Craniofacial Features in Richieri-Costa-Pereira Syndrome.

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 (73.8o ± 4.86o vs 78.85o ± 4.53o, 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.

Dezessete anos de acompanhamento imaginológico de pseudocistos recorrentes do seio maxilar em paciente com fissura palatina / Seventeen years imaging follow-up of recurrent pseudocysts of the maxillary sinus in a cleft patient

Relata-se um caso de paciente portador de fissura palatina apresentandopseudocistos em seios maxilares. As lesões foram diagnosticadas em radiografias panorâmicas obtidas com finalidade odontológica por um período de 17 anos, ora no lado direito, ora no lado esquerdo. Em algumas imagens as lesões não eram vistas. O presente relato demonstra a natureza silenciosa dos pseudocistos, além da possibilidade de resolução espontânea e reaparecimento.

It is reported a case of pseudocysts in maxillary sinuses in a cleft patient. The lesions were diagnosed on random panoramic radiographs for a period of 17 years, either in the right or left maxillary sinus. In some images the lesions were not seen. This report demonstrates the silent nature of pseudocysts,besides the possibility of spontaneous resolution and recurrence.

Ocorrência de anodontia do incisivo central superior em pessoas com fissura de lábio e/ou palato / Occurrence of partial anodontia of the maxillary central incisor in people with cleft lip and palate

As lesões lábio-palatinas acometem o terço médio da face, e causam problemas no desenvolvimento da arcada dentária, levando a um prejuízo estético e funcional. Relatos de hipodontia do incisivo lateral superior associado à presença de fissura são comuns, mas quando se considera a anodontia do incisivo central superior, são poucos os estudos específicos com um levantamento preciso. Sendo o incisivo central um dente de grande importância dentro do arco dentário, resolveu-se pesquisar a ocorrência de sua anodontia, assim considerar as situações em que ela ocorre. Os objetivos deste trabalho foram: Verificar a ocorrência de anodontia do incisivo central superior nos pacientes do HRACUSP; avaliar a prevalência da hipodontia dos incisivos centrais superiores de acordo com o gênero. Verificar a relação entre a hipodontia do incisivo central superior com a presença de fissura ou algum distúrbio na área afetada.O trabalho foi realizado utilizando-se uma amostra de 600 radiografias panorâmicas de pacientes do HRAC-USP, na faixa etária entre 6 e 8 anos. A metodologia consistiu-se na observação da radiografia panorâmica, com auxílio do negatoscópio. Concluiu-se que:- A hipodontia do incisivo central superior é rara, ocorrendo em 1,5 por cento da população observada.- A relação com hipodontia do incisivo lateral superior foi de 55,5 por cento.- Entre os pacientes com hipodontia do incisivo central, 55,5 por cento eram do gênero masculino, e 44,4 por cento do feminino.- A hipodontia do incisivo central superior pode estar relacionada a distúrbios como: holoprosencefalia, retardo do crescimento, anomalias na linha média e outros.

Cleft lip and palate affect the middle third of the face andbring about problems in the development of the dental arches, leading to esthetic and functional impairment. Reports of hypodontia of the maxillary lateral incisor associated with the presence of a cleft are common; however, few studies have specifically addressed the hypodontia of the maxillary central incisor. Since the central incisor is a very important tooth in the dental arch, the occurrence of hypodontia of this tooth, as well as its etiology, should be investigated.This study aimed to verify the occurrence of hypodontia of maxillary central incisor in patients at HRAC-USP; to evaluate the prevalence of hypodontia of maxillary central incisor according togender; and to assess the relation ship between hypodontia of maxillary central incisor and presence of the cleft or other disorders atthe affected area.The study was conducted on a sample of 600 orthopantomograms of patients of HRAC-USP, aged 6 to 8 years.The method comprised radiographic interpretation of the orthopantomogram, with aid of a film viewer.The following conclusions could be drawn:- Hypodontia of the maxillary central incisor is rare and affected 1.5 percent of the study population.- The relationship with hypodontia of the maxillary lateral incisor was 55.5percent.- Among the patients with hypodontia of maxillary central incisor, 55.5 percent were males and 44.4 percent were females.- Hypodontia of the maxillary central incisor may be related to disorders such as holoprosencephaly, delayed growth, midline alterations, and others.