小结
定义
病史和体格检查
关键诊断因素
- 呕吐
- 暴发性排出液态且恶臭的粪便
- 腹胀
- 胎粪排出延迟
- 发热
- 生长迟缓
其他诊断因素
- 脓毒性休克
危险因素
- 唐氏综合征
- IIA 型多发性内分泌肿瘤
- 男性
- 基因突变（10 号和 21号染色体）
诊断性检查
首要检查
- 腹部平片
- 对比灌肠
需考虑的检查
- 直肠活检
- 肛门直肠测压
治疗流程
典型（直肠乙状结肠）/长段疾病：无小肠结肠炎
典型（直肠乙状结肠）/长段疾病：发生小肠结肠炎
全结肠无神经节细胞症
短段疾病
撰稿人
作者
Andrea Bischoff, MD
Pediatric Surgeon
Director of the International Center for Colorectal and Urogenital Care
Children's Hospital Colorado
Associate Professor of Surgery
University of Colorado
Aurora
CO
利益声明
AB is a co-author of several references cited in this topic.
Alberto Peña, MD
Pediatric Surgeon
International Center for Colorectal and Urogenital Care
Children's Hospital Colorado
Professor of Surgery
University of Colorado
Aurora
CO
利益声明
AP is a co-author of several references cited in this topic.
鸣谢
Dr Andrea Bischoff and Dr Alberto Peña would like to gratefully acknowledge Dr Marc A. Levitt, a previous contributor to this topic. MAL declares that he has no competing interests.
同行评议者
Ivo de Blaauw, MD, PHD
Pediatric Surgeon
Radboud University Nijmegen
Academic Medical Center
Nijmegen
Netherlands
利益声明
IdB declares that he has no competing interests.
Jose Manuel Tovilla, MD
Colorectal Pediatric Surgeon
Chief of the Colorectal Clinic
Hospital Infantil de Mexico Federico Gomez
Mexico
利益声明
JMT declares that he has no competing interests.
Daniel P. Doody, MD
Pediatric Surgeon
Department of Pediatric Surgery
Massachusetts General Hospital
Boston
MA
利益声明
DPD declares that he has no competing interests.
鉴别诊断
- 胎粪充塞综合征
- 囊性纤维化（胎粪性肠梗阻）
- 左小结肠综合征
更多 鉴别诊断指南
- ERNICA 直肠乙状结肠先天性巨结肠的管理指南
- 先天性巨结肠症相关性小肠结肠炎的诊断和管理指南
更多 指南
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