|Year : 2016 | Volume
| Issue : 2 | Page : 137-138
A rare case of large bilateral optic nerve glioma in a child without neurofibromatosis type 1
Virna M Shah, Sukumar Sharanya
Department of Neuro Ophthalmology, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Coimbatore, Tamil Nadu, India
|Date of Web Publication||20-Mar-2017|
Virna M Shah
Department of Neuro Ophthalmology, Aravind Eye Hospital, Avinashi Road, Coimbatore - 641 014, Tamil Nadu
Source of Support: None, Conflict of Interest: None
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 2019 Sep 20];28:137-8. Available from: http://www.kjophthal.com/text.asp?2016/28/2/137/202468
| Introduction|| |
Pediatric optic nerve glioma is notorious for being unpredictable in terms of both the presenting history and the clinical course. We report a rare case of large bilateral optic nerve gliomas, which went unnoticed by the parents.
| Case Presentation|| |
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|
Click here to view
| Discussion|| |
Optic pathway tumors are rare and are often seen in young children. Because of their central location and either via direct extension or mass effect, optic nerve gliomas account for endocrine abnormalities and neurologic complications. Hence, the clinical picture can be very unpredictable. Exophthalmos and diminution of vision are the usual presentation in progressive disease. Bilateral optic nerve glioma is usually associated with neurofibromatosis type 1, 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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
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.
Shapey J, Danesh-Meyer HV, Kaye AH. Diagnosis and management of optic nerve glioma. J Clin Neurosci 2011;18:1585-91.
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.
Nair AG, Pathak RS, Iyer VR, Gandhi RA. Optic nerve glioma: An update. Int Ophthal 2014;34:999-1005.
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.