True hermaphroditism

Objective: True hermaphroditism is a rare cause of atypical genitalia which presents significant diagnostic and management challenges. We present the clinical and laboratory findings and management of four patients with true hermaphroditism. Methodology: Case studies from a teaching hospital and literature review. Results: All four patients had atypical genitalia identified at birth. All had a palpable gonad, only one of which was palpable at birth. Three patien ts were 46XX (SRY- ve) and one 46XY (SRY + ve). Three patients were raised as females (two 46XX and one 46XY) and one as a male. All four patients wer e found to have an ovotestis present. Conclusions: The management of true hermaphroditism is controversial and re quires a multidisciplinary approach. It has many implications for both the parent and child. We discuss the issues involved for the patients and their parents.