Volmar Keith E, Nguyen Tom C, Holcroft Cynthia J, Blakemore Karin J, Hutchins Grover M
The Department of Pathology, The Johns Hopkins Medical Institutions, 600 North Wolfe Street, Baltimore, MD 21287, USA.
Virchows Arch. 2003 Feb;442(2):169-72. doi: 10.1007/s00428-002-0730-x. Epub 2002 Nov 30.
The pathogenesis of the prune belly syndrome (PBS) remains controversial, but two theories predominate. The first theory supports an obstructive phenomenon early in gestation leading to irreversible damage to the genitourinary tract and abdominal wall. The second theory suggests mesodermal injury between the 6th and 10th weeks of gestation as the primary abnormality. This paper reports of two fetuses with the PBS phenotype that were examined postmortem at our institution. Thorough examination of the lower urinary tract allowed demonstration of anatomic obstruction of the urethra in both cases. One case illustrated a relatively common pattern of proximal penile urethral obstruction, a flap-like obstruction between the prostatic and penile urethra. The other case provided what we believe to be the first description of PBS caused by severe phimosis.
梅干腹综合征（PBS）的发病机制仍存在争议，但有两种理论占主导地位。第一种理论支持妊娠早期出现的梗阻现象，导致泌尿生殖道和腹壁发生不可逆损伤。第二种理论认为妊娠第6至10周期间的中胚层损伤是主要异常。本文报告了在我们机构进行尸检的两名具有PBS表型的胎儿。对下尿路的全面检查显示，两例均存在尿道解剖性梗阻。其中一例显示了近端阴茎尿道梗阻的相对常见模式，即前列腺尿道和阴茎尿道之间的瓣状梗阻。另一例提供了我们认为是由严重包茎引起的PBS的首次描述。
