Neurofibromatosis in children

Abstract

Neurofibromatosis occurs once in every 3,000 live births. Café au lait spots are the most common presenting lesion. Five spots with a diameter of at least 0.5 cm. should be considered diagnostic in children. Spinal deformity is the most common bony lesion. Scoliosis varies from mild nonprogressive forms to hairpin curvatures. Four types of pseudarthrosis of the tibia can be distinguished, with progressively more serious prognoses. The incidence of neoplasia greatly exceeds that of the general population.