General Medical Information

Sexual Differentiation Disorders

Sarah E. Herbert

Children born with sexual differentiation disorders have always presented medical professionals with difficult decisions, particularly at the time of diagnosis. Optimal management requires a team effort, which includes appropriate medical and mental health specialists. Parents require education and support, not just in the neonatal period but throughout the child’s early years and adolescence. Likewise, the children need developmentally appropriate ongoing support and counseling from the medical professionals treating them and their parents. Problems may arise in the child’s or adolescent’s self-image, relationships with peers, and sexual relationships. More research needs to done to determine if children with sexual differentiation disorders have a higher rate of mental health problems than other children with chronic medical problems or other children in general. Mental health intervention is appropriate to monitor the child’s and family’s adjustment.

Management of children with sexual differentiation disorders has become controversial in recent years. There has been criticism about early surgical intervention and the cultural assumptions about gender that underlie some of the decisions regarding sex assignment. Recommendations have been to assign gender early but to defer surgical intervention when possible until the child or adolescent is older. Parents and medical professionals have the opportunity to see if gender assignment has been appropriate, and the child or adolescent is old enough to participate actively in her care and give assent or consent for treatment.

Much more systematic long-term follow-up needs to be undertaken for children born with sexual differentiation disorders. Outcome should be viewed in terms of appropriateness of gender assignment, social and psychological adaptation during middle childhood and adolescence, and adjustment to adult social and sexual roles.