Department of Pathology, H04.312, University Medical Center Utrecht, PO Box 85500, 3508 GA, Utrecht, The Netherlands.
J Pediatr Surg. 2009 Nov;44(11):2223-9. doi: 10.1016/j.jpedsurg.2009.07.024.
The aim of the study was to analyze the microstructure of penislike clitorises in female pseudohermaphroditism in relation to their pathogenesis.
Penislike clitorises from 2 fetuses and 1 newborn with anorectal malformations and multiple other caudal anomalies and 1 fetus with phallic urethra duplication were histologically examined in toto.
The penislike clitorises revealed basically female features in the histogenesis of the corpora cavernosa and glans and in the absence of penile raphe, septum, fasciae, and periurethral glands. In 2 cases, the "phallic urethra" was completely surrounded by a single corpus cavernosum and in 3 cases by a circular glans. Labia minora and vestibular bulbs had not developed in 3 cases. The urethras had transformed into fusiform or scaphoid megalourethras with stenotic meatuses in 3 specimens and an extra orifice in the fourth specimen.
Penislike clitorises in female pseudohermaphroditism show a fundamental dysgenesis of major structural elements. The pattern favors the hypothesis of an early error in the formation of the cloacal membrane and adjacent cloaca in embryos between 26 and 29 days postovulation that may lead to dysregulation of molecular developmental interactions during the following formation of the genital tubercle.
本研究旨在分析女性假两性畸形中类似阴茎的阴蒂的微观结构与其发病机制的关系。
对 2 例肛门直肠畸形和多种其他尾部异常的胎儿以及 1 例具有阴茎尿道重复的胎儿的类似阴茎的阴蒂进行了全组织学检查。
类似阴茎的阴蒂在海绵体和龟头的组织发生以及缺乏阴茎系带、隔膜、筋膜和尿道周围腺体方面显示出基本的女性特征。在 2 例中，“阴茎尿道”完全被单个海绵体包围，在 3 例中被环状龟头包围。3 例未发育小阴唇和前庭球。3 例尿道已转变为梭形或舟状巨尿道，尿道口狭窄，第 4 例有额外的开口。
女性假两性畸形中的类似阴茎的阴蒂显示主要结构元素的根本发育不良。这种模式支持这样的假说，即在排卵后第 26 至 29 天之间的胚胎中，泄殖腔膜和相邻泄殖腔的早期形成错误可能导致随后的生殖器结节形成过程中分子发育相互作用的失调。
