Dural ectasia of the optic nerve sheath: is it always benign?

Abstract

Abstract:A 36-year-old woman with a 3-month history of progressive visual loss had papilledema, dilatation of the optic nerve sheaths and normal cerebrospinal fluid pressures. She was diagnosed as dural ectasia of the optic nerve sheaths and surgical decompression was performed. In this case report, severe visual loss is described as a serious complication of this rare disease and the importance of early surgical intervention is emphasized.

Idiopathic Inflammatory Neuroretinitis Simulating Optic Nerve Sheath Dural Ectasia

“…A prompt diagnosis has to be made due to the fact that dural ectasia of the optic nerve sheath may sometimes be associated with rapidly progressive and permanent visual loss if not treated at the right time. 9 Our patient had a clinical course typical for neuroretinitis but also had neuroradiologic findings most commonly seen with optic nerve sheath dural ectasia, which initially might have led to an incorrect diagnosis. In the case illustrated here, clinical presentation of the disease process was correctly applied to the interpretation of the neuroradiologic findings which prevented an unnecessary surgical intervention in this particular patient.…”

Dural Ectasia of the Optic Nerve: A Rare Presentation in Neurofibromatosis Type I

“…[46] Other case reports of optic nerve meningoceles (associated with or without NF-1) describe it as tubular or saccular enlargement of optic nerve sheath filled with CSF and harboring a separately visualized normal or thickened optic nerve (imparting a characteristic bull's-eye appearance on coronal T2-weighted images) similar to our case. [78] Thus, a nonenhancing and separately visualized optic nerve of uniform thickness surrounded by an enlarged CSF filled optic nerve sheath should serve to confidently diagnose optic nerve meningocele. Surgical decompression of the optic nerve has been successfully reported to prevent progression of vision loss in patients with acute presentation.…”

Bilateral visual acuity reduction in a child with Marfan syndrome: Is there a pathogenetic role for optic nerve dural ectasia?

“…Dural ectasia in the spinal region is one of the systemic criteria of MFS, but optic nerve dural ectasia in MFS patients has been infrequently described before. 8,9 Bilateral optic nerve dural ectasia is a rare structural anomaly occurring either as a primary entity or associated with intracranial hypertension 10…”

“…Dural ectasia in the spinal region is one of the systemic criteria of MFS, but optic nerve dural ectasia in MFS patients has been infrequently described before 8,9 . Bilateral optic nerve dural ectasia is a rare structural anomaly occurring either as a primary entity or associated with intracranial hypertension 10 and neurofibromatosis type 1 11 . Optic nerve dural ectasia may present with blurred vision, pain, proptosis, an enlarged blind spot, visual field changes, optic head edema, choroidal folds, and optic disk shunt vessels 12 .…”