An unusual cause of duodenal obstruction: mesenteric fibromatosis in a patient with type I Mayer-Rokitansky-Kuster-Hauser syndrome.

ABSTRACT

Patients with mesenteric fibromatosis (MF) are clinically asymptomatic, with little or no focal symptoms until later in their course, at which time they complain of pain, abdominal discomfort, constipation, vomiting, abdominal mass, weight loss, and symptoms due to organ compression. Generally, it occurs as an abdominal mass but may also present in many different ways. In some cases, trauma, previous abdominal surgery, and hormonal stimulation (such as estrogen) may play a role in onset of this neoplasm. Patients with Mayer-Rokitansky-Kuster-Hauser syndrome present primary amenorrhea and may have some other anomalies, including hearing defects, heart defects, skeletal deformities, and genital neoplastic diseases. We diagnosed duodenal obstruction due to MF in a patient with type I Mayer-Rokitansky-Kuster-Hauser syndrome.