THE PRUNE BELLY SYNDROME: REPORT OF 3 CASES

先天性腹肌缺少症候羣：報告三例

陳榮邦(Wing-Pong Chan) ； 李瑤華(Yin-Wah Li)

《中華放射線醫學雜誌》 12卷4期 (1987/12) Pp. 427-431

摘要

The ＂prune belly＂ or ＂traid＂ syndrome is composed of deficiency of the abdominal musculature, cryptorchidism and urinary tract anomaIities. Parker first described the case in 1985. Williams and others have aptly used the term to describe the affected babies. Today, this malformation complex is a well-established entity though the pathogenesis is still controversy. Its incidence is 1:30,000 to 40,000 live births, and affects males almost exclusively. The overall mortality rate is closely related to urethral obstructions. About 20% of the infants are stillborn or 50% die within two years. The diagnosis may remain undetected until roentgenographic investigation for the urinary system anomalies.

並列摘要

台大醫院最近七年內（1981,1~1987,11）共有三例先天性腹肌缺少症候羣，他們都有隱睪症，無腹肌外觀和泌尿系統發育異常。其中兩例超過兩年的存活率，皆無尿道的阻塞。泌尿系攝影用來診斷此症候羣係必需的工具。