Durnford Luke,  Kolanjian Harout,  Jenkins Richard

Final Pr. ID: Poster #: CR-021

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. Read More

Authors:  Durnford Luke , Kolanjian Harout , Jenkins Richard

Keywords:  Mullerian Duct Anomalies, MCDK, Renal Agenesis

Steinhardt Nicole,  Meyers Mariana,  Brown Brandon

Final Pr. ID: Poster #: EDU-007

Fetal MRI is now an important adjunct imaging modality in the evaluation of complex fetal anomalies, including cystic renal disease. The improved resolution and anatomic detail of the renal parenchyma offered by MRI can assist with identification, localization, and characterization of cystic lesions which are less clearly visualized on ultrasound. Advanced imaging adds value through enabling prenatal prognostication and patient counseling. In this presentation, we evaluate the spectrum of renal cystic abnormalities at the microscopic and macroscopic scale, and review the patterns of disease by cyst location, effect on parenchymal integrity, and obstruction of the collecting system. A clearer understanding of the diverse appearances and broad spectrum of outcomes of these fetal anomalies can contribute to more detailed treatment planning and more precise, family-centered care.
In this presentation, we review the varied patterns of cystic renal disease as identified on fetal MRI, highlighting those forms known to be more associated with perinatal morbidity and mortality. Further, we correlate their appearance on MRI with pre- and post-natal US imaging, as well as pathologic findings. Finally, we will describe secondary prenatal imaging biomarkers that may be valuable in counseling and also with both definitive and palliative surgical planning.
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Authors:  Steinhardt Nicole , Meyers Mariana , Brown Brandon

Keywords:  MCDK, ARPKD, Lung