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"code": "Q01",
"name": "Энцефалоцеле",
"icd_name": "Энцефалоцеле",
"gender": 0,
"age_min": 0,
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"cause": [
"0"
],
"periodicity": 1,
"slug": "q01_encefalocele",
"lead": "черепно-мозговая грыжа, содержащая оболочки и вещество головного мозга, но не включающая его желудочки",
"description": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Энцефалоцеле – это порок развития черепа и головного мозга, при котором часть мозгового вещества оказывается вне черепной коробки вследствие дефекта костной ткани.</span></p>",
"etiology": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Черепно-мозговые грыжи возникают вследствие неправильной закладки нервной трубки во внутриутробном развитии. Факторы, которые приводят к этому, четко не выявлены. Предполагается влияние вредных факторов на организм беременной женщины:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #333333; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">употребление наркотиков, алкоголя; курение;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #333333; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">злоупотребление лекарственными средствами;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #333333; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">частые простудные заболевания;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #333333; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">инфекционные болезни во время беременности: токсоплазмоз, краснуха;</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6.5pt; font-family: Verdana; color: #333333; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre;\" dir=\"ltr\">\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">внезапные нарушения эмбрионального развития.</span></p>\r\n</li>\r\n</ul>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Также не стоит исключать и генетическую предрасположенность, ведь характерная аномалия, преобладающая у кровных родственников, часто распространяется и на будущее потомство.</span></p>",
"pathogenesis": "",
"diagnostics": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Первичная диагностика возможна уже в первые дни и часы жизни ребенка, поскольку образование визуализируется сразу после рождения. Любое опухолеподобное выпячивание изначально расценивается как энцефалоцеле с последующей дифференциальной диагностикой. Чаще всего приходится исключать полипы и ангиомы.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Диагноз уточняется на основании данных КТ и МРТ, при этом проводится серия снимков, подтверждающих наличие костного дефекта и присутствие мозговых тканей в выпячивании. Просвечивание (трансиллюминация) позволяет заподозрить наличие в образовании нервных тканей. Кроме того, даже легкое надавливание вызывает беспокойство пациента в связи с резким повышением внутричерепного давления.</span></p>",
"treatment": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Лечение только оперативное. Срочность операции определяется состоянием ребенка. Чаще всего вмешательство по удалению энцефалоцеле планируется в возрасте от 3 лет. Такой срок необходим для того, чтобы понаблюдать за развитием головного мозга, в том числе за участком в составе выпячивания. Кроме того, ребенок этому времени уже достаточно окрепнет, чтобы благополучно перенести такую сложную операцию. Противопоказаниями являются повышенное внутричерепное давление, выраженные психические и неврологические расстройства. Истончение кожи над энцефалоцеле, наоборот, является показанием к срочному хирургическому вмешательству.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Операция осуществляется в два этапа. Сначала выполняется закрытие костного дефекта и отсечение ножки грыжи. В раннем возрасте отверстие в черепе можно закрыть лоскутом надкостницы, при более поздних операциях (в возрасте более 3 лет) применяется костный трансплантат.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Второй этап операции – косметическое иссечение поверхностно расположенной оставшейся части грыжевого мешка. Проводится через 15-20 дней после первого этапа (3-6 месяцев в случае интракраниального доступа). Тактику операции в каждом конкретном случае определяют в зависимости от степени вовлеченности мозговых тканей в энцефалоцеле.</span></p>",
"prevention": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Врожденные деформации лица предупредить невозможно. Если в семье родился ребенок с подобной деформацией, то родителям желательно пройти медико-генетическое консультирование, чтобы иметь возможность оценить риск появления такой же аномалии у следующего ребенка.</span></p>",
"clinical_picture": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Симптомы энцефалоцеле могут варьироваться от одного человека к другому в зависимости от многих различных факторов, включая размер, местоположение, количество и вид мозговой ткани, выступающей из черепа. Энцефалоцеле — врожденный порок развития, т.е. присутствующий при рождении ребенка. Расположение энцефалоцеле очень важно, так как есть определенные клинические последствия для лечения и прогноза для переднего и заднего энцефалоцеле. Задние энцефалоцеле чаще связаны с неврологическими проблемами. Энцефалоцеле передней части черепа обычно не содержат мозговой ткани и, как правило, имеют лучший прогноз.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Наиболее распространенной областью черепа для развития энцефалоцеле является верхняя часть от лба до нижней части спины черепа в области затылочной кости. Энцефалоцеле также может возникать вблизи пазух, лба и носа или около основания черепа.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Симптомы, которые могут развиться, включают:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">задержки в достижении основных этапов развития;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">умственную отсталость;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">неспособность к обучению;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">задержки роста;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">эпилепсию;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">ухудшение зрения;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">несогласованные произвольные движения (атаксия);</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">гидроцефалию, состояние, при котором избыток спинномозговой жидкости в черепе вызывает давление на мозг.</span></li>\r\n</ul>\r\n<p> </p>",
