Ziegler M M, Royal R E, Brandt J, Drasnin J, Martin L W
Division of Pediatric Surgery, Children's Hospital, Cincinnati, Ohio.
Ann Surg. 1993 Oct;218(4):504-9; discussion 509-11. doi: 10.1097/00000658-199310000-00010.
That total intestinal aganglionosis (extended Hirschsprung's disease) is uniformly incompatible with life as reported in 1985, is challenged by this series of patients treated over the last 7 years with an alternative therapy, extended myectomy-myotomy of the small bowel.
A total of 16 neonates worldwide presented with intestinal obstruction secondary to total (extending to the stomach) or near total (to 40 cm of jejunum) intestinal aganglionosis confirmed at one or more leveling operations.
A patient questionnaire was answered by the surgeon of all 16 patients.
The sex distribution was eight boys and eight girls. The definitive operation included extending an antimesenteric myectomy-myotomy from the ganglionic-aganglionic transition zone for variable lengths, the operative design being to create sufficient small bowel length to support life (40-cm minimum, total small bowel maximum). The myectomized bowel was terminated as an end-stroma or as an isolated jejuno-ileal segment. Ten of 16 patients have survived (62.5%) whose length of ganglionated bowel varies from 0 to 40 cm (mean, 12.4 cm; median, 6.0 cm). Six patients have died from 1 to 33 months after operation (mean, 9.5 months; median, 5.0 months) of gut-induced infection (n = 5) and respiratory failure (n = 1); their ganglionated bowel length was similar to survivors (range, 0-26 cm; mean, 9.2 cm; median, 8.0 cm). Of the 16 patients, 15 have received enteral nutrients through the myectomized bowel. Of ten survivors, strikingly two are totally gut nourished (2 cm, 7-cm length of ganglionated bowel), six receive from 1/5 to 4/5 of total calories enterally, and one receives minimal enteral feeding.
From these patients we have learned that (1) extended myectomy-myotomy relieves the obstruction of extended Hirschsprung's disease; (2) aganglionic bowel after extended myectomy-myotomy acts as a passive conduit for proximally propulsed nutrients; and (3) aganglionic bowel after extended myectomy-myotomy undergoes adaptive change and is capable of absorbing life-supporting nutrients. These data demonstrate extended myectomy-myotomy to be a therapeutic option for otherwise fatal extended Hirschsprung's disease, either as a potentially definitive therapy or as a putative bridge to intestinal transplantation.
1985年报告称全肠道无神经节症（广泛性先天性巨结肠病）一律无法存活，但过去7年接受替代疗法（小肠广泛性肌切除-肌切开术）治疗的这一系列患者对这一观点提出了挑战。
全球共有16例新生儿因全肠道（延伸至胃）或近乎全肠道（至空肠40厘米处）无神经节症继发肠梗阻，在一次或多次分期手术中得到确诊。
所有16例患者的外科医生都回答了一份患者问卷。
性别分布为8名男孩和8名女孩。确定性手术包括从神经节-无神经节过渡区开始进行不同长度的系膜对侧肌切除-肌切开术，手术设计目的是创造足够的小肠长度以维持生命（最短40厘米，最长为全部小肠）。切除肌层的肠段以端侧吻合或孤立的空肠-回肠段结束。16例患者中有10例存活（62.5%），其有神经节肠段的长度在0至40厘米之间（平均12.4厘米；中位数6.0厘米）。6例患者在术后1至33个月死亡（平均9.5个月；中位数5.0个月），死因是肠道感染（5例）和呼吸衰竭（1例）；他们有神经节肠段的长度与存活者相似（范围0至26厘米；平均9.2厘米；中位数8.0厘米）。16例患者中有15例通过切除肌层的肠段接受肠内营养。在10名存活者中，显著的是有2名完全通过肠道获得营养（有神经节肠段长度分别为2厘米和7厘米），6名通过肠内获得总热量的1/5至4/5，1名接受极少的肠内喂养。
从这些患者身上我们了解到：（1）广泛性肌切除-肌切开术可缓解广泛性先天性巨结肠病的梗阻；（2）广泛性肌切除-肌切开术后的无神经节肠段可作为近端推进营养物质的被动通道；（3）广泛性肌切除-肌切开术后的无神经节肠段会发生适应性变化，能够吸收维持生命的营养物质。这些数据表明广泛性肌切除-肌切开术是治疗原本致命的广泛性先天性巨结肠病的一种治疗选择，既可以作为一种潜在的确定性治疗方法，也可以作为肠道移植的一种假定桥梁。
