Schönberger Jan, Möhlenbruch Markus, Seitz Angelika, Bußmann Cornelia, Bächli Heidi, Kölker Stefan
Universitätsklinikum Heidelberg, Zentrum für Kinder- und Jugendmedizin, Klinik Kinderheilkunde I, Sektion für Neuropädiatrie und Stoffwechselmedizin, Germany.
Universitätsklinikum Heidelberg, Neurologische Klinik, Abteilung für Neuroradiologie, Germany.
Eur J Paediatr Neurol. 2017 Jul;21(4):678-681. doi: 10.1016/j.ejpn.2017.02.004. Epub 2017 Feb 20.
Spontaneous intracranial hypotension is a rarely diagnosed cause of headache, especially in children and adolescents. It is due to cerebrospinal fluid (CSF) leakage via spinal fistulae occurring without major trauma.
An adolescent patient presented with a 3-month history of strictly postural headache. Cranial magnetic resonance imaging (MRI) showed pronounced Chiari-like prolapse of the cerebellar tonsils, narrow ventricles and enlarged cerebral veins. On spinal MRI, myelographic sequences revealed a large collection of CSF around the first sacral roots. CT myelography proved extensive spinal CSF leakage. Hence, we applied epidural patches at multiple levels. Afterwards, symptoms and radiologic findings, including Chiari-like displacement, completely resolved.
A Chiari-like descent of the cerebellar tonsils alone does not secure the diagnosis of a Chiari I malformation. Especially if other findings indicate spinal CSF leakage, a systematic work-up should be initiated. In most cases, interventional techniques seal the leak successfully, resulting in a favorable outcome.
自发性颅内低压是一种罕见的头痛病因，在儿童和青少年中尤为如此。它是由于脑脊液（CSF）通过脊髓瘘管漏出，且无重大创伤。
一名青少年患者有3个月严格体位性头痛病史。头颅磁共振成像（MRI）显示小脑扁桃体明显的Chiari样脱垂、脑室狭窄和脑静脉扩张。脊髓MRI上，脊髓造影序列显示第一骶神经根周围有大量脑脊液聚集。CT脊髓造影证实存在广泛的脊髓脑脊液漏。因此，我们在多个节段应用了硬膜外贴片。之后，症状和影像学表现，包括Chiari样移位，完全消失。
单纯小脑扁桃体的Chiari样下移不能确诊为Chiari I型畸形。特别是当其他表现提示脊髓脑脊液漏时，应启动系统的检查。在大多数情况下，介入技术能成功封闭漏口，取得良好预后。
