Persistent Mullerian duct syndrome and bilateral cryptorchidism

Abstract

Introduction: persistent Müllerian duct syndrome (PMDS) is a rare condition occasionally encountered in men with normal phenotype but with influencing internal sexual male development. This disorder is characterized by the presence of female reproductive structures such as cervix, fallopian tubes, uterus and upper part of vagina in normal males. PMSD is commonly diagnosed incidentally after testis examination for cryptorchidism or during any pelvic surgery. We report a case of PMDS along bilateral cryptorchidism that present with acute abdomen. Case presentation: A 16-year-old boys presented with abdominal pain and history of appendectomy one month ago and reoperation with collection diagnosis. In examination bilateral undescended testis (UDT) was discovered. An abdominal and pelvis CT scan with and without intravenous and oral of contrast material was performed, which showed a blind-ending, fluid-filled tubular structure arising just posterior to the prostate and extending into the both sides of the urinary bladder. Total excision of the uterus and bilateral fallopian tubes was performed. The patient was prepared for orchidopexy at another time. Conclusions: PMDS is a challenging condition to diagnose. Isolated UDT is the main presenting features of PMDS. Malignant transformation of the testis is the main risk facing those patients. Recent studies have also suggested the possibility of malignancy of PMDS components. Though it is rare, every surgeon should be fully prepared to manage this anomaly especially when operating upon UDT or cryptorchidism.

Keywords

• Undescended testis
• Orchidopexy
• Acute abdomen