里奇-科斯塔-佩雷拉综合征的颅面特征。

Craniofacial Features in Richieri-Costa-Pereira Syndrome.

机构信息

Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Rua Silvio Marchione, Sao Paulo, Brazil.

Oral Oncology Center, São Paulo State University, Rua Jose Bonifácio, Araçatuba, Sao Paulo, Brazil.

出版信息

Cleft Palate Craniofac J. 2021 Nov;58(11):1370-1375. doi: 10.1177/1055665620987749. Epub 2021 Jan 28.

DOI:10.1177/1055665620987749

PMID:33504197

Abstract

OBJECTIVE

Patients with Richieri-Costa-Pereira syndrome (RCPS) present severe craniofacial alterations and frequently require orthodontic and surgical procedures. Thus, this study aims to describe the craniofacial relationships in patients with RCPS.

DESIGN

Panoramic radiographs and lateral cephalometric teleradiographs of 7 patients with RCPS and 7 age- and sex-matched nonsyndromic patients were analyzed. Cephalometric measurements were used to determine the size of apical bases, the relationship between them, the pattern of craniofacial growth, and the facial heights of the patients. Interobservers' concordance was verified by intraclass coefficient. For comparison between the groups, paired test was employed. values <.05 indicated statistical significance.

RESULTS

Average age of patients with RCPS was 18.5 years. Six patients were female. All patients with RCPS had Pierre-Robin sequence while 2 also presented cleft mandible. Most patients with RCPS had missing lower central incisors (100%), lower lateral incisors (85.7%), lower second premolars (85.7%), and/or upper lateral incisors (57.1%). Concordance between observers was excellent for all cephalometric measurements (0.87-0.99). Patients with RCPS presented severe craniofacial alterations when compared to control group: sella-nasion-B point (SNB) angle (73.8 ± 4.86 vs 78.85 ± 4.53, = .029), maxillary length (7.89 cm ± 0.58 cm vs 16.36 cm ± 0.75 cm, = .001), mandibular length (9.90 cm ± 0.46 cm vs 20.61 cm ± 0.45 cm, = .001), upper anterior face height (5.41 cm ± 0.50 cm vs 9.40 cm ± 0.47 cm, = .001), lower anterior face height (5.48 cm ± 0.75 cm vs 11.66 cm ± 0.55 cm, = .001), and posterior face height (6.70 cm ± 0.33 cm vs 13.65 cm ± 1.06 cm, = .001). There was no difference in SNB, A point-nasion-B point, pogonion-nasion-B point, and mandibular place angles between the groups ( > .05).

CONCLUSION

Patients with RCPS present deficient development of maxilla and mandible when compared with nonsyndromic patients.

摘要

目的

里基耶里-科斯塔-佩雷拉综合征（RCPS）患者存在严重的颅面畸形，经常需要进行正畸和手术治疗。因此，本研究旨在描述 RCPS 患者的颅面关系。

设计

对 7 例 RCPS 患者和 7 例年龄和性别匹配的非综合征患者的全景片和侧位头颅 teleradiographs 进行了分析。头影测量用于确定根尖基的大小、它们之间的关系、颅面生长模式和患者的面高。通过组内系数验证观察者之间的一致性。为了比较两组之间的差异，采用配对 t 检验。<.05 表示有统计学意义。

结果

RCPS 患者的平均年龄为 18.5 岁。6 名患者为女性。所有 RCPS 患者均存在 Pierre-Robin 序列，其中 2 例还存在下颌裂。大多数 RCPS 患者缺失下中切牙（100%）、下侧切牙（85.7%）、下第二前磨牙（85.7%）和/或上侧切牙（57.1%）。所有头影测量的观察者间一致性均极好（0.87-0.99）。与对照组相比，RCPS 患者存在严重的颅面畸形：蝶鞍-前鼻棘点-下颌骨后缘点（SNA）角（73.8±4.86 对 78.85±4.53，=0.029）、上颌长度（7.89cm±0.58cm 对 16.36cm±0.75cm，=0.001）、下颌长度（9.90cm±0.46cm 对 20.61cm±0.45cm，=0.001）、上前牙区高度（5.41cm±0.50cm 对 9.40cm±0.47cm，=0.001）、下前牙区高度（5.48cm±0.75cm 对 11.66cm±0.55cm，=0.001）和后面高（6.70cm±0.33cm 对 13.65cm±1.06cm，=0.001）。两组间 SNA、A 点-前鼻棘点-下颌骨后缘点、颏顶点-前鼻棘点-下颌骨后缘点和下颌平面角无差异（>0.05）。