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  51      Downloads  29
Abstract
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.
Keywords
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
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.
Reference
[1]
Baskin LS. Restoring normal anatomy in female patients with atypical genitalia. Semin Perinatol. 2017; 41 (4): 227-31. doi: 10.1053/j.semperi.2017.03.011. PubMed PMID: 28535944.
[2]
Diamond DA YR. Disorders of Sexual Development: Etiology, Evaluation, and Medical Management. In: Wein AJ KL, Partin AW, Peters CA, editor. Campbell-Walsh Urology. 4. Philadelphia, PA: Elsevier Saunders; 2016. p. 3469-97.
[3]
Szymanski KM, Whittam B, Kaefer M, Frady H, Casey JT, Tran VT, et al. Parental decisional regret and views about optimal timing of female genital restoration surgery in congenital adrenal hyperplasia. J Pediatr Urol. 2018; 14 (2): 156 e1- e7. doi: 10.1016/j.jpurol.2017.11.012. PubMed PMID: 29330019.
[4]
Auchus RJ, Witchel SF, Leight KR, Aisenberg J, Azziz R, Bachega TA, et al. Guidelines for the Development of Comprehensive Care Centers for Congenital Adrenal Hyperplasia: Guidance from the CARES Foundation Initiative. Int J Pediatr Endocrinol. 2010; 2010: 275213. doi: 10.1155/2010/275213. PubMed PMID: 21274448; PubMed Central PMCID: PMCPMC3025377.
[5]
Speiser PW, Azziz R, Baskin LS, Ghizzoni L, Hensle TW, Merke DP, et al. A Summary of the Endocrine Society Clinical Practice Guidelines on Congenital Adrenal Hyperplasia due to Steroid 21-Hydroxylase Deficiency. Int J Pediatr Endocrinol. 2010; 2010: 494173. doi: 10.1155/2010/494173. PubMed PMID: 20981249; PubMed Central PMCID: PMCPMC2963799.
[6]
Dindo D, Demartines N, Clavien PA. Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg. 2004; 240 (2): 205-13. doi: 10.1097/01.sla.0000133083.54934.ae. PubMed PMID: 15273542; PubMed Central PMCID: PMCPMC1360123.
[7]
Austin PF, Bauer SB, Bower W, Chase J, Franco I, Hoebeke P, et al. The standardization of terminology of lower urinary tract function in children and adolescents: Update report from the standardization committee of the International Children's Continence Society. Neurourol Urodyn. 2016; 35 (4): 471-81. doi: 10.1002/nau.22751. PubMed PMID: 25772695.
[8]
Anger J, Lee U, Ackerman AL, Chou R, Chughtai B, Clemens JQ, et al. Recurrent Uncomplicated Urinary Tract Infections in Women: AUA/CUA/SUFU Guideline. J Urol. 2019; 202 (2): 282-9. doi: 10.1097/JU.0000000000000296.PubMedPMID: 31042112.
[9]
Speiser PW, Arlt W, Auchus RJ, Baskin LS, Conway GS, Merke DP, et al. Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2018; 103 (11): 4043-88. doi: 10.1210/jc.2018-01865. PubMed PMID: 30272171.
[10]
Dangle PP, Lee A, Chaudhry R, Schneck FX. Surgical Complications Following Early Genitourinary Reconstructive Surgery for Congenital Adrenal Hyperplasia-Interim Analysis at 6 Years. Urology. 2017; 101: 111-5. doi: 10.1016/j.urology.2016.11.027. PubMed PMID: 27889492.
[11]
Stites J, Bernabe KJ, Galan D, Felsen D, Poppas DP. Urinary continence outcomes following vaginoplasty in patients with congenital adrenal hyperplasia. J Pediatr Urol. 2017; 13 (1): 38 e1- e7. doi: 10.1016/j.jpurol.2016.10.012. PubMed PMID: 27932079.
[12]
Almasri J, Zaiem F, Rodriguez-Gutierrez R, Tamhane SU, Iqbal AM, Prokop LJ, et al. Genital Reconstructive Surgery in Females With Congenital Adrenal Hyperplasia: A Systematic Review and Meta-Analysis. J Clin Endocrinol Metab. 2018; 103 (11): 4089-96. doi: 10.1210/jc.2018-01863. PubMed PMID: 30272250.
[13]
Meyer-Bahlburg HF, Dolezal C, Baker SW, New MI. Sexual orientation in women with classical or non-classical congenital adrenal hyperplasia as a function of degree of prenatal androgen excess. Arch Sex Behav. 2008; 37 (1): 85-99. doi: 10.1007/s10508-007-9265-1. PubMed PMID: 18157628.
[14]
Berenbaum SA, Korman Bryk K, Duck SC, Resnick SM. Psychological adjustment in children and adults with congenital adrenal hyperplasia. J Pediatr. 2004; 144 (6): 741-6. doi: 10.1016/j.jpeds.2004.03.037. PubMed PMID: 15192620.
Browse journals by subject