Phillips Timothy M
Department of Urology, Pediatric Urology, Wilford Hall Medical Center, Lackland AFB, TX 78236, USA.
Semin Pediatr Surg. 2011 May;20(2):113-8. doi: 10.1053/j.sempedsurg.2010.12.007.
Cloacal exstrophy, one of the most severe congenital anomalies compatible with life, occurs in up to 1 in 200,000 lives births. The condition affects nearly every major organ system with severe neurologic, skeletal, gastrointestinal, and genitourinary ramifications. With increased understanding of the anatomy and embryology combined with refinements in prenatal diagnosis and postnatal care, there is now near-universal survival of patients with cloacal exstrophy. Functional and cosmetic outcomes have improved with modifications in surgical technique. However, debate continues regarding the issue of gender identity, and long-term data are still accruing with respect to the best strategy for management. Despite the extensive malformations noted, many patients have gone on to live fruitful lives.
泄殖腔外翻是最严重的可存活先天性畸形之一，发生率约为每20万例活产中有1例。这种疾病几乎影响每个主要器官系统，会产生严重的神经、骨骼、胃肠道和泌尿生殖系统后果。随着对解剖学和胚胎学的认识不断增加，以及产前诊断和产后护理的改进，现在泄殖腔外翻患者几乎都能存活。手术技术的改进使功能和美容效果得到了改善。然而，关于性别认同问题的争论仍在继续，关于最佳治疗策略的长期数据仍在积累。尽管存在广泛的畸形，但许多患者仍过上了充实的生活。
