкий стул, а иногда и вздутие кишечника.
Значительно меняется течение заболевания, развивающегося на фоне применения антибиотиков. Клинические признаки острого аппендицита становятся менее выраженными. Важно иметь в виду, что стушевывание остроты клинических проявлений у этого контингента больных не всегда свидетельствует о купировании деструктивного и гнойного процесса, у этой группы пациентов часто образуются аппендикулярные абсцессы.
Распознавание острого аппендицита у детей имеет свои особенности. При обследовании живота ребенка необходимо обращать внимание на три основных симптома, выраженных в правой подвздошной области: болезненность при пальпации, защитное мышечное напряжение и симптом Щеткина - Блюмберга. Все остальные симптомы имеют вспомогательное значение.
Пальпацию живота всегда необходимо начинать с левой подвздошной области в направлении против часовой стрелки. При наличии острого аппендицита можно отметить усиление болезненности при пальпации правой подвздошной области (симптом Филатова). Это очень важный признак, получивший в практике название "локальная болезненность".
Вторым основным симптомом острого аппендицита является защитное мышечное напряжение в правой подвздошной области (defanse musculaire). Чтобы убедиться в отсутствии или наличии ригидности мышц передней брюшной стенки, важно каждый раз при перемене точки пальпации держать руку на животе, дожидаясь вдоха больного. Это позволяет отдифференцировать активное напряжение от пассивного (истинного), которое более четко улавливается при сравнительной повторной пальпации. Можно воспользоваться и другим приемом: врач кладет правую руку на левую подвздошную область больного, а левую руку - на правую подвздошную область и, попеременно нажимая справа и слева, старается определить разницу в тонусе мышц.
В заключение исследования необходимо определить наличие симптома Щеткина - Блюмберга. Данный симптом определяют путем постепенного глубокого давления на переднюю брюшную стенку с последующим быстрым отнятием руки в различных отделах живота. При положительном симптоме Щеткина - Блюмберга ребенок реагирует на возникающую боль.
При атипичных локализациях червеобразного отростка меняются характер течения воспалительного процесса, локализация и интенсивность главных местных симптомов, что нередко является поводом для ошибочного диагноза. В сомнительных случаях во избежание диагностической ошибки необходимо динамическое наблюдение за больным в условиях стационара. Правильно оценить субъективные и объективные данные атипичного острого аппендицита у детей при первом осмотре бывает трудно. Необходимо учитывать, что дети школьного возраста в некоторых случаях склонны скрывать боль, боясь операции, иногда, наоборот, агравируют. Это имеет большое практическое значение, так как немало хирургов производят аппендэктомию у детей по расширенным показаниям, т.е. предпочитают гипердиагностику, которая не всегда оправдана.
Во всех случаях необходимо пальцевое ректальное бимануальное исследование. Это дает возможность выявить наличие осложнения (аппендикулярный инфильтрат) или уточнить диагноз, особенно в препубертатном и пубертатном периодах у девочек (фолликулярные и лютеиновые кисты, перекрут кисты яичника, боль при неустановившемся менструальном цикле).
Обследование через прямую кишку при наличии острого аппендицита позволяет выявить болезненность стенки прямой кишки спереди и справа, а в некоторых случаях и нависание свода справа. Эти данные и постоянно обнаруживаемая локальная болезненность при пальпации являются в сочетании с другими симптомами определенным критерием для установления диагноза.
Изменение количества лейкоцитов при аппендиците у детей, как и у взрослых, не всегда характерно, но в сочетании с другими факторами имеет определенное значение. Чаще всего (около 65%) отмечается повышение содержания лейкоцитов до 15 - 17 • 10-9/л со сдвигом лейкоцитарной формулы влево.
Гиперлейкоцитоз (20 - 30 • 10-9/л) наблюдается у 7 % больных. В остальных случаях количество лейкоцитов бывает в пределах нормы или даже ниже.
В последние годы большое значение придается разработке объективных методов диагностики острого аппендицита. Это обусловлено тем, что при использовании традиционных методов исследования остается высоким процент гипо- и гипердиагностических ошибок.
Из неинвазивных методов диагностики заслуживает внимания электромиография (ЭМГ) передней брюшной стенки, которая позволяет дать количественную и качественную оценку симптома мышечного напряжения при различных формах острого аппендицита. Метод позволяет точно регистрировать кардинальный признак острого аппендицита - защитное мышечное напряжение. Диагностические ошибки по данным ЭМГ составляю! около 6%. Электромиографический диагноз следует считать исключительно синдромологическим. Если с помощью ЭМГ-исследования можно установить наличие воспалительного процесса в брюшной полости, его распространенность, а нередко и локализацию, то установить достоверно этнологию удается не во всех случаях. В этом отношении несомненное преимущество имеет лапароскопия.
Щадяшая методика лапароскопии с учетом возрастных особенностей детей, применение педиатрических моделей лапароскопов позволяют принципиально по-новому подойти к диагностике аппендицита. Применение пункционной лапароскопии при сомнительных результатах других методов исследования позволяет не только безошибочно установить наличие или отсутствие воспаления в червеобразном отростке, но и при исключении диагноза острого аппендицита провести щадящую ревизию органов брюшной полости и у 1/3 больных выявить истинную причину болевого абдоминального синдрома.
Особенности клиники и диагностики острого аппендицита у детей раннего возраста. Вариабельность и своеобразие течения острого аппендицита у детей раннего возраста зависят не только от реактивности детского организма, но и от возрастных анатомо-физиологических особенностей.
Во-первых, вследствие функциональной незрелости нервной системы в этом возрасте почти все острые воспалительные заболевания имеют сходную клиническую картину (высокая температура, многократная рвота, нарушение функции кишечника). Во-вторых, воспалительный процесс в червеобразном отростке у детей протекает чрезвычайно бурно. В то же время механизмы отграничения его слабо выражены. В-третьих, существуют специфические трудности в обследовании детей раннего возраста. Беспокойство, плач, сопротивление осмотру затрудняют выявление основных местных симптомов острого аппендицита. Для того чтобы своевременно поставить диагноз острого аппендицита, необходимо знать особенности клиники и диагностики этого заболевания у детей раннего возраста. До настоящего времени у врачей существует ошибочное мнение, что острый аппендицит у детей до 3 лет встречается крайне редко. При первичном осмотре больных участковым педиатрам, врачам поликлиник, скорой и неотложной помощи необходимо проявлять повышенную настороженность к жалобам детей на боль в животе.
Если у детей старшего возраста ведущее значение имеют жалобы на боль в правой подвздошной области, то у детей первых лет жизни прямых указаний на боль нет и судить о наличии этого симптома можно лишь по ряду косвенных признаков. Наиболее важным из них является изменение поведения ребенка. Более чем в 75% случаев родители отмечают, что ребенок становится вялым, капризным, малоконтактным. Беспокойное поведение больного следует связать с нарастанием боли. Непрерывность боли ведет к нарушению сна, что является характерной особенностью заболевания детей младшего возраста и встречается почти у 1/3 больных.
Повышение температуры при остром аппендиците у детей первых лет жизни отмечается почти всегда (95 %). Нередко температура достигает 38 -39°С. Довольно постоянным симптомом является рвота (85%). Для детей раннего возраста характерна многократная (3-5 раз) рвота, что относится к особенностям течения заболевания в этом возрасте. Своеобразие этих симптомов у маленьких детей в начале заболевания объясняется недифференцированностью реакции центральной нервной системы ребенка на локализацию и степень воспалительного процесса.
Почти в 15% случаев отмечается жидкий стул. Расстройство стула наблюдается в основном при осложненных формах аппендицита и тазовом расположении червеобразного отростка. Жалобы на боль в правой подвздошной области у детей в этом возрасте почти не встречаются. Обычно, боль локализуется вокруг пупка, как и при любом интеркуррентном заболевании, протекающим с абдоминальным синдромом. Такая локализация связана с рядом анатомо-физиологических особенностей: неспособностью точно локализовать место наибольшей болезненности вследствие недостаточного развития корковых процессов и склонностью к иррадиации нервных импульсов, близким расположением солнечного сплетения к корню брыжейки. Важную роль играет быстрое вовлечение в воспалительный процесс мезентериальных лимфатических узлов.
При диагностике ориентируются на те же основные симптомы, что и у детей старшего возраста (пассивное мышечное напряжение и локальная болезненность в правой подвздошной области). Однако обнаружить указанные признаки у детей первых лет жизни чрезвычайно трудно. Они обусловлены возрастными особенностями психики и в первую очередь двигательным возбуждением и беспокойством при осмотре. В этих условиях почти невозможно определить локальную болезненность и отдифференцировать активное мышечное напряжение от пассивного.
Поскольку эти симптомы являются наиболее важными, а у маленьких детей нередко и единственными, указывающими на локализацию патологического процесса, особое значение следует придавать их выявлению. Определенную роль играет умение найти контакт с маленьким ребенком. Это касается детей, которые уже начинают говорить. Обследованию ребенка предшествуют беседы, доступные его пониманию, в результате чего ребенок успокаивается и появляется возможность его обследования. Необходимо подчеркнуть, что важное значение имеет и сама методика пальпации передней брюшной стенки. Обследовать живот нужно не спеша, мягкими движениями теплой руки, вначале едва касаясь передней брюшной стенки, а затем постепенно увеличивая давление. При этом пальпацию необходимо начинать с заведомо здорового места, т.е. с левой подвздошной области по ходу толстой кишки. При проведении пальпации живота важно внимательно следить за поведением ребенка.
Появление двигательного беспокойства, реакции мимической мускулатуры могут помочь оценить болезненность осмотра.
Для обнаружения местных признаков при остром аппендиците у детей предложены специальные методы обследования (одновременная сравнительная пальпация в обеих подвздошных областях, глубокая пальпация на вдохе и т. д.).
Широкое распространение у хирургов получил осмотр ребенка во время сна. Иногда во время пальпации правой подвздошной области можно отметить симптом "отталкивания": ребенок во сне отталкивает своей рукой руку исследующего. Однако для наступления физиологического сна часто требуется много времени. Учитывая быстроту нарастания воспалительных изменений в брюшной полости у детей раннего возраста, столь длительное выжидание может оказаться особенно нежелательным.
В этой ситуации, особенно у беспокойных детей, оправдан метод осмотра детей в состоянии медикаментозного сна, создаваемого путем ректального введения 3% раствора хлоралгидрата (в возрасте до 1 года - 10-15 мл, от 1 года до 2 лет - 15-20 мл, от 2 до 3 лет - 20-25 мл).
Данный метод практически не имеет противопоказаний. Мы произвольно вызываем достаточно глубокий сон и тем самым создаем оптимальные условия для исследования. При этом сохраняются пассивное мышечное напряжение передней брюшной стенки и локальная болезненность, симптомы легко выявляются, поскольку исчезает двигательное возбуждение, снимаются психоэмоциональная реакция и активное напряжение.
Определение симптома Щеткина - Блюмберга во время естественного и медикаментозного сна проводят в заключение обследования. При положительном симптоме ребенок или просыпается, или болезненно реагирует, продолжая спать.
Ректальное пальцевое исследование у младших детей дает меньше диагностической информации и вносит ясность лишь при наличии инфильтрата, который в этом возрасте встречается относительно редко. Тем не менее пальцевое ректальное исследование необходимо проводить всем детям раннего возраста, так как во многих случаях оно помогает отдифференцировать ряд других заболеваний (инвагинация, копростаз, кишечная инфекция и др.
У детей младшего возраста при остром аппендиците чаще всего отмечается повышение количества лейкоцитов в периферической крови в пределах 15-20 • 10 -9/л. Нередко наблюдаются и явления гиперлейкоцитоза (25 -30 • 10 • 9/л и выше).
Учитывая трудность диагностики острого аппендицита у детей раннего возраста и высокий процент диагностических ошибок при этом заболевании, необходимо шире использовать в таких случаях дополнительные методы исследования, включая лапароскопию.
Дифференциальная диагностика острого аппендицита у детей даже старшего возраста имеет принципиальные отличия от таковой у взрослых. Это связано с тем, что острый аппендицит в детском возрасте в своем клиническом проявлении (особенно при атипичных вариантах расположения червеобразного отростка) схож с большим числом заболеваний, не требующих оперативного вмешательства. Еще больше заболеваний, как соматических, так и хирургических с локализацией в брюшной полости и вне ее, "маскируются" под острый аппендицит.
Большое практическое значение имеет вопрос, касающийся длительности наблюдения за больным в случаях неясной клинической картины и возникающих при этом трудностях дифференциальной диагностики. У детей никогда не следует рассчитывать на ограничение воспалительного процесса в червеобразном отростке. Важно в кратчайшие сроки провести все необходимые клинические исследования, привлекая при необходимости к консультациям врачей смежных специальностей (педиатр, инфекционист, отоларинголог). Обычно бывает достаточно 2 - 6 ч активного наблюдения для окончательного установления диагноза. В некоторых случаях эти сроки могут быть изменены.
Сложность диагностики у детей объясняется еще и тем, что в зависимости от возраста меняется и спектр заболеваний, от которых приходится Дифференцировать острый аппендицит. У детей старшей возрастной группы клиническую картину острого аппендицита чаще всего симулируют заболевания желудочно-кишечного тракта желчевыделительной и мочевыделительной систем, копростаз, острые респираторно-вирусные заболевания, пневмония, заболевания половых органов у девочек, врожденные и приобретенные заболевания илеоцекального угла, детские инфекции, геморрагический васкулит (болезнь Шенлейна - Геноха).
В младшем возрасте (преимущественно у детей первых 3 лет жизни) дифференциальную диагностику чаще проводят с острыми респираторно-вирусными инфекциями, копростазом, урологическими заболеваниями, пневмонией, желудочно-кишечными заболеваниями, отитом, детскими инфекциями.
Ошибки обусловлены как недостаточной осведомленностью о вариантах клинического течения острого аппендицита, так и трудностями распознавания этого заболевания у детей, особенно раннего возраста.
Использование объективных методов исследования в пооперационной диагностике сомнительного по клиническим признакам острого аппендицита позволяет довести до минимума процент гипо- и гипердиагностических ошибок и соответственно резко сократить число неоправданных аппендэктомий. При такой тактике уменьшается вероятность послеоперационных осложнений и улучшается диагностика заболеваний, вызывающих абдоминальный болевой синдром.
Лечение. Хирургическая тактика при остром аппендиците у детей принципиально мало отличается от таковой у взрослых. Тем не менее, имеется ряд особенностей при оперативном лечении различных форм острого аппендицита, которые наиболее выражены в первые годы жизни ребенка.
Обезболивание у детей, особенно раннего возраста, должно быть только общим. Важным моментом, предшествующим проведению наркоза, является психологическая подготовка больного.
Опыт доказал, что у детей вполне обоснована аппендэктомия лигатурным способом (без погружения культи в кисетный шов). Лигатурный метод не более опасен, чем погружной, но обладает рядом преимуществ: ускоряет время операции и уменьшает опасность перфорации стенки слепой кишки при наложении кисетного шва. Последнее обстоятельство особенно важно у детей раннего возраста, у которых стенка кишки тонкая.
Важно также избежать опасности деформации илеоцекального клапана (баугиниевая заслонка), который у маленьких детей расположен близко к основанию отростка: при наложении кисетного шва может возникнуть ее недостаточность или стенозирование.
В последние годы во многих клиниках нашей страны успешно выполняется лапароскопическая аппендэктомия. Она имеет ряд неоспоримых преимуществ перед традиционной операцией. Это и меньшая травматичность вмешательства, и меньшая частота послеоперационных осложнений, и хороший косметический эффект.
Лапароскопическая аппендэктомия наиболее эффективна при лечении осложненных форм острого аппендицита. Исключение широкой лапаротомии значительно уменьшает страдания больного, способствует более раннему восстановлению нарушенных функций организма. Снижается частота послеоперационных осложнений, практически исключаются раневые осложнения. Значительно уменьшается риск развития послеоперационного спаечного процесса в брюшной полости, сокращается пребывание больного в стационаре и улучшается косметический результат.
Прогноз. При ранней диагностике острого аппендицита у детей прогноз заболевания, как правило, благоприятный. Летальность в среднем составляет 0,2 - 0,3%. Однако при поздней диагностике заболевания и, особенно в ранней возрастной группе, эти показатели значительно выше (3 - 5%).

ПЕРИТОНИТ

Основные вопросы темы:
1.	Основные причины перитонита у детей
2.	Аппендикулярный перитонит
3.	Клиника и диагностика
4.	Лечение 

Вопросы контроля исходного уровня:
1.	Строение брюшины
2.	Патогенез перитонитов
3.	Основные принципы лечения перитонитов

