A Central Gartner's Duct Cyst, Unilateral Renal Agenesis, Didelphic Uteri and Paratubal Cyst Presenting as Pain and Fever: A Case Report and Review of the Literature

Background: A Gartner’s duct cyst (GDC) is a benign vaginal cystic lesion that arises from the mesonephric duct. GDC are usually small asymptomatic cysts that occur along the lateral walls of the vagina, but may enlarge. The ureteral bud also arises from the mesonephric duct. If the ureteral bud is located high or low with respect to the metanephric blastema, a dysplastic or hypoplastic kidney may result. The rare combination of GDC and unilateral renal agenesis has been reported in the literature, half of which are also associated with m€ ullerian duct abnormalities. Case: A 13 year-old virginal pre-menarchal female with normal secondary sexual maturation was evaluated by her pediatrician for a twoday history of left lower abdominal pain and fever. A pelvic sonogram showed a 6 cm cystic structure in the pelvis, a “normal” uterus, two normal ovaries and left renal agenesis. She was referred to the ED because of the fever and pelvic mass. On external genital examination, the patient’s hymen was patent and a 6 cm cystic mass was palpable along the anterior vagina wall. Laboratory workup included a negative pregnancy test and normal WBC. A CT scan showed a didelphic uterus, left renal agenesis, compensatory enlargement of the right kidney, and a 6 cm cystic mass in the vesicouterine space. The patient continued to experience low grade fevers in the ED and was admitted for IV antibiotics. The following day she defervesced but continued to complain of severe LLQ pain. She was scheduled for an examination under anesthesia and any other indicated procedure. Under general anesthesia, the anterior vaginal wall cystic mass persisted, and two cervices were palpated in the vaginal fornices, confirmed by vaginoscopy. By hysteroscopic visualization, the left uterine cavity with single tubal ostia was seen. The right cervical canal deviated markedly, so the right endometrial cavity was not visualized. Because of the patient’s complaint of LLQ pain, a diagnostic laparoscopy was done which documented the two nondilated divergent uteri, two normal ovaries, and a 10 mm torsed, necrotic paratubal cyst in the LLQ which was removed and submitted for pathology (Fig. 1). A six cm subserosal mass was noted between the bladder and vagina, simultaneous vaginal palpation and laparoscopic visualization revealed that the lesion was likely in the vesicouterine space. Cystoscopy demonstrated normal bladder mucosa with a single right ureteral orifice. The patient’s pain resolved following removal of the necrotic, torsed paratubal cyst. An MRI documented presence of a large Gartner’s duct cyst (Fig. 2). Comments: Our patient provides an example of a rare constellation of diagnoses: a Gartner’s duct cyst, didelphic uteri, unilateral renal agenesis, and torsion of a paratubal cyst. Following her full evaluation only the small torsed paratubal cyst was responsible for her symptoms. It is important to consider the possibility of Mullerian, renal and urologic abnormalities when evaluating a patient with a pelvic mass, and consider that the presence of a mass may be a red herring in the patient’s presentation.