Case notes
A 25-year-old woman with primary amenorrhea reported that a painful bulge intermittently appeared in her left groin. Her medical history also included known congenital left renal agenesis, tricuspid atresia, pulmonary stenosis, and a hypoplastic right ventricle that had required repair. Physical examination revealed Tanner V thelarche and adrenarche, a 1-cm vaginal dimple, and a left inguinal hernia. The patient’s karyotype was 46, XX.
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Pelvic ultrasound showed a right ovary. No left ovary, uterus, or vagina could be seen. Magnetic resonance imaging disclosed a right uterine remnant without functional endometrium and a normal right ovary. The left ovary appeared to be ectopically located; it was 5 cm below the inguinal ring, in the patent processus vaginalis ( Figure 1 ) . Diagnostic laparoscopy confirmed the presence of a normal right ovary and a small, nonobstructed, right uterine horn.
The left ovary was adjacent to the internal inguinal ring, along with the source of the hernia: a chronically incarcerated left uterine remnant. The left uterine horn was partially reduced laparoscopically ( Figure 2 ) . The left round ligament, utero-ovarian ligament, and uterine vessels were isolated and ligated laparoscopically. The hernia sac was opened, and the left uterine remnant was mobilized and removed through the left herniorrhaphy incision. Pathology confirmed myometrial tissue without endometrial stroma or glands. The patient’s pain completely resolved.
