Cloacal dysgenesis with imperforated anus and didelphus uterus

Luc de Catte, MD

Dept of Obstetrics and Gynecology, Academic Hospital, Free University of Brussels, Laar­beeklaan 101, 1090 Brussels, Belgium

This fetus was scanned for the first time at 35 weeks of gestation. There was anhydranmnios. We thought at that time that both kidneys were absent, but they appeared to be very dysplastic. The two identical ovoid shaped structures represented a didelphic uterus, filled with mucus (1 MB video-clip). The connection between these uterine horns and a cystic perineal mass (hydrometrocolpos) became visible after intrauterine instillation of saline.


The fetus was delivered with cloacal dysgenesis sequence, Potter sequence, hydrometrocolpos, a didelphic uterus, hypoplastic thorax, lung hypoplasia, and flexion deformities of hands and feet.

Pathological examination revealed also a anal atresia, abnormal female external genitalia, absence of a vaginal introïtus, and clitoridomegaly. The bladder showed a hypertrophic wall. The blind ending sigmoid inserted onto the posterior uterine wall.

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