Presentation
Left proptosis with decreased vision bilaterally.
Patient Data
Age: 5 years
Gender: Female
From the case:
Neurofibromatosis type 1 - with optic pathway gliomas
{"current_user":null,"step_through_annotations":true,"access":{"can_edit":false,"can_download":true,"can_toggle_annotations":true,"can_feature":false,"can_examine_pipeline_reports":false,"can_pin":false},"extraPropsURL":"/studies/80392/annotated_viewer_json?c=1696822044\u0026embed_domain=external.radpair.com%25252527%2525255b0%2525255d%25252527%2525255b0%2525255d\u0026lang=gb"}
Both optic nerves, optic chiasma, and right optic tract are enlarged and of intermediate signal intensity on T1WI, iso-to high signal intensity on T2WI, and FLAIR with enhancement mainly in the optic nerves. There are abnormal hyperintensities on FLAIR, and T2WI with no enhancement of the dentate nuclei, posterior aspect of the thalami, globi pallidi, and the corporeal segment of the corpus callosum.
Case Discussion
The association of optic pathway gliomas and areas of T2/FLAIR hyperintensity with no contrast enhancement within the white matter and basal ganglia or corpus callosum are most consistent with neurofibromatosis type 1 or Von Recklinghausen disease.
Unable to process the form. Check for errors and try again.