Pycnodysostosis: Characteristics of teeth, mouth and jaws

IF 2.4 3区医学 Q2 DENTISTRY, ORAL SURGERY & MEDICINE

Orthodontics & Craniofacial Research Pub Date : 2024-03-26 DOI:10.1111/ocr.12782

Nikolaos Ferlias, Hans Gjørup, Mia Aagaard Doherty, Thomas Klit Pedersen

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Abstract

Objectives

To describe the clinical and radiographic oro-dental characteristics of patients with pycnodysostosis (PDO).

Materials & Methods

A short interview and clinical examination of seven patients with PDO were performed as well as assessment of the temporomandibular joints and masticatory muscles using the diagnostic criteria for temporomandibular disorders, DC-TMD form. A full set of records were taken including photos and intraoral scan. Finally, existing cone beam computed tomography (CBCT) images and radiographs were also studied.

Results

All patients presented with bimaxillary micrognathia, five had a convex profile, and two had a straight profile. In addition, posterior open bite, Angle Class III molar relation with accompanying anterior crossbite and a grooved median palate were common findings. No patient showed symptoms of temporomandibular disorder (TMD) apart from some clicking. Finally, the main radiographic findings were the obtuse mandibular angle, the frontal bossing, the elongation of the coronoid/condylar process and the presence of hypercementosis with obliterated pulp chambers.

Conclusion

The examined patients with PDO were characterized by dental crowding, malocclusion (anterior crossbite, posterior open bite), hypercementosis, obliterated pulp chambers and deviations in mandibular morphology. In conclusion, patients with PDO have a specific need for dental and orthodontic monitoring with focus on crowding and posterior open bite. The patients will benefit from a long-term orthodontic plan including extractions.

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侏儒症：牙齿、口腔和颌骨的特征。

材料与方法描述侏儒症（PDO）患者的临床和影像学口腔特征：对 7 名 PDO 患者进行简短访谈和临床检查，并使用颞下颌关节紊乱诊断标准（DC-TMD 表）对颞下颌关节和咀嚼肌进行评估。还进行了全套记录，包括照片和口腔内扫描。最后，还研究了现有的锥形束计算机断层扫描（CBCT）图像和X光片：结果：所有患者都有双颌小颌畸形，其中五人的颌面呈凸形，两人的颌面呈直线形。此外，后方开牙合、角Ⅲ类臼齿关系伴前方交叉咬合和中腭凹槽也是常见的检查结果。除了一些咔哒声外，没有患者出现颞下颌关节紊乱（TMD）症状。最后，主要的影像学检查结果是下颌钝角、额突、冠状突/髁突伸长以及牙髓腔阻塞的牙髓增生：受检的 PDO 患者具有牙齿拥挤、错颌（前交叉咬合、后开放咬合）、牙釉质发育不全、牙髓腔阻塞和下颌形态偏差等特征。总之，PDO 患者特别需要牙科和正畸监测，重点是拥挤和后方开放性咬合。患者将受益于包括拔牙在内的长期正畸计划。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Orthodontics & Craniofacial Research 医学-牙科与口腔外科

CiteScore

5.30

自引率

3.20%

发文量

审稿时长

>12 weeks

期刊介绍： Orthodontics & Craniofacial Research - Genes, Growth and Development is published to serve its readers as an international forum for the presentation and critical discussion of issues pertinent to the advancement of the specialty of orthodontics and the evidence-based knowledge of craniofacial growth and development. This forum is based on scientifically supported information, but also includes minority and conflicting opinions. The objective of the journal is to facilitate effective communication between the research community and practicing clinicians. Original papers of high scientific quality that report the findings of clinical trials, clinical epidemiology, and novel therapeutic or diagnostic approaches are appropriate submissions. Similarly, we welcome papers in genetics, developmental biology, syndromology, surgery, speech and hearing, and other biomedical disciplines related to clinical orthodontics and normal and abnormal craniofacial growth and development. In addition to original and basic research, the journal publishes concise reviews, case reports of substantial value, invited essays, letters, and announcements. The journal is published quarterly. The review of submitted papers will be coordinated by the editor and members of the editorial board. It is policy to review manuscripts within 3 to 4 weeks of receipt and to publish within 3 to 6 months of acceptance.