Volume 4, Issue 2, December 2020, Page: 45-50
Feminizing Genitoplasty In Congenital Adrenal Hyperplasia: 20-year Experience of Regional Comprehensive Care
Katie Lynn Canalichio, Department of Pediatric Urology, Seattle Children’s Hospital, Seattle, United States
Jennifer Ahn, Department of Pediatric Urology, Seattle Children’s Hospital, Seattle, United States
Anne-Marie Elizabeth Amies Oelschlager, Department of Adolescent Gynecology, University of Washington, Seattle, United States
Patricia Yvonne Fechner, Department of Pediatric Endocrinology, Seattle Children’s Hospital, Seattle, United States
Elizabeth McCauley, Department of Behavioral Medicine, Seattle Children’s Hospital, Seattle, United States
Linda Ramsdell, Department of Genetics, Seattle Children’s Hospital, Seattle, United States
Paul Merguerian, Department of Pediatric Urology, Seattle Children’s Hospital, Seattle, United States
Margarett Shnorhavorian, Department of Pediatric Urology, Seattle Children’s Hospital, Seattle, United States
Received: Jun. 10, 2020;       Accepted: Jun. 28, 2020;       Published: Jul. 13, 2020
DOI: 10.11648/j.ijcu.20200402.12      View  273      Downloads  67
Background: There is need for data on long-term patient outcomes following feminizing genitoplasty (FG) in females with congenital adrenal hyperplasia (CAH). We aim to describe postoperative and longitudinal psychosocial, endocrine, gynecologic, and urologic outcomes. Methods: We performed a retrospective review of past 20 years looking at females with CAH status post FG. Patients without follow-up were excluded. We captured demographic data, intraoperative details, 30-day complication rates, and longitudinal outcomes. Results: Twenty patients met inclusion criteria. Prader scores ranged from 3-5. Median age at surgery was 1.0 year (IQR 0.6, 1.3). Median follow-up was 4.5 years (IQR 0.7, 9.6). Genetic testing was performed in 15/20 and all had causative variants in the CYP21A2 gene. Thirty-day surgical complications occurred in 5/20 patients (Clavien I-IIIb). All patients over 3 years were toilet trained, with only 1 patient requiring surgery for urinary incontinence. In post-pubertal patients, 5/6 had vaginal stenosis at median age 17.8 years (IQR 14.9, 21.1). Four patients received treatment for behavioral/psychiatric diagnoses. No patient had gender dysphoria. Conclusion: FG is safe as demonstrated by our low major complication rate. Our experience highlights the value of multidisciplinary care through adolescence and young adulthood to support long-term medical and psychosocial outcomes in females with CAH.
Congenital Adrenal Hyperplasia, Feminizing Genitoplasty, Surgical Outcomes, Urinary Incontinence, Vaginal Stenosis
To cite this article
Katie Lynn Canalichio, Jennifer Ahn, Anne-Marie Elizabeth Amies Oelschlager, Patricia Yvonne Fechner, Elizabeth McCauley, Linda Ramsdell, Paul Merguerian, Margarett Shnorhavorian, Feminizing Genitoplasty In Congenital Adrenal Hyperplasia: 20-year Experience of Regional Comprehensive Care, International Journal of Clinical Urology. Vol. 4, No. 2, 2020, pp. 45-50. doi: 10.11648/j.ijcu.20200402.12
Copyright © 2020 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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