Child Kidney Dis.  2022 Dec;26(2):101-106. 10.3339/ckd.22.041.

Acute dural venous sinus thrombosis in a child with idiopathic steroid-dependent nephrotic syndrome: a case report

• Park SJ
• Baik HW
• Cho MH
• Kang JH

• KMID: 2538742
• DOI: http://doi.org/10.3339/ckd.22.041

Abstract

Nephrotic syndrome (NS) is a hypercoagulable state in which children are at risk of venous thromboembolism. A higher risk has been reported in children with steroid-resistant NS than in those with steroid-sensitive NS. The mortality rate of cerebral venous sinus thrombosis (CVST) is approximately 10% and generally results from cerebral herniation in the acute phase and an underlying disorder in the chronic phase. Our patient initially manifested as a child with massive proteinuria and generalized edema. He was treated with albumin replacement and diuretics, angiotensin-converting enzyme inhibitor, and deflazacort. Non-contrast computed tomography showed areas of hyperattenuation in the superior sagittal sinus when he complained of severe headache and vomiting. Subsequent magnetic resonance imaging revealed empty delta signs in the superior sagittal, lateral transverse, and sigmoid sinuses, suggesting acute CVST. Immediate anticoagulation therapy was started with unfractionated heparin, antithrombin III replacement, and continuous antiproteinuric treatment. The current report describes a life-threatening CVST in a child with steroid-dependent NS, initially diagnosed by contrast non-enhanced computed tomography and subsequently confirmed by contrast-enhanced magnetic resonance imaging, followed by magnetic resonance venography for recanalization, addressing successful treatment.

Keyword

Anticoagulation; Case reports; Magnetic resonance venography; Nephrotic syndrome; Thrombosis

Figure

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  Fig. 1. Brain computed tomography (CT) scan findings. (A) The sagittal non-enhanced CT image shows areas of segmentally increased attenuation (red circle), consistent with acute thrombi in the superior sagittal sinus. (B) The axial image on pre-contrast CT shows areas of abnormal hyperattenuation (red arrow), consistent with acute thrombi in the right transverse sinus. (C) The coronal non-enhanced CT image of the patient shows a central hyperattenuation (red circle) in the superior sagittal sinus, surrounded by slightly engorged cortical veins. (D) The coronal non-enhanced CT image reveals an area of significantly hyperintense signal (red arrowhead) indicative of acute thrombosis in the superior sagittal sinus.

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  Fig. 2. Brain magnetic resonance imaging findings. (A) Contrast-enhanced magnetic resonance imaging reveals areas of filling defects in the superior sagittal sinus due to acute thrombosis. (B) The empty delta signs are positive in the superior sagittal (red arrow), lateral transverse (yellow arrow), and sigmoid sinuses (blue arrow), suggesting acute dural sinus thrombosis. No cerebral infarct is found.

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  Fig. 3. Brain magnetic resonance venography manifestations. (A) Three weeks later, contrast-enhanced magnetic resonance venography demonstrates recanalization (red arrows) in the superior sagittal sinus that was obstructed due to acute thrombus. (B) The right transverse sinus still shows a lack of flow (red circle), possibly resulting from congenital hypoplasia of cerebral vasculatures or partially remaining thrombosis.

Reference

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