Transverse testicular ectopia with persistent Mullerian duct syndrome: an operative eureka

Transverse Testicular Ectopia (TTE) is a rare entity in which both testes descend on the same side, and can be found in ectopic locations. When present with Persistent Mullerian Duct Syndrome (PMDS), a yet rarer entity, the persistence of Mullerian duct derivatives i.e. fallopian tubes, uterus, cervix and upper two-thirds of vagina occurs alongside testicular ectopia. There have only been about a hundred and fifty reported cases of TTE; a fifth of these accompanied by PMDS.

Two middle-aged male patients presented with two separate complaints of inguinoscrotal swellings. In both patients, ultrasonography showed a hernial defect protruding into the scrotum on one side and the testis absent on the contralateral side. During hernia surgery, Mullerian duct remnants were found. Diagnosis of TTE with PMDS was established. Bilateral orchidectomy was done and Mullerian derivatives were excised.

There is controversy over the treatment of TTE with PMDS. Some authors,in addition to hernia repair, advocate the preservation of Mullerian structures because of risk to injury to vas deferens while others advocate resection of these structures due to risk of carcinoma. In pediatric patients, orchidopexy should be done to preserve fertility.However, in the older age group, orchidectomy should be done due to an increased risk of testicular carcinoma.

TTE should be suspected in cases of unilateral inguinal hernia with contralateral undescended testes. Orchidectomy is recommended in patients older than 12 years old, otherwise, orchidopexy should be done. No Mullerian duct remnants should be left in situ.