Воспаление брюшины - перитонит - в детском возрасте имеет значительный удельный вес. Тяжесть течения разлитого перитонита у детей во многом определяется не только характером местного процесса, но в большей степени теми бурными и глубокими нарушениями внутренней среды организма, которые он обусловливает.
В общепринятых классификациях перитонит подразделяют следующим образом. По этиологии: асептический и инфекционный; по путям заноса -перфоративный, септический (контактный, гематогенный) и криптогенный; по степени распространенности процесса - общий и местный. Общий может быть диффузным и разлитым, местный - неограниченным и отграниченным.
Причины перитонита у детей разнообразны. В большинстве случаев он является результатом инфицирования со стороны органов брюшной полости. Кроме того, существуют так называемые гематогенные и криптогенные перитониты, причину которых установить трудно. Перфоративные перитониты у детей старше 1 года чаще всего развиваются на фоне острого аппендицита. Возможна также перфорация дивертикула Меккеля при его воспалении.
В зависимости от происхождения перитонита, длительности заболевания и возраста ребенка значительно меняются течение и прогноз. Особенно быстро и злокачественно протекает перитонит в раннем возрасте, в котором местные и отграниченные формы воспаления брюшины встречаются реже, чем разлитые.
АППЕНДИКУЛЯРНЫЙ ПЕРИТОНИТ
Перитонит - частое и наиболее тяжелое осложнение острого аппендицита в детском возрасте, которое возникает в 8 - 10% всех случаев заболевания, причем у детей первых 3 лет жизни в 4 - 5 раз чаще, чем в более старшем возрасте. В последние годы удалось снизить летальность среди больных, госпитализированных в начальных фазах заболевания, однако при запущенных формах летальность остается очень высокой.
Из множества предложенных классификаций наибольшее распространение получил принцип подразделения перитонита по стадийности течения процесса и распространенности поражения брюшины, выраженности пареза кишечника.
Наиболее целесообразным следует считать подразделение перитонита на местный и разлитой. Местный перитонит в свою очередь подразделяют на отграниченный и неограниченный. Последний характеризуется воспалительными изменениями в области поражения с возможным затеком экссудата в смежную область по пути естественного распространения (правый латеральный канал и полость малого таза). При разлитом перитоните воспалительные изменения брюшины выходят за пределы очага не по пути естественного распространения. Выход воспалительного процесса за пределы правого бокового канала практически следует считать разлитым перитонитом.
Наибольшее распространение при перитоните получило выделение трех баз его течения - реактивной, токсической и фазы полиморфных нарушений, которые отражают тяжесть клинических проявлений заболевания.
Фазность течения перитонита подтверждают изменения центральной гемодинамики, микроциркуляции, иммунологической реактивности.
Патогенез разлитого перитонита - сложная цепь функциональных и морфологических изменений ряда систем и органов.
Анатомо-физиологические особенности детского организма сказываются на течении аппендикулярного перитонита. Чем меньше ребенок, тем быстрее гнойный процесс распространяется на все отделы брюшины. Этому способствуют низкие пластические свойства брюшины, недоразвитие большого сальника. Быстрее нарастают интоксикация и обменные нарушения, многие реакции из защитных переходят в патологические.
Ведущая роль в патогенезе перитонита принадлежит микробному фактору и состоянию иммунореактивности организма. Характер и тяжесть изменений зависят от выраженности патологических процессов. Большое значение следует придавать бактериологической идентификации перитонита. Установлено, что в большинстве случаев перитонит является полимикробным заболеванием. Доминирующая роль в развитии заболевания принадлежит Е. coli, но большое значение имеют и другие микроорганизмы: патогенные кокки, протей, синегнойная палочка, клебсиелла и др. При целенаправленном исследовании более чем у 1 '3 больных высевается анаэробная флора. Этот факт необходимо всегда учитывать, так как обычная антибиотикотерапия неэффективна при анаэробной инфекции.
Одним из ведущих факторов в развитии патофизиологических сдвигов, наступающих при перитоните, является резорбция брюшиной токсических продуктов гнойного экссудата из брюшной полости. Поверхность брюшины у детей, особенно раннего возраста, относительно больше чем у взрослых. Установлено, что в начальных стадиях перитонита резорбция брюшины резко повышена и остается высокой во все фазы перитонита. Вследствие этого происходит интенсивное всасывание в кровь и лимфу бактериальных токсинов и продуктов распада микробных тел-. Обладая протеолитическим свойством. эти вещества вызывают распад белка и выделение большого количества гистамина и гистаминоподобных веществ. Именно всасывание токсических продуктов обусловливает цепь разнообразных патофизиологических нарушений, включающих дегидратацию, расстройства циркуляции, гипертермию, нарушения кислотно-щелочного состояния и пр.
При аппендикулярном перитоните у детей довольно быстро развиваются дегидратация и расстройства циркуляции. Выделяющиеся в большом количестве вещества оказывают сосудорасширяющее действие, что увеличивает проницаемость сосудистой стенки и способствует выходу воды и низкомолекулярных белков из сосудистого русла. Вследствие этого усиливается экссудация жидкости и белка в брюшную полость. Под действием протеолитических ферментов наблюдаются торможение перистальтики кишечника, усиление тонуса симпатической нервной системы. Параллельно происходит значительная потеря воды из-за частой рвоты, жидкого стула. Потеря жидкости усугубляется также за счет перспирации и одышки. При парезе кишечника происходит массивное скопление жидкости в его просвете. Большая потеря жидкости из кровеносного русла приводит 'к значительному уменьшению объема циркулирующей крови и, следовательно, гемоконцентрации. Гиповолемия обусловливает снижение снабжения органов и тканей кислородом. В ответ на это происходит спазм периферических сосудов и сосудов почек, вызывающих перераспределение крови с сохранением питания важных органов, главным образом сердца и головного мозга ("централизация крови").
В ответ на это возникает компенсаторная тахикардия, приводящая к перенапряжению сердечной мышцы и нарушению работы сердца.
Снижение почечного кровотока способствует уменьшению фильтрации мочи в почечных клубочках и возникновению почечной ишемии. В дальнейшем расстройства функции почек приводят к гидропонным и метаболическим нарушениям. Из-за спазма кожных сосудов снижается теплоотдача, что способствует развитию гипертермии. Значительное повышение температуры тела (39-40°С и выше) отмечается у большинства больных гнойным перитонитом. В основном это связано с нарушением равновесия между процессами теплорегуляции и теплоотдачи, а также расстройствами тканевого обмена. У детей раннего возраста при гипертермии в связи с повышенным расходом энергии очень быстро истощаются энергетические запасы, возникают нарушения обмена и расстройства функций дыхания и сердечно-сосудистой системы. Все это может привести к недостаточности функции надпочечников.
Одним из важных патофизиологических звеньев при развитии перитонита является нарушение метаболизма. Основным источником образования энергии в организме являются углеводы. При перитоните нарушается восполнение энергетических ресурсов с пищей, и начинают использоваться углеводы из собственных запасов организма. Это происходит главным образом за счет гликогена печени. Затем в качестве источника энергии начинают использоваться белки и жиры, распад которых в условиях тканевой гипоксии приводит к накоплению недоокисленных продуктов. Постепенно начинает страдать дезинтоксикационная функция печени. При тяжелом течении Перитонита отмечаются нарушения белкового обмена. Потери белка происходят с экссудатом, диффузией его в просвет кишечника. Развивается его диспротеинемия.
Образование кислых продуктов изменяет рН крови - возникает метаболический ацидоз. Вначале метаболические сдвиги компенсируются в достаточной степени дыханием (за счет компенсаторной одышки и усиленного выделения углекислоты) и почками (путем реабсорбции натрия и выделения избыточных кислых радикалов). Компенсаторные механизмы очень быстро истощаются в условиях перитонита из-за ограничения дыхательных экскурсий вследствие пареза и вздутия кишечника, а также на почве гемодинамических нарушений. Ацидоз переходит в стадию декомпенсации.
При перитоните происходят также значительные нарушения электролитного обмена. Рвота и жидкий стул приводят к потере жидкости и электролитов - калия, натрия, хлора.
У детей раннего возраста чаще отмечаются гипоосмия при дефиците ОЦК, гипонатриемия и повышение внутриклеточного натрия.
По мере потери калия и хлора на фоне уменьшения объема плазмы возникают нарушения КОС, заключающиеся в развитии внутриклеточного ацидоза на фоне внеклеточного алкалоза (механизм Дэрроу). Обеднение клетки калием способствует нарушению синтеза АТФ и уменьшению запасов энергии, что приводит к ослаблению сократительной силы мышцы сердца и дыхательной мускулатуры. Возникают одышка, тахикардия, снижается ударный объем сердца и развивается недостаточность кровообращения с явлением общей гипоксии тканей. Дефицит калия снижает тонус гладкой мускулатуры, приводя к развитию и прогрессированию пареза желудочно-кишечного тракта.
Следовательно, основными патофизиологическими процессами при перитоните являются гиповолемия и нарушение центральной и периферической гемодинамики, изменение водно-электролитного баланса, КОС, нарушение функции жизненно важных органов. При тяжелом перитоните эти нарушения можно рассматривать как перитонеальный шок многокомпонентного патогенеза.
Большинство указанных нарушений отражены в клинической симптоматике. Необходимо лишь учитывать, что у детей до 3 лет защитные механизмы быстро переходят в патологические и общие клинические симптомы превалируют над местными. Течение разлитого перитонита сопровождается также выраженными нарушениями иммунологической реактивности организма ребенка, которые носят четкий фазовый характер.
В реактивной фазе перитонита отмечается напряжение факторов естественной неспецифической реактивности, повышение активности Ь-лизинов, лейкоцитов, лимфоцитов, нарастание уровня иммуноглобулинов в сыворотке крови, увеличение абсолютного количества розеткообразуюших клеток, снижение относительного количества Т- и В-лимфоцитов. В токсической фазе происходит дальнейшее повышение общего уровня неспецифических показателей, однако отмечается изменение количества функционально активных лейкоцитов, падение уровня сывороточных иммуноглобулинов. В фазе полиорганных нарушений отмечается полная несостоятельность защитных сил, катастрофическое снижение показателей как специфических, так и неспецифических иммунологических реакций.
Клиника и диагноз. В анамнезе, как правило, отмечаются боль, рвота, повышение температуры, В дальнейшем боль может несколько стихать, но температура держится, хотя подчас и невысокая; общее состояние также может несколько улучшиться, но никогда не восстанавливается до удовлетворительного. После светлого промежутка наступает ухудшение: вновь усиливается боль в животе, появляется рвота, прогрессивно ухудшается общее состояние. Наличие такого промежутка связано, по-видимому, с деструкцией и некрозом нервных окончаний в червеобразном отростке. Наступление периода ухудшения объясняется вовлечением в воспалительный процесс всей брюшины в результате перфорации отростка или нарушения целостности конгломерата при "прикрытой" перфорации. Возможно развитие перитонита и без перфорации червеобразного отростка вследствие прохождения флоры через измененную стенку. Быстрота наступления перфорации и длительность светлого промежутка зависят от возраста больного: чем меньше ребенок, тем быстрее наступает перфорация и короче период мнимого улучшения. Резко стирает остроту клинических проявлений аппендицита применение антибиотиков, что увеличивает вероятность развития перитонита. Антибиотики не могут остановить уже начавшийся деструктивный процесс, но применение их уменьшает выраженность болевого симптома, температурной реакции и общих нарушений, в то время как воспаление брюшины прогрессирует. Поэтому применение антибиотиков, особенно у детей раннего возраста, до установления причины боли животе категорически противопоказано.
При осмотре ребенка с аппендикулярным перитонитом отмечается значительная тяжесть общего состояния. Кожные покровы бледные, иногда имеют "мраморный" оттенок. Глаза блестящие, язык сухой с белым налетом. Обычно отмечается одышка, выраженная тем больше, чем меньше ребенок. Нередко можно установить расхождение между частотой пульса и степенью повышения температуры, что свидетельствует о коллаптоидных реакциях на фоне значительной интоксикации. Живот вздут, резко болезнен при пальпации во всех отделах, четко выявляются защитное мышечное напряжение и симптом Щеткина - Блюмберга, наиболее выраженные в правой подвздошной области.
Иногда бывают тенезмы, жидкий учащенный стул небольшими порциями. болезненное и учащенное мочеиспускание. При ректальном исследовании выявляют резкую болезненность и нависание стенки прямой кишки.
У детей раннего возраста общее состояние в первое время может быть нарушено незначительно, что связано с хорошими компенсаторными возможностями сердечно-сосудистой системы в этом возрасте. На первый план могут выступать явления дыхательной недостаточности. Через некоторое время наступает декомпенсация сердечно-сосудистой и дыхательной систем, а также метаболических процессов, в результате чего состояние больного начинает прогрессивно ухудшаться. В раннем возрасте при аппендикулярном перитоните чаще наблюдается жидкий стул, иногда зеленого цвета со слизью.
Лечение перитонита у детей представляет трудную задачу и складывается из трех основных этапов: предоперационной подготовки, оперативного вмешательства и послеоперационного периода.
Немедленное оперативное вмешательство при перитоните в условиях значительных нарушений внутренней среды организма является серьезной ошибкой. Эти сдвиги могут усугубляться во время операции и в послеоперационном периоде под влиянием операционной травмы, погрешностей анестезии и дальнейшего прогрессирования патологического процесса. Цель предоперационной подготовки - уменьшить нарушения гемодинамики, КОС и водно-минерального обмена.
Основой предоперационной подготовки является борьба с гиповолемией, дегидратацией. Степень обезвоживания может быть определена по следующей формуле с использованием показателей гематокрита (Э. К. Цыбулькин).
Для детей старше 3 лет:
Н больного - Fit в норме
V=	- 1/5 Р ,
100 - Ht в норме
где Р - масса тела.
Для детей до 3 лет:
Н больного - Ht в норме
V=____________________ - 1/3 Р
100 - Нг в норме

При проведении инфузионной терапии прежде всего назначают растворы гемодинамического и дезинтоксикационного действия (гемодез, реополиглюкин, полиглюкин, альбумин, раствор Рингера, плазма крови). Объем и качество инфузионной терапии зависят от тяжести перитонита, характера гемодинамических нарушений и возраста больного. Весь комплекс предоперационных мероприятий должен быть выполнен в довольно сжатые сроки (не более 2-3 ч).
Терапию антибиотиками широкого спектра действия начинают уже в предоперационном периоде. Зондирование и промывание желудка способствуют уменьшению интоксикации, улучшению дыхания, профилактике аспирации. Как дополнение к указанным мероприятиям, особенно у детей первых месяцев жизни, важную роль играет борьба с гипертермией, пневмонией, отеком легких, судорогами.
К оперативному вмешательству приступают, когда компенсированы и стабилизированы процессы гемодинамики, КОС, водно-солевой обмен, а температура тела не превышает субфебрильных цифр.
Оперативное вмешательство при перитоните проводят с целью устранения первичного очага, санации и дренирования брюшной полости.
После удаления червеобразного отростка тщательно промывают брюшную полость теплым изотоническим раствором хлорида натрия или раствором фурациллина. Промывную жидкость аспирируют, и эту процедуру повторяют несколько раз.
Дальнейшая тактика зависит от глубины изменений в кишечнике и брюшине. Наиболее важна в этой ситуации оценка нарушений перистальтики и кровообращения.
Особенности хирургической тактики при аппендикулярном перитоните определяются его фазой.
В реактивной фазе при операции обычно обнаруживают обильный гнойный выпот в брюшной полости. Петли кишечника слегка вздуты, гиперемированы, без наложения фибрина и циркуляторных нарушений. Несмотря на то что воспалительный процесс распространяется на всю брюшную полость, преобладает еще местная симптоматика, организм как бы
сам справляется с возникшими нарушениями, используя свои собственные резервы. Среди интраоперационных мероприятий в реактивной фазе важная роль отводится тщательной санации брюшной полости. Затем операционную рану ушивают наглухо с оставлением в малом тазу силиконового дренажа или полиэтиленовой трубки от одноразовой системы для переливания крови. Дренаж вводят через дополнительный разрез-прокол в правой подвздошной области несколько выше и латеральнее "аппендикулярного" разреза. Для его правильной фиксации брюшную стенку прокалывают в косом (под углом 45°) направлении, после чего дренаж укладывают в правый латеральный канал и максимально на дно малого таза (у мальчиков между прямой кишкой и мочевым пузырем; у девочек между прямой кишкой и маткой). Диаметр отверстий отдела трубки, находящегося в малом тазу, не должен превышать 0,5 см. При меньших отверстиях дренаж быстро забивается, а при больших - возможно присасывание кишечной стенки, сальника, жировых подвесок. На коже дренажная трубка фиксируется шелковыми швами.
При токсической фазе перитонита в брюшной полости можно обнаружить большое количество гноя, значительный парез вплоть до паралича, выраженные циркуляторные расстройства с наложениями фибрина на петлях кишечника.
Хирургическая тактика при этой фазе перитонита в общих принципах аналогична предыдущей, но имеет свои особенности. Одномоментное промывание брюшной полости должно быть более тщательным и с применением большого количества промывного раствора. При значительном парезе кишечника производят различные методы его декомпрессии: интубацию тонкой кишки через гастростому, цекостому, ретроградно через заднепроходное отверстие или "сцеживание" содержимого тонкой кишки в толстую.
Как и при экссудативной фазе перитонита, аспирационное дренирование полости малого таза осуществляют также с помощью дренажной трубки. Корень брыжейки тонкой кишки обкалывают 0,25 % раствором новокаина. Показана перидуральная анестезия, которую проводят в послеоперационном периоде.
В фазе полиморфных нарушений на операции выявляют глубокие функциональные нарушения кишечника. Ввиду его паралича отмечаются стаз и расширение тонкой кишки за счет скопления жидкости и газов. При этом чаще наблюдают отграниченные абсцессы в брюшной полости. После разделения петель кишечника целесообразно не ушивать срединную лапаротомную рану (лапаростомия). После тщательной санации брюшной полости дренируют силиконовой трубкой полость малого таза. Кишечник прикрывают полиэтиленовой пленкой с множественными отверстиями до 5 мм, сверху накладывают пропитанные вазелиновым маслом салфетки, над которыми отдельными швами без натяжения сближают кожу с апоневрозом, закрывая лишь края салфетки. Отсутствие компрессии на кишечник и возможность свободного выхода инфицированного экссудата из брюшной полости через рану способствуют улучшению микроциркуляции в кишечнике, восстановлению перистальтики и купированию воспалительного процесса. В этой фазе перитонита особое значение придается декомпрессии желудочно-кишечного тракта. Через 2-3 сут. проводят повторную операцию: удаляют салфетки и пленку, рану санируют и брюшную стенку ушивают наглухо через все слои П-образными матрацными шелковыми швами. Края раны отделяют от спаянного кишечника настолько, чтобы при сшивании не деформировать петли кишок.
Послеоперационное лечение. После операции больному создают возвышенное положение в кровати путем поднятия ее головного конца под утлом 30°, что облегчает дыхание ребенка и способствует оттоку экссудата в нижние отделы живота.  Под согнутые колени подкладывают валик, чтобы ребенок не сползал вниз.
Чрезвычайно важно тщательно контролировать сердечную деятельность (пульс, артериальное давление, ЭКГ), дыхание, белковый состав крови, гидроионный баланс, КОС крови. В течение первых 2-3сут через 2-4ч контролируют температуру тела, пульс, артериальное давление, частоту дыхания. Тщательно измеряют объем выпитой и парентерально введенной жидкости, а также выделенной с мочой и рвотными массами.
Чрезвычайно важными показателями течения воспалительного процесса брюшной полости, помимо общего состояния (реакция на окружающее, аппетит, нормализация функции желудочно-кишечного тракта), являются динамика температурной реакции и картина периферической крови. Комплекс лечебных мероприятий складывается из следующих моментов: борьба с токсикозом и инфекцией, устранение гемодинамических нарушений и гиповолемии, коррекция гидроионных и метаболических сдвигов, ликвидация
анемии и гипопротеинемии.
Большое значение следует придавать профилактике и лечению респираторных нарушений. В комплекс этих мероприятий входит декомпрессия желудка путем введения в него зонда. Постоянное нахождение зонда в первые 2-3 сут после операции предупреждает аспирацию, способствует снижению внутрибрюшного давления, повышая вентиляционные возможности дыхательной системы.
Предупреждение и лечение дыхательной недостаточности необходимы в течение всего ближайшего послеоперационного периода. По показаниям проводят катетеризацию трахеобронхиального дерева с последующим отсасыванием слизи и введением антибиотиков. Это дает возможность предупредить развитие ателектазов и как их следствие - пневмонии.
В раннем послеоперационном периоде вновь могут возникнуть нарушения гомеостаза, обусловленные операционной травмой и продолжающимся воспалительным процессом. Основными из них являются нарушение гемодинамики, КОС и водно-солевого баланса. Установлено, что более чем у 1/3 больных с разлитым перитонитом имеются подобные нарушения с явлениями выраженного метаболического ацидоза. Крайне редко, при очень тяжелых формах перитонита, отмечаются явления и метаболического алкалоза. Коррекция гемодинамических изменений и метаболического ацидоза не отличается от проводимой в предоперационном периоде и достигается внутривенным введением плазмы, высокомолекулярных препаратов. 4% раствора бикарбоната натрия.
Метаболический алкалоз корригируют путем внутривенного введения 7,5 % раствора калия хлорида в среднем от 8 до 10 мл (0,5 мл на 1 кг массы тела) в разведении.
Респираторный ацидоз, возникающий в послеоперационном периоде, устраняют дозированной оксигенотерапией и эвакуацией желудочного содержимого с помощью поставленного зонда. Коррекция нарушений водно-солевого обмена не отличается от проводимой перед хирургическим вмешательством.
Успех лечения при перитоните во многом зависит от рационального применения антибиотиков. Показаны антибиотики широкого спектра действия. Смену их производят в зависимости от чувствительности микрофлоры.
Важным звеном в послеоперационном периоде у больных с перитонитом является нормализация функции кишечника. При тяжелом перитоните парез кишечника нередко держится в течение нескольких дней. Для борьбы с послеоперационным парезом применяют повторные гипертонические клизмы, подкожно назначают средства, стимулирующие перистальтику (0,05 % раствор прозерина по 0,1 мл на 1 год жизни), внутривенно переливают гипертонические растворы глюкозы (10-20 мл 40 % раствора), 10 % раствор натрия хлорида (по 2 мл на 1 год жизни) и растворы калия хлорида. Инфузию последнего осуществляют в растворе глюкозы капельно. Наиболее безопасная концентрация хлорида калия - 1 % раствор. Необходимо следить за регулярностью стула: при его задержке 1 раз в 2 дня назначают очистительные клизмы.
С учетом возможности образования в послеоперационном периоде инфильтратов и абсцессов в брюшной полости необходим контроль за температурной реакцией, содержанием лейкоцитов периферической крови.
У всех больных даже при отсутствии жалоб целесообразно периодически производить пальцевое исследование прямой кишки для своевременного обнаружения тазового инфильтрата или абсцесса, так как применение антибиотиков сглаживает клинические их проявления.
Лечение перитонита у детей, особенно раннего возраста, - задача. требующая индивидуального подхода с учетом многих факторов. Только массивная комплексная терапия может быть эффективна при этом тяжелом заболевании.

