Endocrine Abstracts

Abstract: Gliomas of the optic pathways are rare tumors that are mainly seen in children. We report the case of a 4 year old boy followed since the age of 6 months for a nystagmus. It is a boy born at term by vaginal way resulting from parents not consanguineous, the parents noted the nystagmus when their child was 6 months old. After opinion of the ophthalmologist, the child is treated only by optical correction, it is only at the age of 4 years following the installation of headache that a brain MRI is performed revealing a tissue damage process expansive solido cystique, supra sellaire developed at the expense of the chiasma, the cystic portion of which is posterior and the anterior solid portion measuring 31 × 38 × 36.6 mm. The child was referred to neurosurgery for treatment and then referred to our service. The ophthalmological examination finds a nystagmus without strabismus or exophthalmos. Visual acuity could not be appreciated given the age of the child. Examination of the fundus revealed a bilateral sectoral optic atrophy. The somatic examination did not find café au lait spots or other lesions that could suggest neurofibromatosis. The neurological examination is normal, in particular no intracranial hypertension syndrome. Endocrine examination is without abnormalities.

Discussion: Tumors of the optic pathways are dominated by glioma and then meningiomas. Gliomas are the most common brain tumors in children and adolescents, boys are affected twice as often as girls. These tumors include forms of very good prognosis such as pilocytic astrocytoma, and types much more difficult to treat such as infiltrating glioma of the brainstem. Low-grade (benign) gliomas are more common in younger people, while malignant gliomas affect older children or adolescents. The decrease in visual acuity is the first sign of ophthalmological appeal, however the decrease in visual acuity may go unnoticed in young children in whom the tumor may be revealed by nystagmus or strabismus.

Conclusion: The therapeutic management of gliomas of the optic pathways goes from simple clinical and radiological monitoring, to surgical treatment or chemotherapy or radiotherapy.