Indirect inguinal hernia with uterine tissue in a male: A case of persistent Mullerian duct syndrome and literature review

Abstract

A 32-year-old male presenting with a direct inguinal hernia was found to have uterine tissue extending through the inguinal canal, warranting a diagnosis of persistent Mullerian duct syndrome (PMDS). PMDS is an extremely rare form of internal male pseudo-hermaphroditism in which female internal sex organs, including the uterus, cervix and proximal vagina, persist in a 46XY male with normal external genitalia. The condition results from a congenital insensitivity to anti-Mullerian hormone, or lack of anti-Mullerian hormone, leading to persistence of the female internal sex organs in a male. Clinically, this condition is associated with cryptochoridism. Controversy persists regarding the appropriate treatment of PMDS, since resection of the remnant structures is associated with potential morbidity, but retention risks development of occasional malignancies. We review the literature and discuss various aspects of pathophysiology, diagnosis, and management of PMDS.

Fig. 1

Tubulo-muscular structure shown to the right of the spermatic cord originating from the internal inguinal ring. Class B persistent Mullerian duct syndrome characterized by presence of fallopian tubes and uterus in one hemiscrotum and an undescended testis tethered to fallopian tube on the contralateral side.

Fig. 2

Flexible endoscopic view of tubular structure demonstrating a smooth musuclar structure extending into the pelvis.

Fig. 3

Excised uterine tissue shown alongside Adson forceps.

Fig. 4

The central lumen is lined by pseudostratified monomorphic columnar cells with light eosinophilic cytoplasm, elongated nuclei and small nucleoli. Occasional cilia are seen on epithelial cells. Stroma surrounds the epithelial component. The morphologic features are consistent with a uterus with poorly developed endometrium and myometrium.

Fig. 5

The central lumen is lined by pseudostratified columnar cells with muscle bundles seen deep to this layer. The morphologic features are consistent with a uterus with poorly developed endometrium and myometrium.