先天性肛门直肠畸形伴短结肠的病理

Pathology of Congenital Anorectal Malformation Associated With Short Colon

目的探讨先天性肛门直肠畸形伴短结肠的病理。方法对16例先天性肛门直肠畸形行尸检,发现2例有肛门直肠畸形合并短结肠,行解剖组织学方法观察。结果2例均为男性,高位无肛伴短结肠。直肠盲端极度扩张,壁薄如纸,仅为正常肠壁的1/2～1/4,属Ι型。镜下,肌间和粘膜下神经丛和神经节细胞极少。结论先天性肛门直肠畸形伴短结肠较罕见,本组发生率为12.5%(2/16)。高位无肛伴短结肠、无瘘者,短结肠极度扩张,壁菲薄如纸,肠壁各层发育薄弱,肌间和粘膜下神经丛和神经节细胞极少。无肛畸形合并膀胱瘘、尿道瘘、阴道瘘者,短结肠可有蠕动功能,肠壁全层肥厚水肿,粘膜充血及溃疡,粘膜下层和肌间可能有神经丛和神经节细胞,其改变可为正常、减少或缺如。术前经X线摄片或造影确诊。新生儿期在短结肠处造瘘,待3～6个月后行根治术。

Objective To explore the pathology of congenital anorectal malformation associated with short colon malformation.Methods Autopsies of sixteen cases of congenital anorectal malformation were made and2cases of anorectal deformity associated with short colon was found.Anatomicohistologic examinations were used to observe the pathomorphologic changes.Results The two cases with short colon malformation were both males and high anus atresia.The distal rectums dilated extremely and the thickness of intestinal walls were only1/2～1/4of the normal intestines,which belonges to type I.Under microscopic observation,the nerve plexuses and ganglions in intermuscular and submucous layers were scarce.Conclusions Congenital anorectal malformation associated with short colon is scarce with a low incidence of12.5%(2/16).This disease was divided into IV types.In the group of the patients with high anus atresia accompanied by short colon without fistula,short colon dilated extremely;intestinal walls was very thin;the development was postponed;there were few nerve plexuses and ganglions in intermuscular and submucous layers.Other group of the patients with anus atresia associated with bladder fistula,urethral fistula or vaginal fistula,short colon might have peristaltic function.The intestines were edema ,hyperemia or have ulceration.In intermuscular and submucous layers,there might be nerve plexuses and ganglions,and its change were normal,reduced or deficient.This disease could be diagnosed through X-ray examination before operations.Preliminary surgery is colostomy at the short colon site in infancy and definitive surgical repair is undertaken when infant age exceeded3～6months.