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"etiology": "<p>К причинам врожденной гидроцефалии относятся пороки развития ликворной системы : </p>\r\n<ul>\r\n<li>атрезия отверстий, соединяющих желудочки мозга (Мажанди и Люшка); </li>\r\n<li>дефекты в строении подпаутинного пространства; </li>\r\n<li>сужение (стеноз) сильвиевого водопровода; </li>\r\n<li>краниовертебральные аномалии (аномалии границы между основанием черепа и верхнешейным отделом позвоночника) - аномалия Киари, врожденная базилярная импрессия. </li>\r\n</ul>\r\n<p>Причинами врожденной гидроцефалии могут также быть внутриутробные инфекции (токсоплазмоз, врожденный сифилис, цитомегалия, краснуха), родовая травма. </p>",
"pathogenesis": "<p>Традиционно модель гидроцефалии трактуется как дисбаланс между продуцированием и абсорбцией черепно-мозговой жидкости. Современные исследования показывают, что основная роль определяется абсорбцией капиллярами мозга. Гемодинамический патогенез гидроцефалии (первичная и вторичная) определяется нарушением венозного оттока (обструктивная гидроцефалия) и уменьшением артериальной пульсации (сообщающаяся гидроцефалия).</p>",
"diagnostics": "<p>Гидроцефалия может быть выявлена у плода в перриод внутриутробного развития при проведении скрининга беременной женщины. УЗИ плода позволяет обнаружить гидроцефалию у ребенка на 16–20 неделе беременности. После рождения у некоторых детей гидроцефалия выявляется сразу в родильном доме специалистом-неонатологом. </p>\r\n<p>Трансиллюминация (диафаноскопия) черепа позволяет выявить наружную гидроцефалию у детей, исключить гидроанэнцефалию и субдуральную гигрому. Суть его в просвечивании головы пучком света от лампы в затемнённом помещении. В их лучах при гидроцефалии начинает светиться вся голова. </p>\r\n<p>Рентгенография черепа – выявляют истончение костной ткани, расхождение швов черепа, на внутренней поверхности черепной коробки присутствует характерный симптом “пальцевидных вдавлений”. </p>\r\n<p>Магнитно-резонансная и компьютерная томография позволяют с большой точностью визуализировать патологические изменения внутри черепа, выявить место, которое препятствует оттоку ликвора. </p>\r\n<p>Ультрасонография – ультразвуковое исследование головного мозга через открытый большой родничок. Позволяет определить степень повышения внутричерепного давления. </p>\r\n<p>Офтальмоскопия – осмотр глазного дна. Выявляют застойные диски зрительных нервов, как следствие повышенного внутричерепного давления. </p>\r\n<p>Для оценки давления цереброспинальной жидкости, исследования ее состава и проведения ликвородинамических проб прибегают к вентрикулярной и люмбальной пункциям. </p>",
"treatment": "<p>Основными принципами лечения гидроцефалии у детей является снижение продукции спиномозговой жидкости и нормализация ее циркуляции по ликвороносным путям, в т. ч. с помощью создания обходных анастомозов. </p>\r\n<p>С целью снижения продукции ликвора и внутричерепного давления проводится дегидратационная терапия диуретиками, ингибиторами карбоангидразы, салуретиками. При прогрессирующем нарастании гидроцефалии у детей в течение 2—3-х месяцев ставится вопрос о хирургическом вмешательстве. </p>\r\n<p>Оптимальный способ оперативного вмешательства определяется этиологией, формой, стадией гидроцефалии у детей. В настоящее время все виды операций при гидроцефалии у детей могут быть поделены на 5 групп: </p>\r\n<ul>\r\n<li>паллиативные вмешательства, позволяющие временно снизить внутричерепное давление и устранить краниоцеребральную диспропорцию (люмбальные и вентрикулярные пункции, наружное вентрикулярное дренирование) </li>\r\n<li>операции, направленные на подавление ликворопродукции (клипирование, коагуляция сосудистого