Persistent Mullerian Duct Syndrome with Transverse Testicular Ectopia Presenting as an Irreducible Inguinal Hernia

 

Abstract

Jordan was the first who described persistent müllerian duct syndrome (PMDS) associated with transverse testicular ectopia (TTE) in 1895 [1]. The clinical presentation is a unilateral undescended testis from birth and an inguinal hernia on the contralateral hemiscrotum. We present the case of a newborn male, with history of bilateral cryptorchidism, presenting with a recent swelling in the left inguinal region. On examination the neonate had a left inguinal hernia and an empty right hemiscrotum. An inguinal approach revealed a hernia sac containing female-resembling gonads by intraabdominal approach. Hernia was repaired and biopsies taken from both gonads. A hormonological screening combined with histology results set the diagnosis of PMDS. Based on the current literature, we decided a second surgical intervention a year later so as to perform orchedopexy and müllerian remnants excision. A two-staged approach can prevent injuries of important structures such as vas deferens and resection of female gonads decreases the risk of malignancy.

 

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