Natural history of optic pathway tumors in children with neurofibromatosis type 1: A longitudinal study,☆☆,

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Abstract

To assess the natural history of optic pathway tumors (OPT) in children with neurofibromatosis type 1 (NF-1), from January 1985 through May 1993 we performed a prospective, longitudinal study of OPT in an unselected population of children with NF-1. Of 227 children with NF-1 seen in a specialty clinic, 176 (77%) underwent neuroimaging. Children in whom tumors were identified were followed closely by both repeated neuroimaging and ophthalmologic examinations to detect tumor growth or visual deterioration. Thirty-three children (19%) were found to have OPT at a median age of 4.2 years. The median age of children who had ophthalmologic complaints was significantly lower than that of children who had no such complaints (1.9 vs 5.3 years; p < 0.001). Although eight tumors were discovered because of ophthalmologic complaints or evidence of precocious puberty, 25 children (76%) were free of symptoms at the time of diagnosis. Twenty-one children (64%) had normal ophthalmologic findings at diagnosis; six children, all with chiasmal tumors, had previously unrecognized decreased visual acuity. Only three children (9%) had evidence of either tumor growth or deteriorating vision after diagnosis; the median duration of neuroimaging follow-up was 2.4 years (range, 0.2 to 7.2 years) and of ophthalmologic examinations 3.4 years (range, 0.2 to 8.1 years). All symptomatic OPT were diagnosed before 6 years of age. We conclude that OPT rarely progress during the next few years in children with NF-1 once the OPT have been discovered. The utility of screening neuroimaging for OPT in symptom-free children with NF-1 appears very limited. (J PEDIATR 1994;125:63-6)

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.

0022-3476/94/$3.00 + 0 9/20/55232

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