Schober Justine M, Carmichael Polly A, Hines Melissa, Ransley Philip G
Department of Psychology, City University, London, United Kingdom.
J Urol. 2002 Jan;167(1):300-4.
Our review addresses the various system abnormalities associated with cloacal exstrophy and revisits the question of gender assignment. Gender assignment decisions and psychological aspects of gender issues have become the "ultimate challenge." Exploration of gender identity, gender role behavior and sexual orientation has just begun.
A comprehensive literature review was performed with all world literature regarding the current management of cloacal exstrophy. Research focused on management principles, outcomes and documentation of concurrent anomalies. We also describe original research evaluating gender identity in our own series of patients raised as girls to illustrate the challenge of treatment and augment the available literature, which is scant.
Abnormalities of the vertebral column ranged from hemivertebra to myelomeningocele. With magnetic resonance imaging, the incidence of spinal dysraphism approached 100% and cord tethering was also more frequently recognized. For children with the short bowel syndrome, advances in antibiotic usage, and parenteral and enteral nutrition have increased the survival rate and reduced morbidity. A neurological component has been recognized for bladder function, bladder neck continence, lower extremity function and erectile capacity. Mitrofanoff-type reconstruction with bladder neck closure and continent catheterizable stoma dramatically increased continence. Diminutive or absent penis has been documented in 30% of males, and no documentation of paternity exists. The majority of females have bicornuate uterus. However, ovaries and tubes were generally normal. No reports of proven fertility exist.
The strategy and timing of surgery relating to gender assignment remain controversial. A desperate need exists for research focusing on gender development and quality of life. Until that time, a cautious watchful approach may be most appropriate as our patients with cloacal exstrophy mature into adulthood.
我们的综述探讨了与泄殖腔外翻相关的各种系统异常，并重新审视了性别分配问题。性别分配决策和性别问题的心理层面已成为“终极挑战”。对性别认同、性别角色行为和性取向的探索才刚刚开始。
对关于泄殖腔外翻当前治疗方法的所有世界文献进行了全面的文献综述。研究重点在于治疗原则、结果以及并发异常的记录。我们还描述了对我们自己一系列被当作女孩抚养的患者的性别认同进行评估的原始研究，以说明治疗的挑战并补充现有文献，现有文献非常稀少。
脊柱异常范围从半椎体到脊髓脊膜膨出。通过磁共振成像，脊柱发育不良的发生率接近100%，脊髓拴系也更常被识别出来。对于患有短肠综合征的儿童，抗生素使用、肠外和肠内营养方面的进展提高了生存率并降低了发病率。已认识到神经因素对膀胱功能、膀胱颈控尿、下肢功能和勃起能力有影响。采用膀胱颈闭合和可控性造口的米氏（Mitrofanoff）型重建显著提高了控尿能力。30%的男性记录有阴茎短小或缺失，且没有生育的记录。大多数女性有双角子宫。然而，卵巢和输卵管通常正常。没有已证实生育的报告。
与性别分配相关的手术策略和时机仍存在争议。迫切需要专注于性别发育和生活质量的研究。在此之前，随着我们的泄殖腔外翻患者成长为成年人，谨慎观察的方法可能最为合适。