сплетения) </li>\r\n<li>операции, направленные на восстановление естественных путей циркуляции ликвора или создание новых путей ликворооттока (удаление опухолей, внутричерепных гематом , вентрикулостомия) </li>\r\n<li>операции, активизирующие ликворосорбцию (рассечение арахноидальных спаек и др.) </li>\r\n<li>шунтирующие операции, предусматривающие отведение ликвора в другие системы организма (вентрикулоперитонеальное шунтирование, люмбоперитонеальное шунтирование, вентрикулоатриарное шунтирование, вентрикулоцистерностомия, кистовентрикулоперитонеостомия, субдуроперитонеостомия). При невозможности выполнения стандартных шунтриующих операций проводятся атипичные шунтирующие вмешательства - вентрикулоплевральное, вентрикулоуретральное вентрикулобиллиарное шунтирование. Ликворошунтирующие операции при гидроцефалии у детей сопряжены с риском нарушения функционирования дренажных систем, гипердренированием или осложнениями воспалительного характера. </li>\r\n</ul>",
"prevention": "<p>Чтобы предупредить развитие врожденной гидроцефалии необходимо: </p>\r\n<ul>\r\n<li>Защищаться от инфекционных заболеваний во время беременности </li>\r\n<li>Не принимать никаких медикаментов во время беременности, кроме тех, которые прописал врач </li>\r\n<li>Профилактическое плановое УЗИ-обследование беременных женщин и нейроультрасонография у младенцев. </li>\r\n</ul>",
"clinical_picture": "<p>У детей из-за большой податливости костей черепа не наблюдается повышения внутричерепного давления, гидроцефалия у них сопровождается увеличением размеров черепа. У новорожденных и детей раннего возраста гидроцефалия характеризуется слишком большим размером головы, выбуханием вен скальпа, напряжением и отсутствием пульсации большого родничка, отеком дисков зрительных нервов. Часто отмечается симптом «заходящего солнца» - ограничение движений глазных яблок кверху. Может наблюдаться расхождение швов черепа. Постукивание по черепу сопровождается характерным звуком (симптом «треснутого горшка»). У детей первого года жизни гидроцефалия приводит к отставанию в физическом и умственном развитии. Они позже начинают держать голову, переворачиваться, сидеть и ходить. </p>\r\n<p>Дети, у которых имеется выраженная гидроцефалия, отличаются шарообразной формой головы, ее слишком большим размером, глубоко посаженными глазами, оттопыренными ушами, истончением кожи головы. Может отмечаться снижение зрения, повышение мышечного тонуса в нижних конечностях, нарушения со стороны черепных нервов. Дети, страдающие гидроцефалией, малоподвижны, не могут следить глазами за родителями. Они раздражены, часто плачут, постоянно сонливы, плохо едят. Их часто тошнит. </p>",
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"code": "Q03",
"name": "Врожденная гидроцефалия",
"icd_name": "Врожденная гидроцефалия",
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"age_min": 0,
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"slug": "q03_vrozhdennaya_gidrocefaliya",
"lead": "Нарушение здоровья, относящееся к группе врожденные аномалии [пороки развития] нервной системы",
"description": " \r\nВрожденная гидроцефалия – это патологическое состояние, которое сопровождается накоплением спинномозговой жидкости в желудочках мозга из-за ее избыточной продукции, нарушения всасывания или препятствие на пути оттока. ",
"etiology": "<p>К причинам врожденной гидроцефалии относятся пороки развития ликворной системы : </p>\r\n<ul>\r\n<li>атрезия отверстий, соединяющих желудочки мозга (Мажанди и Люшка); </li>\r\n<li>дефекты в строении подпаутинного пространства; </li>\r\n<li>сужение (стеноз) сильвиевого водопровода; </li>\r\n<li>краниовертебральные аномалии (аномалии границы между основанием черепа и верхнешейным отделом позвоночника) - аномалия Киари, врожденная базилярная импрессия. </li>\r\n</ul>\r\n<p>Причинами врожденной гидроцефалии могут также быть внутриутробные инфекции (токсоплазмоз, врожденный сифилис, цитомегалия, краснуха), родовая травма. </p>",