ГНОЙНЫЕ ЗАБОЛЕВАНИЯ У ДЕТЕЙ

ПАТОГЕНЕЗ ОСТРОЙ ГНОЙНОЙ ХИРУРГИЧЕСКОЙ ИНФЕКЦИИ

В патогенезе гнойной хирургической инфекции участвуют три важнейших фактора: возбудитель, собственная микрофлора организма больного и его реактивность. По словам отечественного патологоанатома И. В. Давыдовского, гнойная инфекция является "эндогенной аутоинфекцией сенсибилизированного организма". Для реализации воспалительного процесса необходимо, чтобы антигенное раздражение в организме превысило защитные возможности иммунной системы. Это происходит при чрезмерном размножении возбудителя в одном из естественных резервуаров существования микрофлоры в организме (желудочно-кишечный тракт, дыхательные пути, кожа) и транслокации в пораженный орган.
Специфических возбудителей гнойной инфекции в природе не существует. Все они в том или ином количестве и качестве вегетируют в естественных резервуарах организма. Однако при определенных условиях степень их патогенности меняется. К возбудителям гнойной хирургической инфекции (по мере уменьшения частоты) относятся стафилококк золотистый и стрептококк из группы грамположительных микроорганизмов, а также большая группа грамотрицательных микроорганизмов: синегнойная палочка, протей, кишечная палочка, клебсиелла, цитобактер, церрация, анаэробная неклостридиальная флора. В 60% случаев флора в очаге поражения бывает смешанной. Наличие у возбудителей разнообразных ферментов агрессии (гиалуронидаза, фибринолизин, коагулаза), эндотоксинов (гемолизин, лейкоцидин, летальный токсин, энтеротоксин и др.) способствует проникновению их в ткани и органы, обусловливая многообразие форм заболевания и различную тяжесть их течения. Точкой приложения действия эндотоксина, вырабатываемого грамотрицагельной микрофлорой в организме, являются эндотелий капилляров и вегетативная нервная система. Для разных видов микроорганизмов характерна достаточно дифференцированная картина воспаления при морфологическом ее изучении.
Для стафилококка наиболее характерны поражения кожи, подкожной жировой клетчатки, костей, легких, желудочно-кишечного тракта; для возбудителей грамотрицательной группы - поражение кишечника, суставов, мочевых путей. Грамотрицательная микрофлора играет ведущую роль в развитии послеоперационных осложнений и патогенезе постреанимационной болезни. Особенно опасна она для новорожденных, лишенных естественных факторов защиты от нее.
Последнее касается всех звеньев иммунитета, преимущественно у детей раннего возраста. Несмотря на высокие показатели ряда факторов неспецифического иммунитета (С-реактивный белок, лизоцим), титр комплемента быстро истощается, фагоцитоз на многие антигены у новорожденных вследствие ферментной активности фагоцитирующих клеток не завершен. Это способствует внутриклеточному персистированию возбудителей и переносу их в организме. Гуморальная фаза иммунитета также не рассчитана на массовое инфицирование: у новорожденных к моменту рождения IgM отсутствует, (IgG и lgA ребенок получает от матери), а самостоятельно начинает формироваться лишь к 4-месячному возрасту. Формирование специфического иммунитета, иммунологической памяти развивается постепенно и зависит от многих факторов: патологии внутриутробного периода, родов, периода адаптации, становления биоценоза организма, становления первичного звена иммунитета.
У детей раннего возраста снижена способность к отграничению воспалительного процесса вследствие особенностей свертывающей системы крови. Недостаток основных прокоагулянтов, снижение индекса протромбина в 2 раза, уровня фибриногена до 1,7 г/л, проконвертина до 30-50% при тенденции к антикоагулянтным состояниям (увеличение гепариновой, антитромбиновой и фибринолитической активности) значительно ограничивают фибринообразование и плазмокоагуляцию в очаге поражения. Тем самым снижается роль этого фактора в демаркации и создаются условия для распространения воспалительного процесса.
С точки зрения патогенеза и клиники для тяжелых форм гнойной хирургической инфекции характерно развитие ряда синдромов.
Синдром гемодинамических нарушений обусловлен влиянием микробного экзо- и эндотоксина, а также гистамина и гистаминоподобных веществ. При распаде тканей высвобождаются протеолитические ферменты, способствующие усилению лизиса белковых веществ и увеличению количества гистамина и других субстанций, воздействующих на сосуды. Вследствие раздражения аденогипофиза, а возможно и при непосредственном воздействии токсина усиливается выброс катехоламинов, что приводит к спазму сосудов, нарастанию периферического сопротивления. Происходит "шунтирование" крови, т. е. кровь из артериальной системы, минуя капилляры и метартериолы, переходит в венозную. Этим поддерживается объем крови, необходимый для циркуляции в жизненно важных органах (централизация кровотока), хотя тканевый обмен на периферии нарушается.
Реакция "централизации" до определенного момента играет положительную роль, однако при затянувшемся бактериальном стрессе могут происходить функциональные нарушения, связанные с нарушением теплоотдачи (гипертермический синдром) и функции почек.
Фаза спазма периферических сосудов, особенно у детей раннего возраста, достаточно кратковременна, клинически обычно просматривается, сменяется фазой пареза капилляров, запускающей последующие патологические механизмы.
Выбрасываемое в кровь значительное количество адреналина повышает потребность в энергетических ресурсах, возникает клеточное голодание, усугубляющееся повышением катаболизма вследствие усиления выброса глюкокортикоидов. Повышение потребности в энергетических ресурсах ведет к распаду эндогенного белка и жира. При этом образуется много недоокисленных продуктов, чему способствует также гипоксия тканей вследствие расстройства циркуляции. Нарушение периферической гемодинамики и обмена, энергетический голод, повышенное образование недоокисленных продуктов приводят к изменению кислотно-основного состояния в сторону метаболического ацидоза. При значительных объемах поражения воспалительным процессом (флегмона) происходят шунтирование крови и нарушение метаболизма в очаге. В этих условиях резко нарастают нагрузка на дыхательную систему как на компенсаторный аппарат (выведение СО2 и других кислых продуктов) и обеспечение кислородом. Кроме того, особенно при грамотрицательной инфекции, которая обладает выраженным вазопрессорным эффектом на сосуды малого крута кровообращения, развивается шунтирование крови в легких, нарушается газообмен. Многие возбудители являются тропными и к легочной ткани, вызывая воспалительные изменения. Развивается синдром дыхательных расстройств
Тяжелые расстройства микроциркуляции, гипоксия и ацидоз приводят к увеличению сосудистой проницаемости и экстравазатам. В ответ на эти изменения нарушается равновесие свертывающей и антисвертываюшей систем крови. Это нарушение проходит определенные стадии развития, объединяемые в медицине под названием ДВС-синдром (синдром диссеминированного внутрисосудистого свертывания). На фоне развития этого синдрома, сопровождающегося процессом микротромбообразования, еще более ухудшается микроциркуляция в почках и легких; нарастают нарушения процессов детоксикации, дыхания.
Токсины микробных клеток, развивающаяся гипоксия организма сказываются на клетках крови. Возникают анемия, изменения в картине белой крови, соответствующие тяжести и фазе воспалительного процесса, тромбоцитопения.
В результате сложных патогенетических механизмов, происходящих в организме больного, развивается полиорганная недостаточность. Чем большее количество органов и систем задействовано в патологическом процессе, тем серьезнее прогноз заболевания.
Различают молниеносную (токсико-септическую), септико-пиемическую и местную формы гнойной хирургической инфекции. Первые две формы следует рассматривать как варианты хирургического сепсиса - тяжелой интоксикации организма в условиях измененной реактивности. Самое тяжелое проявление хирургической инфекции - септический шок.
Токсико-септическая форма начинается и прогрессирует бурно, проявляется тяжелейшим токсикозом и лихорадкой, симптомами нейротоксикоза. Местные симптомы могут находиться в начальных стадиях развития, преобладает общая симптоматика. Развивается на фоне сенсибилизации, дефицита гуморальных антител. В качестве возбудителей часто встречаются грамотрицательная микрофлора, стрептококк или ассоциация со стафилококком.
Септический шок может наступить при любой форме и в любую фазу гнойной хирургической инфекции. Реакция объясняется поступлением в кровь большого количества микробных эндотоксинов, протеаз, гистаминоподобных веществ, активацией кининовой системы. Это часто связано с присоединением грамотрииательных микробов, нарушением демаркации местного очага, ухудшением антитоксического иммунитета, совпадает со значительным изменением биоценоза организма. Характерны выраженные сердечнососудистые нарушения - коллапс, тахиаритмия, нарушения психики, аллергические реакции.
Септико-пиемическая форма протекает при отчетливом преобладании воспалительной реакции над токсикозом, хотя последний также значителен. Местные реакции появляются рано, очаги могут быть множественными, развиваться одномоментно или последовательно.
Местная форма - преобладание местной воспалительной реакции над общими проявлениями, нормоэргическая реакция. Течение местного процесса характеризуется быстрым наступлением некроза, отека, угнетением местной фагоцитарной реакции, склонностью к распространению инфекции и массивной резорбции продуктов воспаления из местного очага, в кровь и лимфу. Особенно выражен отек у новорожденных, гидрофильность тканей у которых повышена. На возникновение значительного отека влияет повышение проницаемости сосудов под воздействием гипоксии, гистамина и гистаминоподобных веществ, а также эндотоксинов. В условиях отека и накопления жидкости значительно снижаются местные барьерные функции. Эти факторы способствуют резкому нарушению сосудистой трофики, что обусловливает быстрое распространение процесса на здоровые участки. Генерализации инфекции способствуют низкая барьерная функция регионарных лимфатических узлов и хорошо развитая сеть кровеносных и лимфатических капилляров.
Хроническая стадия рассматривается как состояние временного динамического равновесия между макроорганизмом и очагом поражения. Равновесие это, однако, нестойкое и всегда может нарушиться в сторону обострения инфекции. Переходу воспалительного процесса в хроническую стадию способствуют несвоевременность диагностики и поздно начатое лечение, недостаточность иммунологического ответа, массивность поражения. При хронической форме заболевания в результате обострений образуются продукты неполного распада тканей, обладающие антигенными свойствами, могут развиваться аутоиммунные процессы. Переходу процесса в хроническую форму способствует применение стероидных гормонов, вызывающих усиление супрессивной активности иммунокомпетентных клеток и снижение хелперной активности. На этом фоне любая стрессовая ситуация (гиперконтаминация, суперинфекция, особенно вирусной природы, травма, переохлаждение) может привести к обострению инфекционного процесса.

ФЛЕГМОНА НОВОРОЖДЕННЫХ

Основные  вопросы темы:
1.	Этиопатогенез флегмоны новорожденных
2.	Клиника и диагностика
3.	Лечение и прогноз

Вопросы контроля исходного уровня:
1.	Понятие флегмоны
2.	Основные принципы лечения флегмон
У новорожденных наблюдается особая форма гнойного поражения кожи и подкожной клетчатки, характеризующаяся быстрым распространением процесса. Развитию флегмоны и быстрому ее распространению способствуют легкая ранимость эпидермиса, слабое развитие базальной мембраны, относительно большое содержание межклеточной жидкости, богатое развитие сосудистой сети, недоразвитие соединительнотканных перемычек в жировой клетчатке. Проникновению инфекции через кожу способствует ее повреждение при несоблюдении гигиенического режима ухода за ребенком.
Возбудителем является, как правило, стафилококк, далее может присоединиться грамотрицательная, смешанная флора. Типичная локализация поражения - крестцово-копчиковая, лопаточная область, передняя и боковая поверхности грудной клетки.

Таблица 3
Дифференциальная диагностика основных гнойно-септических заболеваний хирургии новорожденных

Симптомы	ФЛЕГМОНА	РОЖА	АДИПОНЕКРОЗ	
Возбудитель	чаще стафилококк	чаще стрептококк	нет	
Типичная	поясничная	нижняя часть	межлопаточная	
локализация	область, спина,	живота,	область, область	
	грудная клетка	промежность, бедро	большого	
			вертела, остистых	
			отростков	
Область	кожа и подкожная	собственно кожа	подкожная клетчатка	
поражения	клетчатка			
Общие	высаженный	выраженный	токсикоза нет,	
симптомы	токсикоз,	токсикоз, нередко	НМК 1-Ш степени,	
	анорексия,	септический шок,	температура - норма	
	беспокойство,	анорексия,		
	гипертермия	гипертермия		
Реакция	гипер лейкоцитоз,	гипер лейкоцитоз,	норма,	
периферической	нейтропения.	нейтрофилез,	норма формулы крови	
крови	лимфоцитоз, соэ-	лимфопения,	и соэ	
	норма	мсмоцитопения, соэ-		
		норма		
ЛИИ	меньше 1	больше 1	норма (0,6- 1,2)	
Местные	быстрое	быстрое	не распространяется,	
симптомы	распространение,	распространение,	красно-цианогичная,	
	гиперемия, отек,	гиперемия.	пятнистая, бугристая	
	инфильтрация.	инфильтрация кожи,	инфильтрация,	
	болезненность,	фестончатые границы,	безболезненность,	
	повышение	наличие цианотичных	температура не	
	температуры;	пятен, при булезной	повышена;	
	после УФО	форме отслойка	после УФО окраска	
	окраск» кожи	эпидермиса;	кожи не меняется	
	не изменяется	после УФО -		
		усиление гиперемии.		
Осложнения	генерализация;	генерализация;	нагноение	
	некроз кожи и	септический шок		
	подкожной			
	клетчатки			

При патоморфологическом исследовании очага поражения отчетливо преобладают некротические процессы. Воспаление начинается в основном вокруг потовых желез. Наиболее резкие изменения отмечаются в глубоких слоях подкожной жировой клетчатки. Быстро тромбируются перифокально расположенные кровеносные сосуды. В их стенках обычно бывают выражены явления эндо- и периартериита. Тромбоз сосудов и молниеносно распространяющийся отек подкожной жировой клетчатки приводят к резкому нарушению ее питания, а также питания кожи с последующим их некрозом.
Ввиду несовершенства иммунологических реакций и особенностей строения кожи и клетчатки отграничения воспалительного процесса не происходит, некроз в течение нескольких часов может захватить значительную поверхность.
Клиника и диагностика. Заболевание часто начинается с общих симптомов. Ребенок становится вялым, беспокойным, плохо спит, отказывается от груди. Температура повышается до 38 - 39°С. На участке поражения появляется пятно красного цвета, которое быстро, в течение нескольких часов, увеличивается.
Кожа вначале имеет багровый цвет, а затем принимает цианотичный оттенок. Отмечаются уплотнение и отек мягких тканей. При развитии процесса, ко вторым суткам, в центре его появляется флюктуация. В дальнейшем нарастает токсикоз, а местный процесс может распространиться на значительном протяжении.
В тяжелых случаях кожа отслаивается, некротизируется и образуются обширные дефекты мягких тканей.
При вскрытии флегмоны выделяется мутная серозная жидкость или жидкий гной обычно вместе с кусочками серого цвета. Иногда клетчатка отторгается значительными участками.
Дифференциальный диагноз проводят с рожистым воспалением и асептическим некрозом подкожной жировой клетчатки.

Адипонекроз возникает в результате сдавления мягких тканей в родовых путях при патологическом течении родов, клинически и лаборатории не имеет общих и местных симптомов воспалительного процесса. Типичны бугристая инфильтрация мягких тканей и пятнистая гиперемия зоны поражения.
Лечение. Необходимо комплексное и своевременное лечение. Антибиотики назначают в зависимости от тяжести состояния и стадии развития местного процесса. При отсутствии выраженных общих симптомов, свидетельствующих о развитии сепсиса, лечение можно начинать с внутримышечного введения оксациллина в дозе 200 000 ЕД на 1 кг массы тела в сутки. В более тяжелых случаях препарат сочетают с.аминогликозидами или назначают цефалоспорины с учетом спектра чувствительности микрофлоры. Больному проводят иммунотерапию (антистафилококковый гаммаглобулин, гипериммунная антистафилококковая плазма, прямое переливание крови; под контролем показателей иммунного статуса назначают Т-активин, декарис, продигиозан, пентоксил, витамины С, Е), дезинтоксикационную и другие виды посиндромной терапии.
Местное лечение заключается в нанесении множественных разрезов в зоне поражения и по границе со здоровыми участками. Такая методика позволяет уменьшить отек в пограничной зоне и является профилактическим мероприятием, цель которого - отграничить распространение процесса.
После операции делают перевязку (через 6 - 8 ч). Если отмечается дальнейшее распространение очага, немедленно вновь наносят множественные мелкие разрезы, также захватывающие здоровые участку кожи. После нанесения насечек накладывают влажную повязку с растворами антисептиков (1% растворы хлорофиллипта, диоксидина, томицида, гипертонический раствор хлорида натрия). Разрезы производят после предварительного обкалывания области флегмоны 0,25% раствором новокаина с антибиотиками. В случаях развития некроза кожи выполняют некрэктомию. При этом образуется раневая поверхность, которая при благоприятном течении заболевания постепенно гранулирует, а затем эпителизируется. Ускорению этого процесса способствуют применение физиотерапии, лучей лазера, ультразвуковая обработка ран, гипероксибаротерапия, общая стимулирующая терапия.

Прогноз при своевременной диагностике и адекватном лечении благоприятный. Наличие грубых деформирующих рубцов после обширных некрозов в более старшем возрасте требует проведения пластических операций.

ЛИМФАДЕНИТ

Основные  вопросы темы:
1.	Этиопатогенез  лимфаденитов
2.	Клиника и диагностика
3.	Лечение и прогноз

Вопросы контроля исходного уровня:
1.	Понятие лимфаденита
2.	Локализация основных групп лимфоузлов
3.	Основные принципы лечения лимфаденита

Воспаление лимфатических узлов (лимфаденит) часто наблюдается у детей, особенно в раннем возрасте. Это связано с функциональной и морфологической незрелостью лимфатического аппарата ребенка (широкие синусы, тонкая нежная капсула лимфатических узлов, повышенная восприимчивость к  инфекции, несовершенство барьерной функции).
У детей» особенно в возрасте от 1 года до 3 лет, лимфаденит чаще всего локализуется в челюстно-лицевой области (подчелюстные, подбородочные, шейные лимфатические узлы). Реже поражаются подмышечные, подколенные, паховые и кубитальные лимфатические узлы.
В патогенезе заболевания большую роль играет предварительная сенсибилизация организма в результате перенесенных  инфекционных и гнойных заболеваний.
Причины, приводящие к лимфадениту, многообразны. Лимфаденит челюстно-лнцевой области у детей крайне редко может быть первичным заболеванием. Чаше всего это реакция лимфатических узлов на ряд воспалительных очагов. Тщательное выявление этих причин обеспечивает успех дальнейшего лечения,
Одонтогенные лимфадениты у детей встречаются реже, чем неодонтогенные. Источником одонтогенной инфекции обычно являются молочные зубы, реже постоянные. Причиной возникновения неодонтогенных лимфаденитов, в основном у детей раннего возраста, являются ОРВИ, грипп, ангина, хронический тонзиллит, отит, экссудативный диатез, пиодермия, травма кожи и слизистых оболочек.
Клиника и диагностика. Характерно появление общих симптомов -недомогания, озноба, повышения температуру тела до 38 - 39L'C, учащения пульса, потери аппетита, головной боли, нарушения сна.
Пораженный лимфатический узел (узлы) плотный, увеличенный, резко болезненный при пальпации. В дальнейшем заболевание стихает под влиянием своевременного лечения или же острый серозный лимфаденит переходит в острый гнойный с расплавлением лимфатического узла. Размягчение и скопление гноя можно определить пальпаторно по флюктуации.
Трудности диагностики могут возникнуть в связи с изменением клинической картины лимфаденита под влиянием антибиотиков: температура падает, исчезают острый отек и болезненность, НО увеличение лимфатического узла остается. В дальнейшем процесс протекает вяло, и нередко в узле развивается абсцедирование.
Дифференциальный диагноз необходимо проводить со специфическими процессами в лимфатических узлах и системными заболеваниями крови (лейкоз, лимфогранулематоз), опухолями. Из специфических лимфаденитов встречаются лнмфаденоактиномикоз и туберкулез лимфатических узлов. При туберкулезном лимфадените начало заболевания обычно неострое, отмечается более длительное течение без высокой температуры. Кроме того, при  туберкулезе чаще наблюдаются поражения группы узлов, иногда в виде пакета. Правильная оценка клинических симптомов, анамнез, позволяющий установить входные ворота инфекции, острое начало заболевания позволяют поставить правильный диагноз банального лимфаденита,
У детей раннего возраста при поражении паховых узлов нередко опухоль принимают за ущемленную паховую грыжу. Отсутствие стула, рвота, обилие явления позволяют отличить грыжу от лимфаденита.
Эпифизарный остеомиелит бедренной кости иногда приходится дифференцировать от воспаления глубоких тазовых лимфатических узлов (высокая температура, боль, сгибательно-приводящая контрактура бедра). При обследовании ребенка удается установить, что в тазу над пупартовой связкой определяется болезненный инфильтрат, а в суставе сохраняются движения, хотя и в ограниченном объеме. Если клиническая картина остается неясной, для расправления контрактуры накладывают вытяжение и производят рентгенограмму, которая исключает поражение кости.
Лечение. Особое внимание придается устранению первичного очага инфекции. Консервативное лечение включает применение антибиотиков, десенсибилизирующую и общеукрепляющую терапию, а также назначение протеолитических ферментов совместно с физиотерапевтическими процедурами.
При гнойных лимфаденитах выполняют разрез. При тяжелом течении лимфаденита с явлениями токсикоза, особенно у маленьких детей, разрезы производят, не дожидаясь размягчения воспалительного инфильтрата. Разрез длиной 2-3 см достаточен для дренирования гнойной полости. Лишь обширные аденофлегмоны являются показанием к более широким разрезам.

