Unusual neuroenteric cysts: diagnosis and management

Six cases of unusual neuroenteric (NE) cysts, occurring in children aged 15 months to 18 years, are reported here. Three of the cysts were extramedullary, while the other three were intramedullary. Two of the extramedullary cysts were located at the lumbosacral region, an unusual site. Only one patient had the cyst located in a ventral relation to the cord in the cervicothoracic region. None of the patients had communication with the mediastinum or intraabdominal viscera. Stigmata of spinal dysraphism were seen in three patients. All the children had variable neurological involvement and it was difficult to differentiate NE cyst from other causes of spinal cord or cauda equina lesion, particularly in the absence of some dysraphic marker. One patient presented with minimal neurological deficits and painful torticollis; this patient was suspected to have an atlantoaxial dislocation. All patients were investigated with MRI, and the only diagnostic feature of an NE cyst was an intradural cyst with an anterior vertebral body anomaly in a single case. It was not possible to radiologically differentiate NE cyst from other intraspinal cysts in the rest of the patients. All patients were operated on by the posterior route; an attempt to excise the cyst nearly in toto was made. Evidence of neural tethering most probably due to cyst contents was seen in three patients. All patients improved after surgery. None showed recurrence of the cyst at follow-up of 3 months to 2 years.