Patterns of Congenital Lower Urinary Tract Obstructive Uropathy: Relation to Abnormal Prostate and Bladder Development and the Prune Belly Syndrome
Patterns of Congenital Lower Urinary Tract Obstructive Uropathy: Relation to Abnormal Prostate and Bladder Development and the Prune Belly Syndrome
复制标题
先天性下尿路梗阻性尿路病的模式：与前列腺和膀胱发育异常以及李子腹综合征的关系
DOI：
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发表时间：
2001
影响因子：
1.9
通讯作者：
G. Hutchins
中科院分区：
文献类型：
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作者：
Keith E. Volmar;M. Fritsch;E. Perlman;G. Hutchins
下尿路梗阻的发病机制是有争议的，特别是它与前列腺发育异常和梅干腹综合征（PBS）的关系。为了澄清这一问题，我们检查了11名在我们机构尸检的PBS男性（妊娠17-38周）。完整包埋下尿路并制备成连续组织切片。11例中8例为下尿路机械性梗阻。在这8例中的5例中，尿道的前列腺和阴茎部分之间的异常成角形成了“瓣阀”结构。这些患者有扩张的薄壁膀胱和前列腺以及中度至重度的肾发育不良。8例中有1例在前列腺尿道中段有瓣膜样梗阻，伴有复杂的泄殖腔畸形和薄壁膀胱，另1例在阴茎口有上皮栓，最后1例有后尿道瓣膜。其余3例无机械性梗阻。然而，每例患者都有巨大膀胱，膀胱壁明显增厚、间质纤维化和平滑肌束紊乱。在10例病例中，前列腺没有或只有稀疏的扁平腺体。这些结果表明，PBS中前列腺的异常发育可能被解释为压力诱导的发育不良，而不是原发性发育不良。研究结果进一步表明，前列腺发育异常和梅干腹综合征可能是由于各种类型的解剖阻塞或功能性阻塞的巨大膀胱。
The pathogenesis of lower urinary tract obstruction is disputed, particularly its relation to both abnormal prostatic development and the prune belly syndrome (PBS). In an attempt to clarify this issue we examined 11 males (17–38 weeks gestation) with PBS who were autopsied at our institution. The lower urinary tract was embedded intact and prepared as serial histologic sections. Of the 11 cases, 8 demonstrated mechanical obstruction of the lower urinary tract. In five of these eight cases, a “flap-valve” structure was formed by an abnormal angulation between the prostatic and penile portions of the urethra. These had dilated, thin-walled bladders and prostates and moderate to severe renal dysplasia. One of the eight cases had a valve-like obstruction at the level of the mid-prostatic urethra associated with a complex cloacal malformation and a thin-walled bladder, another case had an epithelial plug at the penile meatus, and the last of the eight cases had a posterior urethral valve. The three remaining cases showed no mechanical obstruction. However, each had megacystis with marked thickening, interstitial fibrosis, and disarray of smooth muscle bundles in the bladder wall. In 10 cases, the prostate had no or only sparse, flattened glands. These results suggest that the abnormal development of the prostate in PBS may be explained as a pressure-induced dysplasia rather than a primary maldevelopment. The findings further suggest that abnormal prostatic development and the prune belly syndrome may arise from either anatomic obstruction of various types or functional obstruction from megacystis.