Girishan Shabari, Rajshekhar Vedantam
Department of Neurological Sciences, Christian Medical College, Vellore, India.
J Neurosurg Pediatr. 2016 Jan;17(1):86-93. doi: 10.3171/2015.5.PEDS1537. Epub 2015 Oct 2.
OBJECT Intramedullary dermoid cysts are rare tumors of the spinal cord. Presentation with rapid onset of paraparesis or quadriparesis (onset within 2 weeks) is rarer still. The authors present their experience in the management and outcome of patients with such a presentation. METHODS Patient records between 2000 and 2014 were retrospectively reviewed to identify those with intraspinal dermoid cysts who presented with rapid-onset paraparesis or quadriparesis. Their clinical, radiological, operative, and follow-up data were analyzed. RESULTS Of a total of 50 patients with intraspinal dermoid cysts managed during the study period, 10 (20%) presented with rapid-onset paraparesis or quadriparesis; 9 patients ranged in age from 8 months to 2 years, and 1 patient was 25 years old. A dermal sinus was seen in the lumbar region of 4 patients, the sacral region of 3, and the thoracic region of 1, and in 1 patient no sinus was found. All except 1 patient presented with rapid-onset paraparesis secondary to infection of the intramedullary dermoid cyst. One patient presented with rupture of a dermoid cyst with extension into the central canal up to the medulla. Early surgery was done soon after presentation in all except 2 patients. Among the 9 patients who underwent surgery (1 patient did not undergo surgery), total excision of the intramedullary dermoid cyst was done in 3 patients, near-total excision in 4 patients, and partial excision in 2 patients. Of the 9 patients who underwent surgery, 8 showed significant improvement in their neurological status, and 1 patient remained stable. The 1 patient who did not undergo surgery died as a result of an uncontrolled infection after being discharged to a local facility for management of wound infection. CONCLUSIONS Early recognition of a dermal sinus and the associated intraspinal dermoid cyst and timely surgical intervention can eliminate the chances of acute deterioration of neurological function. Even after an acute onset of paraparesis or quadriparesis, appropriate antibiotic therapy and prompt surgery can provide reasonably good outcomes in these patients.
目的 髓内皮样囊肿是脊髓的罕见肿瘤。以截瘫或四肢瘫快速起病(2周内起病)的情况更为罕见。作者介绍了他们对有此类表现患者的治疗经验及结果。方法 回顾性分析2000年至2014年间的患者记录,以确定患有脊髓皮样囊肿且表现为截瘫或四肢瘫快速起病的患者。分析他们的临床、影像学、手术及随访数据。结果 在研究期间接受治疗的50例脊髓皮样囊肿患者中,10例(20%)表现为截瘫或四肢瘫快速起病;9例患者年龄在8个月至2岁之间,1例患者为25岁。4例患者在腰部区域可见皮窦,3例在骶部区域,1例在胸部区域,1例未发现皮窦。除1例患者外,所有患者均因髓内皮样囊肿感染继发截瘫快速起病。1例患者出现皮样囊肿破裂并延伸至中央管直至延髓。除2例患者外,所有患者在就诊后不久即进行了早期手术。在接受手术的9例患者中(1例未接受手术),3例患者实现了髓内皮样囊肿的全切,4例患者实现了近全切,2例患者实现了部分切除。在接受手术的9例患者中,8例患者的神经功能有显著改善,1例患者病情稳定。1例未接受手术的患者在转至当地机构处理伤口感染后因感染失控死亡。结论 早期识别皮窦及相关的脊髓皮样囊肿并及时进行手术干预可消除神经功能急性恶化的可能性。即使在截瘫或四肢瘫急性起病后,适当的抗生素治疗和及时的手术也可为这些患者带来较好的预后。