Abstract
Abstract 11 year old male presented in December 2021 with complaints of headache and visual impairment. On examination [he had] multiple freckles in the right axilla and cafe au lait lesions all over his body with largest being 3cm in the inguinal region. Pretreatment MRI showed minimally enhancing mass seen in the optic chiasm extending anteriorly into the distal optic nerves bilaterally and the optic tract posteriorly with extensive vasogenic edema along the optic pathway into the lateral thalamus and extending into the bilateral lentiform nuclei. H-MR spectroscopy of the mass demonstrated a choline peak. Findings in keeping with optic pathway glioma. The baseline fundoscopy showed a normal anterior segment, slightly pale disc on the posterior segment. The patient was commenced on weekly Vinblastine via chemo port. At 24 weeks of treatment patient was assessed MRI and fundoscopy. MRI showed a mild interval decrease in size of lobulated mass lesion involving the optic chiasma, bilateral optic nerves up to intraorbital segments and optic tracts. The lesion also involves the infundibular stalk and tuber cinereum. No interval change in hyperintense T2/FLAIR signal with no enhancement seen at the lateral geniculate bodies, hippocampi, bilateral lentiform nuclei and lateral thalami. He completed 72 weeks of Vinblastine with good clinical Response. NF 1 can cause tumors to form and optic nerve gliomas that may occur in about 15 percent of children with this disease in about 2/3 of the time the tumors stop growing and disappears however there are some tumors that may require treatment.
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