Abstract
Disorders of Sex Development (DSD) refers to biological differences between phenotypic sex, gonad morphology and chromosomal material. A full-term infant was born with Hypoplastic Left Heart Syndrome (HLHS) and ambiguous genitalia. Karyotype revealed 46XX with no evidence of Y chromosome or SRY. Biopsy of both gonads revealed testicular tissue. Parents decided to rear the child as male. The child underwent extensive cardiac repair and is currently awaiting decision regarding urological repair. This case emphasizes the need for multidisciplinary care in the management of children with DSD associated with other complex medical conditions to ensure a timely diagnosis and multi-system, patient-centered treatment approach.
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