Jehad Almasri1,2, Feras Zaiem3, Rene Rodriguez-Gutierrez4,5, Shrikant U Tamhane6, Anoop Mohamed Iqbal7, Larry J Prokop8, Phyllis W Speiser9, Laurence S Baskin10, Irina Bancos6, M Hassan Murad1,2. 1. Evidence-Based Practice Research Program, Mayo Clinic, Rochester, Minnesota. 2. Robert D. and Patricia E. Kern Center for the Science of Health Care Delivery, Mayo Clinic, Rochester, Minnesota. 3. Department of Pathology, Karmanos Cancer Institute, Wayne State University School of Medicine, Detroit, Michigan. 4. Division of Endocrinology, Department of Internal Medicine, University Hospital Dr. Jose E. Gonzalez, Autonomous University of Nuevo Leon, Monterrey, Mexico. 5. Plataforma INVEST Medicina UANL-KER Unit Mayo Clinic, Autonomous University of Nuevo Leon, Monterrey, Mexico. 6. Division of Endocrinology, Mayo Clinic, Rochester, Minnesota. 7. Division of Pediatrics and Adolescent Medicine, Department of Pediatric Endocrinology, Mayo Clinic, Rochester, Minnesota. 8. Mayo Clinic Libraries, Mayo Clinic, Rochester, Minnesota. 9. Division of Pediatric Endocrinology, Cohen Children's Medical Center and Zucker Hofstra Northwell School of Medicine, Lake Success, New York. 10. Department of Urology, University of California, San Francisco, California.
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 ± 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.
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 ± 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.
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