Abstract
This case report describes a 13-year-old patient suffering from osteogenesis imperfecta (OI) and hypodontia. Different issues related to clinical presentation and orthodontic treatment of patients with OI are discussed in this report.
From Volume 6, Issue 1, January 2013 | Pages 21-24
This case report describes a 13-year-old patient suffering from osteogenesis imperfecta (OI) and hypodontia. Different issues related to clinical presentation and orthodontic treatment of patients with OI are discussed in this report.
Osteogenesis imperfecta (OI) known as ‘brittle bone disease’ is defined as a group of heritable connective tissue disorders, caused by a defect in the synthesis of type I collagen. The most common causes of this condition are mutations of the genes COL1A1 and COL1A2 that encode chains of type I collagen.1 Because of the variety in clinical presentation and the emergence of new genetic mutations, the exact incidence of OI is unknown. However, an incidence rate of 1:5000 to 1:20,000 live births was reported by different authors.2,3
Patients with OI may show a plethora of signs and symptoms related to the defective collagen present in different structures of the body, including bone, sclera, skin, ligaments, blood platelets, auditory ossicles, inner ear and teeth. Thus, the signs and symptoms include:
OI is classified on the basis of clinical, genetic and radiographic features.1 Sillence et al18 divided OI into four types. Since then further types have been added:
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