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Hematometra in a Patient With Cornelia De Lange Syndrome

BACKGROUND: Hematometra is usually the result of developmental anomalies or may be secondary to cervical obstruction. Abnormal uterine contractile function (atony) would be an uncommon cause of hematometra. CASE: An 18-year-old female with Cornelia De Lange syndrome and abdominal pain was found to have a hematometra on ultrasound examination. On pelvic examination, her cervical canal was patent and was easily dilated, but the hematometra did not drain until suprapubic pressure was applied. Two weeks postoperatively, pelvic magnetic resonance imaging showed a markedly thinned uterine myometrium and a recurrent hematometra, prompting the decision to perform a hysterectomy. CONCLUSION: Hematometra in a patient with Cornelia De Lange syndrome may be the result of abnormal uterine contractile function.