gms | German Medical Science

Objective: In the current literature, the number of reported cases of cranial neuroenteric cysts (NC) of the central nervous system (CNS) increases over the last decade. It still is a rare condition with no explicit procedure due to different characteristics of this entity. A definite classification for intracranial NC doesn't exist. In the context of the current literature, we report on one unusual case out of a departmental series of 10 cases of NC of the CNS compiled over 31 yrs. The cyst was located in the cerebellum and the patient is suffering postoperative neurological symptoms until today. The purpose of this report is to discuss the surgical treatment and the role of histopathology in the diagnosis of this rare condition in contrast to arachnoid cysts.

Method: A single-center retrospective analysis found 10 cases of NC between 1983 and 2014. Except for one NC located in the cerebellum of an adult, all other NC were located within the spine. We present the clinical course, operative procedure, post-operative magnetic resonance imaging (MRI) and the clinical follow-up of this cerebellar NC.

Results: A 32-year-old female patient presented with a known cystic formation in the cerebellum for elective resection. The cyst was found 14 yrs earlier when the patient felt some kind of paraesthesia in the right arm. A cranial MRI-scan revealed a cystic formation in the cerebellum with diameter of 1 cm, which was assumed to be an arachnoid cyst. Repeated cranial MRI in July 2012 showed distinct progress of the cystic formation in size. In the meantime the patient presented some episodes of dizziness and gait disturbance. Elective gross total resection was performed in January 2013. The histopathological work up showed a NC without ciliary epithelium and no mucus-secreting cells. The patient has remained relapse free in the follow-up MRI-scans until today, presenting a stable neurological state with persisting balance disorder and vertigo.

Conclusions: Neuroenteric cysts are rare. They tend to grow in size and only histophathological evaluation allows the distinction from dysplastic or carcinomatous cysts. The first line treatment needs to be gross total resection as a subtotal resection may cause symptomatic relapse as it is reported in the current literature. An enlarging cyst with proteinaceous content should be suspected to be NC even intracranially. As a definitive control, it is inverserly related to cyst size. Early intervention is desirable where endoscopic fenestration is insufficient.