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Osteogenesis imperfecta: minimizing complications in oral surgery/orthodontic treatment Kaye Jordan Emily Carter Ciarán P Devine Aliki Tsichlaki Judith Jones Dental Update 2024 48:9, 707-709.
Osteogenesis imperfecta (OI) is a rare genetic condition that affects connective tissue and is primarily characterized by bone fragility. Owing to the progressive nature of the disease, patients require life-long management of the adverse effects and the subsequent complications they can experience. Since the introduction of bisphosphonates as a treatment modality, complications and bone deformities associated with OI have been minimized. However, intravenous bisphosphonates in young patients could present a new era of difficulties for the dental profession. We report on a case of a patient with OI who presented with severe orthodontic crowding and describe the multidisciplinary treatment planning required to minimize potential complications.
CPD/Clinical Relevance: General dental practitioners should be aware of the characteristics of osteogenesis imperfecta and the potential complications associated with dental treatment.
Article
Osteogenesis imperfecta (OI) is a hereditary connective tissue disorder with variable phenotypic presentations.1 There is a reported incidence of 15,000–20,000 live births globally.2 OI is characterized by a decreased amount of normal type 1 collagen and this can be a result of decreased collagen secretion or abnormal collagen production.1 Traditionally OI has been subdivided into four subtypes (Table 1).3 In recent years however, at least three further subtypes have been discovered.1 It has now been suggested that all classifications represent a continuum of the same disease, ranging from mild to severe, with the majority of OI cases classified as type 1 of mild.1
AD: autosomal dominant.
Collagen forms a major portion of connective tissue, which is used to establish the framework for bone formation. With bone being poorly formed, a common descriptor for this condition has aptly been ‘brittle bone disease’. Apart from brittle bones however, other tissues and systems are affected including ocular, dental and cardiac.4,5
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