少汗型外胚叶发育不良儿童的早期可摘义齿修复:附10例患儿1~6年随访

Early removeable prosthetic treatment of ten children with hypohidrotic ectodermal dysplasia:1⁃to 6⁃year follow⁃up

目的研究少汗型外胚叶发育不良(HED)患儿的早期可摘义齿修复方法并评价其临床效果。方法选取中山大学附属口腔医院修复科2014年5月至2020年1月收治的全部HED患儿共10例,其中男性9例、女性1例,初诊年龄4~7岁。患儿口腔表现为先天无牙或绝大多数牙缺失,采用可摘义齿(全口义齿或可摘局部义齿)进行早期修复。义齿的设计除了较好地恢复牙列的功能外,还尽量避免影响患儿口颌系统和天然牙的发育,修复后每隔3~6个月复诊,根据患儿颌骨及天然牙的萌出和发育调改、重衬或重新制作义齿。随访期1~6年。结果所有患儿修复后经1~8周后均可适应义齿。在随后的修复效果满意度调查中,所有患儿在美观、发音及自信心等方面均改善明显或较明显;9例患儿咀嚼功能改善明显或较明显;在义齿固位方面,仅2例患儿认为“义齿稳固”;9例患儿在随访期颌位关系稳定,面型发育正常,1例患儿因故停戴下颌全口义齿8个月后导致上下颌弓呈反牙合关系,面型呈明显的凹面型。结论早期可摘义齿修复可恢复HED患儿的咀嚼、发音等功能,并促进颞下颌关节和颌面部的正常发育;正确的人工牙排列位置和基托磨光面形态是改善可摘义齿固位稳定的重要因素;HED患儿无稳定的颌位关系,应根据颌面部及牙槽嵴的解剖标志确定并建牙合;患儿和家长的依从性对治疗效果极为重要。

Objective To study the early removable prosthetic treatment of hypohidrotic ectodermal dysplasia(HED)and evaluate the clinical outcome.Methods Ten young boys(aging from 4 to 7 years)with HED were treated by a series of prosthetic treatments using removable partial and complete dentures.The strategy of the prosthetic treatment was made on the basis of their age,the development of dentition and jaws.In order to promote the eruption of the partially erupting teeth,the Intaglio surface of the removable dentures was trimmed out in the path of eruption.All children were followed up in our clinic every six months for modifications and/or replacement of the dentures to avoid possible complications resulting from the jaw growth.Results All children adapted to the removable dentures during 1⁃8 weeks.Esthetics,phonetic function and swallowing were satisfactorily promoted.The retention of the dentures was not optimal because of the poor anatomical conditions.Only two patients were satisfied with the retention of their dentures,but nine patients reported that their chewing functions improved significantly.The jaw relationships of all children kept stable except one child who gave up wearing his lower complete denture and developed concave facial pattern.Conclusions Early removable prosthetic treatment may help promote the overall life quality and the growth of temporomandibular joints and maxillofacial region for children with HED.The arrangement and occlusion of artificial teeth and the shape of denture base polishing surface were the most important factors influencing denture stability.The children with HED didn′t have stable jaw relations,so that their jaw relations were established according to relevant anatomic landmarks during the clinic procedure,which was much different from that of adults.Patient compliance was very important for the long⁃term effects.