Azzis O, Fremond B, Dabadie A, Jouan J, Bracq H, Babut J M
Service de Chirurgie infantile, C.H.U. Pontchaillou, Rennes.
Chirurgie. 1996;121(3):225-9; discussion 230.
From 1971 to 1994, 16 cases of total colon Hirschsprung's disease were treated at the University Hospital in Rennes. Diagnosis have been at 2 days to 3 months. Two children had a family history of Hirschsprung disease among which one associated megacolon and multiple endocrine neoplasia. This family had a mutation of the RET proto oncogene. Six children died before complete surgical cure, among whom 4 before total parenteral nutrition. Six were treated according to Lester Martin, 3 according to Duhamel, and 1 to Swenson. Diarrhea and occlusions happened during the first postoperative years. None had any enterocolitis. Eight of 9 followed children are continent. Technique had no influence on long term outcome. Early neonatal occlusion management seems to decrease enterocolitis's incidence. We abandoned Lester's technique and kept Duhamel's technique. The problems encountered during ileostomy period do not encourage us to forward the age of definitive surgery procedure.
1971年至1994年期间，雷恩大学医院共治疗了16例全结肠型先天性巨结肠病患者。诊断时间为出生后2天至3个月。其中两名患儿有先天性巨结肠病家族史，其中一名合并巨结肠和多发性内分泌肿瘤。该家族存在RET原癌基因突变。6名患儿在完全手术治愈前死亡，其中4名在接受全胃肠外营养前死亡。6名患儿采用莱斯特·马丁术式治疗，3名采用杜哈梅尔术式治疗，1名采用斯文森术式治疗。术后最初几年出现腹泻和肠梗阻。无一例发生小肠结肠炎。9名接受随访的患儿中有8名排便正常。手术方式对长期预后无影响。早期新生儿肠梗阻的处理似乎可降低小肠结肠炎的发生率。我们放弃了莱斯特术式，保留了杜哈梅尔术式。回肠造口术期间遇到的问题不支持我们提前确定最终手术的年龄。
