Cerebral venous sinus thrombosis in children with nephrotic syndrome
A 4-year-old boy, weighing 14 kg with steroid-responsive nephrotic syndrome of 1-year duration developed vomiting and drowsiness for 2 days, with fresh re-appearance of edema (relapse of nephrotic state). He had vomiting, 5-6 times a day, after each feeding attempt. He was on tapered treatment with prednisolone 5 mg per day, but developed fresh relapse. On admission, dose of prednisolone was increased to 20 mg daily. Past medical history was negative for migraine headaches, head trauma, febrile illness, substance abuse, vitamin intake or thrombophlebitis. Family history was unremarkable.\nOn admission, his vital signs were normal with pulse rate of 80/min, regular, and blood pressure of 110/80 mmHg. General examination revealed periorbital edema with mild ascitis. He was drowsy, apathetic, resenting disturbance and liking to be lying undisturbed. Neurologic examination revealed uncooperative child, conscious but resenting interference with normal cranial nerve examination and good tone and power in all four limbs with normal sensation and normal deep tendon reflexes. There was no neck rigidity, fundus examination revealed blurring of disc margin with early papilledema. The rest of systemic examination was normal.\nInvestigations showed hemoglobin 11.1 gm/dl with packed cell volume of 36%, total leucocytes count of 12,600/mm3, platelet count 2.8 lac/mm3, serum creatinine of 0.42 mg/dl, blood urea of 18 mg/dl and serum albumin of 2.1. Serum electrolytes were normal. Coagulation profile showed prothrombin time of 11 s with international normalized ratio (INR) of 1.12 and activated partial thromboplastin time of 28 s. Protein C and protein S levels were normal. Urine analysis showed 2+ proteinuria, 24 h urine protein excretion was 2.0 gm/day. Serum C3 and C4 complements levels were normal. Renal ultrasonography revealed normal sized kidney without dilatation of pelvicalyceal system.\nComputer tomography scan brain without contrast was normal, so MRI brain and MR venography was done which demonstrated thrombosis of superior sagittal, left transverse and left sigmoid sinuses. There were no cerebral parenchymal or brainstem lesions [].\nHe was treated with daltaparin, a low molecular weight heparin 1000 U (75-100 U/kg each dose 12 h) subcutaneous twice daily for 5 days, and oral anticoagulant warfarin was added on fourth day in dose of 0.75 mg twice a day. Dose of warfarin was adjusted to keep INR between 2.0 to 2.5 (in dose range of 0.05-0.1 mg/kg). The nephrotic state was treated with 50 ml of 20% human albumin i/v daily for four days with prednisolone 20 mg/day and patient had increased dieresis on 4th day of admission and child had nil proteinuria after 10 days. The child responded well to treatment, his drowsiness and vomiting improved in 2 days, and he recovered completely in 2 weeks. Repeat MRI brain with MRV performed after 2 months showed complete resolution of thrombus from all the cerebral venous sinuses. His warfarin dose was reduced after resolution, and was continued in lower dose 0.5 mg twice a day for further 6 months, though INR was difficult to keep at two. At present, patient is off anticoagulant treatment without any neurological complaints [].