Stephen L X, Hamersma H, Gardner J, Beighton P
Faculty of Dentistry, University of Western Cape, Cape Town, South Africa.
Int Dent J. 2001 Aug;51(4):287-90. doi: 10.1002/j.1875-595x.2001.tb00840.x.
Documentation of the oral and dental manifestations of Sclerosteosis.
Sclerosteosis is a member of the family of genetic craniotubular hyperostoses. This severe progressive sclerosing bone dysplasia has important orofacial manifestations and a wide geographical distribution. Comprehensive oral and dental evaluation of eight affected adults in the Afrikaner community of South Africa was undertaken.
Gross asymmetrical hypertrophy of the mandible was present in all eight patients, bilateral or unilateral facial paralysis with weakness of facial muscles due to facial nerve entrapment was present in six. Drooling of saliva and difficulties with mastication were frequent problems in these persons. The teeth were structurally and mechanically normal although there was partial anodontia in two patients and delayed eruption in another. Maxillary (palatal) and mandibular tori were present in every affected person. Due to the hyperostosis of the maxilla and mandible, tooth extraction was often a very difficult matter. There were no instances of post-extraction osteomyelitis of the mandible.
Accurate differentiation of sclerosteosis from the other sclerosing bone dysplasias is crucial for effective dental prognostication and management.
记录骨硬化症的口腔和牙齿表现。
骨硬化症是遗传性颅骨管状骨质增生家族的一员。这种严重的进行性硬化性骨发育异常具有重要的口腔颌面部表现，且分布广泛。对南非阿非利卡人群体中的8名成年患者进行了全面的口腔和牙齿评估。
所有8名患者均出现下颌骨明显不对称肥大，6名患者出现双侧或单侧面神经受压导致的面瘫及面部肌肉无力。流口水和咀嚼困难是这些患者常见的问题。尽管有2名患者存在部分牙缺失，另1名患者牙齿萌出延迟，但牙齿在结构和力学上是正常的。每名患病个体均出现上颌（腭部）和下颌隆突。由于上颌骨和下颌骨的骨质增生，拔牙往往非常困难。未发生下颌骨拔牙后骨髓炎病例。
准确区分骨硬化症与其他硬化性骨发育异常对于有效的牙科预后评估和治疗至关重要。
