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The goals of therapy include family support to best normalize the child’s life despite their handicap and maintain as much mobility as possible.1
correspond to drug therapy cycles. They represent focal areas of increased BMD. Areas between these lines represent bone growth and help demonstrate that bisphosphonates do not halt growth in pediatric patients.39 These areas are also potential stress-risers and locations for future fractures given the relative deceased BMD.
study comparing oral alendronate to intravenous pamidronate showed equivalent bioavailability and equivalent outcomes with respect to BMD and safety despite known poor gastrointestinal absorption.40 A recent randomized control trial comparing a cohort of patients receiving oral risedronate to placebo revealed only marginal improvements in BMD in the treatment group side effects.41 A Cochrane review on bisphosphonates revealed increased BMD seen in all trials with oral therapy; however, there were mixed results on fracture risk reduction.36
trial evaluating the use of IV zoledronic acid in children with OI
Z-scores, decreases in fracture rate, pain relief, and improvements in ambulation.42 A recent Cochrane review evaluated 14 trials involving more than 800 patients was unable to demonstrate pain reduction, improved growth, or functional mobility. Only select studies demonstrated diminished fracture risk. Further, there is limited data regarding the optimal method, therapeutic duration, and long-term safety of this group of drugs.43 Adequately powered trials with a fracture of bisphosphonates on OI.
they are not without their potential hazards. Bisphosphonates
can potentially inhibit bone formation after fracture and osteotomy. One study on pamidronate demonstrated delayed bone healing after osteotomy that was not seen after fracture. The delay in healing was associated with older age and tibial osteotomies.44 Another hypothetical complication associated with bisphosphonate use is osteonecrosis of the jaw. This complication is associated with chronic and long-term bisphosphonate use and mainly in cancer patients and its association in the OI population remains hypothetical.45 A recent systematic review of the literature from 1946 to 2013 revealed no evidence supporting a causal relationship between bisphosphonates and osteonecrosis of the jaw in children and adolescents with OI.46 Adults on chronic bisphosphonates are subject to pathologic subtrochanteric femur fractures. A recent case series on six patients with OI treated with cyclic pamidronate treatment demonstrated similar stress fracture patterns further highlighting concerns about prolonged bisphosphonate use and its effect on remodeling suppression.47
In adults, osteoanabolic treatment with teriparatide has been evaluated with some studies showing increases in BMD with mild types OI variants.48,49 No current data exists on its use in children due to rat studies demonstrating an increased risk of neoplasms.50
SURGICAL TREATMENT
Initial fracture management consists of conservative treatment with casting. Fracture healing in OI patients is normal. The period of immobilization should be as short as possible so that muscle function can be restored and secondary osteoporosis can be prevented.7
The most common surgical procedure employed for long bones is intramedullary nailing or rodding. The goals of surgery include restoration of the anatomic axis and mechanical axis of bone, fracture prevention, and deformity correction. Surgery is done
to give the child the best possibility of weight-bearing. Most techniques use the multilevel osteotomies in conjunction with
an implant that “shish-kabobs” the bone segments to correct the 51 The device used has evolved remarkably from non-expandable, 52,53 The growth resulted in angulation and fracture at levels of non-splinted bone and a shortened interval to revision surgery.54
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Del Med J | June 2016 | Vol. 88 | No. 6
