Osteogenesis imperfecta

Fracture prevention and prevention of scoliosis

• Bisphosphonates are used in patients with OI for secondary prevention of fractures, although the evidence to support reduction of risk of fracture is limited. In children with OI, bisphosphonates have been shown in some studies to have beneficial effects on areal and volumetric bone mineral density, quality of life, fracture incidence, and progression of scoliosis.[35]Anissipour AK, Hammerberg KW, Caudill A, et al. Behavior of scoliosis during growth in children with osteogenesis imperfecta. J Bone Joint Surg Am. 2014 Feb 5;96(3):237-43. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6948836 http://www.ncbi.nlm.nih.gov/pubmed/24500586?tool=bestpractice.com [62]Bains JS, Carter EM, Citron KP, et al. A multicenter observational cohort study to evaluate the effects of bisphosphonate exposure on bone mineral density and other health outcomes in osteogenesis imperfecta. JBMR Plus. 2019 May;3(5):e10118. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6524673 http://www.ncbi.nlm.nih.gov/pubmed/31131341?tool=bestpractice.com [76]Glorieux FH, Bishop NJ, Plotkin H, et al. Cyclic administration of pamidronate in children with severe osteogenesis imperfecta. N Engl J Med. 1998 Oct 1;339(14):947-52. https://www.nejm.org/doi/full/10.1056/NEJM199810013391402 http://www.ncbi.nlm.nih.gov/pubmed/9753709?tool=bestpractice.com [77]Bishop N, Adami S, Ahmed SF, et al. Risedronate in children with osteogenesis imperfecta: a randomised, double-blind, placebo-controlled trial. Lancet. 2013 Oct 26;382(9902):1424-32. http://www.ncbi.nlm.nih.gov/pubmed/23927913?tool=bestpractice.com [78]Rauch F, Plotkin H, Zeitlin L, et al. Bone mass, size, and density in children and adolescents with osteogenesis imperfecta: effect of intravenous pamidronate therapy. J Bone Miner Res. 2003 Apr;18(4):610-4. http://www.ncbi.nlm.nih.gov/pubmed/12674321?tool=bestpractice.com [79]Rauch F, Travers R, Glorieux FH. Intracortical remodeling during human bone development--a histomorphometric study. Bone. 2007 Feb;40(2):274-80. http://www.ncbi.nlm.nih.gov/pubmed/17049943?tool=bestpractice.com

Ear protection

• Provide advice to all patients with OI regarding ear protection (such as use of ear plugs or ear muffs in situations where environmental noise may be high, and regarding controlling volume on devices that send sound directly to the ear), to defer or minimize hearing loss wherever possible.

Other complications

• Screening for development of audiologic, cardiovascular, pulmonary, and dental abnormalities may help to prevent development and progression of associated complications. See Monitoring.