Background

Cloacal exstrophy (CE) is the most severe end of the Exstrophy-Epispadias Complex malformations spectrum. Improvements in postnatal management and well-established operative techniques have resulted in survival rates approaching 100%. This systematic review aims to define the prevalence of long-term active medical problems affecting CE patients after the first decade of life.

Methods

PubMed/Medline, Embase, Scopus, and ISI Web of Knowledge databases were used for the literature search. Original articles related to medical, surgical, and psychosocial long-term problems in CE patients >10 years of age were included in the study. Quality assessment of the articles was performed through the Newcastle-Ottawa Scale. Prevalence estimates and 95% CI were assessed for each outcome.

Results

Twelve studies were included. The most common long-term active problems identified were: urinary incontinence with a prevalence ranging from 9.1% to 85%; sexual function issues related to vaginal anomalies with a prevalence ranging from 8.3% to 71.3%, and uterine anomalies, with a prevalence from 14.3% to 71%; gender identity issues in 46, XY patients raised female had a prevalence from 11.1% to 66.7%. There is no documented history of paternity. Impairment of ambulatory capacity was recorded in 13.8% of patients. Only one paper studied psychological well-being, reporting significantly higher levels of depression among gender reassigned patients.

Conclusions

Teenagers and adults born with CE have well defined long-term problems compared to the general population. Recognition and expert management are crucial to improve care and quality of life during and after the transition into adulthood.

中文翻译：

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泄殖腔外翻患者的长期活动性问题：系统评价

背景

泄殖腔外翻 (CE) 是外翻-外翻瓣复合体畸形谱中最严重的一端。产后管理的改进和成熟的手术技术使生存率接近 100%。该系统评价旨在确定在生命的第一个十年后影响 CE 患者的长期活动性医疗问题的流行率。

方法

PubMed/Medline、Embase、Scopus 和 ISI Web of Knowledge 数据库用于文献检索。该研究纳入了与大于 10 岁的 CE 患者的内科、外科和社会心理长期问题相关的原创文章。文章的质量评估通过纽卡斯尔-渥太华量表进行。评估了每个结果的患病率估计值和 95% CI。

结果

包括十二项研究。最常见的长期活动性问题是： 尿失禁，患病率从 9.1% 到 85% 不等；与阴道异常相关的性功能问题，患病率从 8.3% 到 71.3% 不等，与子宫异常相关，患病率从 14.3% 到 71%；性别认同问题在 46 名 XY 患者中，女性提出的患病率从 11.1% 提高到 66.7%。没有记载的亲子关系史。13.8% 的患者记录到行走能力受损。只有一篇论文研究了心理健康，报告称性别重新分配患者的抑郁症水平明显更高。

结论

与普通人群相比，出生时患有 CE 的青少年和成年人有明确的长期问题。认可和专家管理对于在过渡到成年期间和之后改善护理和生活质量至关重要。