Management of idiopathic intracranial hypertension in children utilizing venous sinus stenting

Abstract

Background: Venous sinus stenting (VSS) is an accepted and minimally invasive treatment for adult idiopathic intracranial hypertension (IIH) associated with lateral sinus stenosis (LSS). The efficacy and safety of venous sinus stenting (VSS) in children with IIH has not been established.

Methods: This is a retrospective analysis of IIH patients 18 years of age or younger with LSS treated with VSS at our institution. Included patients have fulminant disease course or are refractory or intolerant to medical management.

Results: Eight patients were identified; 4 males and 4 females. Mean age is 13.4 years (range 4-18). All patients had severe headaches, 5 had blurred vision, 3 had diplopia and 3 had pulsatile tinnitus. Papilledema was present in 4 patients. Three patients had prior surgical procedures. Four patients were intolerant to medical management, 3 were refractory and 1 had fulminant course. Cerebral venography demonstrated severe stenosis of the dominant sinus in 6 patients and of bilateral co-dominant sinuses in 2 patients. Six patients had intrinsic stenosis and 2 had extrinsic stenosis. Venous sinus stenting (VSS) resulted in improvement of symptoms, papilledema and normalization of CSF opening pressure in 7 patients. No immediate complications were observed. Mean follow-up period is 21 months (range 6-42). Two patients required re-stenting; one responded well and the other had persistent symptoms and underwent subsequent surgical procedures of CSF diversion, suboccipital decompression and duraplasty which were also ineffective.

Conclusion: VSS may provide a viable option for pediatric IIH patients who are intolerant to medication, have failed conservative management or prior surgical interventions, or present with fulminant disease.

Keywords: Pediatric; idiopathic intracranial hypertension; papilledema; venous sinus stenting.

Figure 1.

Axial (a) and coronal plane (b) contrast-enhanced MRV images demonstrate focal short segment intrinsic stenosis of the right distal transverse sinus related to underlying arachnoid granulation. Intrinsic stenosis is again appreciated on frontal (c) and lateral (d) projections during catheter venography. Following stent placement (e), the previously seeeffects of intrinsic stenosis have resolved with normalization of sinus caliber (F).

Figure 2.

Axial (a) and 3 D sagittal plane (b) contrast-enhanced MRV images demonstrate long segment extrinsic stenosis of the right transverse sinus related to overlying brain parenchyma. Extrinsic stenosis is again appreciated on frontal (c) and oblique (d) projections during catheter venography. Following stent placement (e), the previously seen effects of extrinsic stenosis have resolved with normalization of sinus caliber (f).