De Win Gunter, Dautricourt Stéphanie, Deans Rebecca, Hamid Rizwan, Hanna Moneer K, Khavari Rose, Misseri Rosalia, Mueller Margaret G, Roth Joshua, Spinoit Anne-Françoise
University of Antwerp, Faculty of Medicine and Health Science, Antwerp, Belgium.
Department of Urology, University Hospital Antwerp, Wilrijkstraat 10, 2650, Edegem, Belgium.
World J Urol. 2021 Apr;39(4):1021-1027. doi: 10.1007/s00345-020-03461-z. Epub 2020 Sep 28.
With advances in treatment modalities and medical knowledge, girls with congenital urologic disorders are living well into adulthood. Although, sexual and reproductive function in this population is still poorly understood. The aim is to review existing literature about fertility and sexuality in women with congenital genitourinary disorders, including spina bifida (SB), bladder exstrophy-epispadias complex (BEEC) and congenital adrenal hyperplasia (CAH).
This review represents the joint SIU-ICUD (Société Internationale d'Urologie-International Consultation on Urological Disease) consultation on congenital lifelong urology. The results of this analysis were first presented at a joint consultation of the SIU and ICUD at the 2018 SIU annual conference in Seoul, South Korea. Appropriate experts were asked to write specific sections regarding sexuality and reproductive function in female patients with these complex congenital urogenital disorders. Each expert performed their own literature review which was reviewed by GDW, AFS, Hadley M. Wood and Dan Wood. Expert opinion was obtained where data are non-existent.
Only about half of the individuals with SB express a satisfactory sex life. In women with BEEC, cosmetic concerns surrounding genital appearance and function may increase psychological distress, including severe depression, suicide and sexual dysfunction. Professional health care is key for improving self-esteem and to interact in the biopsychosocial model of the quality of life. Patients with SB and BEEC should be informed about all the potential risks and difficulties before, during and after pregnancy. Screening for pelvic organ prolapse is important as it can exacerbate their already existing sexual dysfunction, difficulties achieving pregnancy and challenges with clean intermittent catheterization.
Lifelong multidisciplinary follow-up and management are complex but necessary. As these patients grow into their adolescence, they may have the desire to become involved in personal relationships and have sexual interactions. Their healthcare team needs to be increasingly sensitive to these aspects.
随着治疗方式和医学知识的进步，患有先天性泌尿系统疾病的女孩能够顺利步入成年期。然而，人们对这一群体的性和生殖功能仍知之甚少。本文旨在综述有关先天性泌尿生殖系统疾病女性（包括脊柱裂、膀胱外翻-尿道上裂综合征和先天性肾上腺皮质增生症）生育能力和性方面的现有文献。
本综述是国际泌尿外科学会（SIU）-国际泌尿外科疾病咨询委员会（ICUD）关于先天性终身泌尿外科疾病的联合咨询结果。该分析结果首次在2018年韩国首尔举行的SIU年会上的SIU和ICUD联合咨询会议上公布。邀请了相关专家撰写关于患有这些复杂先天性泌尿生殖系统疾病女性患者性和生殖功能的特定章节。每位专家都进行了自己的文献综述，由GDW、AFS、哈德利·M·伍德和丹·伍德进行审核。在缺乏数据的情况下获取了专家意见。
只有约一半的脊柱裂患者表示性生活满意。在膀胱外翻-尿道上裂综合征女性中，围绕生殖器外观和功能的美容问题可能会增加心理困扰，包括严重抑郁、自杀和性功能障碍。专业的医疗保健对于提高自尊以及在生活质量的生物心理社会模型中进行互动至关重要。脊柱裂和膀胱外翻-尿道上裂综合征患者在怀孕前、期间和之后都应被告知所有潜在风险和困难。筛查盆腔器官脱垂很重要，因为它会加重她们已有的性功能障碍、受孕困难以及清洁间歇性导尿的挑战。
终身多学科随访和管理虽复杂但很有必要。随着这些患者步入青春期，他们可能渴望建立人际关系并进行性互动。他们的医疗团队需要对这些方面越来越敏感。