ОСТРЫЙ ГЕМАТОГЕННЫЙ ОСТЕОМИЕЛИТ

Основные  вопросы темы:
1.	Этиопатогенез флегмоны новорожденных
2.	Клиника 
3.	Дифференциальная диагностика
4.	Особенности остеомиелита у детей первых месяцем жизни
5.	Лечение и прогноз

Вопросы контроля исходного уровня:
1.	Понятие остеомиелита
2.	Особенности строения костной ткани у детей
Развитие гематогенного остеомиелита обусловливается гноеродной микрофлорой, но основным возбудителем являются стафилококк (до 90%) или ассоциации стафилококка с кишечной палочкой, протеем и синегнойной палочкой. Инфицирование организма и проникновение микробов в кровяное русло возможны через поврежденную кожу, слизистые оболочки, лимфоидное глоточное кольцо. Определенное значение имеют гнойничковые заболевания кожи, воспаление носоглотки, а также латентно протекающая инфекция. У детей грудного возраста входными воротами инфекции часто служит пупочная ранка.
В некоторых случаях остеомиелит возникает в результате перехода гнойного процесса на кость из прилегающих мягких тканей или других органов (одонтогенный остеомиелит, связанный с кариесом зубов; остеомиелит ребра вследствие эмпиемы плевры; остеомиелит фаланг пальцев, при панариции и т.д.).
Большую роль в процессе развития остеомиелита у детей играют возрастные анатомические особенности строения и кровоснабжения костей: значительно развитая сеть кровеносных сосудов, автономность кровоснабжения эпифиза, метафиза и диафиза, наличие большого количества мелких разветвлений сосудов, идущих радиарно через эпифизарный хрящ к ядру окостенения.
У детей первых 2 лет жизни преобладает эпифизарная система кровоснабжения, в то время как метафизарная начинает развиваться уже после 2 лет. Эпифизарная и метафизарная системы обособлены, но между ними имею гея анастомозы. Общая сосудистая сеть образуется только после окостенения зоны эпифизарного роста.
Для детей младше 2-3 лет характерно поражение метаэпифизарных зон. С возрастом, когда начинает усиленно развиваться система кровоснабжения метафиза, чаще всего страдает диафиз.
Патогенез острого гематогенного остеомиелита до настоящего времени полностью не изучен. Важная особенность воспалительного процесса состоит в том, что оно замкнуто ригидными стенками костной трубки; это приводит к сдавливанию вен. а затем и артерий. Косвенным доказательством такой трактовки нарушения кровообращения кости служит боль, которая является следствием гипертензии в костномозговом канале. Величина внутрикостного давления при остром остеомиелите достигает 300 - 500 мм вод. ст. (при норме 60 - 100 мм вод. ст. у здоровых детей).
Если остеомиелитический процесс не диагностируют в стадии воспаления в пределах костномозгового канала, то с 4 - 5-х суток от начала заболевания, гной распространяется по костным (гаверсовым) канальцам и питательным (фолькманновским) каналам под надкостницу, постепенно отслаивая ее. В более поздние сроки (8-10 сут и позже) гной и продукты распада продолжают отслаивать периост, затем гной прорывается в мягкие ткани, образуя межмышечные и подкожные флегмоны. В этих случаях идет речь о запущенной диагностике остеомиелита, лечение которого представляет значительные сложности. Боль, как правило, стихает при самопроизвольном вскрытии субпериостального абсцесса в окружающие мягкие ткани, так как снижается давление в костной трубке.
Клинические проявления и тяжесть течения острого гематогенного остеомиелита у детей очень разнообразны и зависят от многих факторов: реактивности организма, вирулентности микробной флоры, возраста больного, локализации поражения, сроков заболевания, предшествующего лечения. Большое значение имеет выраженность процессов сенсибилизации. Если возникновение остеомиелита совпало с максимумом гиперергической фазы иммуногенеза в сенсибилизированном организме, на первый план выступает бурная общая реакция, несколько сходная с анафилактическим шоком. В других случаях общие проявления выражены не в такой степени.
В соответствии с указанными обстоятельствами различают три основные формы острого гематогенного остеомиелита: токсическую (адинамическую). септико-пиемическую и местную.
Токсическая (адинамическая) форма протекает чрезвычайно бурно с явлениями эндотоксического шока. При этом, как правило, наблюдается коллаптоидное состояние с потерей сознания, бредом, высокой температурой (до 40 - 41 °С), иногда судорогами, рвотой. Отмечается одышка без четко определяемой клинической картины пневмонии. При исследовании сердечнососудистой системы обнаруживают нарушение центрального и периферического кровообращения, снижение артериального давления, а вскоре возникают сердечная недостаточность и явления миокардита. На коже нередко появляются мелкоклеточные кровоизлияния. Язык сухой, обложен коричневым налетом. Живот чаще всего вздут, болезненный в верхних отделах. Печень увеличена.
Из-за преобладания общеклинических симптомов тяжелого токсикоза бывает крайне трудно установить местные проявления заболевания, а тем более точную локализацию первичного поражения кости. Лишь спустя некоторое время, когда наступает улучшение общего состояния, можно выявить местный очаг. В этих случаях удается отметить умеренно выраженный отек пораженной области, болевую контрактуру прилежащего сустава, повышение местной температуры, а иногда и усиленный рисунок подкожных вен. Обнаружение этих изменений служит поводом для проведения диагностической пункции предполагаемого очага поражения. При наличии острого гематогенного остеомиелита удается зарегистрировать повышение внутрикостного давления, хотя гной в костномозговом канале при остеоперфорации почти всегда отсутствует.
Летальные исходы при этой форме острого остеомиелита до последнего времени наблюдаются часто, несмотря на проведение массивной инфузионной терапии, включающей назначение антибиотиков широкого спектра действия, иммунопрепаратов и даже оперативного вмешательства в очаге поражения.
Септико-пиемическая форма острого гематогенного остеомиелита протекает с общими септическими явлениями, также достаточно ярко выраженными. Однако у больных этой группы можно значительно раньше выявить костные поражения. Начало заболевания также острое, отмечается повышение температуры до 39 - 40°С, нарастают явления интоксикации, нарушаются функции жизненно важных органов и систем. Возможны спутанное сознание, бред, эйфория. С первых дней заболевания появляется боль в пораженной конечности. Болевой синдром достигает значительной интенсивности из-за развития внутрикостной гипертензии. Нередко возникают септические осложнения вследствие метастазирования гнойных очагов в различные органы (легкие, сердце, почки, а также другие кости).
Местная форма острого гематогенного остеомиелита характеризуется преобладанием местных симптомов гнойного воспаления над общеклиническими проявлениями заболевания. В эту же группу следует включить атипичные формы остеомиелита.
Начало заболевания в типичных случаях довольно острое. На фоне кажущегося благополучия появляется резкая боль в конечности. Обычно дети старшего возраста достаточно точно указывают на место наибольшей болезненности. Ребенок старается удержать больную конечность в определенном положении, так как любое движение усиливает боль. Если очаг располагается близко к суставу, то в процесс вовлекаются связочный аппарат и периартикулярные ткани. Это приводит к выраженной и стойкой контрактуре сустава.
Температура с самого начала заболевания повышается и в дальнейшем держится на высоких цифрах (в пределах 38 - 39°С) Общее состояние ребенка быстро ухудшается, снижается аппетит, усиливается жажда, что свидетельствует о развитии интоксикации.
При осмотре больной конечности выявляются первые признаки воспалительного процесса: припухлость в области поражения, сплошная инфильтрация тканей и усиление венозного рисунка кожи. Среди постоянных
местных признаков остеомиелита главным является резко выраженная локальная болезненность при пальпации и особенно при перкуссии над местом поражения. Отек и болезненность распространяются и на соседние участки.
Такие симптомы, как гиперемия кожных покровов и особенно флюктуация в области поражения, являются поздними признаками и свидетельствуют о запущенности остеомиелита.
Значительные диагностические трудности возникают при остеомиелитическом поражении костей, образующих тазобедренный сустав. В первые дни заболевания местные симптомы бывают нечетко выражены из-за мощного мышечного каркаса этой области. При внимательном осмотре удается установить, что нижняя конечность несколько согнута в тазобедренном суставе. Отмечаются также отведение и некоторая ротация наружу. Движения в тазобедренном суставе болезненны. Сустав и кожные покровы над ним умеренно отечны.
Очень тяжело протекает остеомиелит подвздошной кости и позвонков. С самого начала заболевания выражены интоксикация и высокая температура. При исследовании удается определить отечность и наибольшую болезненность при пальпации и перкуссии в очаге поражения. В сомнительных случаях надо шире использовать диагностическую костную пункцию с последующим цитологическим исследованием пунктата.
В ранней диагностике острого гематогенного остеомиелита важное значение имеет также определение внутрикостного давления. Установление факта внутрикостной гипертензии позволяет подтвердить этот диагноз даже при отсутствии гноя под надкостницей или в костномозговом канале.
В последние годы для более раннего и точного определения локализации и распространенности воспалительного процесса применяют метод радионуклидного сканирования костей с последующей компьютерной обработкой полученных данных. С этой целью используют короткоживущие радионуклиды, обладающие костной тропностью (технеций).
При исследовании крови наблюдаются лейкоцитоз (30 - 40 х 10-9/л) со сдвигом формулы крови влево и токсическая зернистость нейтрофнлов. Отмечается значительное увеличение СОЭ (до 60 мм/ч), которая держится длительное время.
Имеются выраженные изменения и в белковом спектре сыворотки крови, которые заключаются в диспротеинемии, увеличении глобулиновых фракций и возникновении гипоальбуминемии. При длительном и тяжелом течении заболевания развивается анемия, обусловленная угнетением костного мозга длительным воздействием токсинов.
Отмечаются также нарушения со стороны свертывающей системы крови (повышается концентрация фибриногена и фибринолитическая активность, ускоряется время рекальцификации, уменьшается время свертывания, повышается протромбиновый индекс).
Рентгенологические признаки острого гематогенного остеомиелита, как правило, выявляются не ранее чем на14 - 21-й день от начала заболевания. Самые ранние рентгенологические признаки остеомиелита можно обнаружить лишь на хорошей структурной рентгенограмме. Обычно отмечаются разрежение и смазанность кости, удается увидеть и остеопороз в области, соответствующей зоне воспаления. Губчатое вещество кости имеет крупнопятнистый рисунок из-за рассасывания костных перекладин и слияния межбалочных пространств вследствие усиленной резорбции. В дальнейшем деструктивные полости расширяются, наблюдаются разволокнение расплывчатость и неровность контуров кортикального слоя. Наиболее достоверным признаком является линейный периостит.
Периостальная реакция обычно значительно распространена и определяется в виде тонкой полосы, иногда вуалеподобной тени, идущей рядом с кортикальным слоем. Выраженность периостальной реакции зависит от локализации очага. Наибольшая периостальная реакция наблюдается при диафизарном поражении, менее выраженная.-, при метафизарном и еще менее выраженная - при эпифизарном.
При дальнейшем прогрессировании воспалительного процесса происходят некроз и лизис костной ткани с замещением ее гноем, грануляциями. Эти изменения, как правило, начинаются с метафиза, постепенно процесс распространяется на диафиз.
Дифференциальный диагноз острого гематогенного остеомиелита чаше всего приходится проводить с ревматизмом, флегмоной, костным туберкулезом, травмой. Для ревматизма характерны летучие боли в суставах, типичные изменения в сердце, подтверждающиеся данными электрокардиографии. При внимательном осмотре и пальпации области поражения удается отметить при ревматизме в отличие от остеомиелита преимущественную локализацию болей и припухлость не над костью, а над суставом.
Флегмона также может протекать с клинической картиной, напоминающей остеомиелит. При флегмоне гиперемия и поверхностная флюктуация появляются гораздо раньше, чем при остеомиелите. Если флегмона локализуется вблизи сустава, может образоваться контрактура. Она будет менее стойкой и в отличие от таковой при остеомиелите обычно расправляется при осторожных пассивных движениях. Окончательный диагноз в ряде случаев может быть поставлен только при разрезе.
Дифференциальный диагноз с костным туберкулезом в типичных случаях несложен. Туберкулезное поражение костей в настоящее время встречается довольно редко и характеризуется постепенным началом. Ребенок, несмотря на боль в конечности, продолжает ею пользоваться. Бывают выраженными симптом Александрова (утолщение кожной складки на больной ноге) и атрофия мышц. На рентгенограмме отмечаются остеопороз - симптом "тающего сахара" и невыраженность периостальной реакции. Однако эта реакция может быть четко выражена при смешанной инфекции, когда присоединяется банальная флора. Так называемые острые формы костно-суставного туберкулеза относятся к несвоевременно диагностированным случаям, когда уже наблюдается прорыв гноя в сустав. В этих случаях, помимо рентгенологической картины, поставить правильный диагноз помогает обнаружение специфической флоры в пунктате из сустава.
Иногда приходится дифференцировать острый гематогенный остеомиелит от травмы костей. Большую роль при этом играют тщательно собранный анамнез, отсутствие септических проявлений и данные рентгенологического исследования. Затруднение подчас вызывают поднадкостничные переломы. Однако на повторной рентгенограмме через 6-8 дней начинает определяться нежная костная мозоль на ограниченном участке.
В настоящее время широко применяются комплексное лечение остеомиелита, обоснованное еще Т. П. Краснобаевым. Оно слагается из трех основных принципов:
1.воздействие на макроорганизмы;
2.непосредственное воздействие на возбудителя заболевания;
3.своевременная и полноценная санация местного очага.
Воздействие на макроорганизм должно быть направлено на устранение тяжелой интоксикации и коррекцию нарушенного гомеостаза. Активная дезинтоксикационная терапия включает введение 10 % раствора глюкозы с инсулином, гемодеза, полиглюкина, эуфиллина, нативной плазмы. Для десенсибилизации организма и нормализации сосудисто-тканевой проницаемости вводят препараты кальция, димедрол, супрастин или пипольфен. Для повышения уровня специфического иммунитета в остром периоде остеомиелита проводят пассивную иммунизацию организма ребенка. С этой целью вводят гипериммунную стафилококковую плазму и антистафилококковый гамма-глобулин.
При проведении интенсивной терапии необходимо контролировать электролитный обмен, кислотно-щелочное состояние и функцию мочевыделительной системы. Назначают мероприятия по регуляции белкового и углеводного обмена. Курс лечения также включает стимуляцию защитных сил организма.
При тяжелых формах заболевания наступает угнетение функции коры надпочечников. Гормональные препараты (гидрокортизон или преднизалон) вводят коротким циклом (до 7 дней). Непосредственное воздействие на возбудителя заболевания осуществляют путем назначения антибиотиков широкого спектра действия. Наиболее эффективным является сочетание внутривенного и внутрикостного их применения. Эффективность антибактериальной терапии значительно повышается при ее сочетании с протеолитическими ферментами. На последующий курс назначают антибиотики, обладающие костной тропностью (линкомицин), в возрастной дозировке сроком на 2 - 3 нед. Антибиотики отменяют при нормализации температуры, исчезновении воспалительной реакции в очаге и тенденции к нормализации общего анализа крови.
Своевременная и полноценная санация местного очага. В связи с тем, что развитие тяжелых форм остеомиелита в большинстве случаев обусловлено внутрикостной гипертензией, первостепенное значение приобретает раннее оперативное вмешательство - остеоперфорация. Производят разрез мягких тканей  над местом  поражения длиной  не  менее   10  -   15  см и  рассекают продольно надкостницу. На границе со здоровыми участками кости наносят 2 -3 перфоративных отверстия диаметром 3-5 мм. При этом обычно под давлением выделяется гной, а при длительности заболевания 2 - 3 дня содержимое костномозгового канала может быть серозно-гнойным. В более поздние сроки поступления больных в стационар (5 - 6-е сутки) гной можно обнаружить и в поднадкостничном пространстве (субпериостальный процесс).
Через остсоперфорационные отверстия производят промывание костномозгового канала раствором фурацилина 1:5000 с антибиотиками.
В тяжелых случаях остеомиелита проводят костный диализ в течение первых 2-3 сут. послеоперационного периода путем постоянного капельного внутрикостного введения растворов антисептиков с антибиотиками.
После остеоперфорации болевой синдром значительно уменьшается или исчезает. В этих случаях, пока ребенок находится в постели, нет необходимости в иммобилизации пораженной конечности. Напротив, ранние движения в кровати способствуют улучшению кровообращения и полному функциональному восстановлению суставов, прилегающих к воспалительному очагу.
Вопрос о целесообразности иммобилизации решается на основании динамики рентгенологических изменений пораженной кости. При явных признаках остеодеструкции на пораженную конечность накладывают глубокую гипсовую лонгету.
Следует особо подчеркнуть необходимость раннего комплексного лечения гематогенного остеомиелита в острой фазе. Только в этом случае можно предупредить переход острого процесса в хронический.
Принципы диспансерного наблюдения и долечивание детей с гематогенным остеомиелитом. Гематогенный остеомиелит требует длительного, упорного, этапного и периодического лечения. Только настойчиво проводя в жизнь эти принципы, можно снизить инвалидность детей в результате заболевания остеомиелитом и избежать тяжелых последствий. 

Различают несколько последовательных этапов в лечении гематогенного остеомиелита:
1.	в острой стадии;
2.	в подострой стадии;
3.	в хронической стадии;
4.	в стадии остаточных явлений.
По прошествии острого периода и выписке из стационара ребенка берут на диспансерный учет и не реже одного раза в 2 мес. в течение полугода проводят контрольные осмотры с рентгенологическим контролем. 
В подострой стадии необходимы следующие мероприятия:
1.	повторное иммуностимулирующее лечение;
2.	УВЧ-терапия (до 15 сеансов);
3.	десенсибилизирующая терапия (14 дней);
4.	антибиотикотерапия (14 дней);
5.	анаболические гормоны (21 день);
6.	белковая диета;
7.	осторожное разрабатывание пассивных и активных движений в суставах.
Если подострая стадия не переходит в хроническую, для закрепления лечебного эффекта указанные курсы проводят в общей сложности в течение года. Рекомендуется санаторно-курортное лечение.
При переходе процесса в хроническую стадию продолжают указанное выше лечение, а когда появляется необходимость оперативного лечения, больного госпитализируют. Основная задача этого этапа - повысить иммунобиологическую сопротивляемость организма больного, радикально и полностью ликвидировать воспалительный процесс и вызванные им осложнения.
При выписке из стационара особое внимание уделяют восстановлению нарушенной функции конечности (лечебная физкультура и тепловые процедуры под прикрытием антибиотикотерапии). В этой же стадии показано санаторно-курортное лечение.
ОСОБЕННОСТИ ОСТЕОМИЕЛИТА У ДЕТЕЙ ПЕРВЫХ МЕСЯЦЕВ ЖИЗНИ
Заболевание встречается преимущественно у детей грудного возраста. Однако в этой возрастной группе новорожденные дети поражаются чаще. Очаг поражения в метаэпифизарной зоне и синовиальной оболочке может быть первичным, развившимся на фоне видимого благополучия, и вторичным - на фоне текущего инфекционного процесса - пупочного, легочного, кишечного сепсиса, несмотря на проводимую антибактериальную терапию или вскоре после ее отмены.
Возбудителем первичного процесса в 98% случаев является стафилококк, вторичного - грамотрицательная флора (клебсиелла, кишечная и синегнойная палочки, протей). Гематогенным путем инфекция попадает в метафиз кости, где и развивается воспалительный процесс, однако вследствие особенностей кровоснабжения метаэпифизарной границы у детей раннего возраста он распространяется на ростковую зону и эпифиз, располагающийся внутри сустава. Поэтому основные клинические симптомы обусловливаются развитием артрита.

Рис. 27,28

Клиника и диагностика. Метаэпифизарный остеомиелит, как правило, начинается остро с повышения температуры, вялости, отказа от еды, щажения больной конечности, при этом ребенок держит ее в вынужденном положении.
При осмотре выявляют припухлость над зоной поражения, деформацию близлежащего сустава, местное повышение температуры. Гиперемия появляется позже, активные движения отсутствуют. Пальпация и пассивные движения больной конечности вызывают резкую болезненность. Отмечается симптом "псевдопареза", когда кисть или стопа на стороне поражения свисают, движения резко ограничиваются.
Местная форма остеомиелита может тканей вокруг сустава. В некоторых случая септикопиемических очагов при сепсисе. Морфологически в кости при остеомиелите преобладают некротические процессы, сопровождающееся; рассасыванием костной ткани в области метафиза, повреждением ростковой зоны и суставных поверхностей. Однако в этом возрасте репаративные процессы выражены хорошо, в связи с чем перехода этой формы остеомиелита в хроническую не происходит. Функциональные нарушения в отдаленные сроки могут быть значительно выраженными и объясняются нарушением роста конечности, деформациями в суставах.
Рентгенологические признаки остеомиелита выявляются раньше, чем при других формах. Уже на 8 - 10-й день можно 'найти ряд характерных симптомов: утолщение мягких тканей на стороне поражения, расширение суставной щели, легкую периостальную реакцию. Очаги деструкции в метафизе на рентгенограммах выявляются лишь на 3-й неделе от начал;: заболевания, а о степени разрушения эпифиза кости и зоны роста можно с достоверностью судить лишь после того, как появится ядро окостенения.  Наиболее часто поражаются дистальный и проксимальный концы проксимальный конец плечевой и большеберцовой костей.
В случаях проникновения инфекции в синовиальную оболочку суставов развивается гнойный артрит без вовлечения в процесс костей. При этом первичная симптоматика очень напоминает описанную выше, однако сустав увеличивается более значительно, уплотняются и отекают периартикулярные ткани. На рентгенограмме увеличивается за счет выпота суставная щель, раньше появляется и более выражена контрактура сустава. При пункции сустава получают гнойный выпот в большем количестве, чем при остеомиелите, где он в начале заболевания может быть реактивным. Обычный процесс в суставе при артрите протекает более медленно и при правильно проведенном лечении исчезает без функциональных последствий.
Лишь в редких случаях при поздней диагностике воспалительный процесс может перейти на суставные поверхности кости (развивается остеоартрит), которые на рентгенограмме выглядят "изъеденными".
Дифференциальная диагностика основных гнойно-септических заболеваний в хирургии новорожденных.
Лечение. При метаэпифизарном остеомиелите лечение имеет свои особенности. Общая терапия осуществляется по принципам лечения острой гнойной хирургической инфекции с учетом возраста ребенка. Применяют антибиотики направленного спектра действия и обладающие тропностью к костной ткани, дезинтоксикационную и иммунотерапию.
Важную роль играет иммобилизация, которую осуществляют с помощью вытяжения по Шеде (на нижней конечности) или повязки Дезо (на верхней конечности).
Хирургические манипуляции в области ростковой зоны и гипсовые люнгеты у новорожденных не применяют. При явной клинической картине артрита лечение проводят с помощью пункций, удаления выпота m сустава с последующим введением антибактериальных препаратов.
При локализации процесса в проксимальном эпифизе бедренной кости по стихании острого воспалительного процесса с целью профилактики патологического вывиха бедра и формирования coxa vara применяют повязки-распорки.
Дети, перенесшие острый гематогенный метаэпифизарный остеомиелит, должны находиться под диспансерным наблюдением врача-ортопеда или хирурга.

ПАТОЛОГИЯ ВЛАГАЛИЩНОГО ОТРОСТКА БРЮШИНЫ.
ПАХОВЫЕ ГРЫЖИ И ВОДЯНКИ ОБОЛОЧЕК ЯИЧКА У ДЕТЕЙ

Основные  вопросы темы:
1.	Понятие влагалищного отростка брюшины
2.	Клиника и диагностика грыж
3.	Дифференциальная диагностика грыж и водянок оболочек яичка
4.	Классификация грыж
5.	Методы лечения 

Вопросы контроля исходного уровня:
1.	Понятие об эмбриогенезе.
2.	Основные виды лечения грыж.

