Visnapuu Vivian, Peltonen Sirkku, Ellilä Tero, Kerosuo Eero, Väänänen Kalervo, Happonen Risto-Pekka, Peltonen Juha
Department of Anatomy, University of Turku, Kiinamyllynkatu 10, FIN 20520 Turku, Finland.
Eur J Med Genet. 2007 Jul-Aug;50(4):274-80. doi: 10.1016/j.ejmg.2007.04.001. Epub 2007 Apr 21.
Neurofibromatosis type 1 (NF1) is a genetic disorder with skeletal involvement. Periapical cemental dysplasia is a rare finding in the normal population.
A total of 55 patients with NF1, 29 female and 26 male patients, were evaluated with orthopantomograms, supplemented with periapical radiographs if necessary. The vitality of the teeth was measured by two different testing methods.
A novel finding was the occurrence of cemental dysplasia affecting the periapical area of vital mandibular teeth in 8 adult women with NF1. Thus, cemental dysplasia was detected in 34.8% of the adult female NF1 patients, while cemental dysplasia was not present in men or children with NF1.
Periradicular cemental dysplasia is indeed a new NF1 related bone lesion type. Our finding suggests that this is the first reported sexual dimorphism in the manifestations of NF1. Cemental dysplasia of NF1 patients should not be confused with periapical findings caused by endodontic pathoses. The former do not require active therapy whereas in the latter root canal treatment is necessary.
1型神经纤维瘤病（NF1）是一种伴有骨骼受累的遗传性疾病。根尖周牙骨质发育异常在正常人群中较为罕见。
对55例NF1患者（29例女性，26例男性）进行全景X线片评估，必要时辅以根尖片。采用两种不同的检测方法测量牙齿活力。
一项新发现是，8例成年NF1女性患者的活髓下颌牙根尖区出现牙骨质发育异常。因此，在成年女性NF1患者中，牙骨质发育异常的检出率为34.8%，而男性或儿童NF1患者未出现牙骨质发育异常。
根尖周牙骨质发育异常确实是一种新的与NF1相关的骨病变类型。我们的发现表明，这是首次报道的NF1表现中的性别差异。NF1患者的牙骨质发育异常不应与牙髓病引起的根尖周表现相混淆。前者无需积极治疗，而后者则需要进行根管治疗。
