Cerebral venous thrombosis in a patient with nephrotic syndrome

Abstract

Introduction: Nephrotic syndrome (NS) is the most frequent glomerular disease of childhood. Thromboembolic complications can occur. Cerebral venous thrombosis (CVT)diagnosis may bechallengingsinceclinical signs are usually nonspecific. Acute phase treatment is anticoagulation. Case report: Apreviously healthy 3-year-old female child, presented with two days of irritability, prostration and periorbital edema.Workup revealed nephrotic proteinuria, megaloblastic anemia, hypercholesterolemia, hypertriglyceridemia, hypoalbuminemia and elevated sedimentation rate. Remission occurred on the 8th dayof steroid therapy. About nine days after remissonshe started with headache, photophobia, nocturnal awakening and morning vomiting. On examination she was irritable, mildly dehydrated, but normotensive, with normal neurologic and funduscopic examination. The head angiotomography showed an extensive venous thrombosis of the superior sagittal sinus. Antithrombotic therapy was started, withclinical and radiologic improvement. Hereditary thrombophilia and dysfibrinogenemias study showed 675 4G/5G and -844A/G variants in the promoter region of the SERPINE1 gene.She remained on anticoagulant therapy for a year. Conclusion: CVTal though rare is an important complication of NS. The hypercoagulant state contributes to this phenomenon due to its multifactorial etiology.The high clinical suspicion with contrast-enhanced CT allowed the early institution of therapy, thus preventing the occurrence of post-thrombotic complications.In the absence of major intracranial hemorrhage anticoagulation is the gold standard of treatment.