Persistent Müllerian duct syndrome with torsion of a transverse testicular ectopia: first reported case

Persistent Müllerian duct syndrome with transverse testicular ectopia is a rare pathologic association. A 7-month-old boy was admitted to the emergency department with a right testicular swelling. Physical examination revealed a tender right testis, the upper limits of which could not be palpated. The left testis was impalpable. An ultrasound revealed a normal right testis with an evident blood flow, overcome by a similar mass but avascular. At surgery, the right hernial sac contained both testes with an infantile uterus and fallopian tubes. The left testis was viable after manipulation. Proximal-salpingectomy with corporeal hysterectomy was performed to allow bilateral scrotal orchiopexy.