A unilateral functioning uterine anlage with Müllerian duct agenesis.
The case reports of two female adolescents with congenital absence of the Müllerian ducts and a unilaterally enlarged uterine anlage containing functioning endometrial tissue are presented. The clinical manifestations may vary depending upon the delay in diagnosis after the onset of endometrial shedding within the anlage. A high index of suspicion and proper diagnostic evaluation may prevent unnecessary hospitalization. In our experience, prompt removal of the functioning uterine anlage affords complete relief of the symptoms.
D000293:Adolescent; D005260:Female; D005836:Genitalia, Female / Q000002:abnormalities* / Q000196:embryology / Q000473:pathology; D006801:Humans; D010146:Pain / Q000209:etiology; D014599:Uterus / Q000002:abnormalities / Q000196:embryology; D014621:Vagina / Q000002:abnormalities / Q000196:embryology