Cranio-spinal enterogenous cysts: clinico-radiological analysis in a series of ten cases

Enterogenous cysts are rare congenital lesions resulting from dysgenesis during the third embryonic week at the time of notochord development and the transitory existence of the neurenteric canal. Enterogenous cysts are usually lined by mucus secreting epithelium resembling that of the gastrointestinal tract. The inclusion of the endodermal elements in the spinal canal may produce extradural, intradural extramedullary or intradural intramedullary cystic lesions. The endodermal inclusions producing cysts in the intracranial compartment are extremely rare, especially in the supratentorial region. Plain X-rays may show concomitant cranio-spinal anomalies. However, CT and MRI scans show better definition and characterisation. Total excision is the procedure of choice in these benign cysts with favourable long term prognosis. Approximately 100 histologically proven cases have been described, with a few exceptions, as isolated case reports the authors report a detailed analysis of the clinico-radiological aspects of a series of ten patients with cranio-spinal enterogenous cysts with a review of the literature.