Gender assignment in patients with disorder of sex development

Abstract

Purpose of review: To examine the sex assignment in patients with atypical external genitalia, a particularly challenging situation, especially when the genital appearance is not compatible with the sex chromosome.

Recent findings: The most important factors that influence sex assignment include the definite diagnosis, genital appearance, surgical options, potential for fertility, risks of gonadal malignancy and, finally, the perception of the patients and their parents. Full disclosure and complete involvement of the parents in making decisions concerning gender assignment and/or genital surgery must be part of the basic medical care for children with disorder of sex development.

Summary: Patients with disorder of sex development should receive long-term care provided by multidisciplinary teams in centers of excellence with ample experience in the management of this disorder.