"pathogenesis": "<p>Традиционно модель гидроцефалии трактуется как дисбаланс между продуцированием и абсорбцией черепно-мозговой жидкости. Современные исследования показывают, что основная роль определяется абсорбцией капиллярами мозга. Гемодинамический патогенез гидроцефалии (первичная и вторичная) определяется нарушением венозного оттока (обструктивная гидроцефалия) и уменьшением артериальной пульсации (сообщающаяся гидроцефалия).</p>",
"diagnostics": "<p>Гидроцефалия может быть выявлена у плода в перриод внутриутробного развития при проведении скрининга беременной женщины. УЗИ плода позволяет обнаружить гидроцефалию у ребенка на 16–20 неделе беременности. После рождения у некоторых детей гидроцефалия выявляется сразу в родильном доме специалистом-неонатологом. </p>\r\n<p>Трансиллюминация (диафаноскопия) черепа позволяет выявить наружную гидроцефалию у детей, исключить гидроанэнцефалию и субдуральную гигрому. Суть его в просвечивании головы пучком света от лампы в затемнённом помещении. В их лучах при гидроцефалии начинает светиться вся голова. </p>\r\n<p>Рентгенография черепа – выявляют истончение костной ткани, расхождение швов черепа, на внутренней поверхности черепной коробки присутствует характерный симптом “пальцевидных вдавлений”. </p>\r\n<p>Магнитно-резонансная и компьютерная томография позволяют с большой точностью визуализировать патологические изменения внутри черепа, выявить место, которое препятствует оттоку ликвора. </p>\r\n<p>Ультрасонография – ультразвуковое исследование головного мозга через открытый большой родничок. Позволяет определить степень повышения внутричерепного давления. </p>\r\n<p>Офтальмоскопия – осмотр глазного дна. Выявляют застойные диски зрительных нервов, как следствие повышенного внутричерепного давления. </p>\r\n<p>Для оценки давления цереброспинальной жидкости, исследования ее состава и проведения ликвородинамических проб прибегают к вентрикулярной и люмбальной пункциям. </p>",
"treatment": "<p>Основными принципами лечения гидроцефалии у детей является снижение продукции спиномозговой жидкости и нормализация ее циркуляции по ликвороносным путям, в т. ч. с помощью создания обходных анастомозов. </p>\r\n<p>С целью снижения продукции ликвора и внутричерепного давления проводится дегидратационная терапия диуретиками, ингибиторами карбоангидразы, салуретиками. При прогрессирующем нарастании гидроцефалии у детей в течение 2—3-х месяцев ставится вопрос о хирургическом вмешательстве. </p>\r\n<p>Оптимальный способ оперативного вмешательства определяется этиологией, формой, стадией гидроцефалии у детей. В настоящее время все виды операций при гидроцефалии у детей могут быть поделены на 5 групп: </p>\r\n<ul>\r\n<li>паллиативные вмешательства, позволяющие временно снизить внутричерепное давление и устранить краниоцеребральную диспропорцию (люмбальные и вентрикулярные пункции, наружное вентрикулярное дренирование) </li>\r\n<li>операции, направленные на подавление ликворопродукции (клипирование, коагуляция сосудистого сплетения) </li>\r\n<li>операции, направленные на восстановление естественных путей циркуляции ликвора или создание новых путей ликворооттока (удаление опухолей, внутричерепных гематом , вентрикулостомия) </li>\r\n<li>операции, активизирующие ликворосорбцию (рассечение арахноидальных спаек и др.) </li>\r\n<li>шунтирующие операции, предусматривающие отведение ликвора в другие системы организма (вентрикулоперитонеальное шунтирование, люмбоперитонеальное шунтирование, вентрикулоатриарное шунтирование, вентрикулоцистерностомия, кистовентрикулоперитонеостомия, субдуроперитонеостомия). При невозможности выполнения стандартных шунтриующих операций проводятся атипичные шунтирующие вмешательства - вентрикулоплевральное, вентрикулоуретральное вентрикулобиллиарное шунтирование. Ликворошунтирующие операции при гидроцефалии у детей сопряжены с риском нарушения функционирования дренажных систем, гипердренированием или осложнениями воспалительного характера. </li>\r\n</ul>",
