Treatments for Mixed Gonadal Dysgenesis in Children

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At Boston Children's Hospital, the first step in treating your child's is to determine the appropriate sex for your child. This is done as a partnership between your team of health care providers and your family. This determination will inform the course of treatment. You, as parents, will be involved every step of the way.

How is a gender assigned?

Gender assignment is important for treatment purposes as well as the emotional well being of the child as he grows older.  Once you and your child's doctor decide on the appropriate gender for your baby, it's a matter of determining what treatments are most beneficial.

Typically, the decision on whether to raise a child with MGD as male or female is based on the child's anatomy. If raised male, the child typically makes enough testosterone and wouldn't need additional hormone therapy, which is an additional benefit.

Raising a child with MGD as female is a bit more complicated. Because she has a testis that's producing testosterone and she doesn't have a normal ovary, surgery will need to be done to remove the testis and the girl will need to be on hormone replacement therapy to receive adequate estrogen.

What treatments are available?

The medical treatment options for DSDs may include:

Surgery

  • Regardless of what sex is assigned to a child, corrective surgery is usually needed to remove or create the appropriate sexual organs.  To avoid any risks associated with anesthesia, corrective surgery usually happens after a child is 6 months old.
  • If the child is raised as male, based on having one decent testis and a decent phallus, it's likely that the female ductal structures on the opposite side would need to be removed.
  • The decision to raise a child as female might be made if the phallus is very small and the doctor believes that the child would function better as a girl. In this case, surgery would be needed to remove the testis

Hormone replacement therapy (HRT)

  • In terms of lifelong management of MGD, 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

Will children with MGD receive hormone therapy throughout their whole lives?

It depends on the gender of the child.  A male child with a testis that's functioning well enough to produce male hormones won't need hormonal therapy. Children reared as females would need hormonal therapy throughout their lives because they don't have a normal ovary.

How do you determine that my child's hormone levels are normal and he's progressing as he should be?

For males, blood can be tested to make sure testosterone levels are normal. A male might not need hormone therapy if his testis functions adequately. A doctor will also perform routine clinical examinations to see how a boy is maculinizing. The questions they will want to answer include: Is the penis growing? Are the existing testes growing? Are they developing pubic hair?

Since females can't produce their own estrogen, doctors will make sure she receives hormone replacement therapy throughout her life. Blood tests can establish that a girl's estrogen levels remain normal.

Coping and support

MGD can be emotionally difficult for parents and children alike. Fortunately, there is a lot of support and help available to you.

  • At Boston Children's Hospital's Gender Management Service (GeMS) Clinic we help families deal with the psychosocial issues of raising infants, children, adolescents and young adults with disorders of sexual differentiation (DSDs). Our expert physicians and clinical staff work closely with your child and your family to find the treatment that works best for everyone involved
  • Children's Coping Program helps children who are being treated on an outpatient basis at the hospital—as well as their families—understand and cope with their feelings about:
    • being sick
    • facing uncomfortable procedures
    • handling pain
    • taking medication
    • preparing for surgery
    • changes in friendships and family relations
    • managing school while dealing with an illness
    • grief and loss

Visit the Behavioral Medicine and Coping Program page or call us at 617-355-6688 to learn more.

Other sources of support include:

The Children's For Patients and Families website offers information on the wide array of support services available to families at Children's:

  • Parent to parent: Want to talk with someone whose child has also been diagnosed with MGD? Children's can put you in touch with other families who have been through the same experience you and your child are facing.
  • Faith-based support: A diagnosis of hypospadias may affect how and when your son is circumcised. If this is a religious consideration for you, or if you are simply in need of spiritual support, we will help connect you with the Children's chaplaincy.
  • Social work and mental health professionals: Our social workers and mental health clinicians have helped many other families in your situation. We can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties.

For teens

Adolescence can be stressful—even for physically healthy teens. Having a condition like MGD during adolescence further complicates life for teenagers.

  • Support for teen boys: As a boy with MGD reaches adolescence, he may look and feel different from other males his age. Young Men's Health (YMH) is a website that provides health information for teen boys and young men.
  • Support for teen girls:  Girls with MGD can experience their own set of difficulties when they enter puberty and may exhibit more masculine behavior than other girls their age. The Center For Young Women's Health offers the latest gender-specific information about sexual and emotional health.
  • The Medical Coping Team at Boston Children's Hospital works with teens and their families to help them adjust to the stress caused by chronic illness. Our experienced team of pediatric psychologists, psychiatrists and other mental health professionals provide effective, compassionate evaluation, education, counseling and therapy to help teens cope.

 

Parents consider treatment to delay son's puberty
Norman Spack, MD, Associate Physician in Medicine, speaks with NPR's “All Things Considered” about gender identity treatment on National Public Radio. Read part one of the article on gender identity issues
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