Treatments for Disorders of Sexual Differentiation in Children

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Contact the Department of Urology

  • 1-617-355-7796

How are disorders of sex differentiation treated?

The treatment options disorders of sex differentiation (DSD) vary depending on the complexity of your child's disorder. The medical treatments may include hormone replacement therapy, surgery and psychosocial support.

Surgery

Surgical interventions depend on what type of DSD your child has. In some cases, surgery may be required. In other cases, surgery remains an elective (optional) procedure.

Situations where surgery is required:

  • conditions where ambiguous genitalia interfered with a child's sexual and reproductive function (In this case, appropriate sex organs may need to be removed or created in order for them to function properly.)
  • Sconditions like mixed gonadal dysgenesis, where sex assignment is needed (This sex assignment is important for treatment purposes as well as the emotional well being of the child as they grow older.)
  • if a gonad is cancerous or is at risk for becoming cancerous

Reconstructive surgery to improve the aesthetic appearance of the genitals is often elective. Girls who are born with a condition that causes male-appearing genitalia, like congenital adrenal hyperplasia, often undergo reconstructive surgery to reduce the clitoral size and make the vaginal opening better defined. Male reconstructive genital surgery is used to straighten the penis and move the urethra to the tip of the penis.

Hormone replacement therapy

In terms of lifelong management of DSDs, the goal is to keep hormone levels at a normal level. In order to do this, your child may need to take a daily form of cortisol medication, such as: 

  • dexamethasone
  • fludrocortisone
  • hydrocortisone

Girls may need hormonal therapy throughout their lives, which is given as a daily pill.

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