1. Pediatric surgeon, Service of Pediatric Surgery, Hospital Municipal Jesus

Manuscript received 30.07.03, accepted for publication 30.09.03. Objective: We present a case of split notochord syndrome, an extremely rare form of spinal dysraphism. Description: We treated a 2 month-old boy presenting with an extensive lumbosacral deformity, hydrocephalus and apparent enteric segments in the dorsal midline, accompanied by an enteric fistula and imperforated anus. The malformation was diagnosed as split notochord syndrome. The baby died as a result of sepsis before surgical treatment could be attempted. Comments: Split notochord syndrome is the rarest form of neuroenteric cyst described until this moment (< 25 cases in the literature). It is frequently associated with anorectal malformation, intestinal fistulae and hydrocephalus. Prognosis is not necessarily poor and survival is possible if digestive malformations, hydrocephalus and the dysraphism itself are treated simultaneously. J Pediatr (Rio J). 2004;80(1):77-80: Split notochord syndrome, spinal dysraphism, myelodysplasia, neuroenteric cyst. A rare variant of neuroenteric cyst: split notochord syndrom