Mayer-Rokitansky-Kuster-Hauser Syndrome Presenting as an Inguinal Mass and Hernia in the Female Patient

Abstract

In Brief Objectives: Complete mullerian agenesis is the second most common condition usually diagnosed during a work-up for primary amenorrhea. A detailed gynecologic history and pelvic examination are the foundations for establishing the diagnosis. Methods: An 18-year-old nulligravida presented to the General Surgery Service complaining of right-sided lower abdominal pain. Upon dissection of the right inguinal canal, it was noted that the hernia sac contained the right ovary and fallopian tube. An intraoperative consult to the Gynecology Service was obtained. The hernia was reduced and the ovary returned to the pelvis. Pelvic examination under anesthesia revealed a severely shortened vagina. A diagnostic laparoscopy was performed revealing complete mullerian agenesis. Postoperative evaluation included a normal karyotype and normal intravenous pyelogram (IVP). Results: The differential diagnosis of inguinal masses in women should include mullerian tract abnormalities. Conclusions: Complete mullerian agenesis is an uncommon gynecologic condition, occurring in one out of every 4000 female births.1 This condition is usually discovered during a work-up for primary amenorrhea and is the etiology of 15% of cases of primary amenorrhea.2,3 The likelihood of diagnosis is enhanced after careful examination reveals a shortened vaginal canal absent the cervix and uterus.4 Inguinal hernias are not uncommon in women. Usually they are a consequence of congenital abnormality in the abdominal wall, which leads to formation of a hernia sac containing subcutaneous tissue and small bowel. When inguinal hernias contain ovarian and fallopian tissue, there are often associated female genital tract abnormalities.5 Consider mullerian tract abnormality in the differential diagnosis of inguinal mass in women.