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Surgical Treatment of Congenital Adrenal Hyperplasia

Genital reconstructive surgery in CAH-related ambiguous genitalia.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Üroloji department. Book Appointment →

What is Surgical Treatment of Congenital Adrenal Hyperplasia?

Congenital adrenal hyperplasia (CAH), most commonly 21-hydroxylase deficiency, leads to virilisation of external genitalia in 46,XX (female) infants. Hyperandrogenism in the intrauterine period results in ambiguous genitalia ranging from clitoromegaly to penile clitoris depending on Prader scale stages.

Reconstructive surgery comprises clitoroplasty (clitoral reduction), vaginoplasty (formation of vaginal opening) and labioplasty. Optimal timing is controversial; some clinics prefer puberty-onset surgery (patient autonomy), while others recommend infancy.

Symptoms

Ambiguous genitalia (Prader I-V)
Clitoromegaly
Vaginal opening absence/stenosis (urogenital sinus)
Posterior labial fusion
Severe virilisation in 46,XX karyotype
Recurrent UTI (urogenital sinus)

Risk Factors

Severe form of 21-hydroxylase deficiency
Insufficient prenatal dexamethasone treatment
11-beta-hydroxylase deficiency
3-beta-hydroxysteroid dehydrogenase deficiency
Family history of CAH

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Newborn with ambiguous genitalia
  • Pre-puberty CAH girl evaluation
  • Vaginal stenosis with menstrual difficulty
  • Genital surgery decision and timing
  • Multidisciplinary team coordination

Treatment Methods

01
Karyotype + 17-OHP + electrolyte (initial assessment)
02
Genitography (urogenital sinus mapping)
03
Glucocorticoid + mineralocorticoid replacement
04
Optimal timing — multidisciplinary discussion
05
Clitoroplasty (preserving neurovascular bundle)
06
Vaginoplasty (low or high urogenital sinus)
07
Labioplasty (separation of fused labia)
08
Vaginal dilation programme (post-operative)
09
Psychosocial support (gender identity, sexuality)
10
Patient-family-centred decision-making

Which Department to Visit?

You can visit our Üroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Üroloji Department

Let us help you

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.