Effect of Bisphosphonates on Function and Mobility Among Children With Osteogenesis Imperfecta: A Systematic Review

Abstract

Osteogenesis imperfecta (OI) is a rare genetic connective tissue disorder that results in bone fragility and deformity. Management is multi‐disciplinary. Although pharmacologic intervention with bisphosphonates (BP) is a standard of care for individuals with severe OI, no consensus or reviews were found that focus on the effects of bisphosphonates on function and mobility. PubMed, CINAHL, Cochrane Library, Web of Science, and PEDro databases were searched for eligible articles for this review. Methodological quality was assessed using the Cochrane Collaboration's tool for risk of bias. Twenty‐six studies (801 children) were reviewed and five showed a low risk of bias. Included studies showed significant variability among clinical protocols for administering BP. Randomized controlled trials did not demonstrate a significant improvement in function and mobility with oral BP administration, while non‐randomized open‐label uncontrolled studies demonstrated that oral and intravenous BP administration objectively improved function and mobility. The most common outcome measure used by the studies included in this review was the Bleck score. Effect sizes (d = 0.28 ‐ 4.5) varied among studies. This systematic review also summarized the apparent confounding variables affecting results of previous studies and provided suggestions to improve the quality of future studies.

A Multicenter Study of Intramedullary Rodding in Osteogenesis Imperfecta

“…Some studies have postulated that use of bisphosphonates could facilitate a rodding surgical procedure 11 , 13 , 14 , 39 and benefit mobility, muscle force, and well-being 40 , 41 , and other studies found no changes in mobility 19 , 42 , 43 . A recent systematic review concluded that children given intravenous bisphosphonates have increased mobility 44 . However, in this study, we found no correlation between the use of bisphosphonates and mobility outcomes in the rodded group.…”

Intraoperative Hardware Failure of the Fassier–Duval Rescue System in a Pediatric Patient with Osteogenesis Imperfecta

“…Patients with osteogenesis imperfecta suffer from brittle bones, joint hyperlaxity, and a number of different soft tissue defects [9]. Bisphosphonates remain the mainstay of medical treatment for these patients [10][11][12]. e orthopaedic surgical management utilizing intramedullary fixation to treat long bone deformity and prevent future fractures has been thoroughly described and has demonstrated successful long-term clinical and radiographic outcomes [1,3,5,13].…”

Stem Cell Therapy as a Treatment for Osteogenesis Imperfecta

“…Bisphosphonates are used to reduce bone resorption and increase bone mineral density. The effect of bisphosphonates in OI has been debated, and according to a recent systematic review, randomized controlled trials did not demonstrate a significant improvement in function and mobility with oral bisphosphonate administration, whereas nonrandomized open-label uncontrolled studies demonstrate that oral and intravenous bisphosphonate administration objectively improved function and mobility [ 9 ].…”