A girl in early adolescence with autism presented with 3 months of abdominal pain and 36 hours of anuria. She had recently received treatment for urinary tract infections, anxiety and menorrhagia (she had undergone menarche a few months earlier). Due to the pain, she had pulled out an incisor. Bladder scan showed 923 mL, creatinine was 829 mmol/L but urethral catheter insertion did not drain urine. An unenhanced CT scan revealed an absent left kidney, didelphys uterus and right-sided hydroureteronephrosis caused by haematocolpos in keeping with a diagnosis of OHVIRA syndrome and ureteric obstruction of a single kidney causing acute renal failure. She underwent vaginal septoplasty, drainage of the haematocolpos and right ureteric stent.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10826531PMC
http://dx.doi.org/10.1136/bcr-2023-257922DOI Listing

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