An Unusual Case of Optic Atrophy in a Child due to Intracranial Space Occupying Lesion

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Introduction

▪ Eye is the window to look into brain as eye is the prolongation of the brain. ▪ Ocular symptoms and signs appear with significant frequency in patients with Intracranial Space Occupying Lesions (ICSOL), which help in correlating the site of the lesion. ▪ Compressive optic neuropathy (CON)- caused by intrinsic or extrinsic compression anywhere along the optic nerve. ▪ Additionally, demyelination, ischemia, metabolic, and traumatic insult to optic nerve. ▪ Most common sign - slow progressive monocular visual loss, ▪ sometimes associated with headaches. ▪ Optic nerve atrophy - an important ophthalmological sign, associated with serious systemic conditions; a significant bearing on the overall morbidity.

A Case Report A 9-year-old female child presented with complaints of gradually progressive, painless, loss of vision in left eye (LE) and headache, for one and half month.

OD OS UCVA 6/9 PL+, PR inaccurate BCVA -0.50D/- 0.50DX180 6/6 No improvement Pupil RRR RAPD III Fundus disc pallor Chalky white disc, distinct margins H/o - diminution of vision ▪ evening rise of temperature ▪ rigors ▪ weight loss ▪ loss of appetite ▪ seizures.

Fundus picture showing disc pallor (OD) & optic atrophy (OS).

Investigations & Management Montoux test: positive (17mm)

MRI Brain – Ill defined conglomerated lesion in left parieto-occipital region, midline shift, leptomeningeal enhancement suggestive of tuberculoma.

▪ After initial work up, patient was referred to neurosurgery department for further management. ▪ Patient underwent decompressive craniotomy & excision of mass lesion with biopsy.

Post-op NCCT – Craniotomy defect in left parieto- temporal region with edematous changes.

CSF Analysis Appearance turbid TLC 28 cell/ cu. Mm Neutrophils 2% Lymphocytes 90% Protien 250 mg/dl Glucose 20 mg/dl Biopsy report – necrotic brain parenchyma, giant cell granuloma were seen.

Result ▪ RE has partial optic atrophy and LE has optic atrophy. ▪ Post neurosurgery, tuberculoma was found and patient has been put on anti tubercular drugs – isoniazid, rifampicin, pyrazinamide, streptomycin and steroids.

Discussion

▪ ICSOLs are one of the common causes of optic atrophy. ▪ The presentation is usually non-specific and thus may go undiagnosed and untreated. ▪ Optic atrophy is an irreversible consequence following ocular symptoms, thus, making it necessary to screen for the presence of ICSOL in patients presenting with optic atrophy.

Ophthalmoscopic Classificaton of Optic Atrophy Sign Primary Secondary Consecutive Previous swelling of the optic disc No Preceded by long-standing swelling of the optic disc. No Disc colour White: diffuse or sectoral pallor. Grey Waxy pale Disc margins Distinct Blurred Normal, attenuated arteries Fibrosis (gliosis) of the disc None Gliosis of the optic nerve head None Cause • compression of the optic nerve or chiasm• hereditary optic neuropathy • nutritional optic atrophy • chronic papilloedema• papillitis• anterior ischaemic optic neuropathy • chorioretinal disease e.g. retinitis pigmentosa• central retinal artery occlusion Osaguona, Vivian B. “Differential diagnoses of the pale/white/atrophic disc.” Community Eye Health 29 (2016): 71 - 74.

Study Mean age / Range of age (years)

Fundus findings ICSOL (cause) % of patients presenting with optic atrophy due to ICSOL Chinta S. et al. [1] 6.87 Optic atrophy Craniopharyngioma (44%), arachnoid cyst (16.6%), optic glioma (5.56%) 5.56% Repka. et al. [3] 7 Optic atrophy Craniopharyngioma (22%), pituitary adenoma (5%) 29% Hema N. A. et al. 26.72 Optic atrophy, optic neuritis, papilloedema Astrocytoma (44%), schwannomas (14%) meningothelial tumours (12%) 9.7% Olueye T.S. et al. 40.8 Optic atrophy (unspecified ICSOL) 8% Bajracharya K. et al. [2] 53.6 Optic atrophy (unspecified ICSOL) 4% Nalawade K. et al. [4] 0-60 Optic atrophy, papilloedema, optic neuritis, retinal haemorrhages.

Intracranial hematoma (85.7%), brain abscess (10%), brain tumors (4%) 12% Krishna V. et al. [5] 1-80 Optic atrophy (unspecified ICSOL) 10% Present Study 9 Optic atrophy, disc pallor Tuberculoma (not applicable)

Conclusion

▪ Tubercular perineuritis and retrobulbar neuritis though uncommon can lead to primary optic atrophy and permanent visual loss. ▪ Ocular manifestations are reversible in cases of early diagnosis and prompt treatment. If optic atrophy is present, it is incurable. ▪ Prompt diagnosis and initiation of treatment results in good visual outcome. ▪ Interdepartmental co- ordination is required for management of such patients.

Take Home Message ▪ Optic atrophy is the end stage of a process causing damage to the optic nerve. ▪ The management goal is to intervene before optic atrophy is noted or to save remaining function. ▪ Thus, depending upon the underlying cause for the optic nerve damage.