Superior sagittal sinus thrombosis: a rare complication of nephrotic syndrome.MS Tullu, CT Deshmukh, SU Save, BK Bhoite, BA Bharucha
Department of Paediatrics, Seth G. S. Medical College and K. E. M. Hospital, Mumbai, India. , India
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 0010734351
Source of Support: None, Conflict of Interest: None
A two and half year-old-male child, known case of steroid responsive nephrotic syndrome presented with fever and vomiting of acute onset. He was diagnosed to have superior sagittal sinus thrombosis on a contrast computerised tomographic scan of brain. Recovery was complete without anticoagulant therapy. Superior sagittal sinus thrombosis is an extremely rare complication of nephrotic syndrome.
Keywords: Case Report, Child, Preschool, Human, Male, Nephrotic Syndrome, complications,Sagittal Sinus Thrombosis, etiology,radiography,Tomography, X-Ray Computed,
Nephrotic syndrome is associated with a hypercoagulable state arising due to various factors like - elevated levels of plasma fibrinogen, elevated levels of factors V, VII, VIII and X, thrombocytosis and accelerated thromboplastin generation. Other factors favouring thrombosis include - decreased antithrombin III, hyperlipidaemia, steroid therapy, haemoconcentration, etc.,,. Haemostatic abnormalities in nephrotic syndrome are variable and despite a hypercoagulable state, the individual clotting factors can be paradoxically decreased.
The incidence of thromboembolic complications in nephrotic syndrome in children is far less frequent than in adults,. This may be due to the fact that membranous glomerulonephritis is the most frequent lesion in adults and is also the lesion in which thrombosis is most common. In children, minimal change nephrotic syndrome is the commonest lesion and has the least risk of thrombosis. The incidence of thromboembolic complications in children with nephrotic syndrome is very low and has been reported to be 1.8% in 3377 cases in an European study. Only a few isolated reports exist in the literature describing sagittal sinus thrombosis,,,, and we report a case of superior sagittal sinus thrombosis.
A two and half year-old-male child, known case of nephrotic syndrome, presented with fever and irritability for 3 days and vomiting for 1 day. He was diagnosed as a case of nephrotic syndrome about 40 days back and started on prednisolone, as per the regimen recommended by International Study of Kidney Disease in Children. At presentation, he was on treatment with alternate day, single dose of prednisolone. There was no history of diarrhoea or dehydration.
On examination, the vital parameters were normal and blood pressure was 130/90 mm Hg. The child had steroid facies and neurological examination revealed brisk knee jerks and extensor plantar reflexes. The fundus showed bilateral papilloedema.
The relevant investigations were as follows - haemoglobin- 10.1 gm%, total leucocyte count- 8,600 cells/cmm (55% neutrophils, 43% lymphocytes, 1% eosinophils, 1% monocytes), platelet count- 3.8 lac/cmm, serum cholesterol- 386 mg%, serum albumin- 3 gm%, prothrombin time- 13 sec (control- 14 sec), and activated partial thromboplastin time-26 sec (control-42 sec). Renal parameters were normal. Cerebrospinal fluid (CSF) examination revealed few polymorphs on smear and culture was negative. Computed tomographic (CT) scan of brain revealed superior sagittal sinus thrombosis with the classical 'delta sign' [Figure - 1]. No evidence of thrombosis was found outside the central nervous system.
The child was treated with nifedipine (for hypertension), intravenous ceftriaxone (in view of polymorphs in the CSF), antipyretics and anticonvulsants. The fever and irritability decreased in 4 days and the patient was discharged later with no neurological impairment. CT scan brain was not repeated. Subsequently, the child has been followed up for 1 year and is diagnosed as a frequent relapser. He is presently on levamisole therapy.
Dural sinus thrombosis is extremely rare in nephrotic syndrome and was seen in only one out of 700 nephrotic patients followed at the Bai Jerbai Wadia Hospital for Children, Mumbai. In addition to the factors previously mentioned, factors like intravascular volume depletion, high haematocrit and local trauma can contribute to thromboembolic complications,. Our patient had steroid therapy as an additional risk factor.
Focal neurological deficits, though reported by previous workers, were not seen in our case,,. A contrast enhanced CT scan is a good modality for detecting dural sinus thrombosis and shows a relative lucency within the venous sinus ('delta sign') representing the thrombus. Magnetic resonance imaging (MRI) may be a better modality for diagnosis and follow up of dural sinus thrombosis,.
The management of dural sinus thrombosis is directed towards proper fluid management and prevention of factors that aggravate thrombosis. Anticoagulation is difficult in these patients as a large amount of heparin is lost in urine. Higher doses of heparin coupled with administration of antithrombin III concentrates may be required to achieve adequate anticoagulation. Recently, low molecular weight heparin and antithrombin III replacement has been suggested as a rational approach to treatment. Our patient recovered without anticoagulant therapy. The role of fibrinolytic agents in dural sinus thrombosis has not been well studied. Prophylactic anticoagulation may be useful in selected patients to prevent thrombotic complications.
Most patients treated early with adequate supportive care survive without neurological deficits,,. Death has been reported rarely,.
We thank our Dean, Dr. R G Shirahatti for giving us permission to publish this case report.
[Figure - 1]