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Year : 2016  |  Volume : 28  |  Issue : 2  |  Page : 137-138

A rare case of large bilateral optic nerve glioma in a child without neurofibromatosis type 1

Department of Neuro Ophthalmology, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Coimbatore, Tamil Nadu, India

Date of Web Publication20-Mar-2017

Correspondence Address:
Virna M Shah
Department of Neuro Ophthalmology, Aravind Eye Hospital, Avinashi Road, Coimbatore - 641 014, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kjo.kjo_2_17

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We report a rare case of atypical large bilateral malignant optic nerve glioma in a child with no clinical evidence of neurofibromatosis.

Keywords: Infant, neurofibromatosis,optic nerve glioma

How to cite this article:
Shah VM, Sharanya S. A rare case of large bilateral optic nerve glioma in a child without neurofibromatosis type 1. Kerala J Ophthalmol 2016;28:137-8

How to cite this URL:
Shah VM, Sharanya S. A rare case of large bilateral optic nerve glioma in a child without neurofibromatosis type 1. Kerala J Ophthalmol [serial online] 2016 [cited 2023 Feb 8];28:137-8. Available from: http://www.kjophthal.com/text.asp?2016/28/2/137/202468

  Introduction Top

Pediatric optic nerve glioma is notorious for being unpredictable in terms of both the presenting history and the clinical course.[1] We report a rare case of large bilateral optic nerve gliomas, which went unnoticed by the parents.

  Case Presentation Top

A 4-year-old female patient presented with complaints of diminution of vision in both eyes since 2 weeks. It was sudden in onset, painless, and not associated with headache, vomiting, giddiness, loss of consciousness, head, or ocular trauma. The patient's mother gave a history of noticing squinting in the left eye since birth and that the child had difficulty fixing her gaze while looking at objects. She also noticed mild bulging of left eye since 1 year.

The mother does not give any history of maternal illness or drug intake during the antenatal period, and the patient was delivered by caesarean section, at full term, and cried immediately following delivery. The child had no developmental delay and had attained milestones as per age. Family history was noncontributory.

On general examination, the child was of normal stature. Central nervous system examination was normal. Anterior examination revealed rotatory nystagmus in both eyes with 45° exotropia, grade 4 relative afferent pupillary defect, and mild paraxial proptosis (Hertel's exophthalmometry – 19 mm) in left eye. The visual acuity in the right eye was 6/36, and in left eye, there was perception of light. Fundus examination revealed optic disc pallor in both eyes. Magnetic resonance imaging scan revealed bilateral large optic nerve glioma with intracranial extension [Figure 1]. Neurofibromatosis was ruled out clinically.
Figure 1: (a and b) T1 and T2 MRI images showing diffuse mass-like enlargement on both optic nerves involving prechiasmal (left > right), chiasmal, and postchiasmal segments with posterior extension to optic radiation

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  Discussion Top

Optic pathway tumors are rare and are often seen in young children.[2] Because of their central location and either via direct extension or mass effect, optic nerve gliomas account for endocrine abnormalities and neurologic complications.[3] Hence, the clinical picture can be very unpredictable.[4] Exophthalmos and diminution of vision are the usual presentation in progressive disease. Bilateral optic nerve glioma is usually associated with neurofibromatosis type 1,[5] which was ruled out in our case. Radiotherapy, chemotherapy, and surgical excision are the key modalities of treatment, however, long-term survival is very poor.[5]

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Conflicts of interest

There are no conflicts of interest.

  References Top

Awdeh PM, Kiehna EN, Merchant TE. Visual outcomes in paediatric optic pathway glioma after confocal radiation therapy. Int J Radiat Oncol Biol Phys 2012;84:46-51.  Back to cited text no. 1
Shapey J, Danesh-Meyer HV, Kaye AH. Diagnosis and management of optic nerve glioma. J Clin Neurosci 2011;18:1585-91.  Back to cited text no. 2
Opocher E, Kremer L, Perilongo C. Prognostic factors for progression of childhood optic pathway glioma: A systematic review. Eur J Cancer 2006;42:1807-16.  Back to cited text no. 3
Nair AG, Pathak RS, Iyer VR, Gandhi RA. Optic nerve glioma: An update. Int Ophthal 2014;34:999-1005.  Back to cited text no. 4
Singh AD, Traboulsi E, Schoenfield. Neuro-oculocutaneous syndromes (phakomatoses). In: Singh AD, Damato BE, Pe'er J, Murphree AL, Perry JD, editors. Clinical ophthalmic oncology. Philadelphia: Saunders Elsevier; 2007. pp. 385-401.  Back to cited text no. 5


  [Figure 1]


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