Hirschsprung's Disease Presenting with Diffuse Intestinal Pneumatosis in a Neonate

以彌漫性腸壁積氣表現之一新生兒先天性巨結腸症

謝武勲(Wu-Shiun Hsieh) ； 楊鵬弘(Peng-Hong Yang) ； 黃振盛(Cheng-Sheng Huang) ； 王超然(Chao-Ran Wang)

《Acta Paediatrica Taiwanica》 41卷6期 (2000/12) Pp. 336-338

https://doi.org/10.7097/APT.200012.0336

摘要

新生兒時期，腹部X光檢查出理腸炎。我們報告一例女性足月新生兒，出生第二天內腹部X光即出現彌漫性腸壁內積氣，臨床上則有腹脹、含膽汁之嘔吐等疑腸陰塞的現象。腸壁內積氣在48小時內消失，但患兒經直腸壓力及下腸道顯影檢查後疑似新生兒先天性巨結腸症，經開刀及病理切片檢查後證實。術後，病人出現短腸症候群，且不幸在3個月大時死於呼吸道吸入性意外。本報告主要在提醒新生兒腹部X光出現腸壁積氣的現象時，除了懷疑可能罹患壞死性腸炎外，應該將其他造成腸陰塞之各種病因作鑑別診斷。

關鍵字

腸壁積氣 ； 先天性巨腸症 ； 壞死性腸炎 ； 短腸症候群 ； 新生兒

並列摘要

In the neonate, pneumatosis intestinalis is almost always associated with necrotizing enterocolitis. The manifestation of diffuse intestinal pneumatosis in Hirschsprung's disease has been reported rarely. It may occur as a result of Hirschsprung's disease complicated with enterocolitis. We report a two-day- old female baby born at term with the problems of failure to pass meconium, progressive abdominal distension and bile stained vomiting. There was an early roentgenographic presentation of pneumatosis intestinalis which might have led to a diagnosis of necrotizing enterocolitis. However, the intestinal pneumatosis resolved within 48 hours. After anorectal manonletry and contrast enema examination, an ileostomy was performed at the age of 23 days, and multiple biopsies of intestine showed aganglionosis tip to the ileum at the level of 85cm above the ileocecal valve. Unfortunately, the patient developed short bowel syndrome after operation and died suddenly after an accidental choking at the age of three months. This case suggests that Hirschsprung's disease may have an tin usual early roentgenographic presentation with diffuse intestinal pneumatosis in the first few days of life. Anorectal manometries and suction biopsies are crucial for further diagnosis.

並列關鍵字

pneumatosis intestinalis ； Hirschsprung's disease ； necrotizing enterocolitis ； short bowel syndrome ； neonate