Intersex conditions—also known as disorders of sex development (DSD)—occur when a child's internal and external reproductive organs develop abnormally. The defect lies within the chromosomes, gonads, or anatomical sex. In some cases, a person may have male chromosomes (XY) but the genitals appear female or ambiguous. In other cases, a person with female chromosomes (XX) may have external genitals that look male.
This can pose a problem for treatment since a clear differentiation must be made regarding social gender, sexual orientation, and biological sex. While surgery and other medical treatments are available for intersex conditions, but can be delayed to give the child a chance to participate in decisions about their gender.
Complexities of Treatment for Intersex Conditions
Many of the symptoms of intersex conditions are physical—such as ambiguous genitalia, or a mismatch between the sexual identity of the internal and external genitals—but these conditions also have a strong psychological component. As a result, treatment ideally involves a team of health care professionals who have experience in treating intersex conditions.
In the past, treatment involved assigning a gender to the child as early as possible. This was often based not on the gender determined by the chromosomes, but on the external genitals. Tissue from the other gender—such as testicular or ovarian—was removed.
Recently, however, health professionals have begun to take into account issues other than physical appearance. Experts realize that gender is affected by many factors, including psychological, chromosomal, hormonal, behavioral, and neural. Although it is easier to reconstruct female genitalia, choosing the “easiest” surgical route is not always the best choice.
Decisions concerning treatment typically center around four major factors: first, the child's diagnosis and its causes; second, gender assignment or deciding if a child will be considered male or female; third, when genital surgeries or treatments may be appropriate; and four, how and when information about the child's medical condition will be disclosed to the child.
Treatment Options for Intersex Conditions
Gender Assignment
Because children with intersex conditions often have both male and female organs, parents may be faced with the decision of gender assignment. A physician will conduct extensive testing concerning hormones and anatomy. A medical professional, such as a psychiatrist, can inform parents how gender identity typically develops in children with a similar diagnosis. A psychiatrist also can offer advice on how parents can approach raising a child with gender assignment. In rare instances, children are not diagnosed until later in childhood or adolescence. In this instance, parents can work with the multidisciplinary team to determine desired treatment options, if any.
Hormone Therapy
Another approach to managing intersex conditions is using hormone therapy to compensate for the sex hormones a child may not be producing.
For example, drugs are prescribed for individuals with congenital adrenal hyperplasia (CAH), the most common cause of disorders of sexual development. In this condition, the adrenal glands do not produce enough of the hormones cortisol and aldosterone. As a result, children with CAH require medications called glucocorticoids—such as hydrocortisone—to boost cortisol and aldosterone levels. These drugs also reduce the overproduction of androgens (male hormones), which cause male characteristics to form in females with CAH.
Hormone therapy may also be considered if the child is raised male and are required if the child is raised female and has had the gonads removed.
Surgery for Intersex Conditions
Most surgical treatments to address DSD as a baby are related to a possible medical emergency, such as when the abnormally formed tissue has cancerous cells present. In such instances, surgery to remove malignant or cancerous tissues may be indicated.
Physicians and parents can work together to determine if surgeries to reinforce gender assignment at a young age are appropriate. Physicians may recommend waiting until a child is of an age to decide if cosmetic surgeries are desired.
In females with male-looking genitalia, surgical treatment may be done to reconstruct the labia, reduce the size of an abnormally enlarged clitoris, or increase the length or opening of the vagina. In males with female-looking genitalia, surgery can be performed to remove an underdeveloped ovary. Additionally, since the urethra usually exits in some location other than the tip of the penis, reconstructive surgery for the condition (hypospadias) may be required. Reassignment of the gender to female is also considered in certain cases.
Outlook of Treatment for Intersex Conditions
The outlook of treatment for intersex conditions depends upon the type of disorder of sexual development and the course of treatment. Many times, it is easier to treat the physical symptoms than the psychological issues that accompany having a intersex condition. For this, long-term counseling is often provided.
With congenital adrenal hyperplasia, in particular, proper surgical and medical treatment can produce a good outlook. Still, complications from CAH include reduced height, decreased fertility, and early death if not prescribed appropriate amounts of glucocorticoids.
References
Donohoue PA. (2011). Disorders of sex development (intersex). Nelson Textbook of Pediatrics, 19th ed.
Ocal, Gonul. (2011). Current Concepts in Disorders of Sexual Development. Journal of Clinical Research and Pediatric Endocrinology. 3(3). 105-114.
Vidal I, Gorduza DB, Haraux E, et al. (2010). Surgical options in disorders of sex development (dsd) with ambiguous genitalia. Best Pract Res Clin Endocrinol Metab 24(2):311-24.
Allen L. (2009). Disorders of sexual development. Obstet Gynecol Clin North Am 36:25-45.
Accord Alliance. (2008). Clinical Guidelines for the Management of Disorders of Sex Development in Childhood.
Hughes, IA. (2008). Disorders of Sex Development: A New Definition and Classification. Best Practices in Research and Clinical Endocrinology and Metabolism. 22(1). 119-134.
