A Complicated Hematometra and Fallopian Tubal Occlusion in Uterine Didelphys With Unilateral Cervical Atresia

Abstract

Uterine anomalies are uncommon Mullerian malformations without known etiology. Many hypotheses have been put forward to explain these anomalies such as the involvement of genetic, environmental, and pharmacological issues [1]. The incidence is reported to be between 0.5–5.0% of all women [2]. However, a congenital uterine anomaly involving cervical atresia is extremely rare. We report a patient, diagnosed with a uterine anomaly, who underwent a primary laparotomy for unilateral endometrioma without resectioning of the rudimentary horn. Postoperatively, the patient received 6 months of gonadotropin-releasing hormone agonist (GnRHa) treatment as a conservative therapy. After completion of the GnRHa therapy, endometriosis recurred and a hematometra developed. This occurred after her menstrual cycle had resumed for 3 months post GnRHa treatment. The patient underwent a second laparotomy and the rudimentary horn was resectioned. Recovery after the operation was uneventful. The final diagnosis of the uterine anomaly in this patient was uterine didelphys with unilateral cervical atresia. A 23-year-old nulligravid woman had complained of dysmenorrhea since menarche at the age of 14 years in 1999 and this had been partially relieved with medical management. At the age of 22 years during April 2007, the patient presented with acute abdominal pain in the left lower quadrant, in which an abdominal mass was palpable. Pelvic ultrasonography by a primary gynecologist revealed a 10 × 10 × 10 cm complex mass of the left adnexa with multiple cysts and septa. A bimanual pelvic examination showed a single vagina and single cervix. Computed tomography (CT) revealed a multicystic mass associated with the left ovary, possibly endometrioma, and two uterine horns indicating the possibility of a bicornuate uterus (Figures A and B). The right kidney was enlarged and the left kidney was absent. An elevated serum CA-125 level of 8,640 U/mL was noted. The patient underwent exploratory laparotomy by the primary gynecologist. The findings included a 10 cm left ovarian endometrioma, severe endometriosis involving the cul-de-sac and the pelvis, as well as layering of hemosiderin throughout the uterine horns, the pelvis and the omentum. Fibrous adhesions involving the sigmoid colon, small intestine and pelvic sidewall were also noted. The right uterine horn and the right fallopian tube were normal in appearance; a right ovary could also be identified. The uterine anomaly was thought to be a bicornuate uterus. Left partial oophorectomy and lysis of the adhesions were performed. The pathological findings revealed ovarian endometrioma and endometriotic cysts that had endometrial epithelium and hemosiderin-laden macrophages. Postoperatively, the patient recovered quickly and was prescribed 6 months of GnRHa therapy.