Prune Belly Syndrome: Report of Twelve Autopsy Cases and Possible Pathogenesis
作者:
Kenji KAWAMOTO,
Takayoshi IKEDA,
Takeshi MATSUO,
Hiroshi MAEDA,
Naomasa OKAMOTO,
Yukio SATOW,
Naotaka AKIMOTO,
Juing‐Yi LEE,
期刊:
Congenital Anomalies
(WILEY Available online 1985)
卷期:
Volume 25,
issue 1
页码: 1-15
ISSN:0914-3505
年代: 1985
DOI:10.1111/j.1741-4520.1985.tb00629.x
出版商: Blackwell Publishing Ltd
关键词: prune belly syndrome;absence of abdominal muscles;pathogenesis
数据来源: WILEY
摘要:
AbstractTwelve autopsy cases (9 males, 3 females) of the prune belly syndrome are presented. Principal anomalies of this syndrome are a prune‐like abdominal feature and a giant bladder. Urethral atresia was observed in most but two cases. Imperforate anus and rectovesical fistula were observed in 8 and 7 cases respectively. Associated anomalies were those which may or may not be embryologically related to the principal anomalies.As regards the pathogenesis, the authors propose that a primary defect may occur during any developmental stage of the somitic mesoderm, genital tubercle and urethra. In conclusion, the prune belly syndrome may be of spectrum anomalies depending on the stage specificity mainly in the abdominal wall and genito‐urinary org
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