Bone abnormalities occurring in the follow-up of the patients with neurofibromatosis type 1.

摘要: Neurofibromatosis type 1 (NF1), also called von Recklinghausen disease or peripheral neurofibromatosis, is a common autosomal dominant disorder characterized by multiple neurofibromas, "cafe au lait" spots and Lisch nodules of the iris with variable clinical expression. Osseous anomalies appeared in patients NF1 including dysplasia, scoliosis pseudoarthrosis. We propose research osseous involvement at 11 patients, seven female four male ages from 9 to 60 which cutaneous aspect has complete form, hyperpigmented neurofibromas only more than six spots. All suffered radiological exams, CT- MRI-scan. The results were different case extreme severe deformations, especially children, unapparent involvement, incidental found occasion our investigation. CONCLUSIONS. abnormalities specific disease, like scoliosis, pseudoarthrosis, often gentle but sometimes extremely severe. most are presented cases when these development early childhood. Other times asymptomatic, their finding been incidental. want have separate mention for maxillary mandible according information not rare form.