eMedicine Specialties > Pediatrics: Surgery > Urology

Ambiguous Genitalia and Intersexuality: Treatment & Medication

Author: Joel Hutcheson, MD, Consulting Staff, Departments of Surgery and Urology, Pediatric Surgical Associates
Coauthor(s): Howard M Snyder III, MD, Professor, Department of Surgery, Division of Pediatric Urology, University of Pennsylvania School of Medicine
Contributor Information and Disclosures

Updated: Jul 16, 2009

Treatment

Medical Care

Medical therapy for intersex conditions depends on the underlying cause and is indicated for the conditions associated with ambiguous genitalia, including congenital adrenal hyperplasia (CAH). Supplemental hormone therapy may be implemented if gonadal function is compromised.

Surgical Care

  • In a virilized female, the surgical procedure is termed feminizing genitoplasty and includes vaginoplasty and clitoroplasty.
  • Undervirilized males typically have hypospadias requiring surgical reconstruction. Gender reassignment may be considered in patients with male pseudohermaphrodism and genital inadequacy.

Consultations

  • Geneticist/genetic counselor
  • Endocrinologist
  • Surgeon
  • Obstetrician/urologist
  • Psychologist

Medication

Medications used in intersex conditions depend on the underlying cause.

Glucocorticoids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli. Children with congenital adrenal hyperplasia (CAH) require corticosteroid replacement for survival. Replacement also reduces the production of corticotropin and, therefore, the overproduction of androgens.


Hydrocortisone (Hydrocortone, Cortef)

Drug of choice because of mineralocorticoid activity and glucocorticoid effects.

Adult

30-150 mg/d IV divided q8-12h

Pediatric

10-20 mg/m2/d IV divided into 2 equal doses

CYP450 2D6 and 3A3/4 substrate; corticosteroid clearance may increase with phenytoin, barbiturates, or rifampin treatment or decrease with estrogens; cholestyramine may decrease AUC; corticosteroids may increase digitalis toxicity secondary to hypokalemia; coadministration with potassium-depleting agents (eg, diuretics) may increase risk of hypokalemia; corticosteroids may decrease growth-promoting effect of GH; decreases effects of salicylates and vaccines used for immunization; monitor for hypokalemia with coadministration of diuretics or amphotericin B; antagonizes effects of anticholinergics; may increase anticoagulant effects of warfarin; decreases hypoglycemic effects of sulfonylureas and insulin; increases toxicity of cyclosporine

Documented hypersensitivity; viral, fungal, or tubercular skin infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in hyperthyroidism, osteoporosis, peptic ulcer disease, cirrhosis, nonspecific ulcerative colitis, diabetes, and myasthenia gravis

More on Ambiguous Genitalia and Intersexuality

Overview: Ambiguous Genitalia and Intersexuality
Differential Diagnoses & Workup: Ambiguous Genitalia and Intersexuality
Treatment & Medication: Ambiguous Genitalia and Intersexuality
Follow-up: Ambiguous Genitalia and Intersexuality
Multimedia: Ambiguous Genitalia and Intersexuality
References
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Further Reading

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Keywords

adrenogenital syndrome, ambiguous genitalia, congenital adrenal hyperplasia, CAH, disorders of sexual development, DSD, female pseudohermaphrodite, female pseudohermaphroditism, infertility, intersex conditions, intersex disorders, male pseudohermaphrodite, male pseudohermaphroditism, mixed gonadal dysgenesis, pure gonadal dysgenesis, true hermaphrodite, true hermaphroditism, 46,XY DSD, 46,XX DSD, ovotesticular DSD, 46,XX testicular DSD, 46,XY complete gonadal dysgenesis

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Contributor Information and Disclosures

Author

Joel Hutcheson, MD, Consulting Staff, Departments of Surgery and Urology, Pediatric Surgical Associates
Joel Hutcheson, MD is a member of the following medical societies: American Academy of Pediatrics and American Urological Association
Disclosure: Nothing to disclose.

Coauthor(s)

Howard M Snyder III, MD, Professor, Department of Surgery, Division of Pediatric Urology, University of Pennsylvania School of Medicine
Howard M Snyder III, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Urological Association, and National Kidney Foundation
Disclosure: Nothing to disclose.

Medical Editor

Martin David Bomalaski, MD, FAAP, Pediatric Urologist, Alpine Urology
Martin David Bomalaski, MD, FAAP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, and American Urological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Harry P Koo, MD, Chairman of Urology Division and Director of Pediatric Urology, Virginia Commonwealth University; Professor of Surgery, VCU School of Medicine, Medical College of Virginia; Director of Urology, Children's Hospital of Richmond
Harry P Koo, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, and American Urological Association
Disclosure: Nothing to disclose.

CME Editor

Paul D Petry, DO, FACOP, FAAP, Consulting Staff, Freeman Pediatric Care, Freeman Health System
Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Chief Editor

Marc Cendron, MD, Associate Professor of Surgery, Harvard School of Medicine; Consulting Staff, Department of Urological Surgery, Children's Hospital Boston
Marc Cendron, MD is a member of the following medical societies: American Academy of Pediatrics, American Urological Association, European Society for Paediatric Urology, Johns Hopkins Medical and Surgical Association, New Hampshire Medical Society, Society for Fetal Urology, and Society for Pediatric Urology
Disclosure: Nothing to disclose.

 
 
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