MayerRokitanskyKusterHauser (MRKH) syndrome is a rare congenital syndrome with the absence of uterus and vagina, affecting 1 in 5000 women. The patient has normal secondary sexual characters and the first sign of the syndrome is primary amenorrhea. There are two types of the condition in this syndrome type A without associated anomalies, and type B, which is associated with other gynecological and non-gynecological anomalies. The renal agenesis is most common in type B. The contrast-induced nephropathy is the foremost risk factor in renal agenesis. The contrast induced nephropathy is a serious complication resulting from the administration of contrast media which leads to impairment of renal function. Here we reported a rare case of a 34-year-old female patient with a history of MRKH syndrome and Solitary Ectopic Kidney presenting with fever, dyspnea, and diarrhea. The patient previously admitted to her local hospital for fever, nausea, and vomiting. The diagnostic workup was done with blood investigation, ultrasonography, and CT contrast of the abdomen. The patient's total count, blood urea, and serum creatinine levels showed a substantial increase on the following day. The patient was diagnosed to have Mullerian agenesis type II, with contrast-induced nephropathy and uteropelvic obstruction. Incidentally she was also found to have dengue fever with thrombocytopenia. She was managed with hemodialysis and DJ stenting. Patients with solitary ectopic kidney have a high risk of developing contrast induced nephropathy and one should exercise great prudence when ordering radiological investigations in these patients.
Key words: Mullerian agenesis, Single kidney, Contrast-induced nephropathy, Pelviureteric junction obstruction
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