Fouquet V, De Lagausie P, Faure C, Bloch J, Malbezin S, Ferkhadji L, Bauman C, Aigrain Y
Services de chirurgie Viscérale, de Gastro-entérologie, de Santé Publique, d'anesthésie, d'Anatomopathologie, de Génétique, Hôpital Robert Debre, Paris, France.
J Pediatr Surg. 2002 Jan;37(1):71-5. doi: 10.1053/jpsu.2002.29430.
BACKGROUND/PURPOSE: Total colonic aganglionosis with ileal involvement is estimated at 1 case in 50,000 living births. This pathology has a very variable prognosis, and patients often need long-term parenteral nutrition. The aim of this study is to define prognostic factors for this disease.
This is a retrospective study from 1980 to 1999, based on 26 cases of total colonic aganglionosis with ileal involvement. The authors analyzed birth term, sex, birth weight, ileal involvement in centimeters, delay to correct level enterostomy, the total parenteral nutrition duration, and the need for constant rate nutritional assistance. The authors studied the following items: Weight, height, complications, clinical state, and nutritional issues. The statistic test is: LOG RANK (analysis of censored data and comparison of survival diagram).
The only prognostic factor is the length of ileal involvement. All the children with ileal involvement less than 50 cm, except for 2, did not need long-term nutritional assistance; for those with ileal resection over 50 cm, long-term nutritional assistance was needed. Total colonic aganglionosis is a very serious illness (2 children had a small bowel transplantation, and 2 are waiting for one). The prognosis is even worse when associated with a polymalformation syndrome (50% of the children died).
The major prognostic factor is the length of small bowel not involved in the total colonic aganglionosis.
背景/目的：全结肠无神经节症伴回肠受累在每50000例活产婴儿中约有1例。这种病理情况预后差异很大，患者通常需要长期肠外营养。本研究的目的是确定该疾病的预后因素。
这是一项对1980年至1999年期间26例全结肠无神经节症伴回肠受累病例的回顾性研究。作者分析了出生孕周、性别、出生体重、回肠受累长度（以厘米计）、至正确水平肠造口术的延迟时间、肠外营养总持续时间以及持续速率营养支持的需求。作者研究了以下项目：体重、身高、并发症、临床状态和营养问题。统计学检验为：对数秩检验（删失数据的分析和生存曲线的比较）。
唯一的预后因素是回肠受累长度。所有回肠受累长度小于50厘米的儿童，除2例之外，均无需长期营养支持；而回肠切除长度超过50厘米的儿童则需要长期营养支持。全结肠无神经节症是一种非常严重的疾病（2例儿童接受了小肠移植，2例正在等待移植）。当与多发畸形综合征相关时，预后更差（50%的儿童死亡）。
主要预后因素是未累及全结肠无神经节症的小肠长度。
