A Study of Facial Pattern in Patients with Fanconi Anemia

• Published in: The Cleft palate-craniofacial journal 2014-01, Vol.51 (1), p.83-89
• Main Authors: De Castro Ávila, Lucia Fátima, Martins, Wilson Denis, Cândido, Lisiane, Ignácio, Sergio Aparecido, Bonfim, Carmen Maria S., De Oliveira Ribas, Marina
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Brazil; Cephalometry; Child; Child, Preschool; Cross-Sectional Studies; Dentistry; Face; Fanconi Anemia - complications; Female; Genetic disorders; Humans; Male; Maxillofacial Development; Retrospective Studies; Skeletal system; Studies
• ISSN: 1055-6656; 1545-1569
• DOI: 10.1597/11-321.1

Objective This study is aimed to evaluate craniofacial features in patients with Fanconi anemia (FA) through cephalometric analysis and to classify the facial growth pattern to observe possible facial discrepancies. Design This is a cross-sectional study which employed a quantitative approach to compare linear and angular measurements of cephalometric analysis in lateral teleradiographic images of a clinical type sample of patients with FA. A retrospective cephalometric study was performed using cephalometric analyses of Ricketts and Steiner; growth patterns according to Ricketts' vertical growth pattern (VERT index) were also analyzed. Patients Fifty patients diagnosed with FA who were undergoing anti-aplasia treatment at the outpatient Hematology service at the Federal University of Paraná, Curitiba, Brazil were included in the study. Interventions The patients were evaluated in the School of Dentistry of the Pontifical Catholic University of Paraná (PUCPR), Curitiba, Brazil. Exclusion criteria included patients who had used or were using growth hormone medication, had undergone bone marrow transplant, or had been previously subjected to dental treatment. Main Outcome Measures Cephalometric points were plotted in order to set up linear and angular cephalometric measurements. Angular and linear measurements from 17 factors proposed by Ricketts' cephalometric analysis were assessed. Results Dolicofacial appearance was observed in 52% of individuals; braquifacial in 28%, and mesofacial in 20%. Significant maxillary/mandibular discrepancy was observed. It was concluded that upon anteroposterior evaluation of facial bone structures, the FA sample presented smaller median measurements in most variables evaluated; it also presented mandibular micrognathism and mainly dolicofacial vertical growth pattern. These findings, together with other features such as skin pigmentation and microphthalmia, may lead to a possible recognition of a FA condition from a patient's facial features.