Влагалищный отросток брюшины - это слепое выпячивание брюшины в мошонку через паховый канал, зарастающее к моменту рождения; за счет  влагалищного отростка брюшины образуется влагалищная (серозная) оболочка яичка. 
Грыжи и водянки оболочек яичка (гидроцеле) в паховой области и в области мошонки относятся к частой патологии детского возраста. Грыжи возникают как у мальчиков, так и у девочек и могут быть опасными  и жизнеугрожающими состояниями для ребенка. Возможна гибель яичка, яичника или части кишечника в результате ущемления, странгуляции или заворота. Во избежание этих осложнений  важно своевременно  поставить диагноз и выполнить срочную операцию. 
Эмбриология и анатомия.  Влагалищный отросток, с которым связано образование типичной косой паховой грыжи у детей, появляется на 12-неделе внутриутробного развития. Этот отросток представляет собой как бы дивертикул брюшины (выпячивание), окружающий яичко при его опускании из брюшной полости в мошонку через паховое кольцо.  Часть влагалищного отростка, окутывающая яичко, становится оболочкой яичка  (tunica vaginalis testis). Оставшаяся часть, расположенная внутри пахового канала, в конечном счете облитерируется, таким образом прекращается сообщение между мошонкой и брюшной полостью. Когда по времени происходит эта облитерация - вопрос спорный. В каком-то проценте случаев (по-видимому, около 20 %) влагалищный отросток брюшины остается не облитерированным в течение всей жизни, но не дает никаких клинических проявлений.
Незаращенный влагалищный отросток брюшины еще не является синонимом грыжи, а представляет собой лишь потенциально предрасполагающее к возникновению грыжи состояние. Сама грыжа появляется только тогда, когда кишечник или иное содержимое брюшной полости выходит во влагалищный отросток. Если из брюшной полости во влагалищный отросток проникает только жидкость, то подобная ситуация обозначается термином «сообщающаяся водянка яичка», для которой характерно ее нарастание при повышении активности, вследствие повышения внутрибрюшного давления (крик, плач, напряжение) и уменьшение объема водянки во время сна или других периодов релаксации.
Установлено, что при некоторых заболеваниях частота паховых грыж увеличивается также как и риск послеоперационного рецидива грыжи. Так более высокая, чем в общей популяции, частота грыж отмечается у детей с кистофиброзом легких, у больных с нарушением развития соединительной ткани (синдром Элерса-Данлоса), у детей с врожденным вывихом бедра и у пациентов, получающих хронический перитонеальный диализ, у недоношенных с внутрижелудочковыми кровоизлияниями и у больных с миеломенингоцеле, которым произведено вентрикулоперитонеальное шунтирование.
Прямые и бедренные грыжи у детей чрезвычайно редки и составляют небольшой процент. Диагноз этих грыж редко ставится до операции. У взрослых прямые и бедренные грыжи чаще всего являются приобретенными, что же касается их этиологии у детей, то она остается неясной и вызывает споры. Наиболее важным фактором риска развития этих грыж у детей является перенесенная операция по поводу косой паховой грыжи, которая отмечается в анамнезе у одной трети пациентов детского возраста с прямыми и бедренными грыжами. Риск развития прямых и бедренных грыж так же как и косых, более высок при наличии повышенного внутрибрюшного давления и дефектов развития соединительной ткани.
Что же касается частоты двусторонних грыж в детском возрасте, то сведения здесь весьма противоречивы. Во всех возрастных группах двусторонние грыжи значительно чаще встречаются у девочек – до 50 %. Этот факт, в сочетании с чрезвычайно низкой частотой повреждения репродуктивных органов во время герниорафии (грыжесечения) у девочек, позволяет рекомендовать двустороннее вмешательство у всех девочек.
У пациентов с сочетанной патологией, например с вентрикулоперитонеальным шунтом, асцитом, дефектами развития соединительной ткани, кистофиброзом отмечается высокая частота двустороннего поражения. Поскольку крайне нежелательно подвергать таких детей повторному наркозу, то при перечисленных сопутствующих заболеваниях вполне оправдано двустороннее вмешательство. Операция с обеих сторон целесообразна у мальчиков до 2 лет жизни с четко клинически определяемой грыжей с одной стороны и серьезно подозреваемой с другой.
Клинические проявления. Косая грыжа. Основной признак косой паховой грыжи - выпячивание в паховой области, распространяющееся до дна мошонки и более отчетливо заметное при повышении внутрибрюшного давления (плач, смех, натуживание). Грыжа обычно самостоятельно исчезает при расслаблении, или может быть осторожно вправлена рукой, путем деликатного надавливания, как бы заправляя содержимое грыжи обратно в брюшную полость. Иногда вправлению помогает осторожная тракция (подтягивание) за яичко. Иногда первым клиническим симптомом грыжи бывает внезапное появление её с симптомами ущемления. Однако во многих случаях при тщательном сборе анамнеза  оказывается, что у ребенка все же ранее отмечалась некоторая припухлость в паховой области, либо определялась водянка оболочек  яичка.
При первичном осмотре хирург не всегда может отчетливо определить грыжу, даже если осматривает ребенка стоя, с напряжением передней брюшной стенки, при кашле или во время плача. В этих случаях четкий анамнез в сочетании с наличием пальпируемого утолщенного тяжа, пересекающего лонный бугорок (симптом шелковой перчатки), усиленный кашлевой толчок и расширенное паховое кольцо, пропускающее мизинец косвенно свидетельствуют о существовании грыжи и служат показанием к операции.
Прямая грыжа. Прямая грыжа редкая, но встречающаяся в детском возрасте патология. Прямая грыжа представляет собой выпячивание в паховой области, которое при напряжении и натуживании выбухает в большей степени к бедренным сосудам. В одной трети случаев у детей с прямой грыжей в анамнезе была операция по поводу косой грыжи с этой же стороны, что позволяет предполагать, что одной из причин образования прямой грыжи является повреждение задней стенки пахового канала во время первичной  герниографии. Поскольку грыжа выпячивается через дефект в дне пахового канала, медиальнее надчревных сосудов, то операция заключается в укреплении дна подшиванием поперечной фасции или сухожилия у связки Купера, так же как и у взрослых пациентов.
Водянка яичка. Водянка оболочек яичка бывает сообщающаяся и  не сообщающаяся. При сообщающейся водянке есть сообщение с брюшной полостью, а поэтому данная патология должна рассматриваться как грыжа, а соответственно, и лечиться как грыжа. В типичных случаях в анамнезе отмечается возникновение в области мошонки припухлости, которая то появляется, то исчезает. Осторожное надавливание часто способствует перемещению жидкости из мошонки в брюшную полость, но при повышении внутрибрюшного давления, как правило, вновь появляется в мошонке.  
Несообщающаяся водянка может определяться уже при рождении или развиться через несколько месяцев и, даже, лет без каких либо очевидных причин. В анамнезе обычно отмечаются признаки сообщающейся водянки с увеличенным объемом мошонки и постепенно меняющимися размерами. Пока водянка не становится очень большой, никакое лечение не показано, необходимо лишь наблюдение. Почему водянка может значительно увеличиваться в размерах – вопрос спорный. Возможен клапанный механизм накопления жидкости в мошонке. Узкое (точечное) сообщение оболочек яичка с брюшной полостью нарушает возвращение жидкости из мошонки, куда она попадает при эпизодах повышения внутрибрюшного давления (плач, крик, кашель и т.д.) 
Техника операции. Разрез кожи в паховой области по кожной складке. Гемостаз, венозные сосуды коагулируются. Фасция Скарпа рассекается, обнажается апоневроз наружной косой мышцы, очищается от жира, определяется наружное паховое кольцо. У детей младшего возраста нет необходимости рассекать апоневроз, поскольку большая грыжа увеличивает размер пахового кольца.
Грыжевой мешок обычно лежит внутри пахового канала – кпереди или слегка медиальнее по отношению к семенному канатику. Поэтому для извлечения мешка и семенного канатика в рану безопаснее захватывать мягким зажимом ткани, расположенные больше кпереди. Осторожное отделение передних волокон m.spermaticus  позволяет извлечь мешок и связанный с ним семенной канатик до уровня кожи.
Несомненно, принципиально важным и главным этапом грыжесечения является отделение элементов канатика от грыжевого мешка. Учитывая то, что  эти элементы  у детей  имеют малые размеры, многие хирурги используют во время операции бинокулярные лупы с Х2 или Х3 кратным увеличением.
Поскольку семенные сосуды (артерия и вена) выходят из внутреннего кольца, будучи расположены несколько латерально, то хирург, прежде всего, на них и наталкивается. Раздвигая москитом ткани, выявляют характерную белесоватую ткань грыжевого мешка, берут ее на зажимы. Сосуды отводят кзади, отделяя их от мешка, который подтягивают мягким зажимом. 
По мере отделения сосудов освобожденный мешок перехватывают зажимами, отводя, (ротируя) его медиально. В результате обнажается семявыносящий проток, тесно прилежащий к мешку. Сам проток не следует брать зажимом, лучше отводить прилежащие ткани кзади, чтобы отделить проток от мешка. Большую осторожность   и деликатность необходимо проявить, чтобы не повредить во время выделения семявыносящий проток, его артерию или семенные сосуды. Далее мешок захватывают зажимами и рассекают (убедившись, что в просвете  грыжевого мешка нет содержимого).
При ущемленных грыжах оценивают состояние ущемленных органов (жизнеспособность кишки, яичника, сальника). Если имеются признаки некроза кишки выполняется резекция. После рассечения мешка канатик и яичковые сосуды отделяют до основания мешка, последний перекручивают (на 1-2 оборота) для большего удобства прошивания и перевязывают. Внутреннее кольцо обычно не нуждается в реконструкции. Только огромные грыжевые ворота требуют некоторого обуживания пахового канала. Ориентиром является размер пахового кольца, пропускающий кончик пальца. В случае водянки оболочек яичка разрез оболочек следует продлить до дна мошонки. При этом необходимо контролировать положение семенного канатика и Яичковых сосудов и делать разрез в стороне от этих анатомических структур.
Семявыносящий проток иногда имеет извитой ход вдоль наружного края мешка и может быть поврежден или пересечен, если не соблюдать осторожность. Лучше оставлять часть мешка, чем рисковать повреждением семявыносящего протока,  чрезмерно резецируя мешок. Яичко возвращают в мошонку путем осторожной тракции за него через кожу мошонки. Если имеется сочетанное неопущение яичка, то одновременно производят орхипексию.
Обезболивание в послеоперационном периоде возможно виде инъекций анальгетиков, возможно, их пероральное (через рот) применение. Однако после наркоза иногда возникает  рвота и вместе с рвотными массами может эвакуироваться и лекарство. Поэтому более эффективно применение парацетамола в свечах. Следует избегать использование аспирина в связи с его антикоагулянтными свойствами. Возможно обкалывание раны местными анестетиками пролонгированного действия (бупивикаин), хотя этот метод не позволяет анестезировать глубжележащие слои.
Осложнения грыж и грыжесечения. Ущемление и странгуляция. Ущемленная грыжа развивается в том случае кожа содержимое грыжевого мешка (обычно у мальчиков – кишечник,  и девочек – яичник, фалопьева труба, кишечник) не вправляются в брюшную полость. Ущемление намного чаще (70 %) возникает у маленьких детей в возрасте до 1 года. К 8 годам жизни и в более старшем возрасте вероятность ущемления становится очень низкой. Картина ущемления включает в себя резкое беспокойство, сильные схваткообразные боли в животе, иногда рвоту, сначала без примеси желчи, но затем рвота становится более интенсивной, и в рвотных массах появляется примесь не только желчи, но при большой давности ущемления даже кишечного содержимого, что свидетельствует о странгуляции (сдавления кишки с нарушением кровообращения). При осмотре на стороне поражения в паховой области определяется довольно плотное образование, которое  может располагаться только в паховой области или распространяется на мошонку. Обычно ребенку ранее ставился диагноз грыжи, или родители не обращались к врачу, но сами отмечали периодическое появление припухлости в паховой области. В редких случаях ущемление бывает первым проявлением грыжи.
С патофизиологической точки зрения ущемление развивается в результате постепенно возникающего отека органов, находящихся в замкнутом пространстве пахового канала, что приводит к нарушению венозного и лимфатического оттока, и сдавления. В конечном счете, давление в канале начинает превышать артериальное перфузорное давление и наступает гангрена и некроз содержимого грыжи. При развитии указанных циркуляторных нарушений  грыжевое выпячивание становится более плотным и болезненным может появиться гиперемия отек кожи над грыжевым выпячиванием, ухудшается общее состояние ребенка. При наличии всех выше перечисленных  изменений применяется термин «странгуляция». Это осложнение требует немедленного оперативного вмешательства. Однако необходимость резекции кишки в связи с нарушениями её кровоснабжения возникает очень редко – в 0-1,4 % случаев.
Ущемление грыжи без признаков странгуляции  может быть устранено неоперативным путем в 80 % случаев, что позволяет провести инфузионную терапию, улучшить состояние ребенка, способствует уменьшению отека грыжевого мешка и канатика, а это в свою очередь делает последующее грыжесечение технически менее трудным и более безопасным. При нарушениях кровообращения или некрозе кишечника вправить кишку практически невозможно, поэтому если грыжу удалось вправить, это говорит о том, что вряд ли имеются нарушения кровообращения.
Неоперативное вправление состоит из следующих основных компонентов: седация (обычно парентерально вводимыми препаратами), придание ребенку возвышенного (приподнятого) положения нижней части туловища и прикладывание  холода (лед) в  грыжевому выпячиванию с целью уменьшения  отека. У новорожденных и недоношенных детей лед применяют с большой осторожностью из-за риска развития у них гипотермии.
Седация (внутримышечно или внутривенно) для вправления грыж используется  большинством детских хирургов. Для этих целей применяют следующие препараты: промедол + димедрол; димедрол + пипольфен + аминазин; фентанил + морфин.
Перечисленные сильные седативные препараты должны вводиться больному лишь при условии тщательного наблюдения за пациентом врачами, знакомыми с возможными побочными действиями этих средств. Необходимо проводить мониторинг и пульсоксиметрию. Обязательно должна быть наготове вся аппаратура, необходимая для подачи кислорода, аспирации и реанимации на тот случай, если это понадобится. Указанные препараты особенно рискованно принимать у недоношенных детей, пациентов с признаками дегидратации или находящихся в бессознательном состоянии, поскольку у таких детей может возникнуть угнетение дыхания или апноэ.
Если грыжа не вправляется на протяжении 1-2 часов, несмотря на осторожные попытки мануального вправления (важно попытаться  эвакуировать воздух из кишки в грыжевом мешке обратно в кишечник), показано экстренное оперативное вмешательство.
Принцип операции практически такие же, как и при обычном грыжесечении, с некоторыми добавлениями. Грыжу не следует пытаться вправлять после введения в наркоз, ибо важно во время операции оценить состояние содержимого грыжевого мешка. В некоторых случаях грыжа вправляется спонтанно до вскрытия грыжевого мешка. Если при этом содержимое (выпот) не геморрагическое и не имеет неприятного запаха, а непосредственно прилежащая к внутреннему паховому кольцу петля кишки без признаков некроза, то лапаротомию производить не следует.
Хотя многие хирурги пытаются осуществить эту операцию в ране через паховый доступ предварительно расширив  его. Более удобно сделать резекцию кишки через отдельный лапаротомный разрез с извлечением нежизнеспособной кишки в брюшную полость.
Завершение грыжесечения при ущемленной грыже может сопровождаться трудностями, связанными с отеком, а так же тем, что грыжевой мешок «рвется» при ушивании, а элементы семенного канатика определяются весьма не отчетливо. Поэтому следует соблюдать большую осторожность, чтобы при выделении не повредить сосуды и семявыносящий проток и чтобы надежно ушить мешок, избежав «прокалывания» при этом элементов семенного канатика.
При ущемлении или странгуляции сдавление содержимого грыжи очень быстро может привести к нарушению кровоснабжения яичка, а если ущемление длительное, то в результате порой развивается атрофия яичка. Частота данного осложнения колеблется от 2,6 до 5 %. Однако во время операции яичко выглядит цианотичным значительно чаще (до 29 %), но, к счастью, после ликвидации ущемления и устранения сдавления кровообращение яичка восстанавливается. При сомнительной жизнеспособности яичка его не следует удалять. Яичко в таких случаях опускают мошонку и завершают операцию.

ОСТРЫЕ ЗАБОЛЕВАНИЯ ЯИЧЕК

Основные вопросы темы:
1.	Основные причины острых заболеваний яичка
2.	Клиника и диагностика ОЗЯ
3.	Дифференциальная диагностика между  заболеваниями
4.	Методы лечения ОЗЯ

Вопросы контроля исходного уровня:
1.	Анатомия мошонки и яичка
Острое заболевание яичка, или синдром отечной и гиперемированной мошонки, это комплекс симптомов, характеризующийся болями в области мошонки, отеком мошонки и её органов, покраснением кожи и болезненностью при пальпации.
 Этот симптомокомплекс встречается в любом возрасте, однако чаще всего в препубертате, в возрасте 9-11 лет. Он может быть обусловлен несколькими заболеваниями, имеющими схожую клиническую картину, и был объединен в один синдром, так как во всех случаях наличия такой клиники принята единая тактика лечения: требуется операция по экстренным показаниям.
 Причиной синдрома отечной и гиперемированной мошонки чаще всего становится перекрут гидатиды яичка, перекрут яичка и острый орхоэпидидимит.
 Перекрут гидатиды яичка
В области самого яичка или в области его придатка иногда встречаются выросты, они обычно не превышают по размерам 1-2 мм, могут быть и на тонком, и на довольно широком основании. Эти выросты носят название гидатид яичка и придатка яичка. По своей сути это дериваты, оставшиеся в результате внутриутробного развития половой системы мальчика. Они не имеют никакой функции, но иногда становятся причиной неприятностей. Гидатида на узкой ножке может перекрутиться вокруг своей оси, при этом резко нарушается её кровоснабжение и происходит некроз.
Провоцируют перекрут занятия спортом, прыжки, резкие движения ногой при пинке по мячу. Заболевание начинается с небольших, но постепенно нарастающих болей в области мошонки и нарастанием местных изменений. Перекрут гидатиды сопровождается выраженным болевым синдромом и местной реакцией ввиде отека, гиперемии и болезненности мошонки, максимальными эти явления становятся  ко вторым суткам.
В отдельных случаях в начале заболевания при осмотре гидатида довольно хорошо пальпируется, в дальнейшем нарастающий отек смазывает картину, диагноз становится затруднительным. Клинику трактуют как острое заболевание яичка и ребенка в любом случае оперируют. Гидатида обнаруживается на операции на придатке или в области яичка как образование до 1-1.5 см, нередко багрово-черного цвета. Её в ходе скрототомии и ревизии мошонки удаляют, на этом операция заканчивается. Жалобы у больного тоже. Интересно, что после операции практически все мальчики отмечают прекращение боли в яичке, наличие послеоперационной раны на мошонке беспокоит их гораздо меньше, чем существовавший до операции перекрут гидатиды.
 Перекрут яичка
Перекрут яичка встречается в любом возрасте, описаны случаи даже внутриутробного перекрута, когда соответствующая клиника обнаруживалась у новорожденного ребенка. Однако чаще всего это случается в препубертате, в 9-11 лет. Это обусловлено тем, что у мальчиков в этом возрасте начинается бурный рост яичек, в то же время кремастерный рефлекс всё ещё очень выражен. Чаще всего ребенок во время игры совершает резкое движение ногой (например, пнув мяч), и чувствует боли в яичке. Боли усиливаются, нарастает отек, болезненность и покраснение мошонки. При перекруте яичко заворачивается вокруг своей оси на семенном канатике, причем завернуться оно может не на один, а на два и даже три оборота. При этом сосуды яичка бывают пережаты, резко нарушается его кровоснабжение. В первые часы от случившегося перекрута, пока отек мошонки не значителен, при пальпации иногда удается поставить точный диагноз до операции. Как правило, яичко находится в мошонке несколько выше противоположного, а его придаток пальпируется не на обычном месте. В дальнейшем отек нарастает и диагноз становится затруднителен. Ребенка жизненно необходимо прооперировать в первые 12 часов от начала заболевания, если операция выполняется в более поздние сроки, прогноз для яичка становится не определенным. Иногда оно и на третьи сутки ещё жизнеспособно, но иногда уже через 12 часов оно черное и не жизнеспособное. При скрототомии и ревизии мошонки обнаруживается отечное яичко, перекрученное вокруг своей оси, оно обычно цианотичное, при устранении перекрута после блокады семенного канатика кровообращение восстанавливается, яичко становится пестрым, а потом нормальным по цвету. В случае некроза яичка это не происходит, несмотря на все мероприятия, в таких случаях яичко приходится вынужденно удалить.
 Орхоэпидидимит
Орхоэпидидимит (воспаление яичка и придатка яичка) у детей встречается редко, обычно случается после ОРВИ. Клинически не отличим от остальных острых заболеваний яичка. В принципе, это заболевание и можно было бы лечить консервативно, без оперативного вмешательства, но сходство клинической картины и редкость его как причины синдрома отечной и гиперемированной мошонки обязывают провести операцию в любом случае, так как риск пропустить перекрут яичка в данном случае не оправдан.
 Клиника
Клиническая картина синдрома отечной и гиперемированной мошонки во всех случаях схожа. Ребенок жалуется на боль в яичке. В области половины мошонки появляются постепенно нарастающие боли, покраснение и отек, болезненность при пальпации. Начало заболевания ребенок иногда не связывает ни с чем, иногда отмечают резкое движение ногой до начала болей или падение. Вообще анамнез, как правило, собрать не просто, так как дети стесняются рассказывать о случившемся. Бывает, они и родителям говорят о том, что болит яичко, только на вторые-третьи сутки.
Мало того, со стороны родителей нередко встречается не адекватная оценка проблемы, что служит причиной позднего обращения к врачу (в отношении перекрута яичка – это катастрофа).
Общее состояние детей не страдает. При осмотре обращает на себя внимание особая походка ребенка, он идет, широко расставляя ноги, если ему нужно сесть – он садится, широко разведя колени из-за боли в яичке. При осмотре мошонки обнаруживается отек, болезненность и покраснение половины мошонки.
 Лечение  
Лечение синдрома отечной и гиперемированной мошонки во всех случаях оперативное, под общим обезболиванием производится скрототомия и ревизия мошонки. При операции удаляют перекрученную гидатиду или расправляют перекрут яичка. Если при перекруте яичка оно жизнеспособно, производится фиксация его к дну мошонки, что предотвращает повторный перекрут. Если яичко пришлось удалить, с профилактической целью производится фиксация второго, не пострадавшего яичка. При обнаружении орхоэпидидимита при необходимости мошонку дренируют.

