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},
"code": "Q75.0",
"name": "Краниосиностоз",
"icd_name": "Краниосиностоз",
"gender": 0,
"age_min": 1,
"age_max": 10,
"cause": [
"0"
],
"periodicity": 1,
"slug": "q75.0_kraniosinostoz",
"lead": "преждевременное срастание у ребенка одного или нескольких швов, разделяющих черепные кости",
"description": "",
"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;\">Природа данного заболевания на сегодняшний день мало изучена. Но существует большое количество теорий возникновения краниостеноза. Теории говорят о таких причинах:</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: 6pt; 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;\">гормональные</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6pt; 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;\">внутриутробные</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6pt; 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;\">наследственные нарушения</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6pt; 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;\">сдавление головы плода в матке и пр.</span></p>\r\n</li>\r\n</ul>\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;\">Одним из факторов патогенеза официально признан медициной дефект в гене рецептора фактора роста фибробластов. Основные швы свода черепа:</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: 6pt; 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;\">коронарный</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6pt; 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;\">сагиттальный</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6pt; 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;\">метопический</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 6pt; 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;\">лямбдовидный</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;\">Если костный шов поражен, фиксируют компенсаторный рост костей перпендикулярно его оси. В результате этого процесса возникает характерная деформация – своя для каждого из замкнутых швов.</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;\">При обращении родителей ребенка с жалобами на диспропорциональный череп, беспокойство, тошноту, рвоту, врач проводит осмотр, при котором проводится краниометрия – измерение антропометрических показаний головы с помощью сантиметровой ленты. Обращает на себя внимание нарушение темпов роста черепа. Затем для уточнения состояния пациента требуется консультация невропатолога, офтальмолога, генетика. Параллельно с этим назначаются инструментальные исследования:</span></p>\r\n<p> </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: 8pt; font-family: Verdana; color: #141412; 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;\">Рентгенография черепа (краниография) является основным методом диагностики. При краниосиностозе выявляется деформация черепа, отсутствие одного или нескольких швов, диспропорция толщины свода черепа, а также признаки внутричерепной гипертензии – пальцевидные вдавления, усиленный сосудистый рисунок.</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #141412; 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;\">Осмотр глазного дна. При этом исследовании на глазном дне видны отек соска зрительного нерва и признаки венозного застоя.</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #141412; 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;\">Ультразвуковая функциональная венография. При краниосиностозе отмечается затруднение венозного оттока сосудов головного мозга и косвенные признаки внутричерепного повышенного давлении.</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #141412; 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;\">Электрокардиография, эхокардиография и ультразвуковое исследование внутренних органов проводится с целью выявления сопутствующих аномалий развития, а также как подготовка перед операцией для выявления общего состояния организма ребенка.</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #141412; 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;\">К современным методам исследования относится компьютерная томография, с помощью ее можно узнать об отсутствии какого-либо шва, наличии повышенного внутричерепного давления, различных компенсаторных изменений. Метод является очень точным и позволяет зарегистрировать минимальные отклонения.</span></p>\r\n</li>\r\n<li style=\"list-style-type: disc; font-size: 8pt; font-family: Verdana; color: #141412; 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;\">В сложных случаях краниосиностоза, при сочетанном поражении швов черепа показано выполнения ангиографии для выявления коллатеральных путей кровотока с целью уменьшения операционные риски.</span></p>\r\n</li>\r\n</ul>",
"treatment": "<p><span id=\"docs-internal-guid-54e7d665-7fff-5c5f-1473-68d104101a15\"><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Основное лечение краниосиностоза – хирургическая коррекция костной деформации черепа. Оптимальное время для проведения оперативного вмешательства – первые 6-9 месяцев жизни ребенка. Данные сроки обусловлены тем, что в этом периоде наблюдается наиболее интенсивное развитие тканей головного мозга, которому может препятствовать деформация черепной коробки. Кроме того, кости черепа в этом возрасте быстро восстанавливают свою структуру без развития осложнений. Объем и техника операции зависят от формы краниосиностоза и сопутствующих патологий. В 2-3-х летнем возрасте коррекция проводится исключительно с целью ликвидировать косметический дефект. Помимо хирургического лечения осуществляется изменение рациона ребенка в соответствии с возрастными требованиями. При развитии интеркуррентных заболеваний показана медикаментозная терапия.</span></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;\">Специфической профилактики для данной патологии не существует. Неспецифические меры подразумевают медико-генетическую консультацию семьи и планирование беременности, охрану здоровья женщины при вынашивании ребенка, рациональное питание, отказ от вредных привычек и исключение всех потенциальных этиологических факторов развития краниосиностоза.</span></p>",
"clinical_picture": "<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;\">Основным признаком краниосиностоза является деформированный череп, при этом голова принимает неестественную форму, отмечается ее чрезмерный рост в сторону, противоположную от места стеноза.</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;\">Затем в костную деформацию начинает вовлекаться и лицевая часть черепа, особенно страдает область глаз.</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;\">У детей проявляются признаки внутричерепной гипертензии. В младенческом возрасте она определяется по вынужденному положению головы, беспричинному крику ребенка, тошноте и рвоте.</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;\">При преждевременном закрытии нескольких черепных швов возникает гидроцефалия - нарушение оттока цереброспинальной жидкости из желудочков головного мозга. Признаками гидроцефалии является несоответствующий возрасту рост головы, напряженный выбухающий родничок, косоглазие.</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;\">Если в процесс вовлекается венечный шов, происходит деформация черепа в фронтально-орбитальной зоне, что приводит к быстро прогрессирующему ухудшению зрения.</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;\">При синдромных вариантах краниосиностоза из-за недоразвития верхней челюсти у детей часто отмечается нарушение дыхания, особенно ночью, а также задержка речевого развития.</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;\">Чем длительнее течет краниосиностоз, тем сильнее страдает функции коры головного мозга. Вначале дети не отстают в психомоторном развитии, но с течением времени начинают проявляться симптомы задержки, вплоть до утраты уже приобретенных навыков (зависит от варианта заболевания).</span></p>\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;\">Краниосиностоз может отмечаться и в детском возрасте 8 – 10 лет, тогда симптомы не так выражены, развивается компенсированная форма болезни, нуждающаяся в грамотной диагностике.</span></p>",
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