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Table of Contents
CORRESPONDENCE
Year : 2020  |  Volume : 3  |  Issue : 2  |  Page : 86-88

Cerebral sinovenous thrombosis in steroid-dependent nephrotic syndrome


Division of Pediatric Nephrology, Institute of Child Health, Kolkata, West Bengal, India

Date of Submission19-Jul-2020
Date of Acceptance08-Oct-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Subhankar Sarkar
Department of Pediatric Nephrology, Institute of Child Health, 11, Dr Biresh Guha Street, Kolkata - 700 017, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2589-9309.305926

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How to cite this article:
Sarkar S, Singh P, Akhtar MS, Sinha R. Cerebral sinovenous thrombosis in steroid-dependent nephrotic syndrome. Asian J Pediatr Nephrol 2020;3:86-8

How to cite this URL:
Sarkar S, Singh P, Akhtar MS, Sinha R. Cerebral sinovenous thrombosis in steroid-dependent nephrotic syndrome. Asian J Pediatr Nephrol [serial online] 2020 [cited 2021 Jan 26];3:86-8. Available from: https://www.ajpn-online.org/text.asp?2020/3/2/86/305926



Sir,

Thromboembolism is a described complication of nephrotic syndrome; however, cerebral sinovenous thrombosis (CSVT) is rare, with an incidence of 0.67 per 100,000 children per year.[1] Risk factors identified include membranous nephropathy, urinary loss of anticoagulants, hepatic overproduction of procoagulants, immobility, hypoalbuminemia, hemoconcentration, infection, iron deficiency, previous episodes of thromboembolism, and genetic predisposition leading to procoagulant state.[2],[3] Two children with steroid-dependent nephrotic syndrome presented to our center with relapse, generalized edema, and persistent drowsiness and were found to have CSVT, likely secondary to hemoconcentration. The two patients, an 8-year-old girl and a 4-year-old boy, were diagnosed with steroid dependence 6 and 12 months previously, respectively. Case 1 presented with severe headache, vomiting, and drowsiness for 4 days. Case 2 had acute gastroenteritis and severe headache for the last 3 days. Both patients had been on therapy with mycophenolate mofetil twice daily and prednisolone on alternate days. For edema, oral furosemide had been administered for 2 days each, without seeking medical attention. There was no history of head trauma, fever, substance abuse, neck pain, rigidity, or photophobia. Family history was unremarkable. Blood investigations (cases 1 and 2) showed hyponatremia (127 and 129 mEq/L), hyperkalemia (5.5 and 5.6 mEq/L), hypoalbuminemia (1.4 and 1.2 g/dL), increased hematocrit (64% and 47%), and decreased fractional excretion of urea (10% and 12%) without evidence of sepsis or impaired renal function. Cerebral magnetic resonance (MR) imaging and venography showed CSVT involving superior sagittal sinus and right transverse and sigmoid sinuses in case 1 and involving superior sinus and right sigmoid and transverse sinuses in case 2 [Figure 1]. Relapse was treated with prednisolone at 60 mg/m2 per day until remission. Both patients received therapy with low molecular weight heparin (LMWH) subcutaneously for 6 months. Intravascular hypovolemia was managed with intravenous hydration and infusion of 20% human albumin, which led to correction of electrolyte abnormalities and fall in hematocrit. Subsequently, diuretics were used judiciously in conjunction with albumin infusions to control edema. Procoagulant screen, including levels of protein C and protein S, was normal in case 1; repeat evaluation of these and other parameters are awaited in both patients. MR imaging, repeated at 6-month follow-up, showed resolution of thrombosis in both cases.
Figure 1: Findings on magnetic resonance imaging and venography of the brain in the two cases

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CSVT is a serious, albeit uncommon, complication of childhood nephrotic syndrome. We have summarized important studies that reported thrombosis in nephrotic syndrome in [Table 1].[4],[5],[6],[7],[8],[9],[10],[11],[12] Severe hypoalbuminemia, increased hematocrit, and low antithrombin levels are reported risk factors; inherited thrombophilia was rare.[4],[5],[8] Severe hypoalbuminemia and hemoconcentration were present in our cases.
Table 1: Thrombosis in nephrotic syndrome

