An Uncommon Finding: Ovotestes in a True Hermaphrodite

Abstract

A true hermaphrodite is an individual who is found to have both testicular and ovarian tissue; ovotestes is the most common gonad. True hermaphroditism is a rare intersex disorder reported in less than 500 patients. This patient population typically presents with ambiguous genitalia, hypospadius, and often an associated bifid scrotum; 90% go on to develop gynecomastia. Although some patients are diagnosed in the newborn period, only 20% are diagnosed prior to the age of 5 years. In this case report, the authors describe these classic findings and the use of advanced ultrasound technology, which contributed to this rare diagnosis.