title: Surgical outcomes and complications of reconstructive surgery in the female congenital adrenal hyperplasia patient: What every endocrinologist should know.
abstract: Surgical management of classical congenital adrenal hyperplasia (CAH) in 46, XX females has evolved significantly. Virilization of the genitalia of 46, XX females with CAH begins prenatally as a result of excess fetal androgen production. Improved understanding of anatomy and surgical outcomes has driven changes in surgical techniques as well as the timing of surgery. For endocrinologists treating these patients, it is important to understand the outcome of genitoplasty, identify patients who need further treatment and direct these patients to experienced surgeons. We performed a literature search on PubMed of publications addressing CAH and genital reconstruction published in the English language from 1990 to the present. In accordance with our institutional review board, we performed a retrospective analysis of clitoroplasty and/or vaginoplasty procedures performed by a single surgeon at our institution from 1996 to 2015. We found that genital reconstruction in 46, XX CAH patients is associated with few immediate post-operative, infectious, and urinary complications. Vaginal stenosis is a common complication of vaginal reconstruction and requires evaluation by an experienced surgeon. Clitoral pain or decreased sensation can be associated with clitoral recession and clitorectomy. Outcomes in sexual satisfaction and gender identity can also be impacted by surgical technique and success. Long term follow up and patient reported feedback are crucial to our understanding and management of this special group of patients. Improved awareness and understanding of the complications of genital surgery will allow endocrinologists to know what to ask patients and be ready to provide them with a resource with the understanding and experience to help them improve their quality of life.
authors.affiliation: The Comprehensive Center for Congenital Adrenal Hyperplasia, The Komansky Center for Children's Health, Department of Urology, New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY, United States. Electronic address: lilywang1115@gmail.com.
authors.forename: Lily C
authors.initials: LC
authors.lastname: Wang
authors.affiliation: The Comprehensive Center for Congenital Adrenal Hyperplasia, The Komansky Center for Children's Health, Department of Urology, New York Presbyterian Hospital, Weill Cornell Medical College, New York, NY, United States.
authors.forename: Dix P
authors.initials: DP
authors.lastname: Poppas
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D041321:Chromosomes, Human, X; D002987:Clitoris / Q000601:surgery; D003251:Constriction, Pathologic; D004704:Endocrinology / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D013509:Gynecologic Surgical Procedures / Q000379:methods*; D006801:Humans; D017060:Patient Satisfaction; D011788:Quality of Life; D019651:Plastic Surgery Procedures / Q000379:methods*; D012189:Retrospective Studies; D014621:Vagina / Q000473:pathology; D014770:Virilism / Q000601:surgery; D014844:Vulva / Q000601:surgery
keywords: Complications; Congenital adrenal hyperplasia; Genital reconstruction; Outcomes