title: Orthotic treatment of positional brachycephaly associated with osteogenesis imperfecta.
abstract: Osteogenesis imperfecta is an inherited disorder of the connective tissue characterized primarily by fractures with no or small causal antecedents and extremely variable clinical presentation. The disorder requires a global and, therefore, multidisciplinary therapeutic approach that should aim, among other aspects, at the prevention and treatment of deformities resulting from osteogenesis imperfecta. Due to limitations related to bony deformities, it can be difficult to place these infants in a variety of positions that would help remediate skull deformities, so a cranial orthosis becomes the therapy of choice. The aim of this study was to demonstrate the results obtained during treatment with a cranial remolding orthosis (helmet) in babies with osteogenesis imperfecta. Case Description and Methods: For the first time in the scientific literature, this study describes the use of a cranial orthosis for the treatment of infants with osteogenesis imperfecta. Both children had severe asymmetrical brachycephaly documented by laser digital scanning and were submitted to treatment with a cranial remolding orthosis. Outcomes and Conclusion: The study showed that there was a significant improvement in cranial proportion and symmetry, with a reduction in the cephalic index at reevaluation. It is concluded that the orthotic therapy is an effective therapeutic modality to improve the proportion and minimize the asymmetry in children with osteogenesis imperfecta. Clinical relevance The clinical relevance of such a description is that children with osteogenesis imperfecta may have numerous deformities and minimizing them can be an important factor. This report showed a beneficial result as the orthotic therapy modality improved the proportions and minimized the asymmetry. This treatment offers too high levels of satisfaction to parents and brings these children closer to normal indices.
authors.affiliation: 1 Heads-Clinica Dr. Gerd Schreen, São Paulo, Brazil.
authors.forename: Carolina G
authors.initials: CG
authors.lastname: Matarazzo
authors.affiliation: 1 Heads-Clinica Dr. Gerd Schreen, São Paulo, Brazil.
authors.forename: Gerd
authors.initials: G
authors.lastname: Schreen
authors.affiliation: 2 Hospital Israelita Albert Einstein, Sao Paulo, Brazil.
authors.forename: Camilla D do
authors.initials: CDD
authors.lastname: Lago-Rizzardi
authors.affiliation: 3 Universidade Federal de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Maria Stella
authors.initials: MS
authors.lastname: Peccin
authors.affiliation: 4 Universidade de Sao Paulo, Sao Paulo, Brazil.
authors.forename: Fernando Cg
authors.initials: FC
authors.lastname: Pinto
mesh_terms: D003398:Craniosynostoses / Q000000981:diagnostic imaging / Q000209:etiology / Q000628:therapy*; D005260:Female; D006260:Head Protective Devices; D006801:Humans; D007223:Infant; D008297:Male; D009989:Orthotic Devices; D010013:Osteogenesis Imperfecta / Q000150:complications*
keywords: Osteogenesis imperfecta; infant; posterior plagiocephaly; skull