"prevention": "<p>Чтобы предупредить развитие врожденной гидроцефалии необходимо: </p>\r\n<ul>\r\n<li>Защищаться от инфекционных заболеваний во время беременности </li>\r\n<li>Не принимать никаких медикаментов во время беременности, кроме тех, которые прописал врач </li>\r\n<li>Профилактическое плановое УЗИ-обследование беременных женщин и нейроультрасонография у младенцев. </li>\r\n</ul>",
"clinical_picture": "<p>У детей из-за большой податливости костей черепа не наблюдается повышения внутричерепного давления, гидроцефалия у них сопровождается увеличением размеров черепа. У новорожденных и детей раннего возраста гидроцефалия характеризуется слишком большим размером головы, выбуханием вен скальпа, напряжением и отсутствием пульсации большого родничка, отеком дисков зрительных нервов. Часто отмечается симптом «заходящего солнца» - ограничение движений глазных яблок кверху. Может наблюдаться расхождение швов черепа. Постукивание по черепу сопровождается характерным звуком (симптом «треснутого горшка»). У детей первого года жизни гидроцефалия приводит к отставанию в физическом и умственном развитии. Они позже начинают держать голову, переворачиваться, сидеть и ходить. </p>\r\n<p>Дети, у которых имеется выраженная гидроцефалия, отличаются шарообразной формой головы, ее слишком большим размером, глубоко посаженными глазами, оттопыренными ушами, истончением кожи головы. Может отмечаться снижение зрения, повышение мышечного тонуса в нижних конечностях, нарушения со стороны черепных нервов. Дети, страдающие гидроцефалией, малоподвижны, не могут следить глазами за родителями. Они раздражены, часто плачут, постоянно сонливы, плохо едят. Их часто тошнит. </p>",
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},
"code": "Q54",
"name": "Гипоспадия",
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3
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"lead": "Нарушение здоровья, относящееся к группе врожденные аномалии [пороки развития] половых органов",
"description": "Гипоспадия — это врожденное нарушение анатомического строения мочеиспускательного канала, при котором его отверстие открывается не в типичном месте (то есть на головке), а на задней поверхности полового члена, в промежности или на мошонке. ",
"etiology": "<p> К возможным причинам развития гипоспадии относятся: </p>\r\n<ul>\r\n<li>нарушение внутриутробного развития плода на 7-15 неделях, которое возникает из-за различных факторов: </li>\r\n<li>воздействие неблагоприятных факторов окружающей среды: курение, алкоголь, некоторые лекарственные препараты, неполноценное питание (недостаток белков, витаминов); </li>\r\n<li>резкие гормональные колебания в организме матери во время беременности, которые часто возникают из-за стрессов, нервных перегрузок, отсутствия режима труда и отдыха; </li>\r\n<li>многоплодная беременность; </li>\r\n<li>частые беременности матери; </li>\r\n<li>внутриутробные инфекции плода; </li>\r\n<li>наследственные причины (генетические нарушения). </li>\r\n</ul>",
