Journal of American Association for Pediatric Ophthalmology and Strabismus
Major articleOrbital optic nerve gliomas in children with neurofibromatosis type 1
Section snippets
Methods
NF-1 was confirmed in all patients on the basis of the diagnostic criteria established by the National Institutes of Health Consensus Development Conference.10 Patients were identified by compiling a list of all patients who had optic pathway tumors seen in the Neurofibromatosis Clinic of Children’s Memorial Hospital since 1985. All available records were reviewed to identify patients with symptomatic tumors at presentation or during the study period. This study was approved by the hospital’s
Results
The charts of 644 patients with NF-1 were reviewed. A total of 69 patients with optic pathway tumors were identified during the study period. Of those, 12 symptomatic orbital optic nerve gliomas were identified, all of which had led to proptosis (Table 1). There were eight girls and four boys. All were Caucasian. These 12 patients were diagnosed with NF-1 at an average age of 20 months. The tumors were discovered at an average age of 26 months. The rapid onset of proptosis led to the diagnosis
Discussion
Optic pathway tumors in patients with NF-1 and sporadic non-NF-1-associated optic pathway tumors have different biologic properties that distinguish their initial clinical manifestations and their natural history.12 Although both occur in very young children, sporadic tumors generally lead to extensive morbidity requiring treatment. In contrast, NF-1-associated tumors often have a period of rapid growth leading to decreased visual acuity or precocious puberty, after which tumor growth may
Acknowledgments
This work was supported in part by an unrestricted grant from Research to Prevent Blindness, NY (Northwestern University).
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