ПОРОКИ РАЗВИТИЯ ПОЛОВОГО ЧЛЕНА
Основные вопросы темы:
1.	Основные заболевания полового члена
2.	Клиника и диагностика  ЗПЧ
3.	Дифференциальная диагностика между  заболеваниями
4.	Методы лечения 

Вопросы контроля исходного уровня:
1. Анатомия полового члена и уретры

Самой частой врожденной патологией полового члена является гипоспадия. В среднем она встречается у 1 из 300 новорожденных мальчиков. Гипоспадия у девочек встречается крайне редко. Наружное отверстие мочеиспускательного канала при этом в виде большого овального отверстия, переходящего в желобок передней вагинальной стенки, открывается во влагалище.
Гипоспадия - (от греч. hypospao - оттягиваю вниз, разрываю снизу) врождённое недоразвитие мочеиспускательного канала с замещением недостающего участка соединительной тканью и искривлением полового члена в сторону мошонки, наружное отверстие канала может как находиться на своём нормальном месте (головка полового члена), так и быть смещено вниз, вплоть до нахождения снизу на теле члена, на мошонке или в промежности. Сам пенис при этом также может быть анатомически недоразвитым, маленьким, тонким или приросшим к мошонке. Обычно крайняя плоть расщеплена и накрывает головку в виде капюшона. В наиболее резко выраженных случаях гипоспадии половой член настолько мал, тонок и приращён к мошонке, что трудно отличить измененный половой член от гипертрофированного клитора и возможны ошибки в определении пола. 
Ввиду того, что недоразвитая часть уретры представлена нерастяжимым рубцом (по виду напоминает плотный шнурок), возникает искривление полового члена к мошонке, особенно при эрекции. Иногда угол искривления настолько велик, что делает невозможным половую жизнь. Замечено, что этот врожденный порок нередко сочетается с крипторхизмом - неопущением яичка в мошонку.
Причинами гипоспадии могут быть наследственные факторы, внутриутробные инфекции, повышенный уровень эстрогенов у матери во время формирования у плода половых органов и мочеиспускательного канала.
Виды гипоспадии и клинические проявления.
Жалобы больных зависят от их возраста и от вида гипоспадии. 
Если детей беспокоит главным образом расстройство мочеиспускания, то взрослых - затруднение или невозможность полового акта. 
Выделяют головчатую (наиболее частая), околовенечную, стволовую и промежностную формы гипоспадии. 
Головчатая - у больных на месте нормального расположения наружного отверстия мочеиспускательного канала определяется лишь углубление или бороздка до венца полового члена. Наружное отверстие открывается на уровне венца полового члена. Наружное отверстие часто бывает суженным. Искривление полового члена отмечается редко. Жалобы возникают лишь при наличии сужения наружного отверстия или при слишком сильно наклоненной головке, когда моча попадает на ноги.
Околовенечная форма - в отличие от предыдущей, наружное отверстие уретры располагается дальше от венечной борозды. Чаще встречаются жалобы на нарушение мочеиспускания.
Стволовая форма - у больных отмечается расположение наружного отверстия уретры на различных уровнях стволовой части полового члена. Характерно искривление пениса, которое зависит от уровня расположения отверстия уретры. Отмечается сужение наружного отверстия уретры. Жалобы на нарушение мочеиспускания (струя направлена книзу), что затрудняет опорожнение мочевого пузыря. 
Мошоночная форма - одна из наиболее тяжелых форм. Характерно резкое недоразвитие и искривление пениса. Половой член напоминает клитор. Расщепленная мошонка усиливает это сходство. Мочеиспускание при этом возможно только сидя, моча разбрызгивается, что вызывает раздражение кожи внутренних поверхностей бедер. 
Новорожденных с мошоночной гипоспадией иногда ошибочно принимают за девочек или ложных гермафродитов.
Промежностная форма гипоспадии - при данной патологии мошонка расщеплена, половой член резко уменьшен в размерах и искривлен, часто скрыт между складками мошонки. Мочеиспускательный канал открывается позади мошонки, на промежности. Наружное отверстие имеет вид широкой воронки. Головка резко недоразвита. Крайняя плоть расщеплена. Промежностная гипоспадия часто сочетается с крипторхизмом, что еще больше затрудняет определение пола. Дети рано начинают понимать свою неполноценность, становятся замкнутыми, раздражительными, уединяются. После полового созревания появляются жалобы на невозможность совершения полового акта.
"Гипоспадия без гипоспадии"- довольно редкий порок развития. Заключается в укорочении дистального отдела мочеиспускательного канала. Это приводит к искривлению полового члена, которое усиливается при эрекции. При этом наружное отверстие уретры отходит в физиологичном месте. Мошонка, крайняя плоть развиты правильно. 
Диагностика типичной гипоспадии особых трудностей не вызывает. Осмотра бывает достаточно.
Но иногда бывает трудно отличить мошоночную и промежностную гипоспадию от женского ложного гермафродитизма. В таких случаях необходимо определять генетический пол ребенка.
Из рентгенологических методов применяют генитографию (для обнаружения матки и придатков), уретрографию для выявления мочеполового синуса. Значительные возможности имеет ядерно-магнитно-резонансная и ультразвуковая диагностика. 
В особо затруднительных случаях для выявления яичников производят лапароскопию или лапаротомию.
Иногда гипоспадия сопровождается сужением наружного отверстия мочеиспускательного канала и приводит к заболеваниям мочевого пузыря и почек. Также она может сочетаться с врожденной патологией мочевыделительной системы, поэтому каждый больной ребенок нуждается в обследовании в специализированном медицинском центре.
Лечение гипоспадии.
При всех формах гипоспадии, кроме головчатой (если нет значительного искривления полового члена или стеноза), показана пластическая операция. 
Принцип всех оперативных вмешательств заключается в следующем - переместить наружное отверстие мочеиспускательного канала в его физиологическое (правильное) положение. 
К настоящему времени предложено много различных оперативных методов лечения, но общим для всех является правило производить операцию уже в первые годы жизни, т е. еще до появления необратимых процессов в кавернозных телах полового члена.
Оперативное лечение для каждого больного индивидуально и назначается хирургом-урологом.
Раньше оперативное лечение гипоспадии проводилось в несколько этапов, и затягивалось на несколько лет. Нередко больные оперировались по 10 - 15 раз. Известен случай, когда пациент перенес 24 операции.
К счастью, современные технологии позволяют устранить гипоспадию за один прием и реально помочь детям с любой формой этого порока, даже если они раньше не раз оперировались.

Выполнение операции
Если имеет место искривление полового члена, то сначала осуществляют его выпрямление путем тщательного иссечения рубцовых тканей и/или выполнения пликации белочной оболочки (наложения стягивающих швов на ее выпуклую часть).
Затем осуществляют создание недостающей части мочеиспускательного канал с использованием кожи полового члена (крайней плоти), мошонки или свободного кожного лоскута предплечья (в особо тяжелых случаях).
При мошоночной гипоспадии возможно выполнение пластики уретры тканью, искусственно выращенной из так называемых стволовых клеток, взятых у ребенка до операции.
Операцию при гипоспадии рекомендуется выполнять в возрасте 1-2 года, пока мальчик еще не осознает своей проблемы, отсутствует психогенная реакция на заболевание (комплекс неполноценности), что позволяет избежать психологической травмы маленького пациента. Кроме того, результаты оперативного лечения взрослых, даже с применением современной техники, несколько хуже.
Это происходит потому, что ткани полового члена развиваются в этом случае в неблагоприятных условиях (деформированы недоразвитой уретрой) и степень рубцовых изменений более выражена.
Результаты оперативного лечения у детей лучше и по функциональным показателям, например по качеству мочеиспускания (полноценное опорожнение мочевого пузыря, отсутствие разбрызгивания струи мочи) и по эстетическому состоянию полового члена.
Возможные осложнения.
Учитывая анатомические особенности перемещенных при первой операции лоскутов, возможно развитие осложнений, связанных с нарушением кровоснабжения искусственной уретры. 
Таким образом, известны случаи многократных хирургических вмешательств до 15 - 24 операций по поводу коррекции гипоспадии. 
В редких случаях возможно появление типичных для всех оперативных вмешательств осложнений, таких как кровотечение, воспаление послеоперационной раны, болевой синдром.
Послеоперационный период.
В ближайшем послеоперационном периоде на срок 10 дней ребенку показан постельный режим. В мочевой пузырь устанавливается катетер для отведения мочи и заживления мочеиспускательного канала. 
Чаще всего результат операции благоприятный. Это позволяет добиться нормальной социальной адаптации и избежать развития психологических и сексуальных проблем после периода полового созревания.
Даже при хороших результатах операции прооперированных детей необходимо периодически показывать урологу, особенно в период полового созревания, когда происходит бурный рост половых органов. Так как в это время иногда могут выявляться скрытые деформации, нуждающиеся в исправлении.

ЗАБОЛЕВАНИЯ ПОЛОВОГО ЧЛЕНА
Оценка состояния крайней  плоти (КП) в детской практике остается предметом дискуссий педиатров, детских хирургов, урологов. При осмотре детей зачастую не уделяется должного внимания возрастным особенностям.. Отсутствует единая точка зрения в определении понятия фимоз  в  детской  практике, нет четко сформулированных границ между нормой и патологией, не отработана тактика  лечения и меры профилактики заболеваний крайней плоти. Нет единства врачей в отношении к синехиям (сращениям головки с внутренним листком крайней плоти). Многие хирурги не знают, как поступить со скоплением смегмы в препуциальном мешке,  насколько опасно её присутствие для возникновения баланита. Обсуждается тактика ведения больных с гнойно-воспалительными заболеваниями крайней плоти. Необходимо ли обведение и выведение головки при острых воспалительных процессах. Практические  рекомендации по методам общей гигиены и способами  ухода за мальчиком в грудном и младшем возрасте пишутся зачастую неспециалистами, а людьми далекими от практики. Подобные советы могут создавать для ребенка больше проблем, чем  пользы. Решению всех перечисленных вопросов было посвящено наше исследование.
Термин фимоз - имеет греческое происхождение,  означает "muzzle" (намордник,  дуло, жерло, рыло), это патологическое состояние, при котором невозможно выведение головки (6). Основная причина фимоза у взрослых больных - это наличие плотного, рубцового кольца  крайней  плоти. Лечение  только оперативное, путем иссечения рубцовой ткани. Обобщенное  определение фимоза, применяемое  у взрослых хирургов , в детской практике недопустимо, иной должна быть и тактика лечения. По нашим наблюдениям  у большинства  мальчиков (87 %)  до 3-х лет открыть головку полового члена не удается, а в 43% невозможно даже увидеть часть головки без травмы препуциального мешка. Однако, это не означает, что всем им требуется операция.  Основная причина данного «физиологического» сужения крайней плоти - врожденное узкое отверстие препуциального мешка в месте перехода наружного листка крайней плоти во внутренний. 
Понятие физиологический фимоз не имеет возрастных рамок и может встречаться в любом возрасте (у новорожденного и 16 летнего подростка).  При осмотре можно отметить следующие характерные особенности физиологического фимоза. Кожа крайней плоти не имеет рубцовых изменений, мягкая, хорошо тянется  (Рис.1). Однако при попытках быстрого или грубого выведения головки возникают радиальные трещины в области соединения внутреннего и наружного листков препуциального мешка. Наиболее глубокие разрывы крайней плоти можно наблюдать при одномоментном выведении головки у мальчиков с физиологическим фимозом.
Современные представления о природе синехий и скоплений смегмы в препуциальном мешке отличаются от ранее существовавших в отечественной литературе.  В настоящее время  нет единой точки зрения о тактике хирурга в отношении этих состояний.
  Классическое описание эмбрионального развития крайней плоти в норме существует с 1949 года (Gairdner D). Развитие препуциального  мешка у плода начинается между 3 и 5мес. гестации. И к моменту рождения крайняя плоть закрывает головку полового члена до меатуса (4,8).  Головка полового члена и крайняя плоть развиваются из одних и тех же  тканей дистальной части полового члена в течение 3-го месяца беременности. Крайняя плоть возникает в виде складки кожи в основании головки полового члена и растет, выдвигаясь над этим основанием. Ее рост с дорсальной стороны происходит более интенсивно, чем с вентральной. Поскольку на ранних сроках беременности мочеиспускательный канал не замкнут, а представляет собой уретральный желобок, то крайняя плоть с вентральной стороны развивается из двух парных выростов. (Рис.3 А,Б). Вслед за слиянием  уретральной площадки в трубку, участки крайней плоти движутся по боковым поверхностям головки накрывая  мочеиспускательный канал сверху  сливаются между собой, образуя уздечку. 

Варианты фимоза в детской практике:
1.	Физиологический фимоз   - 3096 (87%)
2.	Гипертрофический фимоз 
3.	(хоботковая крайняя плоть) -  168 (2,7%)
4.	Рубцовый фимоз (классический)

ОПУХОЛИ

Основные  вопросы темы:
1.	Основные виды доброкачественных и злокачественных опухолей
2.	Клиника и диагностика опухолей
3.	Дифференциальная диагностика опухолей
4.	Методы лечения опухолей
Вопросы контроля исходного уровня:
1.	Понятие о доброкачественности и злокачественности опухолей
2.	Основные виды лечения опухолей

Опухолью общепринято называть избыточное разрастание какой-либо ткани организма любой локализации.
Опухоль как болезнь формируется со всеми своими индивидуальными свойствами в зависимости от особенностей конкретного больного и от разнообразных факторов, вызвавших ее. По этим признакам все опухоли можно разделить на доброкачественные и злокачественные.
Само название "доброкачественная опухоль" указывает на благоприятное течение заболевания. Эти опухоли растут, раздвигая окружающие ткани, имеют часто капсулу, не метастазируют и не рецидивируют после радикальных операций. При гистологическом исследовании в доброкачественных новообразованиях выявляются дифференцированные клетки. Однако нередки случаи, когда локализация доброкачественной опухоли в жизненно важном органе создает угрозу для здоровья и даже жизни больного. Некоторые доброкачественные опухоли, особенно эпителиального генеза, могут озлокачествляться.
Злокачественные новообразования, возникнув в организме больного, обычно приводят к его гибели. Особенности злокачественной опухоли как болезни в значительной степени определяются свойством ее клеток. Злокачественная опухолевая клетка отличается от нормальной большим количеством признаков. Наиболее важные среди них - автономность развития, т. е. неподчинение регулирующим воздействиям со стороны организма-хозяина, анаплазия (утрата способности образовывать дифференцированные морфологически зрелые тканевые структуры), способность к инфильтративному рост}' в окружающие ткани, метастазированию по кровеносным и лимфатическим сосудам, а также иммунологический, гормональный, биохимический и другие атипизмы. Таких отличительных признаков к настоящему времени выявлено около 30.
Все признаки злокачественности приобретаются опухолевой клеткой не сразу. Согласно учению L. Fulds о прогрессии опухолей для приобретения какого-либо признака злокачественности опухолевой клетке необходимо определенное время. Этот период для каждой опухолевой клетки различен, в среднем он составляет 10-15 клеточных генераций. Второй постулат этого учения гласит: клетки приобретают признаки злокачественности независимо один от другого. Клетка может достигнуть морфологического, иммунологического, гормонального атипизма, но не иметь еще таких злокачественных признаков, как метастазирование и рецидивирование или наоборот.
Учение L. Fulds, подтвержденное теоретическими и экспериментальными исследованиями, в клинической практике особенно наглядно проявляется в детской онкологии. Нередко возраст больного ребенка является по времени еше недостаточным для того, чтобы опухолевая клетка полностью озлокачествилась. Поэтому у детей раннего возраста на основании только морфологического исследования опухоли трудно прогнозировать течение заболевания. Это одна из особенностей детской онкологии. Другой ее особенностью является редкость патологии. Заболеваемость злокачественными новообразованиями составляет около 15 на 100 000 детского населения в год. Исходя из этого показателя в стране ежегодно можно ожидать около 12 000-13 000 вновь выявленных больных.
Редкость заболевания является первой объективной причиной поздней диагностики злокачественных опухолей у детей. Педиатры, как правило, не вспоминают о возможности злокачественного заболевания, а родители больного ребенка и думать не желают о такой возможности.
Вторая объективная причина поздней диагностики - отсутствие патогмоничных симптомов, т.е. симптомов, характерных только для опухолевой патологии на ранних стадиях заболевания. Даже такой симптом, как "пальпируемая в брюшной полости опухоль" у детей раннего возраста в 80% случаев обусловлен неопухолевой патологией; макрогематурия, которая у взрослых больных в основном обусловлена опухолью, у детей встречается при разнообразных заболевания и крайне редко - у больных с опухолями почек и мочевого пузыря.
Практически все симптомы, которые выявляются у больных в начальных стадиях опухолевого заболевания, могут встречаться при разнообразных и более частых заболеваниях детского возраста, поэтому при первичном обращении к педиатру или детскому хирургу устанавливают иеонкологический диагноз и соответственно назначают неадекватное лечение, которое в ряде случае» может стимулировать рост и метастазирование злокачественной опухоли. Скрываясь под масками широко распространенных детских заболеваний, злокачественные опухоли становятся практически неуловимыми в начальных стадиях.
Третьей причиной поздней диагностики и неудовлетворительных результатов печения злокачественных опухолей у детей является несовершенная организация онкологической помощи детскому населению. В специализированном  отделении,  где  имеются  все условия для  проведения диагностики и комплексного лечения, включая хирургические вмешательства, лучевую терапию, полихимиотерапию, по существующим старым нормативам одна кокка может обслужить в год не более 5 больных. В настоящее время функционирует не более 500 специализированных коек, которые могут обслужить за один год около 2500 детей со злокачественными опухолями. Соответственно около 10 000 детей с первично выявленными злокачественными заболеваниями остаются за пределами специализированной помощи. Конечно, эти дети лежат в стационарах, но они лишены современных противоопухолевых химиопрепаратов, лучевой терапии, их оперируют хирурги общего профиля, не знакомые с принципами онкологического радикализма и абластики.
Вышеуказанные принципы, а также плохо поддающееся лечению течение злокачественного заболевания привели к тому, что являясь редкой патологией по заболеваемости, злокачественные опухоли у детей занимают второе место среди причин детской смертности после травматизма. Это не относится к группе детей до 1 года, среди которых на первые места выходят пороки развития, врожденные заболевания, а также инфекция, и к больным подросткового возраста, у которых первые места занимают травмы и самоубийства.
Вопросы ранней диагностики в педиатрической онкологии являются важнейшими среди всех прочих. Врач-педиатр должен помнить, что за необъяснимыми симптомами, "нетипичным течением" заболевания у ребенка может скрываться новообразование, исключить которое надо в первую очередь. Врач должен проводить каждый осмотр ребенка с позиции онкологической настороженности, предусматривающей следующие моменты:
1.	знание ранних симптомов опухолей, наиболее часто встречающихся в детском возрасте (5 основных локализаций - кроветворные органы, кости, забрюшинное пространство, центральная нервная система, глаза);
2.	знание предраковых заболеваний и их выявление;
3.	скорейшее направление ребенка в специализированное учреждение;
4.	тщательное обследование каждого ребенка, обращающегося к врачу любой специальности для выявления возможного онкологического заболевания.
Особенностью онкологии детского возраста является и своеобразие морфологии злокачественных новообразований. Преобладают опухоли мезенхимального гистогенеза, редко встречаются эпителиальные. Злокачественные заболевания, рассматриваемые в качестве основной патологии во взрослой онкологии, у детей являются казуистикой, а опухоли, которые поражают детей, у взрослых больных встречаются в единичных случаях. Даже на протяжении детского возраста в разных возрастных группах имеются разительные отличия в преобладании той или иной нозологии.
Морфологическими и биологическими особенностями злокачественных новообразований детского возраста, вероятно, определяется большая эффективность химиотерапии и облучения у детей по сравнению со взрослыми больными. Несмотря на то, что около 80% детей поступают на лечение в III-1V стадиях заболевания, с помощью современных методов комплексного лечения удается излечить в среднем около 50% больных.
Даже опытный специалист-онколог на основании только данных осмотра и пальпации опухоли не всегда может при первичном осмотре установить принадлежность новообразования к той или иной группе. Правильное лечение доброкачественных и злокачественных опухолей возможно только после гистологического исследования. При обнаружении опухоли ее идентификация должна быть проведена незамедлительно.

