Transverse Testicular Ectopia: A Rare Presentation with Persistent Müllerian Duct Syndrome

Abstract

Undescended testes can be found in the inguinal channel or in the abdomen. Rarely, undescended testes can present with transverse testicular ectopia (TTE) and very rarely, with residual Müllerian duct (MD) structures. This latter presentation is called persistent MD syndrome (PMDS). PMDS is mostly discovered during surgery for inguinal hernia or cryptorchidism. TTE is a rare congenital anomaly in which both testes descend through a single inguinal canal. Patients with TTE present with symptoms of unilateral cryptorchidism and contralateral inguinal hernia. Herein, we report two TTE cases: one associated with PMDS and the other having only cross ectopia. For patients with inguinal hernia and cryptorchidism associated with TTE, PMDS should be kept in mind and radiologic evaluation with ultrasonography or magnetic resonance imaging of the genitourinary system and karyotyping are recommended. Radiologic evaluation can be helpful in the diagnosis of TTE; however, it cannot diagnose the malignancy itself. Laparoscopy is very useful for both diagnosis and treatment of TTE.

A novel mutation of AMH in three siblings with persistent Mullerian duct syndrome

“…To the best of our knowledge, 21 PMDS cases have been reported from our country until now; transverse testicular ectopia and malignancy have been observed in 11 and 8 of these cases, respectively (15,16) The incidence of malignancy in an ectopic testis with PMDS is 15%, similar to the rate in abdominal testes in an (17). Embryonal carcinoma, seminoma, yolk sac tumor, and teratoma have been reported in patients with PMDS (6).…”

Transverse testicular ectopia with Müllerian duct remnant in an incarcerated congenital inguinal hernia – a case report

“…Moreover; these testes are subjected to a higher risk of malignant transformations arising from Müllerian remnants to squamous cell carcinoma, adenocarcinoma and papillary cystadenocarcinoma. Hence, long term follow-up for tumors is required [9].…”

Laparoscopic management of Müllerian duct remnants in the paediatric age: Evidence and outcome analysis

“…[ 8 ] MDR is associated with hypospadias or intersex disorders in 90% of cases, TTE in approximately 20% of the cases and bilateral cryptorchidism in about 10%. [ 2 4 9 ]…”

“…Assuming cancer occurrence is binomially distributed, we may formulate that we are 90% confident that between 3.1% and 8.4% of males with PMDS will develop a Müllerian malignancy. [ 1 9 ] In literature, three reported cases were found about the malignant alteration of MDRs. Theil et al .…”