Presentation
Decreased vision.
Patient Data
Ill-defined, irregular lesion involving optic chiasma, bilateral optic tracts, and hypothalamus with edema/abnormal T2, FLAIR hyperintensity extending to posterior limbs of both internal capsules. Possible involvement of intracranial segments of both optic nerves is seen. The postcontrast study shows no definite enhancement.
T2, FLAIR hyperintensities are seen in bilateral thalami, pons, bilateral cerebellar hemispheres, and possibly in the right superior cerebellar peduncle.
No obvious diffusion restriction or enhancement is seen in any of the above-mentioned regions.
Case Discussion
The above findings are suggestive of hypothalamic-optochiasmatic glioma with focal areas of signal intensity (FASI) in a case of neurofibromatosis type I. The child had multiple café au lait spots and was later genetically confirmed to have neurofibromatosis type I.
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