N-Fékété C, Ricour C, Martelli H, Jacob S L, Pellerin D
J Pediatr Surg. 1986 Mar;21(3):251-4. doi: 10.1016/s0022-3468(86)80847-8.
From 1960 to 1984, 27 cases of total colonic aganglionosis were treated at the Sick Children's Hospital in Paris; in 19 cases there was ileal involvement, 16 of them extending more than 15 cm above the ileocecal valve. Five had a family history of Hirschsprung's disease. Nine infants died without having had definitive surgery, because of delayed diagnosis, or intractable malabsorption in extensive ileal aganglionosis. Two cases were diagnosed only at the ages of 6 and 13 years. Eleven children had Martin's modification of the Duhamel operation, the oldest of these being now 13 years old; and one girl aged 13 underwent a Swenson operation with ileoanal anastomosis, and one child has had a Kimura procedure. Four infants still have a diverting ileostomy. One out of the 14 operated children died 3 years after operation with fulminating enterocolitis. Late surgical nutritional results are analyzed with regard to the length of the side-to-side ileocolorectal anastomosis, and to the size of the ileorectal anastomosis, on which adequate pouch emptying depends. The essential problem in total colonic aganglionosis is not the surgical management of the condition, but rather its prompt diagnosis and the handling of the neonatal intestinal obstruction.
1960年至1984年期间，巴黎儿童医院共治疗了27例全结肠无神经节症；其中19例伴有回肠受累，16例回肠受累范围超过回盲瓣上方15厘米。5例有先天性巨结肠家族史。9例婴儿因诊断延误或广泛回肠无神经节症导致难以治疗的吸收不良，未进行确定性手术即死亡。2例分别在6岁和13岁时才被诊断出来。11名儿童接受了马丁改良的杜哈梅尔手术，其中年龄最大的现在13岁；一名13岁女孩接受了带回肠肛管吻合术的斯文森手术，一名儿童接受了木村手术。4例婴儿仍有转流性回肠造口术。14名接受手术的儿童中有1例在术后3年死于暴发性小肠结肠炎。根据侧侧回结肠直肠吻合术的长度以及回直肠吻合术的大小（充足的贮袋排空取决于此）对手术后期的营养结果进行了分析。全结肠无神经节症的关键问题不是该病的手术治疗，而是其早期诊断以及新生儿肠梗阻的处理。
