Purpose or Case Report: Mullerian duct anomalies are a spectrum of malformations resulting from complications of mullerian duct development in utero. They are common, seen in up to 5% of the female population, and whilst many are mild and asymptomatic, more severe deformities can result in miscarriage or infertility. They have a common association with mesonephric duct abnormalities, often resulting in concomitant renal agenesis. Some of these complete constellations have been grouped into defined syndromes such as OHVIRA (Herlyn-Werner-Wunderlich), and Meyer-Rockitansky-Kunster-Hauser. This means upon discovery of mullerian abnormalities, further investigations are often required to exclude these associations.
We present a case of a child who presented at birth with a unilateral cystic right flank mass identified antenatally. Post-natal US visualised a cystic mass with no viable renal tissue seen, and MCKD was suspected. This was seemingly confirmed when a DMSA scan demonstrated no functional renal tissue on the side of the mass. Follow up US imaging showed apparent involution of the mass, again consistent with MCKD, and the patient was then asymptomatic until the age of 15. The patient then represented with abdominal pain and was referred for an US. This discovered a large multiloculated cystic mass in the right flank, the same side as the one seen at birth. MRI imaging was then performed which confirmed a large multiloculated cystic mass, but with a rim of enhancing tissue suggestive of ovarian stroma. Incidentally, no renal tissue was seen on the right, and a contralateral unicornuate uterus with a single normal ovary on the left was seen. The lesion was surgically excised, and histology confirmed an ovarian cystadenoma. Previous imaging was retrospectively reviewed, and the initial cystic mass surmised to have represented an ectopic ovary with multiple follicles rather than an MCDK. The DSMA findings were explained by an ipsilateral renal agenesis in the context of mullerian duct abnormalities.
Unicornate uterus with contralateral ectopic ovary and renal agenesis has been reported in the literature but not formally categorised. This case demonstrates the pitfalls of multiple abnormalities in the context of mullerian duct pathology, and how renal agenesis with ipsilateral ectopic ovary can demonstrate near identical appearances to MCDK and poses a significant diagnostic challenge.
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