Takenaka A, Gohji K, Arakawa S, Matumoto O, Kamidono S, Mizutori M, Kitazawa S, Maeda S, Sugiyama T
Department of Urology, Kobe University School of Medicine.
Hinyokika Kiyo. 1989 Aug;35(8):1439-44.
Two autopsy cases of prune belly syndrome are reported. In Japan, 111 cases have been reported. Abdominal muscle deficiency or hypoplasia, urinary tract abnormalities and cryptorchidism are usually recognized. Case 1 was a female stillborn at 33 weeks' gestation and case 2 was a male stillborn at 21 weeks' gestation. Both cases had anomalies of genitourinary and digestive system. There was anatomic obstruction of external urethral orifice in case 1 and overriding urethra in case 2 which meant functional obstruction. In relation to the pathogenesis, we propose the important role of the urethral obstruction, whatever anatomic or functional. Further examination of the lower urinary tract including serial section analysis might be necessary to identify the pathogenesis of prune belly syndrome.
报告了两例梅干腹综合征的尸检病例。在日本，已报告111例。通常可发现腹肌缺损或发育不全、泌尿系统异常和隐睾症。病例1是一名孕33周的死产女婴，病例2是一名孕21周的死产男婴。两例均有泌尿生殖系统和消化系统异常。病例1存在尿道外口解剖性梗阻，病例2存在尿道骑跨，意味着功能性梗阻。关于发病机制，我们提出无论解剖性还是功能性的尿道梗阻都起着重要作用。可能需要对下尿路进行进一步检查，包括连续切片分析，以确定梅干腹综合征的发病机制。