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The clinical manifestations of CSVT are nonspecific, but it should be considered when a child with nephrotic syndrome in relapse presents with neurological symptoms such as seizures, altered consciousness, drowsiness, nausea, vomiting, motor deficit, headache, diplopia, or papilledema.[2],[8] MR imaging with time-of-flight venography is preferred to computed tomography in view of its excellent sensitivity, ability to detect the extent of the thrombus, lack of ionizing radiation exposure, and no risk of contrast nephropathy.[2] Anticoagulation with LMWH, or its later substitution by oral anticoagulants for 6 months or longer, is advocated; longer therapy is given in the presence of risk factors or persisting proteinuria.

The cases illustrate that CSVT should be suspected in children with nephrotic syndrome presenting with neurological abnormality. Relapses associated with hypoalbuminemia, hemoconcentration, and diuretic use predispose to CSVT. MR imaging with time-of-flight venography enables early diagnosis, while LMWH along with management of relapse and hypovolemia enables resolution of findings.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
deVeber G, Andrew M, Adams C, Bjornson B, Booth F, Buckley DJ, et al. Cerebral sinovenous thrombosis in children. N Engl J Med 2001;345:417-23.  Back to cited text no. 1
    
2.
Saposnik G, Barinagarrementeria F, Brown RD Jr., Bushnell CD, Cucchiara B, Cushman M, et al. Diagnosis and management of cerebral venous thrombosis: A statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2011;42:1158-92.  Back to cited text no. 2
    
3.
Zhang LJ, Zhang Z, Li SJ, Meinel FG, Nance JW Jr., Zhou CS, et al. Pulmonary embolism and renal vein thrombosis in patients with nephrotic syndrome: Prospective evaluation of prevalence and risk factors with CT. Radio 2014;273:897-906.  Back to cited text no. 3
    
4.
Lilova MI, Velkovski IG, Topalov IB. Thromboembolic complications in children with nephrotic syndrome in Bulgaria (1974-1996). Pediatr Nephrol 2000;15:74-8.  Back to cited text no. 4
    
5.
Citak A, Emre S, Sâirin A, Bilge I, Nayir A. Hemostatic problems and thromboembolic complications in nephrotic children. Pediatr Nephrol 2000;14:138-42.  Back to cited text no. 5
    
6.
Hoyer PF, Gonda S, Barthels M, Krohn HP, Brodehl J. Thromboembolic complications in children with nephrotic syndrome. Risk and incidence. Acta Paediatr Scand 1986;75:804-10.  Back to cited text no. 6
    
7.
Mehls O, Andrassy K, Koderisch J, Herzog U, Ritz E. Hemostasis and thromboembolism in children with nephrotic syndrome: Differences from adults. J Pediatr 1987;110:862-7.  Back to cited text no. 7
    
8.
Kerlin BA, Blatt NB, Fuh B, Zhao S, Lehman A, Blanchong C, et al. Epidemiology and risk factors for thromboembolic complications of childhood nephrotic syndrome: A Midwest Pediatric Nephrology Consortium (MWPNC) study. J Pediatr 2009;155:105-10.  Back to cited text no. 8
    
9.
Candelaria GT, Belangero VM. Predisposing factors for deep venous thrombosis in children and adolescents with nephrotic syndrome. ISRN Vasc Med 2011;1-5. [DOI: 10.5402/2011/827483].  Back to cited text no. 9
    
10.
Gargah T, Abidi K, Nourchene K, Zarrouk C, Lakhoua MR. Thromboembolic complications of childhood nephrotic syndrome. Tunis Med 2012;90:161-5.  Back to cited text no. 10
    
11.
Suri D, Ahluwalia J, Saxena AK, Sodhi KS, Singh P, Mittal BR, et al. Thromboembolic complications in childhood nephrotic syndrome: A clinical profile. Clin Exp Nephrol 2014;18:803-13.  Back to cited text no. 11
    
12.
Tavil B, Kara F, Topaloglu R, Aytac S, Unal S, Kuskonmaz B, et al. Case series of thromboembolic complications in childhood nephrotic syndrome: Hacettepe experience. Clin Exp Nephrol 2015;19:506-13.  Back to cited text no. 12
    


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