"pathogenesis": "<p>Эмбриогенез уретры представляет собой сложный процесс, включающий закладку и формирование уретральной площадки и уретральной борозды, превращение ее в первичную уретральную трубку, редукцию заднего сегмента первичной уретральной трубки и развитие кавернозного тела уретры. </p>\r\n<p>Разделение указанных этапов формирования уретры во времени делает возможным возникновение аномалий, обусловленных нарушением развития на одном из этапов или всего сложного процесса формирования и эволюции уретральной трубки. Гипоспадия с резким недоразвитием свободной стволовой части полового члена возникает в тех случаях, когда прекращается не только замыкание уретры, но и ее дальнейшая эволюция (рассасывание первичной уретральной площадки, формирование, и рост кавернозного тела уретры). </p>\r\n<p>Это приводит к тому, что в уретре и дистальнее ее остаются эмбриональные тяжи, неспособные к росту, которые замещаются грубой рубцовой тканью. Гипоспадия полового члена с хорошо развитой стволовой частью и незначительными рубцами на волярной поверхности возникает вследствие остановки замыкания уретрального желоба, но продолжающаяся эволюция уретры, рассасывание дорсального сегмента уретральной трубки и не замкнутой уретральной площадки обеспечивают рост уретры и свободного участка полового члена по длине без существенного его искривления. </p>\r\n<p>Патогенез «гипоспадии без гипоспадии» объясняется тем, что при этой аномалии развития после формирования уретры приостанавливается ее дальнейшая эволюция: не проходят редукция заднего сегмента первичной уретральной трубки, развитие поверхностного тела уретры, а вместе с тем и рост ее по длине, что вызывает искривление полового члена. </p>\r\n<p> </p>",
"diagnostics": "<p>Диагностика гипоспадии включает: </p>\r\n<p>Осмотр наружных половых органов: </p>\r\n<ul>\r\n<li>местоположение наружного отверстия уретры (на теле полового члена, на мошонке и т.п.); </li>\r\n<li>выделение мочи во время мочеиспускания из нетипичного места (не из отверстия мочеиспускательного канала на головке полового члена); </li>\r\n<li>отсутствие единой струи мочи или ее ослабление. </li>\r\n</ul>\r\n<p>Исследование хромосомного набора человека (для определения генетического пола ребенка): исследуют хромосомы клеток (структуры клеток, содержащие ДНК – хранилище генов), определяют половой хроматин (специальное вещество, отличающееся по составу у мальчиков и девочек). </p>\r\n<p>УЗИ (ультразвуковое исследование) почек: для исключения врожденных заболеваний почек и мочевых путей. </p>\r\n<p>Цистоуретрография: суть метода в изучении строения уретры и мочевого пузыря. Для этого через катетер (тонкая трубочка), введенный в мочеиспускательный канал, в мочевой пузырь вводится рентгенконтрастное вещество (оно видно на рентгеновских пленках), и выполняется несколько снимков. </p>\r\n<p>Внутривенная урография: суть метода в изучении строения почек и мочевыводящих путей. Для этого внутривенно вводится рентгенконтрастное вещество (оно видно на рентгеновских пленках) и выполняется несколько рентген-снимков почек и мочевого пузыря. </p>",
"treatment": "<p>Лечение хирургическое. Предпочтительно в возрасте 6-18 месяцев, а при головчатой гипоспадии до 3 лет. </p>\r\n<p>Более легкие формы гипоспадии (головчатая, венечная, дистальная стволовая) лечатся с использованием одноэтапных операций. Сложные варианты (стволовая с выраженным искривлением, мошоночная, промежностная) лучше лечить в два, а иногда и в три этапа. </p>\r\n<p>Процесс хирургического лечения гипоспадии состоит из нескольких этапов: </p>\r\n<ol>\r\n<li>Выпрямление полового члена и придание ему естественного вида;</li>\r\n<li>Формирование уретры из эластичного пластического материала лишенного волосяных луковиц;</li>\r\n<li>Формирование головки полового члена;</li>\r\n<li>Выведение уретры на верхушку головки с формированием наружного отверстия уретры нормального расположения и формы;</li>\r\n<li>Устранение транспозиции и расщепления мошонки.</li>\r\n</ol>",
