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Cerebral sinovenous thrombosis and idiopathic nephrotic syndrome in childhood: report of four new cases and review of the literature

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Abstract

Introduction

Nephrotic children are prone to develop thromboembolic complications secondary to an acquired hypercoagulable state. Cerebral sinovenous thrombosis (CSVT) is increasingly recognised in this population, but clinical characteristics and outcome are not well documented.

Patients and methods

The database of the Canadian Pediatric Ischemic Stroke Registry (Toronto Site) containing prospectively enrolled children from 1992–2004 with CSVT identified four children with NS. A pooled literature analysis retrieved 17 additional cases reports.

Results

CSVT presented in the majority of cases during the first flare or within 6 months after the onset of NS and was found to occur more often in SSNS/SDNS (n=13) than in SRNS (n=4). Clinical manifestations were non-specific and consisted primarily of seizures (n=8) and signs of raised intracranial pressure (n=16). Imaging studies revealed a predilection for superior sagittal sinus involvement (n=21) and rare parenchymal lesions (n=4). The most consistent biological risk factors were a severe hypoalbuminaemia (n=14) and, to a lesser extent, decreased antithrombin (AT) levels (n=9/16). Deficiency of other coagulation inhibitors (protein S, protein C) was not identified. Inherited thrombophilia was documented in a single case, suggesting that acquired, more than genetic, coagulation factors are involved. Anticoagulation was safe, and the outcome was good in most patients, and no recurrence of thrombotic event was reported.

Discussion

In conclusion, CSVT is now a well-described complication of NS with potential morbidity. A high index of suspicion is required, especially in young children with NS presenting neurological symptoms. Reliable biological predictors of CSVT are lacking.

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Abbreviations

NS :

Nephrotic syndrome

CSVT :

Cerebral sinovenous thrombosis

SSNS :

Steroid-sensitive nephrotic syndrome

SDNS :

Steroid-dependant nephrotic syndrome

SRNS :

Steroid-resistant nephrotic syndrome

UHF :

Unfractionated heparin

LMWH :

Low molecular weight heparin

AT :

Antithrombin

Lip(a) :

Lipoprotein (a)

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Acknowledgement

The authors gratefully acknowledge the data management assistance of Ann-Marie Pontigon in the preparation of this study.

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Authors and Affiliations

  1. Division of Pediatric Neurology, The Hospital for Sick Children, Toronto, ON, Canada

    Joel Fluss & Gabrielle deVeber

  2. Division of Pediatric Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, Canada

    Denis Geary

  3. Department of Pediatric Neurology, Hôpital Kremlin-Bicêtre, 78, rue du Général Leclerc, 94275 le Kremlin-Bicêtre, Paris, France

    Joel Fluss

  4. Division of Neurology, Children’s Stroke Program, The Hospital for Sick Children, 555 University Avenue, M5G1X8, Toronto, ON, Canada

    Gabrielle deVeber

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  1. Joel Fluss
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Correspondence to Gabrielle deVeber.

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Fluss, J., Geary, D. & deVeber, G. Cerebral sinovenous thrombosis and idiopathic nephrotic syndrome in childhood: report of four new cases and review of the literature. Eur J Pediatr 165, 709–716 (2006). https://doi.org/10.1007/s00431-006-0147-7

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  • DOI: https://doi.org/10.1007/s00431-006-0147-7

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