ДОБРОКАЧЕСТВЕННЫЕ ОПУХОЛИ МЯГКИХ ТКАНЕЙ

По определению специального комитета Всемирной организации здравоохранения к мягким тканям относят все неэпителиальные, внескелетные ткани, за исключением ретикулоэндотелиальной системы и опорных тканей внутренних органов. Опухоли мягких тканей разнообразны по гистологической картине. Около 30% этих опухолей составляют группу неклассифицированных бластом, так как определить их гистогенез не представляется возможным. На основании клинического течения и морфологической картины все опухоли мягких тканей делятся на три группы:
1.	доброкачественные опухоли: гемангиомы, лимфангиомы, фибромы, липомы, невриномы, лейомиомы, рабдомиомы;
2.	условно злокачественные опухоли, часто рецидивирующие после удаления, но не метастазирующие: абдоминальный и экстраабдоминальный десмоид (агрессивный фиброматоз), эмбриональные фибромы, эмбриональная липома, миосаркома, миксоидная липосаркома, гемангиоэндотелиома;
3.	истинно злокачественные опухоли - саркомы, рецидивирующие и метастазирующие: рабдомиобластома, ангиосаркома, синовиальная саркома, липосаркома, лейомиосаркома, злокачественная невринома, злокачественная мезенхиома, неклассифицированные бластомы и др.
ГЕМАНГИОМА
Гемангиома или ангиома - доброкачественная сосудистая опухоль, наблюдающаяся преимущественно в детском возрасте. Она характеризуется довольно быстрым ростом, особенно в первые месяцы жизни ребенка. При этом гемангиома разрушает окружающие ткани, приводя к косметическому, а нередко и функцональному дефекту. Встречается довольно часто: она составляет 45,7% всех опухолей кожи и мягких тканей у детей. Различают капиллярные и кавернозные гемангиомы, а также смешанные, содержащие, кроме ангиоматозного, какой-то другой компонент.
Капиллярная гемангиома. Гистологическая структура характеризуется наличием компактных пластов мелких капиллярного типа сосудов, тесно прилегающих друг к другу. Стенки сосуда образованы базальной мембраной и 1-2 слоями эпителиоподобных клеток. В просвете сосудов содержатся форменные элементы крови. Иногда группы сосудов образуют дольки, разделенные прослойками стромы, богатой полиморфными клетками.
Кавернозная гемангиома построена из множества разных по величине и форме полостей, выстланных одним слоем эндотелиальных клеток. Иногда межполостные перегородки разрываются с формированием сосочкоподобных образований в просвете полостей.
Электронно-микроскопическое исследование гемангиом помогло обнаружить ряд специфических особенностей их морфологии. Опухолевые клетки сходны с эндотелиальными клетками по набору, строению и распределению органелл в цитоплазме. Основная часть цитоплазмы заполнена рибо- и полисомами, а также тонкими фибриллами. Последние в опухолевых клетках представлены в гораздо большем количестве, чем в эндотелиальных клетках нормальных капилляров. Разрушающиеся опухолевые клетки замуровываются аморфным или мелкофибриллярным субстратом, к которому тесно примыкают и внедряются в него коллагеноподобные волокна.
Полученные данные свидетельствуют о том, что клеточные элементы, образующие стенки опухолевых сосудов, имеют структурную организацию, близкую к эндотелиальным клеткам. В межсосудистых участках не находят клеток менее дифференцированных, чем те, которые входят в состав стенок сосудов. Это позволяет считать, что гемангиомы у детей развиваются не из мезенхимального камбия, а за счет пролиферации сосудистого эндотелия. Приведенные данные убедительно свидетельствуют о том, что гемангиому следует рассматривать как опухоль, развивающуюся из эндотелия, а не как порок развития.
Эта опухоль обладает рядом характерных свойств, которые отличают ее от новообразований. Одним из таких отличий является возможность спонтанной регрессии "простых" кожных гемангиом заложенная в микроструктуре самой опухоли. Вот почему, вероятно, действие многих факторов, в частности, тепла, холода, химических веществ, способно остановить рост гемангиомы или полностью излечить ее. Спонтанная регрессия проявляется в относительном замедлении роста опухоли после достижения ребенком возраста 6 мес. Несмотря на возможность остановки роста гемангиомы с последующей инволюцией, дальнейшее развитие ее все же остается непредсказуемым, особенно у новорожденных детей. Поэтому-целесообразно раннее лечение таких больных всеми имеющимися способами.
•	простые ангиомы на коже;
•	кавернозные, располагающиеся под кожей;
•	комбинированные, имеющие кожную и подкожную часть;
•	смешанные, когда ангиома сочетается с другими опухолями, например с лимфангиомой.
Гемангиомы,  как правило, обнаруживают сразу же после рождения ребенка (87,3%) или в первые месяцы жизни; 70% гемангиом встречаются у девочек. Опухоль может располагаться на любом участке тела, очень редко во внутренних органах - печени, легких, мозге, костях. Однако преимущественная локализация (до 80-83%) - верхняя половина тела, включая голову и шею. Наиболее часто (95% всех ангиом) встречаются простые ангиомы, кавернозные составляют около 3% и обширные гемангиомы комбинированного характера чаще сложной анатомической локализации - 2% всех ангиом. Однако, несмотря на столь небольшую частоту, они представляют наибольшие трудности для лечения.
Клиника. Простая ангиома характеризуется пятном разной величины,  красного цвета с оттенками. Пятно всегда возвышается над поверхностью кожи. При пальцевом давлении на край опухоли по границе с непораженной кожей ангиома бледнеет, уменьшается, по прекращении давления вновь восстанавливает свой цвет. У детей раннего возраста, до 3-4 мес, заметен периферический рост опухоли, в чем можно убедиться, сделав абрис опухоли на прозрачной пленке и приложив к тому же месту через 10 -12 дней.
Кавернозная ангиома представляет собой образование, располагающееся в подкожной клетчатке. Кожа над ней не изменена, но под кожей определяется опухоль в виде конгломерата синеватого цвета, иногда видны подходящие к опухоли сосуды. При надавливании на опухоль последняя несколько уменьшается, затем восстанавливает прежние вид и размеры. Иногда при пальпации можно определить дольчатость опухоли. Какой-либо пульсации над опухолью не определяется, хотя кожа может быть на ощупь несколько теплее окружающих тканей. Кавернозные гемангиомы могут быть диффузными, без четких границ или же иметь тонкую соединительнотканную капсулу. Иногда, особенно на лице и шее, а также в околоушной области, эти ангиомы способны к быстрому росту с прорастанием в окружающие ткани, следствием чего могут быть тяжелые функциональные и косметические дефекты.
Комбинированные ангиомы имеют кожную и подкожную части, причем подкожная часть может быть обширной.
Смешанные опухоли встречаются дотольно редко (около 0.5% случаев). Может быть сочетание ангиоматозиого компонента, например, с лимфангиомой, липомой, кератомой, фибромой и другими новообразованиями. Наиболее важной в клиническом отношении является такая особенность гемангиом, как быстрый, порой непредсказуемый рост, особенно в первые 3 мес. после рождения. У недоношенных детей эта особенность выражена наиболее ярко: ангиомы у них растут в 2 -3 раза быстрее, чем у доношенных. Известны наблюдения, когда небольшое пятнышко превращалось за 10 -12 дней в обширную и глубокую гемамгиому. После первого полугодия жизни рост гемапгиомы замедляется, но сказать с полной определенностью о темпах роста опухоли бывает довольно трудно.
В этой связи отдельного внимания заслуживает факт спонтанной регрессии некоторых простых ангиом. Истинная регрессия может наблюдаться в 10-15% случаев, чаще на закрытых участках тела. При этом яркость ангиомы несколько уменьшается, на ней появляются участки беловатого цвета, прекращается периферический рост. Через 6-8 мес ангиома представляет гладкое, не возвышающееся над кожей беловато-розовое пятно, кожа над ним подвергается атрофии, и к 3 -4-му году жизни остается небольшой депигментированный участок кожи.
Следуем подчеркнуть, что спонтанной регрессии подвергается лишь небольшая часть простых ангиом. Кавернозные и комбинированные ангиомы не регрессируют. Поэтому необходим постоянный контроль за состоянием и ростом гемангиом. В процессе своего развития гемангиомы могут изъязвляться и воспаляться. Иногда в результате этих проявлении ангиомы также могут подвергаться обратному развитию.
Грозным осложнением течения гемангиом являются кровотечения. Обычно они наблюдаются у детей с обширными и глубокими комбинированными ангиомами в результате изъязвления или воспалительного процесса, причем остановка кровотечения может сопровождаться довольно большими трудностями. Иногда бывает необходимо экстренное оперативное вмешательство.
Диагноз гемангиомы, как правило, не вызывает больших затруднений. Наличие ярко-красного пятна, возвышающегося над кожей, бледнеющего при надавливании на его край и восстанавливающего цвет, форму и объем, после прекращения давления, свидетельствуют об ангиоме.
Для выявления анатомических нарушений при обширной и глубокой ангиоме, т.е. особенно сложной анатомической локализации, целесообразно выполнить рентгенографию. Однако наиболее информативной в этом отношении является ангиография, которая позволяет выявить сосудистые связи опухоли и определить наиболее рациональные пути ее лечения.
Лечение. Серди многочисленных методов лечения ангиом имеются чисто хирургические способы (иссечение в пределах здоровых тканей с кожной пластикой и без нее) и так называемые консервативные, или неоперативные, способы. Принципиально важным независимо от способа является максимально раннее начало лечения ангиом - с первых дней, недель и месяцев жизни. Не следует надеяться на спонтанную регрессию опухоли. Чем меньше ребенок, тем быстрее увеличивается ангиома и бывает невозможно предвидеть, какой станет небольшая ангиома через 2 кед или месяц.
Выбор способа лечения зависит от характера опухоли (плоская, кавернозная, комбинированная, смешанная), от ее величины и расположения, возраста ребенка, быстроты роста ангиомы, характера осложнений, косметических и функциональных нарушений, вызванных ростом опухоли.
Наиболее эффективным и распространенным способом лечения простых ангиом является их низкотемпературное разрушение (криодеструкция). Остается высокоэффективным замораживание с помощью кристаллической углекислоты (-79°С) путем непосредственной аппликации хладагента на поверхность ангиомы в течение 15-20 сек. В последнее время большое распространение получила аппаратная криодеструкция ангиом, при которой в качестве хладагента используют жидкий азот температуры -1% ° С. Применение аппаратных методов криодеструкции позволяет прогнозирован, результат с высокой точностью. Эффективность лечения достигает 96%.
В тех случаях, когда криогенное лечение трудно выполнимо, например при локализации гемангиомы в области век и глазницы, весьма эффективной оказывается короткофокусная рентгенотерапия. При простых ангиомах большой площади, когда лечение с помощью глубокого холода заняло бы продолжительное время, целесообразно назначение преднизолона через день из расчета 4-6 мг на 1 кг массы тела: в 6 ч утро 1/3 дозы, в 9 ч - 2/3 дозы. Продолжительность курса 20 дней. При  необходимости  курс  может быть повторен неоднократно. Схема не требует постепенной отмены препарата. Необходимы контроль содержания сахара и калия в крови и при необходимости их коррекция.
При лечении кавернозных ангиом следует исходить из локализации опухоли. Если гемангиома располагается в косметически неблагоприятной области (кончик носа, щека, область лба, переносицы), то используют склерозирующую терапию, заключающуюся во введении в ангиому веществ, вызывающих асептический некроз опухоли и ее рубцевание под кожей без рубца и деформации кожи. Для этой цели применяют хинин-уретан, гидрокортизон, 10% раствор натрия хлорида. Наиболее часто используют 70% этиловый спирт, получаемый разведением 96% спирта на 1/4 2% раствором новокаина. Количество вводимого спирта зависит от локализации, формы, величины опухоли и колеблется от 0,5 до 5 мл. Вкол иглы должен быть вне ангиомы; спирт вводят в толщу опухоли и под нее. Иногда на курс приходится выполнять 10-15 инъекций с перерывами между инъекциями от 14 дней до 1 мес.
В случаях, когда не требуется решать вопросы косметики, кавернозная ангиома может быть удалена хирургическим путем, например, при локализации на бедре, плече, передней брюшной стенке, спине.
Более сложной задачей для лечения являются комбинированные гемангиомы. Выбор способа лечения определяется локализацией, величиной и скоростью роста опухоли. При локализации на закрытых участках тела предпочтительнее радиационное хирургическое лечение. Если же ангиома расположена в косметически неблагоприятной области, то можно рекомендовать для лечения метод СВЧ-криодеструкции, заключающийся в облучении опухоли сверхвысокочастотным электромагнитным полем с последующей немедленной криодеструкцией опухоли, что позволяет резко усилить разрушающий эффект криодеструкции с сохранением всех свойств локального охлаждения, особенно органотипической регенерации эпителия кожи. При лечении этой формы ангиом также правомочны склерозирующая, гормональная и лучевая терапия.
Наибольшие трудности для лечения представляют обширные и глубокие ангиомы сложной анатомической (критической) локализации. Эти ангиомы располагаются преимущественно в области головы и шеи, чаще в околоушной области, и характеризуются постоянным, хотя и довольно медленным после первого года жизни ростом. Эти ангиомы склонны к изъязвлению, что дает массивные кровотечения, которые могут приводить к анемии и плохо поддаются склерозирующей гормональной и лучевой терапии.
Хирургическое лечение сопряжено со значительным риском кровотечения и повреждением нервных стволов, в частности, лицевого нерва.
Для выработки оптимального подхода к лечению этой сложной группы больных показана ангиография, с помощью которой определяются характер кровоснабжения опухоли и ее анатомические взаимоотношения. Одним из эффективных способов лечения этой патологии является эмболизация ангиомы таким веществом, как гидрогель, что резко уменьшает кровенаполнение опухоли, снижает возможности коллатерального заполнения ее массы. После этого производят криодеструкцию гемангиомы без ее удаления. Благодаря развитию некробиотического процесса опухоль гибнет, частично рассасывается и остается в виде участков атрофической кожи. Последующие косметические вмешательства необходимы в более старшем возрасте (5-6 лет).
В лечении некоторых видов гемангиом может быть использована комбинация нескольких способов: криогенного, склерозирующего, хирургического, гормонального и лучевого. В ряде случаев применяют интраоперационную криодеструкцию или высокочастотную коагуляцию. Сочетание нескольких способов лечения позволяет добиться более эффективных результатов.
В последние годы исследуется и не без успеха применяется методика локальной СВЧ гипертермии больших ангиом сложной анатомической локализации. Это позволяет частично или полностью отказаться от хирургического лечения и добиться хорошего косметического и функционального результата.
ЛИМФАНГИОМА
Лимфангиома - доброкачественная опухоль врожденного характера, микроскопическая структура которой напоминает тонкостенные кисты различных размеров от узелков диаметром 0,2-0,3 см до крупных образований. Лимфангиомы встречаются значительно реже, чем гемангиомы, и составляют примерно 10-12% всех доброкачественных образований у детей.
Патоморфологическая и клиническая классификация в основном совпадают. Различают простые, кавернозные и кистозные лимфангиомы.
Простая лимфангиома представляет собой разрастание лимфатических сосудов ограниченных участков кожи и подкожной клетчатки.
Кавернозная лимфангиома является наиболее частой формой, наблюдаемой у детей. Структура ее представлена неравномерно наполненными лимфой полостями, образованными из соединительнотканной губчатой основы, содержащей гладкие мышечные волокна, эластический каркас и мелкие лимфатические сосуды, выстланные эпителием.
Кистозная лимфангиома может состоять из одной или множества кист величиной от 0,3 см до размера головы ребенка, которые могут сообщаться между собой. Внутренняя поверхность кист выстлана эндотелием, а стенки содержат плотную соединительную ткань. Могут существовать переходные элементы лимфангиом, возможно сочетание разных форм.
Лимфангиомы чаще всего обнаруживают в первый год жизни ребенка (до 90%), реже в первые 2-3 года. Локализуются они там, где бывают скопления регионарных лимфатических узлов: в подмышечной области, на шее, щеках, губах, языке, паховой области, реже в области корня брыжейки, забрюшинном пространстве, средостении.
Растут лимфангиомы относительно медленно, чаще синхронно с ростом ребенка, но иногда лимфангиомы резко увеличиваются независимо от возраста.
Клиника и диагностика. Простая лимфангиома представляет собой утолщение кожи, слегка бугристое, нерезко очерченное, как правило, с небольшой подкожной частью. Иногда на коже наблюдаются разрастания элементов лимфангиомы в виде небольших узелков. Поверхность лимфангиомы может быть несколько влажной (лимфорея).
Кавернозная лимфангиома определяется по наличию припухлости, нерезким очертаниям, мягкой консистенции. Часто ощущается флюктуация. Кожа может быть спаяна с образованием, но не изменена или мало изменена. При надавливании опухоль может сжиматься, затем медленно наполняться вновь. Смещаемость опухоли незначительна. Рост достаточно медленный. Наиболее характерная локализация кавернозных лимфангиом - шея, околоушная область, щеки, язык, губы. Весьма характерны частые воспалительные процессы, развивающиеся в лимфангиоме. Иногда в результате воспалений лимфангиома останавливает свой рост и даже исчезает.
Кистозная лимфангиома представляет собой эластичное образование, покрытое растянутой кожей, которая, как правило, не изменена. Отмечается отчетливая флюктуация. Иногда через истонченную кожу просвечивает синеватое образование. При пальпации можно уловить неровность стенки кист. Наиболее часто эти лимфангиомы встречаются на шее (причем одна часть опухоли может находиться в средостении в виде "песочных часов"). При этом целесообразно выполнять рентгенографию грудной клетки с целью выявления узла опухоли такой локализации. Кистозные лимфангиомы растут довольно медленно, но при своем росте могут сдавливать сосуды, нервы, а при расположении вблизи трахеи и пищевода - сдавливать эти органы, что требует порой экстренных оперативных вмешательств.
Диагноз лимфангиомы не вызывает затруднений. Клиническая картина довольно характерна, тем не менее для уточнения диагноза, а чаще для уточнения анатомических вариантов расположения выполняют лимфографию. Для этой цели опухоль пунктируют и вводят водорастворимое контрастное вещество 10-20% концентрации. После выполнения исследования контрастное вещество удаляют и полости, куда вводилось это вещество, промывают изотоническим раствором натрия хлорида. Рентгеновские снимки выполняют в двух проекциях, что дает представление о расположении и анатомических взаимоотношениях опухоли.
Дифференциальный диагноз лимфангиомы проводят с брахиогенными кистами шеи, кистами из остатков щитовидно-подъязычного протока. дермоидами, спинномозговыми грыжами, липомами, тератомами, лимфаденитами шеи. Внимательный осмотр больного помогает различить эти заболевания. Шейные кисты располагаются по срединной линии, не достигают больших размеров, связаны с трахеей и слегка смещаются при глотании. Брахиогенные кисты располагаются по краю грудино-ключично-сосцевидной мышцы.
Дермоиды единичны, плотной консистенции, четко отграничены, чаще правильной округлой формы, без тенденции к быстрому росту. Воспалительные изменения в лимфатических узлах характеризуются отечностью, гиперемией, болезненностью, повышением местной температуры. Липомы обычно не флюктуируют, имеют нечеткие границы, кожа над ними не изменена.
Очень важен дифференциальный диагноз со спинномозговыми грыжами, которые располагаются строго медиально, не проявляют тенденции к увеличению, довольно часто сопровождаются неврологическими расстройствами разной степени выраженности. При рентгенографии выявляется порок развития позвонков.
От крестцово-копчиковой тератомы лимфангиому отличают мягкость консистенции, наличие флюктуации, просвечивающаяся через кожу жидкость. Использование для уточнения диагноза рентгенографии позволяет выявить включения, характерные для тератомы и не наблюдаемые при лимфангиомах.
Лечение лимфангиомы в основном хирургическое. Однако в некоторых случаях при небольших лимфангиомах, локализующихся в области носа, губ, околоушной области, проводят склерозирующую терапию, как при гемангиомах.
Хирургическое лечение заключается в иссечении лимфангиомы в пределах неизмененных тканей. Подобные вмешательства легче осуществляются при кистозных лимфангиомах. Кавернозные лимфангиомы часто распространяются в межфасциальные пространства, бывают спаяны с окружающими органами и тканями, поэтому их полное иссечение не всегда возможно. В этих случаях оставшиеся участки лимфангиомы прошивают шелковыми или капроновыми нитями. Весьма эффективна высокочастотная коагуляция (электрокоагуляция) оставшихся участков опухоли в плане предотвращения рецидивирования. Иногда для долечивания используют склерозирующую терапию.
Реже применяют отсасывание содержимого опухоли посредством пункции. Такой способ лечения более рационален у новорожденных при наличии больших лимфангиом на шее, когда затруднены дыхание, глотание, сосание. Это позволяет временно улучшить состояние больного и подготовить его к операции.
Лимфангиомы, подверженные воспалительным процессам, лечат по общим принципам лечения больных с гнойными процессами. Лимфангиому вскрывают, дренируют. Иногда в результате воспалительного процесса лимфангиома может значительно уменьшиться или даже исчезнуть. Оперативное лечение проводят по стихании воспалительного процесса. Озлокачествления лимфангиом не наблюдалось. Оперативные вмешательства по поводу лимфангиом могут быть весьма длительными и тяжелыми, поэтому целесообразно, если нет экстренных показаний, оперировать детей после первого полугодия жизни.