"prevention": "<p>Профилактика гипоспадии включает:</p>\r\n<ul>\r\n<li>Исключение или снижение влияния вредных факторов на организм беременной женщины (курение, алкоголь, необоснованный прием лекарственных препаратов, психоэмоциональных нагрузок (стрессов) и т.д.). </li>\r\n<li>Регулярное посещение женщиной акушера-гинеколога во время беременности, своевременная постановка ее на учет в женской консультации (до 12 недели беременности). </li>\r\n<li>Своевременная подготовка к планируемой беременности. </li>\r\n<li>Своевременное обращение к урологу при выявлении у мальчика гипоспадии – до 6 -тимесячного возраста. </li>\r\n</ul>",
"clinical_picture": "<p>Головчатая форма гипоспадии встречается в 75% случаев и является самой легкой и наиболее частой формой порока. Наружное отверстие уретры расположено низко, обычно сужено (меатостеноз), что затрудняет мочеиспускание. Может отмечаться искривление полового члена, усиливающееся с началом половой жизни </p>\r\n<p>Венечная форма гипоспадии (отверстие уретры расположено по краю головки полового члена) сопровождается нарушением мочеиспускания и выраженным искривлением полового члена. Моча выделяется тонкой струйкой, с усилиями; ребенок постоянно мочится себе на ноги, что заставляет его во время мочеиспускания приподнимать пенис вверх. </p>\r\n<p>Стволовая форма гипоспадии может иметь несколько вариантов, поскольку отверстие уретры может быть расположен на разных уровнях задней поверхности полового члена. Мочеиспускание по мужскому типу (стоя) сильно затруднено: дети вынуждены мочиться сидя или подтягивая половой член вверх, к животу. Значительно выражена деформация полового члена, отмечается болезненность эрекций. Половая жизнь при данной форме гипоспадии возможна, однако, если наружное отверстие уретры расположено ближе к основанию пениса, то при эякуляции сперма не попадает во влагалище. </p>\r\n<p>Мошоночная форма гипоспадии является наиболее тяжелым проявлением патологии. Наружное отверстие уретры открывается на мошонке, расщепляя ее на 2 части. Половой член резко недоразвит и искривлен, напоминает гипертрофированный клитор; мошонка похожа по виду на большие половые губы. При рождении мальчики с данной формой гипоспадии могут быть приняты за девочек с адреногенитальным синдромом (врожденной гиперплазии надпочечников). Мочеиспускание при мошоночной форме гипоспадии может осуществляться только сидя; вследствие недоразвития и деформации пениса половая жизнь становится невозможной. Раздражение кожи мошонки мочой вызывает покраснение и воспаление. </p>\r\n<p>Промежностная форма гипоспадии характеризуется расположением отверстия уретры позади мошонки. У больных определяется малый половой член, расщепление мошонки, что нередко затрудняет определение половой принадлежности ребенка. Промежностная и мошоночная формы гипоспадии чаще, чем другие, сочетаются с крипторхизмом (неопущением яичка), паховой грыжей, водянкой оболочек яичка. </p>\r\n<p>Гипоспадия без гипоспадии: мочеиспускательный канал недоразвит и короче, чем половой член. При этом наружное отверстие уретры открывается в типичном месте – на кончике головки полового члена, но за счет разницы длины уретры и полового члена (он длиннее, чем уретра) происходит искривление пениса. При эрекции пенис выгибается в форме лука, что сопровождается болезненностью, затрудняет или делает невозможным половой акт. </p>\r\n<p>Женская гипоспадия характеризуется влагалищной эктопией наружного отверстия уретры и сопровождается рецидивирующими инфекциями мочевыводящих путей (уретритами и циститами), вульвитами и вульвовагинитами, нередко - признаками гермафродитизма и псевдогермафродитизма. </p>",
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