neurosurgeryНейрохирургияRussian journal of neurosurgery1683-32952587-7569Издательский дом "МедИНК"10.17650/1683-3295-2024-26-4-65-74neurosurgery-1611Research ArticleНАБЛЮДЕНИЕ ИЗ ПРАКТИКИFROM PRACTICEПриобретенная мальформация Киари 1-го типа (псевдомальформация Киари) и синдром внутричерепной гипотензии: клиническое наблюдение и обзор литературыAcquired Chiari malformation type I (pseudo-Chiari malformation) and intracranial hypotension: Clinical observation and literature reviewhttps://orcid.org/0000-0002-3007-6909ГовенькоФ. С.Goven’koF. S.

Федор Стефанович Говенько

194044; ул. Академика Лебедева, 6; 193312; пр. Солидарности, 4; Санкт-Петербург

Fedor Stefanovich Goven’ko

6 Akademika Lebedeva St., Saint Petersburg 194044 4 Solidarnosty Ave., St. Petersburg 193312

fyodor.govenko@yandex.ruhttps://orcid.org/0000-0002-8459-2466МартыновБ. В.MartynovB. V.

194044; ул. Академика Лебедева, 6; Санкт-Петербург

194044; 6 Akademika Lebedeva St.; Saint Petersburg

https://orcid.org/0000-0003-0910-6904ГрищенковА. С.GrishchenkovA. S.

194044; ул. Академика Лебедева, 6; Санкт-Петербург

194044; 6 Akademika Lebedeva St.; Saint Petersburg

ФГБВОУ ВО «Военно-медицинская академия им. С. М. Кирова» Минобороны России; СПб ГБУЗ «Александровская больница»РоссияS. M. Kirov Military Medical Academy, Ministry of Defense of Russia; Alexandrovskaya HospitalRussian FederationФГБВОУ ВО «Военно-медицинская академия им. С. М. Кирова» Минобороны РоссииРоссияS. M. Kirov Military Medical Academy, Ministry of Defense of RussiaRussian Federation2024240120252646574Copyright © Говенько Ф.С., Мартынов Б.В., Грищенков А.С., 20252025Говенько Ф.С., Мартынов Б.В., Грищенков А.С.Goven’ko F.S., Martynov B.V., Grishchenkov A.S.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.therjn.com/jour/article/view/1611

   Одно из осложнений скрытой внутричерепной гипотензии – дистопия миндалин мозжечка, которая может привести к постановке ошибочного диагноза «мальформация Киари 1-го типа».

   Цель работы – обратить внимание врачей на симптомы, течение и диагностику приобретенной мальформации (псевдомальформации) Киари при синдроме внутричерепной гипотензии.

   Работа основана на анализе клинико-неврологических данных и данных магнитно-резонансной томографии головного и спинного мозга женщины 28 лет, полученных при динамическом наблюдении на протяжении ряда лет. При остром начале заболевания с сильной постуральной головной боли, минимальных неврологических симптомах по данным магнитно-резонансной томографии выявлены нарастающая в динамике дистопия миндалин мозжечка с уровня плоскости большого затылочного отверстия до уровня на 15 мм ниже ее и стабильная гидромиелия в грудном отделе, что склоняло к постановке диагноза мальформации Киари 1-го типа. Однако уплощение моста, утолщение твердой мозговой оболочки, тенденция к округлению сагиттального синуса, сужение субарахноидальных пространств, базальных цистерн, пролабирование дна III желудочка в предмостовую цистерну свидетельствовали о возможности внутричерепной гипотензии, обусловленной скрытой ликвореей, что нашло подтверждение при магнитно-резонансной томографии с внутривенным введением гадолинийсодержащего контрастного вещества в режиме сильно взвешенного Т2-трехмерного изображения с инверсией и ослаблением жидкости – выявлена скрытая ликворея на шейном уровне. Установлен диагноз псевдомальформации Киари, который исключает хирургическое вмешательство – декомпрессию задней черепной ямки. При диагностике мальформации Киари 1-го типа и выявлении дистопии миндалин мозжечка всегда следует исключать псевдомальформацию Киари, связанную с иной первичной патологией, в частности со скрытой цереброспинальной ликвореей, следствием которой является внутричерепная гипотензия, которая имеет специфические магнитно-резонансные признаки и требует иной лечебной тактики.

   One of the complications of latent intracranial hypotension is a dystopia of the tonsils of the cerebellum, which can lead to an erroneous diagnosis of “Chiari malformation type 1”.

   The aim of the work is to pay attention to the symptoms, course and diagnosis of acquired (pseudomalformation) Chiari in intracranial hypotension syndrome.

   The work is based on clinical and neurological data and magnetic resonance imaging of the brain and spinal cord of a woman aged 28 years, obtained during dynamic observation over a number of years. At the acute onset of the disease with severe postural headache, minimal neurological symptoms, magnetic resonance imaging revealed a dynamic dystopia of the cerebellar tonsils from the plane of the large occipital foramen 15 mm below it and stable hydromyelia in the thoracic region, which inclined to the diagnosis of Chiari malformation type 1. However, flattening of the bridge, thickening of the dura mater, the tendency to rounding of the sagittal sinus, narrowing of subarachnoid spaces, basal cisterns, prolapse of the bottom of the 3rd ventricle into the prebridge cistern indicated the possibility of intracranial hypotension caused by latent liquorrhea, which was confirmed by magnetic resonance imaging with intravenous administration of gadolinium containing contrast agent in the mode of a strongly weighted T2-three-dimensional image with inversion and weakening of fluid – revealed hidden liquorrhea at the cervical level. A diagnosis of Chiari pseudomalformation has been established, which excludes surgical intervention – decompression of the posterior cranial pit. When diagnosing Chiari malformation type 1 and detecting cerebellar tonsillar dystopia, it is always necessary to exclude Chiari pseudomalformation associated with other primary pathology, in particular, with latent cerebrospinal liquor, the consequence of which is intracranial hypotension, which has specific signs in magnetic resonance imaging, and requires a different therapeutic tactic.

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The authors declare that there are no conflicts of interest present.