Bilateral idopathic optic nerve sheath meningocele associated with unilateral transient cystoid macular oedema

Shanmuganathan, Vijay; Leatherbarrow, B; Ansons, Alec M.; Laitt, Roger

doi:10.1038/sj.eye.6700212

Cited by 26 publications

(22 citation statements)

References 7 publications

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“…[5][6][7] There are some common features of both diseases; however there is no significant evidence suggesting the role of raised intracranial pressure in the etiology of the dural ectasia of the optic nerve. 1,8 Although optic nerve sheath dilatation is one of the radiological features of IIH, the enlargement of the optic nerve sheath to this degree is unusual. The clue for differential diagnosis is the LP opening pressure which should be more than 250 mm H 2 O in IIH.…”

Section: Discussionmentioning

confidence: 99%

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

Bakbak

Dönmez

Kansu

et al. 2009

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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.

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Section: Discussionmentioning

confidence: 99%

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

Bakbak

Dönmez

Kansu

et al. 2009

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“…Isolated optic nerve sheath meningocele is a rare affection and only about 30 cases were reported in the literature. 2,3 The pathophysiology of meningocele remains unknown. 2,4 Hayreh [5] reported that the subarachnoid space was narrower in the optic canal.…”

Section: Discussionmentioning

confidence: 99%

“…But some symptoms will point to complications such as acute or progressive decrease in visual acuity or alteration of the visual field. [1][2][3][4] The main differential diagnoses are optic nerve glioma, meningioma (primary or secondary), and arachnoid cysts. 1,2,4 MRI is an essential element to validate the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…1,4,5 Some authors reported cases of meningocele with abnormalities in intracranial pressure and brought forward the hypothesis that its pathophysiology could be similar to idiopathic intracranial hypertension (IIH). 3 Initials symptoms can be neurological and/or ophthalmological but remain vague. [1][2][3][4] Neurological symptoms usually consist in nonspecific headaches.…”

Section: Discussionmentioning

confidence: 99%

“…3 Initials symptoms can be neurological and/or ophthalmological but remain vague. [1][2][3][4] Neurological symptoms usually consist in nonspecific headaches. Ophthalmological symptoms can reflect a progressive expansion of the meningocele causing orbital pain, acquired hyperopia, and unilateral or bilateral exophthalmos.…”

Section: Discussionmentioning

confidence: 99%

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Optic Nerve Sheath Meningocele: A Case Report

Halimi¹,

Wavreille²,

Rosenberg³

et al. 2013

Neuro-Ophthalmology

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Isolated optic nerve sheath meningocele is a rare affection defined as the cystic enlargement of the optic nerve sheath filled with cerebrospinal fluid. We report the case of a 39-year-old woman presenting with bilateral meningocele uncovered during a routine examination for headache complaints. A 5-year follow-up validated the lesion's clinical and imaging stability. Magnetic resonance imaging (MRI) is an essential tool in the diagnosis of this pathology, alongside characteristic symptoms indicating that the meningocele might have progressively expanded into the orbit. In this case we present a therapeutic approach based on pathophysiological hypotheses and review of the literature.Keywords: Optic nerve, optic nerve sheath meningocele, tear of the optic nerve sheath OBSERVATIONWe report here the case of a 39-year-old woman who initially consulted for nonspecific headaches progressing over the past 10 years. The associated clinical symptoms included pain around the orbit and intermittent eyelid oedema.The initial ophthalmological examination found retained visual acuity in both eyes and exophthalmos on the right side at 22 mm (Hertel exophthalmometer), the left side measures were normal at 19 mm. During the cover test, intraocular pressure and oculomotricity were normal for both eyes. The examination of the anterior segment was eventless on both sides. Ophthalmoscopy of the right eye unveiled deeply located optic disc drusen validated by fluoroscopy with a cobalt filter, B-mode ultrasound, and angiography. The clinical picture brought up a pseudopapilloedema (Figure 1). The examination of the left eye was normal (Figure 2).On the right side, visual field testing in static (Humphrey-Zeiss field analyser) and kinetic perimetry (workhorse Goldman perimeter) showed a widening of the blind spot. The latencies of evoked visual potentials (EVPs) were normal but their amplitudes diminished on both sides. B-mode ultrasound showed a larger cystic dilatation of the optic nerve sheath (Figure 3) on the right side than on the left one. Brain magnetic resonance imaging (MRI) validated an optic nerve sheath meningocele ( Figure 4A, B, and C), with normal ventricles, fluid collection in enlarged subarachnoid spaces, and Grade 1 exophthalmos of the right eye (Cabanis scale).The patient refused a lumbar puncture to check her intracranial pressure. Since the usual analgesics failed to provide pain relief for the headaches, a medical treatment with acetazolamide (half a 250-mg tablet, two to three times a day) was initiated. Pain was properly managed with acetazolamide and the meningocele remained stable during the entire 5-year follow-up, validated by yearly ophthalmology, neurosurgery, as well as MRI examinations. DISCUSSIONThe first description of ''optic nerve sheath meningocele'' was reported by Garrity et al. in 1990. 1

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