Ovarian Teratoma in a Nonverbal Adult With Rett Syndrome

Mature cystic ovarian teratomas, also called dermoid cysts, are the most common ovarian neoplasms in women, especially those of reproductive age. While the general clinical approach and treatment of teratomas do not differ in a patient with a concomitant diagnosis of Rett syndrome, the presentation, surgical preparation, and clinical course can be influenced by the pathophysiology of the condition. We report the case of a 35-year-old nonverbal woman with Rett syndrome presenting with symptoms of abdominal pain, constipation, abdominal distension, nausea, and vomiting. Computed tomography of the abdomen and pelvis revealed a mass in the left lower quadrant, originating from the left adnexa. During an exploratory laparotomy, a large cystic mass was identified in the region of the left adnexa with associated adnexal torsion, and the patient underwent a left salpingo-oophorectomy. Microscopic examination of the adnexal mass confirmed a diagnosis of mature ovarian cystic teratoma. This report adds to the limited literature on gynecologic pathology in Rett syndrome.