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"description": "Микроцефалия – недоразвитие черепа и головного мозга, сопровождающее умственной отсталостью и неврологическими отклонениями. \r\n ",
"etiology": "<p>Первичной считается микроцефалия, развитие которой произошло в результате наследования ребенком дефектных генов от одного или обоих родителей. К хромосомным заболеваниям относятся: </p>\r\n<ul>\r\n<li>Синдром Дауна; </li>\r\n<li>Синдром Эдвардса; </li>\r\n<li>Синдром Патау; </li>\r\n<li>Синдром кошачьего крика и т.д. </li>\r\n</ul>\r\n<p>Вторичная микроцефалия развивается в результате воздействия неблагоприятных факторов в период эмбрионального развития плода, когда происходит закладка и формирование структур головного мозга. Генетический аппарат родителей при этом не нарушен. </p>\r\n<p>Вторичная микроцефалия может быть обусловлена: </p>\r\n<ul>\r\n<li>Вирусными заболеваниями (краснухой, цитомегалией, корью); </li>\r\n<li>Токсоплазмозом; </li>\r\n<li>Злоупотреблением алкоголем; </li>\r\n<li>Злоупотреблением наркотиками; </li>\r\n<li>Приемом некоторых медикаментов; </li>\r\n<li>Голоданием; </li>\r\n<li>Внутриутробной гипоксией плода; </li>\r\n<li>Механическими травмами. </li>\r\n</ul>",
"pathogenesis": "<p>При первичной микроцефалии возникает остановка роста мозга (и вслед за ним черепа) без указаний на дегенеративный процесс или повреждение. Истинная микроцефалия всегда имеет наследственный характер. Она возникает в результате раннего поражения зародышевых клеток и обусловленного этим искаженного развития мозга. При этом отсутствуют признаки перенесенных мозговых заболеваний. </p>\r\n<p>Вторичная (ложная, или церебропатическая) микроцефалия возникает в результате рано перенесенного мозгового заболевания. Чаще оно носит воспалительный или травматический характер. </p>",
"diagnostics": "<p>Диагностика микроцефалии включает: </p>\r\n<p>Жалобы и анамнез: уменьшение размеров головы, задержка в психоречевом развитии, снижение мышления, памяти, внимания, расторможенность, неусидчивость. Перинатальный анамнез отягощен, в анамнезе возможны перенесенные нейроинфекции. Окружность черепа не увеличивается или увеличивается незначительно. <br /> Физикальное обследование: маленькая окружность головы, лоб скошен, затылок уплощен, отмечается выраженная диспропорция между лицевым и мозговым черепом, большие выступающие уши. У 95% больных отмечается неврологическая симптоматика: нарушение мышечного тонуса, спастические парезы, судороги, умственная отсталость, расстройство координации движений, косоглазие. <br /> Инструментальные исследования: на ЭЭГ задержка формирования возрастной корковой ритмики, диффузные изменения электрогенеза головного мозга. </p>",
"treatment": "<p>Современное лечение микроцефалии заключается в основном в симптоматической поддержке больного при помощи медикаментозных препаратов, а также в комплексных реабилитационных мероприятиях, направленных на максимальную адаптацию и социализацию ребенка. В качестве лекарственной терапии для лечения микроцефалии применяются препараты, улучшающие обменные процессы в мозговой ткани, витаминные комплексы и т. д. По показаниям - противосудорожные и седативные препараты. В рамках реабилитационных мероприятий детям с микроцефалией необходимы занятия лечебной физкультурой, массаж, трудотерапия.</p>",
"prevention": "<p>Профилактика микроцефалии у детей предусматривает тщательное планирование беременности, обследование на инфекции (TORCH-комплекс, ПЦР), антенатальную охрану плода. Раннее внутриутробное выявление микроцефалии является основанием для решения вопроса об искусственном прерывании беременности. Медико-генетическое консультирование семей, имеющих детей с микроцефалией, необходимо для оценки потенциального риска при последующих беременностях.</p>",
"clinical_picture": "<p>Объем черепа у ребенка с микроцефалией уменьшен уже при рождении, в дальнейшем его развитие заметно отстает от возрастной нормы. Отмечается преобладание лицевого черепа над мозговым. Типичный внешний вид больного с микроцефалией характеризуется узким и скошенным лбом, выступающими надбровными дугами, большими ушами. Большой родничок и черепные швы закрываются уже в первые месяцы жизни. В дальнейшем больные с микроцефалией обычно отстают в массе и росте (вплоть до карликовости), имеют диспропорциональное телосложение, узкое высокое (готическое) небо, большие редкие зубы. </p>\r\n<p>Неврологические нарушения при микроцефалии могут включать мышечную дистонию, спастические парезы, атаксию, судороги, косоглазие. Часто дети с микроцефалией могут страдать эпилепсией и детским церебральным параличом. Дети с микроцефалией поздно начинают держать головку, сидеть, ползать, ходить. Отмечается грубая задержка речевого развития, нечеткость артикуляции, резкая ограниченность словарного запаса, нарушение понимания обращенной речи. </p>\r\n<p>Степень интеллектуальных нарушений у ребенка с микроцефалией может варьировать от дебильности до идиотии. При нерезко выраженной умственной отсталости больные с микроцефалией могут быть обучаемы, способны к самообслуживанию и выполнению несложных поручений. Однако в большинстве случаев дети с микроцефалией требуют ухода, контроля и надзора со стороны взрослых. </p>\r\n<p>По особенностям темперамента дети с микроцефалией могут быть отнесены к торпидной или эретической группе. В первом случае детям свойственна малоподвижность, вялость, безучастность к окружающему, пассивно-подражательная деятельность; во втором случае – гиперактивность, суетливость, подвижность, неустойчивое внимание. Эмоциональная сфера у больных с микроцефалией остается относительно сохранной: дети приветливы, добродушны; реже - эмоционально неустойчивы и склонны к аффективным вспышкам. </p>",
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