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При этой патологии структуры головного мозга, расположенные в задней черепной ямке, опущены в каудальном направлении и выходят через большое затылочное отверстие [3](https://yandex.ru/medicine/disease/malformatsija-arnolda-kiari)[17](https://www.KrasotaiMedicina.ru/diseases/zabolevanija_neurology/chiari-malformation). Это может привести к сдавлению головного и спинного мозга, нарушая ток спинномозговой жидкости (ликвора) [3](https://yandex.ru/medicine/disease/malformatsija-arnolda-kiari)[6](https://www.invitro.ru/library/chem-boleyut-nashi-deti/54012/). \n\n**Типы аномалии** [3](https://yandex.ru/medicine/disease/malformatsija-arnolda-kiari): \n* **I тип**: миндалины мозжечка смещены в большое затылочное отверстие (реже незначительно смещён и продолговатый мозг) [3](https://yandex.ru/medicine/disease/malformatsija-arnolda-kiari)[8](https://gemotest.ru/info/spravochnik/zabolevaniya/anomaliya-arnolda-kiari/). \n* **II тип**: миндалины мозжечка, его нижняя часть, варолиев мост и растянутый четвёртый желудочек смещаются ниже плоскости большого затылочного отверстия, в направлении спинномозгового канала [3](https://yandex.ru/medicine/disease/malformatsija-arnolda-kiari)[8](https://gemotest.ru/info/spravochnik/zabolevaniya/anomaliya-arnolda-kiari/). \n* **III тип**: все структуры задней черепной ямки смещаются в большое затылочное отверстие [3](https://yandex.ru/medicine/disease/malformatsija-arnolda-kiari)[8](https://gemotest.ru/info/spravochnik/zabolevaniya/anomaliya-arnolda-kiari/). \n\n\n\n## Причины\nТочные причины аномалии до конца не изучены, но учёные сходятся во мнении, что это врождённый порок развития, заложенный ещё на этапе формирования нервной трубки у эмбриона (на 4–8 неделе беременности) [12](https://Megapteka.ru/specials/sindrom-arnolda-kiari-6088). Основная гипотеза — недоразвитие задней черепной ямки, из-за чего в ней не хватает места для мозжечка и ствола мозга [12](https://Megapteka.ru/specials/sindrom-arnolda-kiari-6088). \n\n## Симптомы\nВ большинстве случаев заболевание протекает бессимптомно и выявляется случайно при проведении КТ или МРТ головного мозга по поводу других заболеваний [6](https://www.invitro.ru/library/chem-boleyut-nashi-deti/54012/)[7](https://ProBolezny.ru/anomaliya-arnolda-kiari/). Однако возможны некоторые проявления: \n* боли в шейно-затылочной области, усиливающиеся при повороте головы, кашле и натуживании [1](https://www.neurology.ru/o-centre/struktura/neurosurgery/lechebnaya-rabota/anomaliya-arnolda-kiari/); \n* головные боли [1](https://www.neurology.ru/o-centre/struktura/neurosurgery/lechebnaya-rabota/anomaliya-arnolda-kiari/); \n* снижение остроты зрения [1](https://www.neurology.ru/o-centre/struktura/neurosurgery/lechebnaya-rabota/anomaliya-arnolda-kiari/); \n* шаткость походки, неустойчивость при движении, частые падения [1](https://www.neurology.ru/o-centre/struktura/neurosurgery/lechebnaya-rabota/anomaliya-arnolda-kiari/); \n* нарушение глубокой чувствительности (например, человек с закрытыми глазами не ощущает, в каком положении у него лежат руки) [1](https://www.neurology.ru/o-centre/struktura/neurosurgery/lechebnaya-rabota/anomaliya-arnolda-kiari/); \n* снижение болевой и температурной чувствительности верхних конечностей [1](https://www.neurology.ru/o-centre/struktura/neurosurgery/lechebnaya-rabota/anomaliya-arnolda-kiari/); \n* нарушения дыхания (вплоть до остановки) — в тяжёлых случаях [2](https://fcn-tmn.ru/nozologi/detskoe-otdelenie/anomaliya-arnolda-kiari/). \n\n## Диагностика\n**Стандартным методом диагностики** является магнитно-резонансная томография (МРТ) головного мозга [1](https://www.neurology.ru/o-centre/struktura/neurosurgery/lechebnaya-rabota/anomaliya-arnolda-kiari/)[14](https://MedAboutMe.ru/zdorove/spravochnik/bolezni/malformatsiya_arnolda_kiari/). МРТ позволяет обнаружить характерное смещение миндалин мозжечка, а также наличие или отсутствие другой сопутствующей патологии головного мозга (сирингомиелия, гидроцефалия, опухоли и др.) [14](https://MedAboutMe.ru/zdorove/spravochnik/bolezni/malformatsiya_arnolda_kiari/). \n\n## Лечение\n**Бессимптомно протекающая аномалия** не нуждается в лечении [17](https://www.KrasotaiMedicina.ru/diseases/zabolevanija_neurology/chiari-malformation). **Если аномалия сопровождается неврологическими нарушениями** (парезы, расстройства чувствительности и мышечного тонуса, нарушения со стороны черепно-мозговых нервов и пр.) или не поддающимся консервативной терапии болевым синдромом, показано хирургическое лечение [17](https://www.KrasotaiMedicina.ru/diseases/zabolevanija_neurology/chiari-malformation). \n\n**Некоторые методы хирургического лечения**: \n* **Краниовертебральная декомпрессия** — расширение затылочного отверстия за счёт удаления части затылочной кости, ликвидация сдавления ствола и спинного мозга за счёт резекции миндалин мозжечка и задних половин двух первых шейных позвонков [17](https://www.KrasotaiMedicina.ru/diseases/zabolevanija_neurology/chiari-malformation). \n* **Шунтирующие операции** — дренирование цереброспинальной жидкости из расширенного центрального канала спинного мозга [17](https://www.KrasotaiMedicina.ru/diseases/zabolevanija_neurology/chiari-malformation). 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