Abstract
Meningocele of the optic nerve sheath is an extremely rare condition with a few cases reported in literature. Image studies reveal tubularcystic enlargement of the optic nerve although with the same thickness. Symptoms are often related to the involvement of the optic nerve, leading from slow to accelerated decreasing of the visual acuity. The early surgical treatment is the decompression of the optic nerve sheath, which it could provide improvement of visual function. We are presenting a case report of a patient who showed clinical and radiological signs of this rare pathological condition. Male patient attended at service complaining of proptosis of right eye (OD) since birth, in progress during the last months associated to stabbing pain. Best corrected visual acuity (BCVA) of OD was movements at 50 cm far; OS showed no abnormalities. Nuclear Magnetic Resonance of the OD showed expansive formation with cystic aspect, defined boundaries, located in an intraconal situation on the right orbit cavity and in a closing anatomical relationship to the optic nerve, inducing compression, deformity and anterior displacement of this eye besides presenting signal similar to spine liquor in all sequences obtained. The first hypothesis was meningocele of right optic nerve sheath. Then, patient was referred for surgical decompression.
Highlights
Optic nerve sheath meningocele (ONAM) is an extremely rare condition, with few cases reported in the literature, being it defined as a sheath swelling of the optic nerve by spinal fluid
This paper aims at reporting the case of a patient whose clinical data and imaging exams are highly suggestive of optic nerve sheath meningocele, and stressing the main features of this rare disease
Lunardi et al (1) reviewed the existing literature, finding only about 31 cases of MBNO throughout literature, and they describe the disease as primary cysts of the optic nerve sheath without apical mass or malformation of the skull-orbital junction
Summary
Optic nerve sheath meningocele (ONAM) is an extremely rare condition, with few cases reported in the literature, being it defined as a sheath swelling of the optic nerve by spinal fluid. There is an expansion of the cerebrospinal fluid space around the optic nerve without associated inflammation, cerebral or orbital neoplasia. Imaging exams show a tubularcystic enlargement of the optic nerve / sheath-optical complex with thickening of the optic nerve. The differential diagnosis includes tumors of the optic nerve as meningioma, vascular hamartoma, glioma, neurofibromatosis, Von Hippel-Lindau desease, hemangioendothelioma, or skull-orbital fracture(1). This paper aims at reporting the case of a patient whose clinical data and imaging exams are highly suggestive of optic nerve sheath meningocele, and stressing the main features of this rare disease
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