Coxa Vara in Osteogenesis Imperfecta
Osteogenesis imperfecta (OI) is a heterogenous genetic disorder of connective tissue that causes low bone mass, bone fragility, multiple fractures, and reduced mobility and function. The type and severity of OI are variable. The Sillence classification has designated four types: Type I is a mild form, Type II is lethal at birth, Type III is very severe with multiple fractures often at birth, and Type IV is of mixed severity. Several other types were added later. Long bone deformities, including coxa vara, are typical findings in patients with OI, specifically in the more severely affected.
In this chapter, the management of a 4-year-old child with Type III osteogenesis imperfecta and coxa vara is discussed, including clinical presentation, radiographic findings, details of the surgical management, as well as pearls and pitfalls.
KeywordsCoxa vara Osteogenesis imperfecta Valgus osteotomy Fassier-Duval rods
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