Initial Experience With Percutaneous IM Rodding of the Humeri in Children With Osteogenesis Imperfecta

Grossman, Leonid; Price, Amber L.; Rush, Eric T.; Goodwin, Jennifer L.; Wallace, Maegen; Esposito, Paul W.

doi:10.1097/bpo.0000000000000856

Cited by 14 publications

(23 citation statements)

References 22 publications

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“…However, again these are complex surgeries and post-operative problems are common. High rates of revision surgery are reported from most centers (66).…”

Section: Orthopedic Management Of Deformitymentioning

confidence: 99%

Management of Osteogenesis Imperfecta

Ralston

Gaston

2020

Front. Endocrinol.

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Osteogenesis imperfecta (OI) is the term used to describe a group of rare inherited skeletal disorders characterized by a greatly increased risk of fragility fractures (1). Mutations in several genes can cause OI but the condition is most commonly caused by mutations of COLIA1 or COL1A2 resulting in the production of collagen which is abnormal or present in reduced amounts. Fractures in OI are particularly common during childhood but the elevated fracture risk continues throughout life. Bone mineral density (BMD) can be reduced in OI but the magnitude of increase in fracture risk is far greater than can be accounted for by low BMD, highlighting that a key mechanism of bone fragility is reduced bone quality due to defects of bone matrix and mineralization. A multidisciplinary approach is needed to optimize management of OI, with input from physicians, orthopedic surgeons, physiotherapists, occupational therapists, and other allied health professionals. Orthopedic surgery plays a key role both in the fixation of fractures and in the correction of limb deformities. Bisphosphonates have been widely used in the treatment of children and adults with OI. Although there is good evidence that they increase BMD, it is uncertain to what extent they reduce fracture risk. Clinical trials of bone anabolic drugs such as teriparatide and inhibitors of sclerostin have also been studied; although they increase BMD, studies of these agents have not been powered to look at fracture endpoints. Various other treatment modalities including denosumab, and cell therapy have been explored but haven't gained acceptance in routine clinical practice. There have been huge advances in understanding the pathogenesis of OI but these have not been accompanied by advances in treatment. This signals need for well-designed clinical trials with fracture endpoints in OI, both with existing agents and with the newer therapeutic agents that are now starting to emerge.

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“…However, again these are complex surgeries and post-operative problems are common. High rates of revision surgery are reported from most centers (66).…”

Section: Orthopedic Management Of Deformitymentioning

confidence: 99%

Management of Osteogenesis Imperfecta

Ralston

Gaston

2020

Front. Endocrinol.

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“…The outcomes are very promising. 15,[39][40][41] In all the years, despite improved surgical techniques and more sophisticated implants, the overall complication and reoperation rate has not changed a lot. 25,[27][28][29]31 The causes of failure have not changed either: distal and proximal rod migration, jamming of the telescoping mechanism, bending rods due to the operating mechanical and muscular forces and technical issues with the operation.…”

Section: Discussionmentioning

confidence: 99%

“…25,28,30 In the humerus a revision rate of 34% to 57% was recorded. 15,29 With the improvement of the implants and implication of more advanced surgical techniques the complication and reoperation rates are thought to be less. The reality, however, is that with the improvement of the global medical care in those patients, the activity levels rise.…”

Section: Complications and Causes Of Revision Surgerymentioning

confidence: 99%

“…In the upper extremity, however, telescoping nails are only usable for humeral rodding. 15 The radius and ulna do not generally accept telescoping nails due to the small diameters of the intramedullary canal and the difficult accessibility of the radius. Hence, flexible intramedullary nails, Rush rods or Kirschner (K)-wires A simultaneous correction of both the right humerus and forearm was performed using multiple osteotomies; telescopic nail for humerus; and Kirschner-wires in the radius and ulna (c).…”

Section: Upper Extremitymentioning

confidence: 99%

“…Because of the increased stability of the plated bone area, stress risers are created above and below with predictable deformity occurrence, if they are kept in place as a single implant. While humerus corrections in OI patients are mostly possible from the age of three years, 15 forearm correction requires a minimum size of both bones and the intramedullary canal. The indication for upper extremity correction is currently worked out with the patients themselves and their parents.…”

Section: Upper Extremitymentioning

confidence: 99%

See 2 more Smart Citations

The orthopaedic management of long bone deformities in genetically and acquired generalized bone weakening conditions

Wirth

2019

Journal of Children's Orthopaedics

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PurposeDiseases such as osteogenesis imperfecta, fibrous dysplasia, hypophosphataemic rickets and others lead to soft and weak bones and long bone deformity in affected patients. As a consequence, these patients lose their walking capacity and functional abilities of the upper extremities as well.MethodsIn combination with bisphosphonate treatment and physical rehabilitation programmes surgical interventions are being applied to correct and stabilize the deformed and less mechanically resistant long bones. Intramedullary devices, ideally with an elongating telescopic mechanism, have proven to be the most suitable implants.ResultsThe surgical correction and stabilization of deformed bones in weak bone diseases is very beneficial to the patients. Pain restriction, reduction of fracture events, minimization of consequences of traumatic events and falls have resulted in a significant functional improvement. The patients live on a better activity level with a much-improved individual independence. Despite a high complication and revision rate of the intramedullary rods the gain of quality of life outweighs these negative aspects by far.ConclusionOrthopaedic surgical treatment for deformed bones in patients with weak bone diseases has changed the life of the patients and plays a very important role in the disease management protocols. This paper was written under the guidance of the Study Group Genetics and Metabolic Diseases of the European Paediatric Orthopaedic Society.

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Upper Extremity

Wirth

2020

Osteogenesis Imperfecta

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Initial Experience With Percutaneous IM Rodding of the Humeri in Children With Osteogenesis Imperfecta

Abstract: Level IV-retrospective case series.

Cited by 14 publications

References 22 publications

Management of Osteogenesis Imperfecta

Management of Osteogenesis Imperfecta

The orthopaedic management of long bone deformities in genetically and acquired generalized bone weakening conditions

Upper Extremity

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