Müllerian Anomaly with Hematosalpinx Presenting as Amenorrhea and an Adnexal Mass

Abstract

Amenorrhea is a common symptom that generally presents to the gynecologist for evaluation and therapy during adolescence. Its presence, in combination with an anomaly of the vagina and a pelvic mass, suggests many possible etiologies whose diagnosis and management is critical in the young patient. Here we present such a patient who was treated with a transabdominal hysterectomy and left salpingectomy. A 22-year-old nulliparous female presented with amenorrhea, pelvic pain, and dyspaurenia. Pelvic examination, ultrasonography and magnetic resonance imaging (MRI) showed a left adnexal mass suspicious for an endometrioma or hematosalpinx but further definition was difficult. Surgery confirmed a 10 × 6 cm "chocolate" fluid-filled fallopian tube and a uterine remnant, without a cervix. The vagina was hypoplastic and ended in a blunt pouch. Both ovaries appeared normal. Pathological evaluation of the fallopian tube demonstrated "chocolate" fluid and no tubal endometriosis. The myometrium and endometrium were unremarkable and there was no evidence of endometriosis in the pelvis. Primary amenorrhea secondary to vaginal hypoplasia can, in rare instances, be accompanied by congenital uterine and fallopian tube dysgenesis. The pelvic mass that was seen was of concern because even with advanced radiological studies, and ultrasound, it could not be determined if the patient had a patent cervix or if the uterine tissue could be joined to the vagina to allow normal attempt at fertility. Thus, it was critical to have reviewed the potential surgical options with the patient and her family prior to any intervention. This case is unique in its late presentation, its impact on the patient's reproductive potential, and the possible surgical management of the pelvis.