Idiopathic Hypertrophic Cranial Pachymeningitis Associated with a Dural Arteriovenous Fistula Involving the Straight Sinus: Case Report

Abstract

AbstractOBJECTIVE AND IMPORTANCEReports of idiopathic hypertrophic cranial pachymeningitis have increased as a result of advances in magnetic resonance imaging. This is the first documented case of idiopathic hypertrophic cranial pachymeningitis associated with a dural arteriovenous fistula involving the straight sinus. We discuss possible causes of the association and the treatment options.CLINICAL PRESENTATIONA 64-year-old man presented with a headache and visual disturbance. Gadolinium-enhanced T1-weighted magnetic resonance imaging demonstrated homogeneously stained meninges and prominent enhancement of the tentorium and falx. Angiograms demonstrated a dural arteriovenous fistula of the straight sinus.INTERVENTIONAlthough surgical excision of the straight sinus and subsequent corticosteroid therapy failed to relieve the patient's visual symptoms, subsequent surgical decompression of the optic nerve resulted in improvement and stabilization.CONCLUSIONNarrowing or occlusion of the tentorial sinuses and narrowing of the straight sinus by extensive dural fibrosis of the tentorium and falx, attributable in turn to idiopathic hypertrophic cranial pachymeningitis, may have resulted in the development of a dural arteriovenous fistula. We propose surgical decompression of the optic nerve as an alternative treatment during the active phase of the disease in patients who exhibit resistance to corticosteroid therapy.