ОСТЕОГЕННАЯ САРКОМА
Остеогенная саркома - первичная костная злокачественная опухоль, развивающаяся из примитивной нолипеленшой соединительной ткани, способной к образованию опухолевого остеоида я кости. Преобладающими (щеточными элементами остеосаркомы являются остеоциты, подвергшиеся малигнизации. По расположению в пределах кости опухоли делят на центральные медуллярные остеосаркомы, параоссальные (юкстакортикальные) остеосаркомы, множественный остеосаркоматоз и остеосаркомы мягких тканей. В настоящей главе описана только центральная медуллярная остеагениая саркома.
Остеогенная саркома по частоте занимает одно из первых мест среди всех злокачественных опухолей. В детском возрасте - в основном поражаются больные старше 5 лет.
Чаще всего поражаются кости нижних конечностей, особенно вокруг коленного сустава, и проксимальные метафизы плечевых костей. Если опухоль возникает до закрытия эпифизарной щели, то эпифизарный хрящ обычно служит барьером для распространения  опухоли в эпифиэ. Подобным барьером являются и суставные хрящи. Опухоль может поражать и плоские кости, но крайне редко.
Клиника. Первым симптомом заболевания является боль. Характерно, что боль не связана с движением конечности и наблюдается в покое, довольно быстро становится постоянной, прогрессирует в своей интенсивности и оказывается мучительной и днем, и ночью. Наиболее сильная боль отмечается при локализации опухоли в костях голени. По мере развития процесса возникают и другие клинические проявления; припухлость, пальпируемая опухоль, натяжение кожи и расширение подкожных вен, повышение местной температуры и нарушение функции конечности. Иногда возникают патологические переломы конечности й месте роста опухоли.

Рис. 32 

Многие больные и их родители связывают появление боли и припухлости с травмой, что при первичном обращении к врачу нередко приводит к неправильному диагнозу типа "посттравматическая гематома". Тщательный анализ анамнеза позволяет часто, установить наличие временного промежутка между моментом травмы и болью, возникшей на фоне роста опухоли. Этот "светлый" промежуток может достигать нескольких месяцев.
Остеосаркомы рано метастазируют, преимущественно в легкие. Гематогенные метастазы в печень и другие кости регистрируются редко. Лимфогенное метастазирование для остеогенной саркомы нетипично.
Диагностика. Рентгенологически различают три формы остеосарком: остсолитическую, остеобластическую и смешанную. При остеолитической форме на рентгенограммах пораженной кости выявляется очаг деструкции литического характера различных размеров с нечеткими контyрами, располагающийся чаще всего эксцентрически. В ранней стадии заболевания периостальная реакция может быть невыраженной. В дальнейшем отмечаются разрушение коркового слоя и нарушение целостности надкостницы, отчетливо выявляется периостальная реакция с образованием характерного периостального козырька. При остеобластическоп форме остеосаркомы отмечается наличие склеротического, с неопределенными краями дефекта в  области метафиза. проникающего через кортикальную пластинку и распространяющегося в окружающие мягкие ткани.
Видны периостальный козырек и множественные периостальные разрастания, отходящие перпендикулярно оси кости спикулы, - гак называемый симптом солнечного протуберанца.
Распространение опухоли за пределы кости в окружающие мягкие ткани может быть довольно значительным. Границы мягкотканого компонента опухоли на рентгенограммах достоверно установить не удается. Для этой	 цели необходимо использовать компьютерную томографию.
Суставы при остеогенной саркоме вовлекаются в процесс исключительно редко, так как эпифизарный и суставной хрящи служат естественным барьером для роста опухоли. Если и происходит прорастание опухоли в сустав, то это является следствием распространения опухоли по мягким тканям и сухожилиям. Почти всегда у больных остеогенной саркомой отмечается остеопороз костей пораженной конечности, который развивается в результате как нейротрофических нарушений, так и бездеятельности конечности из-за сильной боли.
Даже в типичных случаях клинико-рентгенологический диагноз остеосаркомы должен быть обязательно подтвержден морфологическим исследованием. Всем больным до решения вопроса о лечении необходимо производить пункционную биопсию толстой иглой (трепанобиопсию) пораженного участка кости или открытую инцизионную биопсию опухоли.
Минимальное обследование больного для выявления метастазов включает рентгенографию легких в прямой, боковых и косых проекциях, сканирование скелета и ультразвуковое исследование печени. Исследования проводят обязательно до начала лечения и периодически на его фоне.
Дифференцировать остеосаркому, особенно в ранних стадиях заболевания, необходимо от опухоли Юинга, хондросаркомы, злокачественной формы остеобластокластомы, остеоид-остсомы, эозинофильной гранулемы. Иногда приходится дифференцировать остеогенную саркому от неопухолевых заболеваний - поднадкостничной гематомы, оссифицирующего миозита, остеомиелита.
Лечение остеогенных сарком у детей в настоящее время комбинированное - сочетание операции и противоопухолевой химиотерапии. Хирургическое лечение в радикальном варианте это ампутация конечности выше непораженного сустава. Однако большая часть больных и их родителей отказываются от подобных калечащих операций. В последнее десятилетие в тех случаях, когда это технически возможно выполняют различные органосохраняюищие операции на фоне проведения интенсивной противоопухолевой химиотерапии. Проведение интенсивной химиотерапии даже после нерадикальных операций (резекции пораженных участков кости) позволяют достигнуть 2-летней выживаемости примерно у 50% больных.

НЕФРОБЛАСТОМА
Нефробластома (опухоль Вильмса) - злокачественная опухоль почки, развивающаяся из метанефрогенной ткани. Нефробластома занимает пятое место по частоте среди всех злокачественных заболеваний у детей. Возрастной пик заболеваемости приходится на 3 года, хотя опухоль считается врожденной. Мальчики и девочки заболевают одинаково часто, опухоль может возникать в любом участке почки. Приблизительно у 5% больных наблюдается первично двустороннее поражение почек. Крайне редко нефробластома может возникать экстраренально по ходу эмбриональной миграции метанефрогенной ткани. Длительное время опухоль растет в капсуле почки, однако даже в начальных периодах роста опухоли может отмечаться ее гематогенное и лимфогенное метастазирование. Метастазами чаще всего поражаются легкие, печень, кости и забрюпшнные лимфатические узлы.
В зависимости от преобладания мезенхимального или нефробластического компонентов различают три основных морфологических варианта нефробластомы:
1.	типичные нефробластомы; мезеихималькый и нефробластический (эпителиальный) компоненты представлены в равном объеме;
2.	нефробластома с преобладанием эпителиального компонента;
3.	нефробластома с доминирующим мезенхимальным компонентом.
В каждом из этих вариантов выделяют подварианты в зависимости от степени дифференцирован преобладающего компонента опухоли. Так, высокодифференцированный эпителиальный компонент, преобладающий в опухоли, характерен для кистозного типа нефробластомы, а в недифференцированном мезенхимальном варианте опухоль может быть представлена участками рабдомиосаркомы, ангиосаркомы, нейросаркомы и др. В самостоятельную группу выделяют мезобластическую нефрому (мезенхимальную гамартому) - опухоль почки, которая встречается исключительно у детей грудного возраста. Обособление мезобластической нефромы обусловлено доброкачественным характером течения данного варианта нефробластомы.
Клиника. В ранних стадиях заболевания трудно заподозрить наличие у ребенка опухоли почки, так как имеющиеся клинические симптомы непостоянны и неспецифичны (некоторая бледность кожных покровов, похудание, снижение аппетита, тошнота, боль в животе, раздражительность).
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Лишь при больших размерах опухоли, когда она отчетливо определяется при пальпации через переднюю брюшную стенку, возникает подозрение на наличие у больного новообразования.

Рис.34

В это время в результате сдавления опухолью близлежащих органов усиливается боль в животе, могут отмечаться признаки частичной кишечной непроходимости, расширение вен передней брюшной стенки, асцит. У некоторых больных можно выявить гематурию и повышение артериального давления, а также анемию и ускорение СОЭ.
Диагностика. Для диагностики нефробластомы наряду с осмотром больного, пальпацией органов брюшной полости и лабораторными исследованиями необходимо использовать обзорную рентгенографию брюшной полости, экскреторную урографию, ультразвуковое исследование брюшной полости и забрюшинной области и ангиографию почек. Уже на обзорной рентгенограмме органов брюшной полости можно в проекции почки определить гомогенную тень, "вынужденный изгиб" позвоночника, смещение петель кишечника в противоположную от опухоли сторону и нечеткие контуры поясничной мышцы на стороне поражения.
На урограммах при опухоли Вильмса определяются увеличение почки, нечеткость ее контуров, изменение ее положении и деформация собирательной систем почки. В ряде случаев при больших размерах опухоли и практически полном поражении паренхимы почки функции ее на урограммах не определяется ("немая" почка). В этих случаях, как и при двустороннем поражении почек и сочетании опухоли с пороками развития почек, обязательным является проведение ангиографического исследования. На серии ангиограмм четко определяются признаки опухоли Вильмса - скопление контрастного вещества в опухоли в виде "озер", расширение, деформация и ампутация сегментарных артерий, нарушение их дихотомического ветвления, быстрый сброс артериальной крови в венозное русло по патологическим артерио-венозным шунтам.
Весьма информативным для хирургов является контрастирование нижней полой вены при проведении каваграфии, так как в ряде случаев удается до операции выявить ее расположение по отношению к опухоли, наличие в вене опухолевого тромба или ее вторичную деформацию за счет сдавления увеличенными метастатическими лимфатическими узлами. С помощью эхографии удается отдифференцировать врожденные и приобретенные кистозные образования почки от опухолей, установить довольно точно размеры новообразования и отношение его к ближайшим органам, а также следить за эффективностью проводимого лечения. Ультразвуковое исследование позволяет также выявить метастазы опухоли в печень и забрюшинные лимфатические узлы и вовремя при динамическом контрольном обследовании обнаружить рецидив заболевания. Метастазы в легкие выявляют с помощью рентгенографии грудной клетки в пяти проекциях (прямая, два боковых и два косых снимка), а поражение костей - путем радиоизотопного исследования скелета.
Различают четыре стадии нефробластомы:
I	стадия - опухоль локализуется внутри почки и не прорастает ее капсулу;
II	стадия - опухоль выходит за пределы почки, но не   прорастает собственную капсулу, метастазы отсутствуют;
III	стадия - опухоль прорастает собственную капсулу, околопочечную клетчатку или поясничные мышцы и прилежащие органы, имеется поражение регионарных лимфатических узлов, разрыв опухоли до или во время операции;
IV	стадия - наличие отдаленных метастазов (в легкие, печень, кости и другие органы). Некоторые авторы двустороннюю нефробластому называют V стадией.

Нефробластому необходимо дифференцировать прежде всего от пороков развития почек (гидронефроз, поликистоз почек и мультикистозная почка, удвоение почки, подковообразная почка и др.) и забрюшинных внепочечных образований (нейробластома, рабдомиобластома, ангиосаркома, тератома). В ряде случаев приходится проводить дифференциальный диагноз с опухолями печени и лимфомой брюшной полости.
Лечение нефробластомы комплексное: 
1.	хирургическое (трансперитонеальная нефрэктомия);
2.	лучевое (предоперационное и послеоперационное облучение ложа опухоли);
3.	химиотерапевтическое (предоперационное и в послеоперационном периоде). 
При выборе метода лечения учитывают стадию заболевания, морфологическое строение опухоли, возраст ребенка. Принципиальная схема лечения нефробластомы в зависимости от стадии заболевания представлена в таблице 5.
Таблица 5
Методы лечения нефробластомы в зависимости от стадии заболевания 

Стадия заболевания	Предоперационная химиотерапия	Предоперационная лучевая
терапия	Операция	Послеоперационная лучевая терапия	Послеоперационная химиотерапия	
I	-(+)	-	+	-	-(+)	
II	+	-	+	-(+)	+	
III	+	-(+)	+	-	+	
IV	+	-(+)	-(+)	+	+	
V	+	-(+)	-(+)	-(+)	+	

У детей в возрасте до 1 года с 1 стадией заболевания лечение может быть ограничено только оперативным удалением опухоли. При II стадии обязательно проведение предоперационного и послеоперационного курсов химиотерапии.
Послеоперационную лучевую терапию проводят в случае разрыва опухоли во время операции, наличия остаточных микрометастазов и т.д. В III стадии заболевания предоперационную лучевую терапию проводят при неэффективности предоперационной химиотерапии или огромных размерах опухоли. Вопрос о целесообразности проведения предоперационной лучевой терапии у больных с IV стадией заболевания решается индивидуально и зависит в основном от размеров опухоли, эффективности предоперационной химиотерапии.
Прогноз при опухоли Вильмса в основном определяется стадией заболевания на момент поступления, морфологическим вариантом нефробластомы и возрастом детей. При I стадии заболевания можно добиться выздоровления не менее чем у 95% детей, при II стадии - у 70-80%, при III стадии - до 50%, при IV-V - до 10%.
Исход бывает более благоприятным при дифференцированных вариантах нефробластомы. При анализе такого прогностического фактора, как возраст пациентов, отмечено, что чем младше ребенок, тем выше вероятность благоприятного исхода.
НЕЙРОГЕННЫЕ ОПУХОЛИ
У детей встречаются как злокачественные, так и доброкачественные нейрогенные опухоли.
Нейробластома - злокачественная опухоль, встречающаяся преимущественно в детском возрасте; составляет от 6 до 8% всех злокачественных новообразований у детей. Термин "нейробластома" собирательный для злокачественных форм нейрогенных опухолей.
Опухоль исходит из клеток симпатической нервной системы и может встречаться в любой области тела, но чаще всего она локализуется в забрюшинном пространстве и заднем средостении. В зависимости от клеточного состава различают четыре основные разновидности нейрогенных опухолей:
1.	симпатогониома - опухоль, состоящая из клеток, напоминающих симпатогонии. Преимущественная локализация - надпочечник. Обладает тенденцией к быстрому росту и раннему метастазированию;
2.	симпатобластама - более зрелая разновидность нейробластомы. Опухоль представлена более дифференцированным по сравнению с симпатогониями типом клеток - симпатобластами
3.	ганглионейробластома (злокачественная ганглионейрома) новообразование, клеточный состав которого представлен незрелыми нейтроцитами различной степени дифферепцировки (симпатогонии, симпатобласты, ганглиозные клетки различной степени зрелости);
4.	ганглионеврома - доброкачественная опухоль, состоящая из зрелых ганглиозных клеток.
Нейробластомы относятся к гормонально активным опухолям и практически всегда продуцируют катехоламины и их метаболиты (дофамин, ДОФА, винилинминдальная и гомованилиновая кислоты).
Клиника. Признаки заболевания на ранних стадиях обычно - проявляются в виде неспецифического симптомокомплекса - синдрома малых признаков: ребенок становится вялым, сонливым, раздражительным, плохо ест, беспокойно спит. По мере развития болезни часто возникают различные вегетативные нарушения, обусловленные повышением тонуса симпатической нервной системы под влиянием гиперпродукции опухолью катехоламинов: повышенная потливость иногда в виде профузного пота, резкая бледность с "мраморным" рисунком кожи, выпадение волос, головная боль, тахикардия, транзиториая артериальная гипертензия. Примерно у 10% больных может возникать диарея различной интенсивности, но обычно она носит профузный характер и сопровождается нарушениями водно-солевого обмена. Диарейный синдром, по-видимому, вызывается действием вазоактивного интестинального полипептида (ВИП), который продуцируется опухолью.
Местные симптомы нейробластомы обусловлены сдавлением и смещением соседних органов и тканей, прорастанием опухоли в них. Опухолевые узлы, располагающиеся в верхнем отделе средостения и надключичной области, вследствие сдавления шейного нервного сплетения или его поражения нередко проявляются только синдромом Горнера- (птоз, миоз и энофтальм на стороне поражения). При расположении нейрогенных опухолей в заднем средостении и забрюшинно они могут прорастать через межпозвоночные отверстия в спинномозговой канал и сдавливать спинной мозг, что приводит к парезам и параличам ниже уровня сдавления.
Склонность нейробластом к бурному раннему метастазированию приводит к тому, что причиной первичного обращения к врачу у многих больных являются симптомы, обусловленные не самой опухолью, а ее метастазами, что нередко затрудняет правильную диагностику. Так, у детей грудного возраста чаще всего признаки метастазирования проявляются в виде гепатомегалии или появления множественных мелких подкожных узелков. Поражение костного мозга проявляется клинически в виде бледности и изменений в анализах крови. Метастазы в мягкие ткани орбиты вызывают экзофтальм и экхимозы век. Костные метастазы приводят к ограничению функции пораженной конечности и сильным болям.
Диагностика. Первичное диагностическое обследование больных с нейробластомой должно включать рентгенографию грудной клетки (прямую и боковую), экскреторную урографию, рентгенографию костей скелета, исследование костного мозга и определение катехоламинов в моче. Весьма информативным для выявления опухоли, располагающейся в забрюшинной области и малом тазу, является ультразвуковое исследование. Дополнительные исследования (миелография, ангиография, сканирование печени) применяют по индивидуальным показаниям. Морфологическое подтверждение диагноза необходимо у всех больных (пункция или биопсия опухоли). Обязательным является также гистологическое исследование увеличенных периферических лимфатических узлов и подкожных опухолевых узелков, если таковые имеются.
При подозрении на наличие забрюшинной опухоли обследование больного начинают с проведения урографии и ультразвукового исследования. При забрюшинных нейробластомах на урограммах почти, всегда видны почки нормальных размеров, обычной формы, с четкими контурами. Наиболее частым и типичным рентгенологическим признаком забрюшинных нейрогенных опухолей является изменение положения почек и мочеточников. Для опухолей надпочечников характерно смещение почек книзу. При нейробластомах, исходящих из симпатических ганглиев парааортальной и паравертебральной областей, почки и мочеточники смещаются латерально. На боковых рентгенограммах обнаруживается смещение почки и мочеточника кпереди. Если опухоль прорастает в почку, то может отмечаться деформация чашечно-лоханочного аппарата в виде его разрыва, как и при опухоли Вильмса. Отсутствие функции почки при забрюшинных новообразованиях непочечного генеза наблюдается крайне редко, как правило, в запущенных случаях и свидетельствует о полном поражении почечной паренхимы или глубоком нарушении кровоснабжения почки.
В сомнительных случаях, когда смещение почек и мочеточников незначительно, а контуры небольшой опухоли отчетливо не прослеживаются, экскреторную урографию повторяют на фоне введения воздуха в забрюшинное пространство (пневморетроперитонеум). При больших опухолях забрюшинного пространства эта методика нецелесообразна, так как воздух плохо проникает на пораженную сторону и не окружает опухоль. Ангиография показана в сложных для диагностики случаях.

Проведение рентгенологического обследования органов грудной клетки в прямой и боковой проекциях позволяет в большинстве случаев установить наличие опухоли и отличить нейрооластому заднего средостения от других новообразований на основании типичных локализаций. Расположение опухоли с четкими контурами в реберно-позвоночной борозде, вторичные изменения в задних отделах ребер, межреберьях и позвоночнике, а также наличие иногда б опухоли известковых включений являются наиболее типичными признаками неврогенной опухоли.

В развитии нейробластомы различают 5 стадий:
I стадия - опухоль локализуется в области первичного очага:
II стадия - опухоль распространяется за пределы первичного очага, но не переходит средней линии; могут быть  поражены лимфатические стороне поражения;
III стадия - опухоль распространяется за пределы средней линии, входят опухоли, распространяющиеся билатерально; лимфатические узлы поражены с обеих сторон позвоночника:
IV стадия - метастазирование отдаленное (кости скелета, черепа, мягкие ткани, лимфатические узлы шеи, паховой области и т. д.);
V стадия - объединяет больных с I или II стадиями, но имеющих метастазы в один из органов (печень, кожу или костный мозг), за исключением метастазов в кости. Эту стадию называют также "первично диссиминированной нейробластомой.
Нейробластомы заднего средостения у детей приходится дифференцировать от других опухолей и объемных образований. Основным дифференциально-диагностическим признаком при этом является типичная локализация того или иного новообразования.
Лечение нейробластомы комплексное. Выбор тактики лечения зависит от стадии заболевания и морфологического варианта нейробластомы (таблица 6) .
Таблица 6.
Методы лечения нейробластомы в зависимости от стадии заболевания

Стадия заболевания	Предоперационная химиотерапия	Предоперационная лучевая
терапия	Операция	Послеоперационная лучевая терапия	Послеоперационная химиотерапия	
I	-	-	+	-(+)	-	
II	-(+)	-(+)	+	+	-(+)	
III	+	+	+	-(+)	-	
IV	+	+	-(+)	-	-	
IV s	+ или	+	-	+ или	-	

Для контроля за эффективностью проводимого лечения необходимо динамическое исследование суточной экскреции с мочой катехоламинов  и их метаболитов. Снижение пли нормализация их на фоне лекарственною лечения может быть первым признаком эффективности проводимой терапии. В настоящее время 2-летняя выживаемость больных с нейробластомой составляет от 35 до 40%.
На прогноз заболевания влияют такие факторы, как локализация опухоли, ее морфологический вариант, стадия заболевания.
Отмечено, что при локализации опухоли в заднем средостении возможность излечения выше. Прогноз заболевания более благоприятен: при преобладании в опухоли высокодифференцированных клеток и при ранних стадиях заболевания.

ЛИТЕРАТУРА:
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2.	Ашкрафт К. У. Детская хирургия. — СПб., 1999. — Т. 3. — С. 11—28.
3.	Баиров Г. А. Хирургия пороков развития у детей. — Л., 1968.
4.	Исаков Ю.Ф. Хирургические болезни детского возраста. – М., 2004