K V Satish Kumar, Mammen Abraham, Varma Karthikeya K
Department of Neonatal and Pediatric Surgery, Malabar Institute of Medical Sciences, Calicut, Kerala, India.
Department of Neonatal and Pediatric Surgery, Malabar Institute of Medical Sciences, Calicut, Kerala, India.
J Pediatr Urol. 2015 Dec;11(6):314-8. doi: 10.1016/j.jpurol.2015.05.030. Epub 2015 Jul 30.
Classical bladder exstrophy affects 1 in 30 000 live births. Results of surgical treatment from different institutions employing various surgical techniques are not uniform, thus there is a need for a consensus on the best technique for bladder exstrophy repair. Surgical correction in bladder exstrophy would be more effective if the exact pathogenetic mechanism was deduced and the procedure was directed to correct the cause, which is responsible for the defect. The anatomy of exstrophy shows that the infraumbilical abdominal wall, the anterior wall of the bladder, and the urethra are split, with splayed out genitalia and musculature along with pubic diastasis. There is no tissue loss and hence embryological defect is unlikely to be the cause of bladder exstrophy. Thus there is a need to examine pathogenesis of bladder exstrophy.
A literature search was made of the various hypotheses for cause of bladder exstrophy, and attempts were made to propose a new hypothesis. The present hypothesis is also the basis for a technique of mobilization of pelvic musculature, done in two stages.
The functional outcomes of 38 children with bladder exstrophy managed over a period of 10 years were reviewed. At a mean follow-up of 4.5 years (range 2.5-8 years), 82% of patients were functionally continent.
The exact embryopathogenesis of bladder exstrophy is unknown. In this study a new hypothesis is proposed, with the aim of tailoring the surgical procedure to correct this defect. Bladder exstrophy epispadias complex (BEEC) is a deformative disruption occurring after embryogenic phase and pubic diastasis, and is central to exstrophy development. A working hypothesis can be formulated in line with our observation so that future experiments based this new hypothesis can aim to elucidate the exact pathogenesis.
经典型膀胱外翻在活产婴儿中的发病率为1/30000。不同机构采用不同手术技术进行手术治疗的结果并不一致，因此需要就膀胱外翻修复的最佳技术达成共识。如果能推断出确切的发病机制并针对导致缺陷的病因进行手术，膀胱外翻的手术矫正将更有效。膀胱外翻的解剖结构显示，脐下腹壁、膀胱前壁和尿道裂开，生殖器和肌肉组织展开，伴有耻骨分离。没有组织缺失，因此胚胎学缺陷不太可能是膀胱外翻的病因。因此，有必要研究膀胱外翻的发病机制。
对关于膀胱外翻病因的各种假说进行文献检索，并尝试提出一种新假说。本假说也是分两阶段进行盆腔肌肉组织动员技术的基础。
回顾了10年间对38例膀胱外翻患儿的功能结局。平均随访4.5年（范围2.5 - 8年），82%的患者功能控尿。
膀胱外翻的确切胚胎发病机制尚不清楚。在本研究中提出了一种新假说，旨在调整手术程序以纠正这一缺陷。膀胱外翻阴茎头型尿道上裂复合体（BEEC）是胚胎发育阶段和耻骨分离后发生的一种畸形破坏，是膀胱外翻发育的核心。可以根据我们的观察形成一个可行的假说，以便基于这一新假说的未来实验能够旨在阐明确切的发病机制。
