Uterus Didelphys with Obstructed Hemivagina and Multicystic Dysplastic Kidney

Abstract

Müllerian malformations include a broad range of anomalies, resulting from the incomplete formation and/or differentiation of the Müllerian ducts. The uterus didelphys with obstructed hemivagina represents the result of a lateral nonfusion of the Müllerian ducts with asymmetric obstruction, and it is almost always associated with renal agenesis. We report a case of incidental discovery of this anomaly in a 12-year-old girl during a routine renal ultrasound. A right multicystic dysplastic kidney had been detected in the prenatal and neonatal period with no evidence of uterine anomaly. Postnatal ultrasound examinations performed regularly had never detected either right renal tissue or the uterine anomaly before menarche. Hematocolpos after menarche made uterine anomaly detection easier. Magnetic resonance imaging performed to evaluate the uterine malformation detected a dysplastic right kidney. We review the present knowledge and recommendations for the diagnosis and treatment of this anomaly, and we emphasize the use of magnetic resonance imaging not only for the evaluation and classification of Müllerian malformations, but also for the detection of the persistence of renal tissue missed with other studies.

Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review

“…In three cases (42,44,70) a different renal anomaly was present (one multicystic, one dysplasic, and one rudimentary kidney), and in two patients the information was not available (53,71). In two women the urinary apparatus was normal (4,45).…”

Unilateral Renal Agenesis and Associated Müllerian Anomalies: A Case Report and Recommendations for Pre-adolescent Screening

“…6 However, almost all cases of duplication of the female genital tract seem to have renal involvement, with an apparent right-sided dominance. 7 Zurawin et al described eight patients with uterus didelphys and obstructed hemivagina over the course of two years. 4 They found that 100% had ipsilateral renal anomalies diagnosed by MRI.…”

An unusual presentation of uterus didelphys with obstructed hemivagina with ipsilateral renal agenesis

“…Uterus didelphys with obstructed hemivagina is the result of a lateral nonfusion of the m€ ullerian ducts with asymmetric obstruction, and it is almost always associated with renal agenesis (6). A high index of suspicion is necessary to diagnose these disorders, and an adequate work-up is essential (7).…”