Abstract Details

Title Proptosis in Optic Pathway Gliomas Associated with Neurofibromatosis: Response to Chemotherapy

Topic Neuro-ophthalmology/Neuro-otology

Presentation(s) P06 - (-)

Poster/Presentation
Number 004

Objective

Background BACKGROUND: Neurofibromatosis type 1 (NF1) is a genetic disorder associated with optic pathway gliomas (OPGs), which can lead to vision loss and proptosis. Chemotherapy is the treatment of choice for clinically and radiologically progressive OPGs. A recent multi-institutional retrospective study revealed that approximately half of subjects with proptosis showed improvement in proptosis with chemotherapy. However, this evaluation was based on the subjective impression of ophthalmologists without radiographic or other confirmation. If shown to be effective, chemotherapy offers a vision-sparing alternative to surgical resection of the optic nerve for proptosis.

Design/Methods DESIGN/METHODS: This retrospective chart review examined the MRIs of subjects identified with proptosis before initiation of chemotherapy in Fisher et al.'s[sup1] multicenter study of NF1-OPG. MRIs of the brain and orbits were examined pre- and post-treatment. The degree of proptosis was measured following established radiographic methodology after each MRI was aligned three-dimensionally along a common plane. Volumetric measurements of intra-orbital tumors were taken using signal-to-noise ratio to determine tumor edge. The change in degree of proptosis and tumor volume pre- and post-chemotherapy were evaluated to determine the effect of treatment and compared to the clinical impression of proptosis change.

Results RESULTS: All patients showed improvement in proptosis following chemotherapy (n=11, p<0.01) with an average of 56% decrease. Tumor volume also shrank with treatment (mean 23% decrease); however, there was no correlation between proptosis and volume change.

Conclusions CONCLUSIONS: These results confirm quantitatively that chemotherapy improves proptosis in NF1-OPG. This information will be useful to families with children with NF1-OPG and proptosis, who are considering chemotherapeutic or surgical interventions.

Authors/Disclosures
Alyssa Nguyen-Phuc PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
David A. Wolk, MD, FAAN (University of Pennsylvania)	Dr. Wolk has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eli Lilly. Dr. Wolk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Functional Neuromodulation. Dr. Wolk has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for GSK. The institution of Dr. Wolk has received research support from Biogen. Dr. Wolk has received publishing royalties from a publication relating to health care. Dr. Wolk has received personal compensation in the range of $5,000-$9,999 for serving as a CME speaker with Eli Lilly.
	No disclosure on file
Grant Liu, MD, FAAN (Div. of Neuro-Ophthalmology)	Dr. Liu has received publishing royalties from a publication relating to health care.

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