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Characteristics of Female Genital Restoration Surgery for Congenital Adrenal Hyperplasia Using a Large-scale

Joshua D Roth1, Jessica T Casey1, Benjamin M Whittam1

  • 1Department of Urology, Section of Pediatric Urology, Riley Hospital for Children at IU Health, Indianapolis, IN.

Urology
|March 6, 2018
PubMed

Insights

Female genital restoration surgery (FGRS) for congenital adrenal hyperplasia (CAH) is typically performed around 10 months of age. This study found low complication and reoperation rates for FGRS in girls with CAH.

Area of Science:

  • Pediatric Surgery
  • Endocrinology
  • Genetics

Background:

  • Congenital adrenal hyperplasia (CAH) is a group of genetic disorders affecting the adrenal glands.
  • Female genital restoration surgery (FGRS) is performed to address anatomical differences in individuals with CAH.

Purpose of the Study:

  • To analyze nationwide data on the timing, cost, length of stay, and complications of FGRS in females with CAH.
  • To evaluate surgical outcomes for FGRS in this population.

Main Methods:

  • Utilized the Pediatric Health Information System database from 2004-2014.
  • Identified patients with CAH using ICD-9 codes for adrenogenital disorders and relevant surgical procedures.
  • Analyzed data on patient demographics, surgical procedures, hospital stay, costs, and complications.

Main Results:

  • 544 females underwent initial FGRS between 2004-2014, with a median age of 9.9 months.
  • Vaginal procedures were most common (92%), followed by perineal (85%) and clitoral (48%) procedures.
  • Mean length of stay was 2.5 days, mean cost was $12,258, with a 13.8% 30-day readmission rate and 4% perioperative complication rate.

Conclusions:

  • Approximately 12% of girls with CAH underwent FGRS, primarily vaginoplasty, at a median age of 10 months.
  • Clitoroplasty was performed in less than half of the patients.
  • FGRS for CAH demonstrated low 30-day complication and immediate reoperation rates.
Abstract

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