Disseminated neurenteric cyst

This case report presents the unusual holospinal dissemination of a neurenteric cyst, which was successfully treated by fenestration and placement of a subarachnoid-peritoneal (SP) shunt. The patient was a 46-year-old Japanese woman with a history of fourth ventricle neurenteric cysts, which were managed with cyst fenestration in 1996 and 2005. She had been doing well until January 2006, when she developed dizziness and an unsteady gait. A neurological examination revealed a disturbance in the deep sensation of the feet. A neuroimaging evaluation demonstrated multiple cystic lesions in the whole spinal canal, which significantly distorted the spinal cord. Because the spinal cord distortion was the most severe in the lower cervical to upper thoracic areas, a unilateral osteoplastic laminotomy with an endoscopic cyst fenestration was performed in these areas, followed by placement of an SP shunt. The pathological diagnosis was a disseminated neurenteric cyst. There was no malignancy, and the patient has been well, with an improved gait and no signs of peritoneal dissemination, for > 1 year. The present case showed a unique extent of dissemination, which was most likely a secondary characteristic. Neurenteric cysts are well known for their tendency to recur, and total removal is usually difficult because of adhesion of the cyst membrane to important structures. The lesion also compromises cerebrospinal fluid circulation. Cyst fenestration combined with SP shunt placement might be a treatment option in such a case.