Kuribayashi K, Nakasu S, Matsumura K, Matsuda M, Handa J
Department of Neurosurgery, Shiga University of Medical Science, Japan.
No To Shinkei. 1993 Aug;45(8):747-51.
Dermoid cysts in the central nervous system are often associated with various congenital disorders, especially dermal sinus and spina bifida. We report a case of dermoid cyst in the fourth ventricle associated with Klippel-Feil syndrome. A 47-year-old man with a long history of headache had been known to have a cystic lesion in the posterior fossa for 12 years. When he was referred to our hospital with complaints of transient tetraparesis, he showed bilateral cerebellar ataxia and minimal left hemiparesis. Furthermore, he was noted to have a webbed neck with a low hairline and facial asymmetry. CT and MRI showed multiple cerebral infarctions as well as a mass lesion in the posterior fossa. Cervical roentgenogram showed a fusion of C 2 and C 3 vertebrae. The tumor was totally removed via a suboccipital approach, and the diagnosis was a dermoid cyst. The present patient had not only dermoid cyst and Klippel-Feil syndrome but also hypertrophy of the zygomatic bone. The pathogenesis of the Klippel-Feil syndrome is presumed to be an intrauterine defect, with a failure of segmentation of mesodermal somites. The zygomatic bone is also derived from the mesoderm somites at early fourth week, too. From these points of view, the disturbance in the mesoderm before the fourth week of gestation might have played an important role in causing a dermoid cyst.
中枢神经系统的皮样囊肿常与各种先天性疾病相关，尤其是皮样窦和脊柱裂。我们报告一例第四脑室内皮样囊肿合并Klippel-Feil综合征的病例。一名有长期头痛病史的47岁男性，已知后颅窝有囊性病变12年。当他因短暂性四肢轻瘫前来我院就诊时，表现为双侧小脑性共济失调和轻度左侧偏瘫。此外，还发现他有蹼颈、低发际线和面部不对称。CT和MRI显示多发脑梗死以及后颅窝有占位性病变。颈椎X线片显示C2和C3椎体融合。通过枕下入路将肿瘤完全切除，诊断为皮样囊肿。该患者不仅患有皮样囊肿和Klippel-Feil综合征，还伴有颧骨肥大。Klippel-Feil综合征的发病机制推测为子宫内缺陷，中胚层体节分割失败。颧骨在妊娠第四周早期也源自中胚层体节。从这些角度来看，妊娠第四周前中胚层的紊乱可能在导致皮样囊肿方面起了重要作用。
