International Journal of Clinical Urology

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Feminizing Genitoplasty in Congenital Adrenal Hyperplasia: 20-year Experience of Regional Comprehensive Care

Received: Jun. 10, 2020    Accepted: Jun. 28, 2020    Published: Jul. 13, 2020
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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.

DOI 10.11648/j.ijcu.20200402.12
Published in International Journal of Clinical Urology ( Volume 4, Issue 2, December 2020 )
Page(s) 45-50
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Congenital Adrenal Hyperplasia, Feminizing Genitoplasty, Surgical Outcomes, Urinary Incontinence, Vaginal Stenosis

References
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[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.
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[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.
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  • APA Style

    Katie Lynn Canalichio, Jennifer Ahn, Anne-Marie Elizabeth Amies Oelschlager, Patricia Yvonne Fechner, Elizabeth McCauley, et al. (2020). Feminizing Genitoplasty in Congenital Adrenal Hyperplasia: 20-year Experience of Regional Comprehensive Care. International Journal of Clinical Urology, 4(2), 45-50. https://doi.org/10.11648/j.ijcu.20200402.12

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    ACS Style

    Katie Lynn Canalichio; Jennifer Ahn; Anne-Marie Elizabeth Amies Oelschlager; Patricia Yvonne Fechner; Elizabeth McCauley, et al. Feminizing Genitoplasty in Congenital Adrenal Hyperplasia: 20-year Experience of Regional Comprehensive Care. Int. J. Clin. Urol. 2020, 4(2), 45-50. doi: 10.11648/j.ijcu.20200402.12

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    AMA Style

    Katie Lynn Canalichio, Jennifer Ahn, Anne-Marie Elizabeth Amies Oelschlager, Patricia Yvonne Fechner, Elizabeth McCauley, et al. Feminizing Genitoplasty in Congenital Adrenal Hyperplasia: 20-year Experience of Regional Comprehensive Care. Int J Clin Urol. 2020;4(2):45-50. doi: 10.11648/j.ijcu.20200402.12

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  • @article{10.11648/j.ijcu.20200402.12,
      author = {Katie Lynn Canalichio and Jennifer Ahn and Anne-Marie Elizabeth Amies Oelschlager and Patricia Yvonne Fechner and Elizabeth McCauley and Linda Ramsdell and Paul Merguerian and Margarett Shnorhavorian},
      title = {Feminizing Genitoplasty in Congenital Adrenal Hyperplasia: 20-year Experience of Regional Comprehensive Care},
      journal = {International Journal of Clinical Urology},
      volume = {4},
      number = {2},
      pages = {45-50},
      doi = {10.11648/j.ijcu.20200402.12},
      url = {https://doi.org/10.11648/j.ijcu.20200402.12},
      eprint = {https://download.sciencepg.com/pdf/10.11648.j.ijcu.20200402.12},
      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.},
     year = {2020}
    }
    

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  • TY  - JOUR
    T1  - Feminizing Genitoplasty in Congenital Adrenal Hyperplasia: 20-year Experience of Regional Comprehensive Care
    AU  - Katie Lynn Canalichio
    AU  - Jennifer Ahn
    AU  - Anne-Marie Elizabeth Amies Oelschlager
    AU  - Patricia Yvonne Fechner
    AU  - Elizabeth McCauley
    AU  - Linda Ramsdell
    AU  - Paul Merguerian
    AU  - Margarett Shnorhavorian
    Y1  - 2020/07/13
    PY  - 2020
    N1  - https://doi.org/10.11648/j.ijcu.20200402.12
    DO  - 10.11648/j.ijcu.20200402.12
    T2  - International Journal of Clinical Urology
    JF  - International Journal of Clinical Urology
    JO  - International Journal of Clinical Urology
    SP  - 45
    EP  - 50
    PB  - Science Publishing Group
    SN  - 2640-1355
    UR  - https://doi.org/10.11648/j.ijcu.20200402.12
    AB  - 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.
    VL  - 4
    IS  - 2
    ER  - 

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Author Information
  • Department of Pediatric Urology, Seattle Children’s Hospital, Seattle, United States

  • Department of Pediatric Urology, Seattle Children’s Hospital, Seattle, United States

  • Department of Adolescent Gynecology, University of Washington, Seattle, United States

  • Department of Pediatric Endocrinology, Seattle Children’s Hospital, Seattle, United States

  • Department of Behavioral Medicine, Seattle Children’s Hospital, Seattle, United States

  • Department of Genetics, Seattle Children’s Hospital, Seattle, United States

  • Department of Pediatric Urology, Seattle Children’s Hospital, Seattle, United States

  • Department of Pediatric Urology, Seattle Children’s Hospital, Seattle, United States

  • Section