Intraspinal neurenteric cysts--report of three paediatric cases

Neurenteric cysts are rare congenital lesions of the spine and are lined with entodermal epithelium. They result from anomalous endodermal-neuroectodermal adhesion in the 3rd week of embryonic life with persistence of canal of Kovalevsky. The nature of the eventual abnormality depends on the extent to which this adhesion subsequently disappears. Persistence of the entire tract results in the extreme form of combined anterior and posterior spina bifida with dorsal enteric fistula and persistence of only a part of the tract producing the isolated intraspinal cyst. The most common location is the cervico-dorsal region, and usually it lies ventral to the spinal cord. The lumbosacral location is uncommon. Associated vertebral anomalies, gut cysts, bowel duplication, the presence of keratin markers and mucin-secreting cuboidal or columnar intestinal epithelium in their walls confirm their entodermal origin.

We describe here three unusual cases of neurenteric cysts in patients aged 5-18 years who had already had symptoms for some time. One of these had a cyst sited predominantly in the sacral canal, another presented with a lumbar neurenteric cyst, and the third patient had an intradural extramedullary thoracic lesion. Two of these children had associated anomalies, the one with lumbar cyst also having a lipomeningomyelocele and spina bifida while the other also had deformed vertebrae. All three patients underwent laminectomy and gross excision of the cysts through a posterior approach.

The diagnosis of neurenteric cysts was confirmed by demonstrating mucin-producing cuboidal or columnar epithelium lining the cystic cavity.