[A rare variant of neuroenteric cyst: split notochord syndrome].
OBJECTIVE
We present a case of split notochord syndrome, an extremely rare form of spinal dysraphism.


METHODS
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.


CONCLUSIONS
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.
Universidade Federal Fluminense, Rio de Janeiro, RJ, Brazil.
Lisieux E
LE
Jesus
Cristiano G
CG
França
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D001006:Anus, Imperforate / Q000175:diagnosis*; D017809:Fatal Outcome; D006801:Humans; D006849:Hydrocephalus / Q000175:diagnosis; D007223:Infant; D007412:Intestinal Fistula / Q000175:diagnosis*; D008297:Male; D009672:Notochord / Q000002:abnormalities*; D016135:Spinal Dysraphism / Q000175:diagnosis*; D013577:Syndrome