Acute labio-scrotal pain in a patient with ovotesticular syndrome. Case report

Ovotesticular syndrome (OTS) belongs to the group of disorders of sex development (DSD). We present a case of a patient with OTS presenting with acute labioscrotal pain. A surgical exploration was indicated, and hemorrhage was identified. A gonadectomy was performed and the final pathology report revealed an ovotestis with a bleeding follicle, normal ovarian parenchyma and atrophic testicular parenchyma. After reviewing the literature there is scarce information on this complex topic, but conservative management could be an option if the risk of a gonadal malignancy is low.