Feminizing genital reconstruction in congenital adrenal hyperplasia

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Abstract

The past several decades have seen multiple advances in the surgical reconstruction for girls born with Disorders of Sexual Differentiation. This surgery can be technically very demanding, and must be individualized for each patient, as the degree of virilization and level of confluence of the vagina and urogenital sinus will dictate the surgical approach.

In this manuscript we present our approach and experience in the surgical options for girls born with Congenital Adrenal Hyperplasia, with special attention regarding clitoroplasty, urogenital mobilization, and vaginoplasty.

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Most cited references 25

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The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study.

Sarah M Creighton, P. G. Ransley, E C Minto … (2003)

The effects on sexual function of surgical removal of parts of the clitoris are unknown. For infants with intersex conditions and ambiguous genitalia being raised female, this surgery is often undertaken in early childhood. Our aim was to assess the effects of surgery on sexual outcome in this population. We did a cross-sectional study to which we recruited 39 adults who had intersex conditions with ambiguous genitalia who were living as female from clinical (n=15) and peer-support (n=24) settings. We obtained data by use of a postal questionnaire, incorporating a validated sexual function assessment inventory. We also obtained hospital notes of 36 respondents who did not want to remain anonymous, and did genital examinations of 19 women. We assessed sexual problems in relation to surgical history and compared the results for our population to those of a healthy control group. Of the 39 individuals enrolled, 28 had been sexually active and all had sexual difficulties. The 18 women who had undergone clitoral surgery had higher rates of non-sensuality (78%) and of inability to achieve orgasm (39%) than did the ten who had not had surgery (20% [p=0.002] and 0% [p=0.03], respectively). Sexual function could be compromised by clitoral surgery. Debate on the ethics of the use of this surgery in children should be promoted and further multicentre research is needed to ensure representative samples and comprehensive outcome assessment. Meanwhile, parents and patients who consent to clitoral surgery should be fully informed of the potential risks to sexual function.

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Anatomical studies of the human clitoris.

L Baskin, A Erol, Y.-W. Li … (1999)

Endogenous or exogenous testosterone exposure to the fetus during gestation may result in masculinization of the external genitalia. Surgical correction requires a clear understanding of normal female anatomy. We report observations from our anatomical dissections on which we base our approach to reduction clitoroplasty. A total of 14 normal human fetal clitoral specimens at 8 to 24 weeks of gestation were serially sectioned after formalin fixation. Every tenth section was stained with Masson's trichrome, smooth muscle alpha-actin and the neuronal markers PGP 9.5 or S-100. Computer reconstruction using imaging software permitted 3-dimensional analysis of the nerves, corporeal bodies and glans clitoris. These specimens were compared with 2 obtained postnatally at feminizing genitoplasty. The normal fetal clitoris consists of 2 corporeal bodies with a midline septum. The ultrastructure of the female corporeal bodies is similar to that of the male counterpart. The glans clitoris forms a cap on top of the distal end of the narrowed corporeal bodies. There is a midline septum starting on the ventral aspect and extending approximately halfway into the glans. Large bundles of nerves course along the corporeal bodies with the highest density on the dorsal aspect or top. No nerves were noted at the 12 o'clock position, although nerves extend completely around the tunica in a fashion similar to that of the fetal penis. Glans innervation is provided by multiple perforating branches entering at the dorsal junction of the corporeal body and glans. The lowest density of nerves in the glans is on the ventral aspect in juxtaposition to the glans septum. In surgical specimens obtained from patients with congenital adrenal hyperplasia nerves were adjacent to the excised tunica of the corporeal bodies, especially on the lateral aspect. A clear understanding of the anatomy of the human clitoris is important for surgical reconstruction. As in the human penis, nerves in the clitoris form an extensive network around the tunica of the corporeal body with a nerve-free zone at the midline 12 o'clock position. Care should be taken to preserve all nerves. Reduction of the glans clitoris should not violate the extensive innervation that predominates on the dorsal aspect of the glans. The normal clitoris has corporeal bodies that are smaller but analogous to those of the penis. One may consider their function when extensive resection is considered.

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Total urogenital mobilization--an easier way to repair cloacas.

Oscar Peña (1997)

The surgical treatment of persistent cloaca is a serious challenge. The operation is technically difficult and the final results for urinary and fecal function are far from excellent. The repair of a cloaca includes, among other maneuvers, the separation of the vagina from the urinary tract. This step is a serious technical challenge and is very time consuming. Devascularization of these structures is the main source of complications such as urethro-vaginal fistula, vaginal stricture, and acquired vaginal atresia. To avoid these complications and to facilitate the cloacal repair, a new technical variation using total urogenital mobilization was performed in 11 patients. In this procedure, after the rectum is separated from the vagina, both the urethra and the vagina are mobilized together as a single unit. The surgical time spent during the reconstruction was reduced by approximately 70%. All patients recovered well from the operations and have been followed up for 1 to 14 months. The blood supply of the vagina and urethra in all cases remained excellent. No patient developed urethrovaginal fistula, vaginal stricture, or acquired vaginal atresia. The cosmetic appearance in these patients is superior to the one achieved with previous techniques. Although this maneuver may not render better urinary or fecal control, the urethra is more accessible for catheterization. These preliminary results suggest that the total urogenital mobilization maneuver provides a definite technical advance in the repair of cloaca malformations.

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Author and article information

Journal

Journal ID (nlm-ta): Indian J Urol

Journal ID (publisher-id): IJU

Title: Indian Journal of Urology : IJU : Journal of the Urological Society of India

Publisher: Medknow Publications (India )

ISSN (Print): 0970-1591

ISSN (Electronic): 1998-3824

Publication date (Print): Jan-Mar 2009

Volume: 25

Issue: 1

Pages: 17-26

Affiliations

Department of Urology, University of Texas Health Science, 7703 Floyd Curl Dr., MC 7845, San Antonio-78229, Texas - USA

[1 ]Indiana University, School of Medicine, Riley Hospital for Children, 702 Barnhill Drive #4230, Indianapolis-46202, Indiana - USA

Author notes

For correspondence: Dr. Mark Patrick Cain, Department of Urology, Indiana University School of Medicine, Riley Hospital for Children, 702 Barnhill Drive #4230, Indianapolis-46202, Indiana – USA. E-mail: mpcain@ 123456iupui.edu

Article

Publisher ID: IJU-25-17

DOI: 10.4103/0970-1591.45532

PMC ID: 2684317

PubMed ID: 19468424

SO-VID: 221adedb-585a-4242-8db3-80b7cf0aab65

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Feminizing genital reconstruction in congenital adrenal hyperplasia

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Most cited references 25

The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study.

Anatomical studies of the human clitoris.

Total urogenital mobilization--an easier way to repair cloacas.

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