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Coxa Vara in Osteogenesis Imperfecta

  • Reggie C. Hamdy
Chapter

Abstract

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.

Keywords

Coxa vara Osteogenesis imperfecta Valgus osteotomy Fassier-Duval rods 

Reference

  1. 1.
    Aarabi M, Rauch F, Hamdy RC, Fassier F. High prevalence of coxa vara in patients with severe osteogenesis imperfecta. J Pediatr Orthop. 2006;26(1):24–8.CrossRefGoogle Scholar

Suggested Reading

  1. Fassier F, Ashby E, Hamdy RC. Case 86: Coxa vara in a nine-year-old boy with osteogenesis imperfecta. In: Rozbruch SR, Hamdy R, editors. Limb lengthening and reconstruction surgery atlas: pediatric deformities. Cham: Springer; 2015. p. 619–22.CrossRefGoogle Scholar
  2. Fassier F, Glorieux FH. Osteogenesis imperfecta. In: DuParc J, editor. Surgical techniques in orthopaedics and traumatology 55-050-D-30. New York: Elsevier; 2003. p. 1–8.Google Scholar
  3. Fassier F, Sardar Z, Aarabi M, Odent T, Haque T, Hamdy R. Results and complications of a surgical technique for correction of coxa vara in children with osteopenic bones. J Pediatr Orthop. 2008;28(8):799–805.CrossRefGoogle Scholar
  4. Fassier FR. Osteogenesis imperfecta. In: Sanjeev S, editor. Pediatric lower limb deformities: principles and techniques of management. Cham: Springer; 2016. p. 255–65.CrossRefGoogle Scholar
  5. Trejo P, Rauch F. Osteogenesis imperfecta in children and adolescents—new developments in diagnosis and treatment. Osteoporos Int. 2016;27(12):3427–37.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Orthopaedic SurgeryMcGill University Health CentreMontrealCanada
  2. 2.The Montreal Children’s HospitalMontrealCanada
  3. 3.Shriners Hospital for Children – CanadaMontrealCanada

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