Renal venous thrombosis (RVT) in infancy occurs in situations associated with reduced renal blood flow and hypercoagulability. The clinical diagnosis is based on finding enlarged kidney(s), haematuria and thrombocytopenia in a setting where the infant is at risk of RVT. Ultrasonography is the imaging modality of choice and should replace the more invasive excretory urography and venography for confirmation of the diagnosis. Impairment of renal function is best documented by radionuclide studies. Treatment is supportive with heparinisation for severe bilateral RVT and inferior vena cava thrombosis. The role of thrombectomy and fibrinolytic therapy is limited in infancy. Survival rates have much improved in recent years. Severe venous infarction leads to atrophy of the affected kidney, which may later be mistaken for congenital renal hypoplasia. RVT may be complicated by hyperreninaemic hypertension, which is curable by nephrectomy.

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