Jesus Lisieux E, França Cristiano G
Universidade Federal Fluminense, Rio de Janeiro, RJ, Brazil.
J Pediatr (Rio J). 2004 Jan-Feb;80(1):77-80.
We present a case of split notochord syndrome, an extremely rare form of spinal dysraphism.
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.
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.
我们报告一例脊索分裂综合征病例，这是一种极为罕见的脊柱裂畸形。
我们治疗了一名2个月大的男婴，他患有广泛的腰骶部畸形、脑积水，并且在背侧中线有明显的肠段，伴有肠瘘和肛门闭锁。该畸形被诊断为脊索分裂综合征。在尝试进行手术治疗之前，婴儿因败血症死亡。
脊索分裂综合征是迄今为止所描述的最罕见的神经肠囊肿形式（文献中<25例）。它常与肛门直肠畸形、肠瘘和脑积水相关。如果同时治疗消化畸形、脑积水和脊柱裂本身，预后不一定很差，存活是有可能的。
