Cunha K S, Rozza-de-Menezes R E, Andrade R M, Almeida Lms, Janini Mer, Geller M
School of Medicine, Fluminense Federal University, Niterói, RJ, Brazil.
Department of Specific Formation, School of Dentistry, Nova Friburgo University Pole, Fluminense Federal University, Nova Friburgo, RJ, Brazil.
J Clin Pediatr Dent. 2015 Winter;39(2):168-71. doi: 10.17796/jcpd.39.2.972220046g774534.
Neurofibromatosis type 1 (NF1) is a common autosomal genetic disorder with a prevalence of 1 in 3,000 births. NF1 is a complex syndrome characterized by many abnormalities and may affect all organ systems. Oral manifestations of NF1 occur frequently, but reports including NF1 children with facial plexiform neurofibromas and oral alterations are scant. Facial plexiform neurofibroma may cause asymmetry, disfigurement and usually arises from the trigeminal nerve. The aim of this paper is to to report three pediatric NF1 cases with facial plexiform neurofibroma presenting with oral manifestations, which were evaluated clinically and radiographically, and also to briefly review the literature. Patients presented with changes in the oral soft tissues, jaws, and teeth ipsilateral to the tumor.
1型神经纤维瘤病(NF1)是一种常见的常染色体遗传病,发病率为每3000例出生中1例。NF1是一种复杂的综合征,其特征是存在许多异常,可能影响所有器官系统。NF1的口腔表现很常见,但关于患有面部丛状神经纤维瘤和口腔改变的NF1儿童的报道很少。面部丛状神经纤维瘤可能导致不对称、毁容,通常起源于三叉神经。本文的目的是报告3例患有面部丛状神经纤维瘤并伴有口腔表现的儿科NF1病例,对其进行临床和影像学评估,并简要回顾文献。患者表现为肿瘤同侧口腔软组织、颌骨和牙齿的改变。