Natural history of optic pathway tumors in children with neurofibromatosis type 1: A longitudinal study☆,☆☆,★
Section snippets
METHODS
The Children's Memorial Hospital Neurofibromatosis Clinic was established in 1985 as a consultation service primarily to assist in the diagnosis and management of neurofibromatosis in children. Children have been referred to the clinic because of (1) NF-1 suspected as a result of the presence of café au lait spots, subcutaneous masses, or characteristic ophthalmologic or orthopedic problems, (2) a previous diagnosis of NF-1, or (3) a first-degree relative with NF-1. All patients initially
RESULTS
As of the end of the study period, 227 patients with NF-1 had been examined by the neurofibromatosis clinic. Of these, 176 (77%) had undergone neuroimaging by either computed tomography or MRI and are the subject of this report. Thirty-three children (19%) were found to have evidence of an optic pathway tumor. The incidence of OPT in children who had no ophthalmologic complaints at diagnosis was 15%. No OPT were subsequently found in children who had never undergone screening neuroimaging.
DISCUSSION
Previous studies that have examined the natural history of OPT have been hampered by the inclusion of children who did not have NF-1 and by the frequent rendering of what would now be considered unnecessary, overaggressive therapy.6, 7, 8 Wright et al.8 were the first to point out that the natural history of OPT in children with NF-1 might be more benign than that of sporadic OPT. The most significant finding of our study of an unbiased population of children with NF-1 is the predominantly
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From the Departments of Pediatrics and Surgery, Northwestern University Medical School, and the Divisions of General Academic and Emergency Pediatrics, Genetics, and Ophthalmology, Children's Memorial Hospital, Chicago, Illinois
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Reprint requests: Robert Listernick, MD, Division of General Academic and Emergency Pediatrics, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614.
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0022-3476/94/$3.00 + 0 9/20/55232