Craniofacial Features in Richieri-Costa-Pereira Syndrome

Milena P Pardo; Gabriela Lopes Dos Santos; Izabel M M Carvalho; Kellen Cristine Tjioe

doi:10.1177/1055665620987749

Craniofacial Features in Richieri-Costa-Pereira Syndrome

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

Authors

Milena P Pardo¹, Gabriela Lopes Dos Santos², Izabel M M Carvalho¹, Kellen Cristine Tjioe^{1

2}

Affiliations

¹ Hospital for Rehabilitation of Craniofacial Anomalies, University of São Paulo, Rua Silvio Marchione, Sao Paulo, Brazil.
² Oral Oncology Center, São Paulo State University, Rua Jose Bonifácio, Araçatuba, Sao Paulo, Brazil.

PMID: 33504197
DOI: 10.1177/1055665620987749

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 t test was employed. P 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^o ± 4.86^o vs 78.85^o ± 4.53^o, P = .029), maxillary length (7.89 cm ± 0.58 cm vs 16.36 cm ± 0.75 cm, P = .001), mandibular length (9.90 cm ± 0.46 cm vs 20.61 cm ± 0.45 cm, P = .001), upper anterior face height (5.41 cm ± 0.50 cm vs 9.40 cm ± 0.47 cm, P = .001), lower anterior face height (5.48 cm ± 0.75 cm vs 11.66 cm ± 0.55 cm, P = .001), and posterior face height (6.70 cm ± 0.33 cm vs 13.65 cm ± 1.06 cm, P = .001). There was no difference in SNB, A point-nasion-B point, pogonion-nasion-B point, and mandibular place angles between the groups (P > .05).

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

Keywords: Pierre-Robin sequence; Richieri-Costa-Pereira syndrome; cephalometry; orthodontic treatment; orthognathic surgery.

MeSH terms

Adolescent
Clubfoot*
Female
Hand Deformities, Congenital*
Humans
Maxilla
Pierre Robin Syndrome*

Supplementary concepts

Richieri Costa Pereira syndrome