Hazan-Molina H, Aizenbud D
Orthodontic and Craniofacial Department, Graduate School of Dentistry, Rambam Health Care Campus, Bruce Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel.
J Clin Pediatr Dent. 2015 Winter;39(2):172-8. doi: 10.17796/jcpd.39.2.rn7147700425t647.
Hutchinson-Guilford progeria syndrome is an extremely rare condition classified as one of the premature ageing syndromes. This case presents a 16-year-old Israeli female patient, suffering from a variant of Hutchinson-Guilford progeria with a history of treatment with oral biphosphnates. The patient presented with typical cranial and facial features of the syndrome including delayed teeth eruption and root development probably due to insufficient jaw growth and severs retrognatic position of the maxilla and mandible. Orthodontic treatment considerations are described along with those required in light of the previous treatment by oral biphosphonates.All primary teeth were extracted in three appointments while creating as minimal trauma as possible to the surrounding tissue and alveolar bone. For now, the patient refuses to begin the orthodontic treatment course. There are no limitations to conduct any dental procedures in progeria patients, however, extreme caution must be exercised during oral surgery due to the inelasticity of tissues and dermal atrophy. Orthodontic procedure commencement should be early enough to manage the delayed development and eruption of teeth. Patients taking oral biphosphonates should be advised of this potential complication. If orthodontic treatment is considered appropriate, plans should be assessed and modified to include compromises.
哈钦森-吉尔福德早衰综合征是一种极为罕见的疾病，被归类为早衰综合征之一。该病例为一名16岁的以色列女性患者，患有哈钦森-吉尔福德早衰综合征的一种变体，有口服双膦酸盐治疗史。患者表现出该综合征典型的颅面部特征，包括牙齿萌出延迟和牙根发育异常，这可能是由于颌骨生长不足以及上颌骨和下颌骨严重后缩所致。文中描述了正畸治疗的考虑因素以及鉴于之前口服双膦酸盐治疗所需要的注意事项。在三次就诊过程中拔除了所有乳牙，同时尽量减少对周围组织和牙槽骨的创伤。目前，患者拒绝开始正畸治疗疗程。对早衰患者进行任何牙科手术并无限制，然而，由于组织缺乏弹性和皮肤萎缩，在口腔手术期间必须格外谨慎。正畸治疗应尽早开始，以处理牙齿发育和萌出延迟的问题。应告知服用口服双膦酸盐的患者这种潜在并发症。如果认为正畸治疗合适，则应评估并修改治疗计划，以做出妥协。
