Normal angulation of skull base in Apert syndrome
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Citações na Scopus
10
Tipo de produção
article
Data de publicação
2018
Título da Revista
ISSN da Revista
Título do Volume
Editora
CHURCHILL LIVINGSTONE
Autores
LU, Xiaona
FORTE, Antonio Jorge
SAWH-MARTINEZ, Rajendra
WU, Robin
CABREJO, Raysa
STEINBACHER, Derek M.
ALPEROVICH, Michael
PERSING, John A.
Citação
JOURNAL OF CRANIO-MAXILLOFACIAL SURGERY, v.46, n.12, p.2042-2051, 2018
Resumo
Apert syndrome is characterized by the severe craniofacial deformities. The subsequent process of skeletal maldevelopment is likely to be influenced by multiple interactions at several levels, at a given time. In this study, we aimed to explore the evolution of cranial basal dysmorphology and the chronology of these deformities in Apert syndrome, by objectively analyzing three-dimensional measurements. Fifty-four CT scans from unoperated patients (Apert, n = 18; control, n = 36) were included in this study, with age range from 3 days to 24 years. Before 6 months of age, Apert's anterior cranial base was widened 60%. Between 6 months and 2 years of age, the whole cranial base length, anterior cranial base length and posterior cranial base length decreased 8%, 8% and 14%, respectively. The greater sphenoid wing angle was wider by 26.0 degrees, and continued into adulthood. The cranial base angles did not produce significant changes throughout life. The extra cranial distances synchronously and almost proportionally shortened after later infancy. The anterior and posterior cranial base length shortened at an almost proportional rate. The malformations of the skull vault are additive effects with cranial base fusion on skull length restriction, but the angulation of the skull base is virtually normal.
Palavras-chave
Apert syndrome, Cranial base, Cephalometries, Development
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