Presentation
A solitary kidney on abdominal workup leads to further evaluation.
Patient Data
Sagittal sonographic image shows an absent kidney in the right renal fossa.
MRI of the pelvis shows uterine didelphys and an obstructed right hemi-vagina filled with blood (hematocolpos). In the presence of an absent right kidney, all findings are consistent with Herlyn-Werner-Wunderlich syndrome.
Case Discussion
Herlyn-Werner-Wunderlich (HWW) syndrome is defined by a triad of abnormalities: type III Müllerian duct anomaly (resulting in a uterus didelphys), an obstructed hemivagina, and mesonephric duct anomalies. Typically, the mesonephric duct anomalies present as renal agenesis, but in some cases, duplicated kidneys, dysplastic kidneys, recto-vesical bands, or crossed fused ectopia may also occur.
Co-authors: Zohaib Khan, MD, and Sravani Gampala, MBBS
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