Perdomo-Pantoja Alexander, Zakaria Hesham Mostafa, Judy Brendan F, Khalifeh Jawad M, Porras Jose L, Azad Tej D, Hwang Brian Y, Witham Timothy F, Bettegowda Chetan, Theodore Nicholas
Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
J Neurosurg Case Lessons. 2021 Dec 13;2(24):CASE21355. doi: 10.3171/CASE21355.
Intracranial deposits of fat droplets are an unusual presentation of a spinal dermoid cyst after spontaneous rupture and are even more uncommon after trauma. Here, the authors present a case with this rare clinical presentation, along with a systematic review of the literature to guide decision making in these patients.
A 54-year-old woman with Lynch syndrome presented with severe headache and sacrococcygeal pain after a traumatic fall. Computed tomography of the head revealed multifocal intraventricular and intracisternal fat deposits, which were confirmed by magnetic resonance imaging (MRI) of the neuroaxis; in addition, a ruptured multiloculated cyst was identified within the sacral canal with proteinaceous/hemorrhagic debris, most consistent with a sacral dermoid cyst with rupture into the cerebrospinal fluid (CSF) space. An unruptured sacral cyst was later noted on numerous previous MRI scans. In our systematic review, we identified 20 similar cases, most of which favored surgical treatment.
Rupture of an intraspinal dermoid cyst must be considered when intracranial fat deposits are found in the context of cauda equina syndrome, meningism, or hydrocephalus. Complete tumor removal with close postoperative follow-up is recommended to decrease the risk of complications. CSF diversion must be prioritized if life-threatening hydrocephalus is present.
脂肪滴的颅内沉积是脊髓皮样囊肿自发破裂后的一种不寻常表现,在创伤后更为罕见。在此,作者报告了一例具有这种罕见临床表现的病例,并对文献进行了系统回顾,以指导对这些患者的决策。
一名患有林奇综合征的54岁女性在一次外伤跌倒后出现严重头痛和骶尾部疼痛。头部计算机断层扫描显示脑室内和脑池内有多灶性脂肪沉积,神经轴磁共振成像(MRI)证实了这一点;此外,在骶管内发现一个破裂的多房囊肿,伴有蛋白质样/出血性碎屑,最符合骶部皮样囊肿破裂进入脑脊液(CSF)间隙。后来在之前的多次MRI扫描中发现了一个未破裂的骶部囊肿。在我们的系统回顾中,我们确定了20例类似病例,其中大多数倾向于手术治疗。
当在马尾综合征、脑膜刺激征或脑积水的背景下发现颅内脂肪沉积时,必须考虑脊髓皮样囊肿破裂。建议完整切除肿瘤并密切进行术后随访,以降低并发症风险。如果存在危及生命的脑积水,必须优先进行脑脊液分流。