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Характеризуется отсутствием передней стенки мочевого пузыря и передней брюшной стенки, что приводит к постоянному контакту мочи с окружающей кожей [1](https://kirsanovkids.ru/napravleniya/poroki-mochevidilitelnoy-sistemi/ekstrofiya-mochevogo-puzirya/)[9](https://yandex.ru/medicine/disease/ekstrofija-mochevogo-puzyrja). \n\nЧастота в популяции — 1 случай на 30–50 тысяч новорождённых [2](https://www.KrasotaiMedicina.ru/diseases/children/bladder-exstrophy)[9](https://yandex.ru/medicine/disease/ekstrofija-mochevogo-puzyrja). В большей степени страдают мальчики, распространённость патологии среди них в 2–6 раз выше, чем среди девочек [2](https://www.KrasotaiMedicina.ru/diseases/children/bladder-exstrophy)[9](https://yandex.ru/medicine/disease/ekstrofija-mochevogo-puzyrja).\n\n\n\n## Причины\nТочная причина экстрофии мочевого пузыря до конца не выяснена [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/). Выделяют несколько факторов риска [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/): \n* **Генетическая предрасположенность** — если в семье уже были случаи экстрофии или других пороков развития мочеполовой системы, риск рождения ребёнка с этой патологией возрастает [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/). \n* **Воздействие токсических веществ во время беременности** — курение, алкоголь, некоторые лекарственные препараты могут повысить вероятность развития экстрофии [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/). \n* **Инфекционные заболевания матери в первом триместре беременности** [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/).\n\n## Симптомы\nНекоторые симптомы экстрофии мочевого пузыря: \n* **Видимые аномалии при рождении** — открытый мочевой пузырь, выступающий через брюшную стенку, внутренняя поверхность мочевого пузыря видна снаружи тела [1](https://kirsanovkids.ru/napravleniya/poroki-mochevidilitelnoy-sistemi/ekstrofiya-mochevogo-puzirya/)[9](https://yandex.ru/medicine/disease/ekstrofija-mochevogo-puzyrja). \n* **Аномалии гениталий**: у мальчиков — расщепление полового члена и расположение мошонки ниже нормы, у девочек — расщепление клитора и расположение влагалища и уретры ниже нормы [1](https://kirsanovkids.ru/napravleniya/poroki-mochevidilitelnoy-sistemi/ekstrofiya-mochevogo-puzirya/)[9](https://yandex.ru/medicine/disease/ekstrofija-mochevogo-puzyrja). \n* **Проблемы с мочеиспусканием** — из-за отсутствия нормальной анатомии мочевого пузыря мочеиспускание может быть затруднено или неконтролируемым [1](https://kirsanovkids.ru/napravleniya/poroki-mochevidilitelnoy-sistemi/ekstrofiya-mochevogo-puzirya/)[9](https://yandex.ru/medicine/disease/ekstrofija-mochevogo-puzyrja). \n* **Возможные инфекции** — постоянное воздействие мочи на кожу может приводить к частым инфекциям и раздражению [1](https://kirsanovkids.ru/napravleniya/poroki-mochevidilitelnoy-sistemi/ekstrofiya-mochevogo-puzirya/)[9](https://yandex.ru/medicine/disease/ekstrofija-mochevogo-puzyrja).\n\n## Диагностика\nДиагностика экстрофии мочевого пузыря может начаться ещё до рождения ребёнка — современные методы пренатальной диагностики, такие как ультразвуковое исследование, позволяют заподозрить эту патологию уже во втором триместре беременности [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/). После рождения диагноз устанавливается на основании характерной клинической картины [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/). Дополнительные методы исследования, такие как рентгенография и МРТ, помогают оценить состояние костей таза и сопутствующих аномалий внутренних органов [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/).\n\n## Лечение\n**Единственный эффективный метод лечения** — **хирургическая коррекция** [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/). Обычно проводится в несколько этапов [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/): \n* **Первый этап** — в первые дни или недели жизни ребёнка, цель — закрыть мочевой пузырь и восстановить переднюю брюшную стенку [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/). \n* **Второй этап** — обычно проводится в возрасте 6–12 месяцев и направлен на коррекцию эписпадии у мальчиков или аномалий наружных половых органов у девочек [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/). \n* **Третий этап** — проводится в возрасте 4–5 лет, включает реконструкцию шейки мочевого пузыря для улучшения контроля мочеиспускания [8](https://www.phag-rostov.ru/articles/ekstrofiya-mochevogo-puzyrya-u-detej-prichiny-operatsiya/). \n\nРанняя хирургическая коррекция даёт наилучшие результаты, но даже после успешных операций многим детям требуется длительное наблюдение 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