Stec Andrew A
Division of Pediatric Urology, Brady Urological Institute, Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.
Semin Pediatr Surg. 2011 May;20(2):66-70. doi: 10.1053/j.sempedsurg.2010.12.011.
The exstrophy-epispadias complex is a complex congenital anomaly that, although rare, remains the largest genitourinary birth defect that is surgically correctable. The primary defect in exstrophy is a derangement in midline developmental that presents with a spectrum of severity. In its mildest form, epispadias, the dorsal urethral unit is not fused and has failed to form into a tube. Next, patients with classic bladder exstrophy present with a bladder and urethra open and continuous with the abdominal wall; also associated is a failure of the abdominal muscles, pelvic ring, and pelvic floor musculature to fuse in the midline. Cloacal exstrophy, the most severe variant, includes exstrophied hindgut tube and a more severe degree of concomitant congenital derangements of musculoskeletal, genitourinary, gastrointestinal, and neurological systems. The embryology of the exstrophy-epispadias complex has been long studied, yet debate still exists over the specific origins of the anomaly. This article covers the embryologic theories of this congenital defect and the subsequent bony pelvic and pelvic floor muscular defects characteristic of exstrophy. Primarily, the anatomic focus will be on classic bladder exstrophy because it is the most common and well studied to date.
膀胱外翻-尿道上裂综合征是一种复杂的先天性畸形，尽管罕见，但仍是最大的可通过手术矫正的泌尿生殖系统出生缺陷。膀胱外翻的主要缺陷是中线发育紊乱，其严重程度不一。最轻微的形式是尿道上裂，背侧尿道单元未融合且未能形成管道。其次，典型膀胱外翻患者的膀胱和尿道开放并与腹壁相连；同时还伴有腹部肌肉、骨盆环和盆底肌肉组织在中线处未能融合。泄殖腔外翻是最严重的变体，包括外翻的后肠管以及更严重程度的肌肉骨骼、泌尿生殖、胃肠道和神经系统先天性紊乱。膀胱外翻-尿道上裂综合征的胚胎学已被长期研究，但关于该畸形的具体起源仍存在争议。本文涵盖了这种先天性缺陷的胚胎学理论以及随后膀胱外翻特有的骨盆和盆底肌肉缺陷。主要地，解剖学重点将放在典型膀胱外翻上，因为它是迄今为止最常见且研究最多的。
