Females with congenital adrenal hyperplasia (CAH) and atypical genitalia often undergo complex surgeries; however, their outcomes remain largely uncertain.
We searched several databases through March 8, 2016 for studies evaluating genital reconstructive surgery in females with CAH. Reviewers working independently and in duplicate selected and appraised the studies.
We included 29 observational studies (1,178 patients, mean age at the time of surgery 2.7 ± 4.7 years, mostly classic CAH). After an average follow-up of 10.3 years, the majority of patients who underwent 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 based on the Female Sexual Function Index (which has a maximum score of 36). Many patients continued to complain of significant impairment of sensitivity in the clitoris, vaginal penetration difficulties and low intercourse frequency. Most patients were sexually active, although only 48% reported comfortable intercourse. The majority of patients (79.4%) and treating healthcare professionals (71.8%) were satisfied with the surgical outcomes. Vaginal stenosis was common (27%), whereas other surgical complications, such as fistulas, urinary incontinence and urinary tract infections, were less common. Data on quality of life were sparse and inconclusive.
The long-term follow-up of females with CAH who underwent urogenital reconstructive surgery shows variable sexual function. The majority of 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.
The Journal of clinical endocrinology and metabolism. 2018 Sep 28 [Epub ahead of print]
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
Evidence-Based Practice Research Program, Mayo Clinic, MN, USA., Department of Pathology, Karmanos Cancer Institute, Wayne State University School of Medicine, MI, USA., Endocrinology Division, Department of Internal Medicine, University Hospital Dr Jose E. Gonzalez, Autonomous University of Nuevo Leon, Mexico., Division of Endocrinology, Mayo Clinic, Rochester, MN, USA., Department of Pediatric Endocrinology, Division of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA., Mayo Clinic Libraries, Mayo Clinic, Rochester, MN, USA., Division of Pediatric Endocrinology, Cohen Children's Medical Center and Zucker Hofstra Northwell School of Medicine, Lake Success, NY., Department of Urology, University of California, San Francisco, CA, USA.