Genital reconstructive surgery in females with congenital adrenal hyperplasia: A systematic review and meta-analysis

Jehad Almasri, Feras Zaiem, Rene Rodriguez-Gutierrez, Shrikant U. Tamhane, Anoop Mohamed Iqbal, Larry J. Prokop, Phyllis W. Speiser, Laurence S. Baskin, Irina Bancos, M. Hassan Murad

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6 Scopus citations

Abstract

Background: Females with congenital adrenal hyperplasia (CAH) and atypical genitalia often undergo complex surgeries; however, their outcomes remain largely uncertain. Methods: We searched several databases through 8 March 2016 for studies evaluating genital reconstructive surgery in females with CAH. Reviewers working independently and in duplicate selected and appraised the studies. Results: We included 29 observational studies (1178 patients, mean age at surgery, 2.7 6 4.7 years; mostly classic CAH). After an average follow-up of 10.3 years, most patients who had undergone surgery had a female gender identity (88.7%) and were heterosexual (76.2%). Females who underwent surgery reported a sexual function score of 25.13 using the Female Sexual Function Index (maximum score, 36). Many patients continued to complain of substantial impairment of sensitivity in the clitoris, vaginal penetration difficulties, and low intercourse frequency. Most patients were sexually active, although only 48% reported comfortable intercourse. Most patients (79.4%) and treating health care professionals (71.8%) were satisfied with the surgical outcomes. Vaginal stenosis was common (27%), and other surgical complications, such as fistulas, urinary incontinence, and urinary tract infections, were less common. Data on quality of life were sparse and inconclusive. Conclusion: The long-term follow-up of females with CAH who had undergone urogenital reconstructive surgery shows variable sexual function. Most patients were sexually active and satisfied with the surgical outcomes; however, some patients still complained of impairment in sexual experience and satisfaction. The certainty in the available evidence is very low.

Original languageEnglish (US)
Pages (from-to)4089-4096
Number of pages8
JournalJournal of Clinical Endocrinology and Metabolism
Volume103
Issue number11
DOIs
StatePublished - Jan 1 2018

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ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Endocrinology
  • Clinical Biochemistry
  • Biochemistry, medical

Cite this

Almasri, J., Zaiem, F., Rodriguez-Gutierrez, R., Tamhane, S. U., Iqbal, A. M., Prokop, L. J., Speiser, P. W., Baskin, L. S., Bancos, I., & Hassan Murad, M. (2018). Genital reconstructive surgery in females with congenital adrenal hyperplasia: A systematic review and meta-analysis. Journal of Clinical Endocrinology and Metabolism, 103(11), 4089-4096. https://doi.org/10.1210/jc.2018-01863