Abstract

Author present a case of persistent mullerian duct syndrome with transverse testicular ectopia with bilateral hernia uteri inguinalis in a 17 years old boy. This is a rare congenital anomaly and an uncommon form of male pseudo-hermaphroditism characterized by the persistence of well-developed Mullerian duct structures in an otherwise normal male with a 46 XY karyotype. Transverse testicular ectopia (TTE) is one of the rarest forms of testicular ectopia. In this condition, both testes are located on one inguinal side and the opposite inguinal canal and scrotum are empty. TTE associated with PMDS is much rarer. The exact cause of PMDS is uncertain. However, it is thought to result from the failure of synthesis or release of Mullerian inhibiting factor (MIF), the failure of end organs to respond to MIF, or a defect in the timing of the release of MIF. Patients with PMDS present with unilateral or bilateral cryptorchidism and an inguinal hernia containing a fallopian tube, uterus and testis. The case was diagnosed on doing diagnostic laparoscopy followed by bilateral inguinal herniorrhaphy and right Orchidectomy.

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