一名患有舟状头畸形的男童在婴儿期接受后颅窝髓母细胞瘤切除术后出现症状性脊髓空洞症，这是一种晚期并发症。
Symptomatic syringomyelia occurring as a late complication of posterior fossa medulloblastoma removal in infancy in a boy also suffering from scaphocephaly.
作者信息
El Hassani Yassine, Burkhardt Karim, Delavellle Jacqueline, Vargas Maria-Isabel, Boex Colette, Rilliet Benedict
机构信息
Service de Neurochirurgie, Hôpital Cantonal Universitaire, 24 rue Micheli-du-Crest, 1211 Geneva 14, Switzerland.
出版信息
Childs Nerv Syst. 2009 Dec;25(12):1633-7. doi: 10.1007/s00381-009-0968-6. Epub 2009 Aug 7.
INTRODUCTION
The association of a medulloblastoma and a syringomyelia has been already described in rare instances albeit without symptoms related to the syrinx.
CASE REPORT
The case of a 23-year-old man operated in infancy for a medulloblastoma and then treated solely with adjuvant chemotherapy is reported. He was also operated in infancy for a scaphocephaly. With a very long time delay, he has developed a Chiari I and a symptomatic cervico-dorsal syringomyelia. The symptoms attributed to the syrinx consisted of a unilateral prurigo over the left arm which was so severe to lead to self-mutilation.
DISCUSSION
Clinical and magnetic resonance imaging follow-up after cervico-dorsal decompression shows a significant improvement of the symptoms together with a reduction of the size of the syrinx. This case is discussed in the light of the presumed pathophysiology of the syrinx and its exceptional clinical presentation.
引言
髓母细胞瘤与脊髓空洞症的关联虽在罕见病例中已有报道，但均无与脊髓空洞相关的症状。
病例报告
报道了一名23岁男性的病例，该患者在婴儿期因髓母细胞瘤接受手术，随后仅接受辅助化疗。他在婴儿期还因舟状头畸形接受了手术。经过很长一段时间后，他出现了Chiari I畸形和有症状的颈胸段脊髓空洞症。归因于脊髓空洞的症状包括左臂单侧瘙痒症，严重到导致自残。
讨论
颈胸段减压术后的临床及磁共振成像随访显示症状有显著改善，同时脊髓空洞大小减小。结合脊髓空洞的推测病理生理学及其特殊临床表现对该病例进行了讨论。
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