Thakar Sumit, Dadlani Ravi, Tawari Manish, Hegde Alangar S
Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences.
Neurol Med Chir (Tokyo). 2014;54(7):567-71. doi: 10.2176/nmc.cr2013-0066. Epub 2013 Nov 20.
Symptomatic cerebellar slump (CS) and external hydrocephalus (EH) are amongst the rarer complications of foramen magnum decompression (FMD) for Chiari I malformation (CM). CS typically presents with delayed onset headache related to dural traction or with neurological deficit offsetting the benefit of FMD. EH, consisting of ventriculomegaly along with subdural fluid collection(s) (SFCs), has been related to cerebrospinal fluid egress from a tiny breach in an otherwise intact arachnoid. We describe the case of a 21-year-old man with CM and syringomyelia who presented with impaired gag, spastic quadriparesis, and raised intracranial pressure 1 week following an uneventful FMD during which the arachnoid had been widely fenestrated. Magnetic resonance imaging (MRI) showed an infratentorial SFC, dilated aqueduct and triventriculomegaly, features of CS, and a residual but resolving syrinx. His symptoms resolved following a high pressure ventriculo-peritoneal shunt. At a 6-month follow-up visit, he was asymptomatic and demonstrated partial resolution of the syrinx, with no recurrence of the SFC. The unusual features in the clinical course of this patient were an atypical CS syndrome presenting with concomitantly resolving syringomyelia, and the development of EH after a wide arachnoidal fenestration. This is the first case in indexed literature describing such a combination of unusual postoperative complications of a FMD. A hypothesis is presented to explain the clinico-radiological findings of the case.
症状性小脑下垂(CS)和外部性脑积水(EH)是 Chiari I 畸形(CM)枕大孔减压术(FMD)较为罕见的并发症。CS 通常表现为与硬脑膜牵引相关的迟发性头痛或出现神经功能缺损,抵消了 FMD 的益处。EH 由脑室扩大以及硬膜下积液(SFC)组成,与脑脊液从原本完整的蛛网膜微小裂口中流出有关。我们描述了一名 21 岁患有 CM 和脊髓空洞症的男性病例,他在一次顺利的 FMD 术后 1 周出现吞咽反射受损、痉挛性四肢瘫和颅内压升高,此次手术中蛛网膜已被广泛开窗。磁共振成像(MRI)显示幕下 SFC、导水管扩张和三脑室扩大、CS 的特征以及一个残留但正在消退的脊髓空洞。在进行高压脑室 - 腹腔分流术后,他的症状得到缓解。在 6 个月的随访中,他无症状,脊髓空洞部分消退,SFC 未复发。该患者临床过程中的不寻常特征是出现非典型 CS 综合征且脊髓空洞症同时消退,以及在蛛网膜广泛开窗后发生 EH。这是索引文献中首例描述 FMD 这种不寻常术后并发症组合的病例。本文提出了一个假说来解释该病例的临床 - 放射学表现。