The Newborn With a Suspected Difference of Sex Development

The care of patients with difference(s) of sex development (DSD) is rapidly evolving, and this is especially true for the care of newborns with a suspected DSD. This review presents a paradigm for care, with an emphasis on a family-focused, shared decision-making approach that seeks to de-escalate the sense of "social crisis" that may arise when a baby is born with concern for a DSD. In addition to providing education and psychosocial support, tasks for the neonatal team include monitoring for hypoglycemia, determining the sex-chromosome complement, testing for congenital adrenal hyperplasia (and if appropriate, initiating treatment), and starting the diagnostic evaluation, recognizing that this evaluation and care will extend beyond the neonatal period and continue in the outpatient setting. Surgery is rarely indicated in the neonatal period, but parents may seek counseling about future surgical options; this counseling should avoid leading parents and families to believe that surgery can "fix" their child's DSD. A key component of care is connecting patients and families with clinicians who have the expertise to provide consultative and ongoing care-ideally a multidisciplinary team that includes mental-health, medical, and surgical expertise-and with written and online resources as well as support groups.