Neonatal Intestinal Perforation Caused by Congenital Defect of the Small Intestinal Musculature: Report of One Case

先天性小腸肌肉缺損導致新生兒腸道破裂：一病例報告

黃博琪(Po-Chi Huang) ； 鄒國英(Kuo-Inn Tsou Yau) ； 吳林榮(Mu-Zon Wu) ； 黃秀芬(Shiu-Feng Huang)

《Acta Paediatrica Taiwanica》 40卷4期 (1999/08) Pp. 271-273

https://doi.org/10.7097/APT.199908.0271

摘要

此篇病例報告描述一個五天大男嬰，因為不明原因的腸阻塞，導致腸道破裂而死亡。死後解剖發現在回腸多處有肌肉缺損的現象。先天性腸道肌肉缺損是一種罕見的疾病，多見於早產或體重很輕之嬰兒，其病理機轉尚未确定。診斷主要靠切下腸子後的病理組織檢查，可見肌肉層局部或完全的缺損。回顧過去英語文獻上的二十個病例及本病例，我們推測週產期之腸道缺血或缺氧性傷害，應是導致先天性腸道肌肉缺損的主要機制。

關鍵字

新生兒腸道阻塞 ； 不明原因腸破裂 ； 先天性腸道肌肉缺損

並列摘要

Congenital defect of the small intestinal musculature is a rare cause of neonatal spontaneous intestinal obstruction or perforation. Its etiology and pathogenesis are still controversial. A male infant presented with intestinal obstruction at two days of age and rapidly progressed to perforation and septic shock. He died at seven days of age. Autopsy finding revealed a perforation hole at twenty-five cm proximal to ileocecal valve. Histology examination demonstrates multifocal deficiency of the inner circular muscle layer three cm around the perforation site. The clinical and histological characteristics are reviewed and discussed. We propose that the muscle defect of small intestine, especially ileum, is secondary to ischemic injury rather than an embryological malformation.

並列關鍵字

Intestinal perforation ； intestinal obstruction ； intestinal musculature ； newborn