Cerebral Venous Thrombosis in a Patient with Nephrotic Syndrome

Introdution: Nephrotic syndrome (NS) is the most frequent glomerular disease of childhood, reaching about 2-7: 100,000 children. This may be associated with thromboembolic complications, mostly with venous origin. The diagnosis of cerebral venous thrombosis is difficult, since the clinic may be non-specific. In the acute phase recommended treatment is low molecular weight heparin for 5 to 7 days.

Case report: A 3-year-old female child, previously healthy presented to the pediatric emergency department with two days of irritability, prostration and periorbital edema. Physical examination showed peri-orbital edema and blood preassure was normal.

Laboratory workup revealed nephrotic proteinuria, megaloblastic anemia, hypercholesterolemia, hypertriglyceridemia and hypoalbuminemia (15.3 mg / dL), and elevated sedimentation rate. Under the diagnosis of Nephrotic Syndrome, she was admitted on steroid therapy.

Remission occurred on the 8th day of therapy, being discharged on the 12th under steroid therapy with Pediatric Nephrology follow-up.

Five days after discharge she started headache with photophobia, nocturnal awakening and morning vomiting. On general observation she was complaining, slightly dehydrated, normotensive, with normal cardiopulmonary evaluation. Neurologic examination was normal without fundoscopic alterations.

Due to this the clinical manifestations and concomitant risk factors angiotomography was performed, showing an extensive venous thrombosis of the superior sagittal sinus with an apparent extension to the transverse sinus and started antithrombotic therapy.

Progressive improvement of headache episodes was observed, being asymptomatic after 3 days of antithrombotic therapy (Enoxaparine).

She remained clinically stable throughout the hospitalization period and was discharged on the 15th day taking prednisolone, enoxaparin and enalapril, with pediatric nephrology and neuropediatrics follow-up.

Hereditary thrombophilia and dysfibrinogenemias were studied.

She remains clinically well without neurologic symptons.

Conclusion: Cerebral venous thrombosis is a rare yet a 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. There is no consensus for prophylactic treatment.