[Syndromes 18. Von Recklinghausen's disease]

Abstract

Von Recklinghausen's disease (neurofibromatosis 1; NF1) is one of the neurofibromatoses and accounts for about 90% of all cases. Inheritance is autosomal dominant with about 30-50% of cases representing new mutations. Characteristic features for NF1 are six or more café-au-lait-spots, neurofibromas, Lisch nodules and axillary freckling. Oral manifestation consists of neurofibromas and intrabony lesions. Due to growth of the oral and facial neurofibromas maldevelopment of the facial skeleton and malocclusion are seen. Surgical correction in young individuals easily leads to recurrence. Contour corrected surgery in grown up individuals is possible.