Congenital adrenal hyperplasia: Current surgical management at academic medical centers in the United States

J Urol. 2015 May;193(5 Suppl):1796-801. doi: 10.1016/j.juro.2014.11.008. Epub 2015 Mar 25.

Congenital adrenal hyperplasia: current surgical management at academic medical centers in the United States.

Abstract

PURPOSE:

Controversy exists on the necessity for and timing of genitoplasty in girls with congenital adrenal hyperplasia. Our knowledge of surgical preferences is limited to retrospective series from single institutions and physician surveys, which suggest a high rate of early reconstruction. We evaluated current surgical treatment for congenital adrenal hyperplasia at academic centers.

MATERIALS AND METHODS:

We queried the Faculty Practice Solutions Center database to identify all female patients younger than 18 years with a diagnosis of congenital adrenal hyperplasia between 2009 and 2012. Procedures were identified by CPT codes for vaginoplasty, clitoroplasty and other genital procedures. Reconstruction type, age at surgery and surgeon volume were analyzed.

RESULTS:

We identified 2,614 females in the database with a diagnosis of congenital adrenal hyperplasia who were seen at a total of 60 institutions. Of infants younger than 12 months between 2009 and 2011 as few as 18% proceeded to surgery within a 1 to 4-year followup. Of those referred to a pediatric urologist 46% proceeded to surgery. Of patients who underwent surgery before age 2 years clitoroplasty and vaginoplasty were performed in 73% and 89%, respectively, while 68% were treated with a combined procedure. A medium or high volume surgeon was involved in 63% of cases.

CONCLUSIONS:

Many patients with congenital adrenal hyperplasia in the database did not proceed to early reconstructive surgery. Of those referred to surgeons, who were possibly the most virilized patients, about half proceeded to early surgery and almost all underwent vaginoplasty as a component of surgery. About two-thirds of the procedures were performed by medium or high volume surgeons, indicative of the surgical centralization of disorders of sexual development.

Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

KEYWORDS:

adrenal glands; adrenal hyperplasia; congenital; disorders of sex development; reconstructive surgical procedures; virilism

PMID:
25817160
[PubMed – in process]

from http://www.ncbi.nlm.nih.gov/pubmed/25817160

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