Title: Functional Outcome of Osteotomy and Telescopic intramedullary nailing in paediatric cases of Osteogenesis imperfecta with Long bone Deformities
Authors: T. Satish Chandra Reddy, Chennakesava Rao Sajja
DOI: https://dx.doi.org/10.18535/jmscr/v7i2.181
Abstract
Introduction: The main feature of osteogenesis imperfecta is an excessive fragility and deformability of the bones owing to reduced mass and bone quality. This leads to angular deformity and frequent fractures. These fractures usually heal normally, and malunion is a common complication with conservative management.
Aims and Objectives: To study the outcome of osteotomy and telescopic intramedullary nailing in paediatric cases of Osteogenesis imperfecta with deformities in long bones.
Methods and Methodology: 6 cases of Osteogenesis imperfecta with deformities of long bones of lower limb were included in the study. All were treated using Telescopic intramedullary nailing after corrrection of deformity by osteotomy. Results were evaluated after 1 year of follow up by using radiological evaluation and functional usage of the limb.
Results: Radiological fracture healing was noticed at 14 weeks in all cases. Allowed full weight bearing after radiological fracture healing. Patients were able to walk without any aids after 6 months. One case had recurrence of deformity at lower tibia after 1 year of follow up.
Conclusion: The staged surgical stabilization performed allows for simultaneous correction of deformities and fracture healing resulting in rapid mobilization and better functional outcome.
Keywords: Osteofenesis imperfect, deformity correction, telescopic nailing.
References
- Glorieux F. Osteogenesis Imperfecta. Best Pract Res Clin Rheumatol.2008 Mar;22(1):85-100. doi: 10.1016/j.berh.2007.12.012.
- Forlino A, Cabral WA, Barnes AM,Marini JC. New perspectives on osteogenesis imperfecta. Nat Rev Endocrinol. 2011.doi:10.1038/nrendo.2011.81.
- Smith R. Osteogenesis imperfecta: from phenotype to genotype and back again.Int J Exp Pathol. 1994; 75:233e41.
- Birke O, Davies N, Latimer M, Little DG,Bellemore M. Experience with the Fassier-Duval telescopic rod: first 24c onsecutive cases with a minimum of 1-year follow-up. J Pediatr Orthop. 2011Jun; 31(4):458-64. doi:10.1097/BPO.0b013e31821bfb50.
- McKusick VA. Mendelian Inheritance in Man. 9th ed., 1990, Baltimore: JohnsHopkins University Press.
- King JD, Boblechko WP. Osteogenesisim perfecta: An orthopaedic description and surgical review. J Bone Joint Surg.1971; 53B:72e89.
- Sillence D. Osteogenesis imperfecta: an expanding panorama of variants. Clin Orthop. 1981; 159:11e25.
- Bălănescu R, Ulici A, Rosca D, Topor L, Barbu M. Use of minimally invasive (percutaneous) Fassier-Duval telescopic rod on an 8 year old patient with Lobstein disease. Chirurgia (Bucur). 2013 Jan-Feb;108(1):120-5.
- Sterian A, Balanescu R, Barbilian A, Ulici A. Osteosynthesis in Osteogenesis Imperfecta, telescopicversus non-telescopic nailing. Journal of Medicine and Life Vol. 8, Issue 4, October-December 2015