Dual Interlocking Telescopic Rod Provides Effective Tibial Stabilization in Children With Osteogenesis Imperfecta

Shin, Chang Ho; Lee, Dong Hoon; Yoo, Won Joon; Choi, In Ho; Cho, Tae Joon

doi:10.1097/corr.0000000000000429

Cited by 12 publications

(18 citation statements)

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“…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. 25,[27][28][29]31 Most of these problems are caused by the weak bones; the disease itself.…”

Section: Discussionmentioning

confidence: 99%

“…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. 25,[27][28][29]31 Most of these problems are caused by the weak bones; the disease itself. Despite of all these negative points the overall activity level of our patients with weak bones has clearly improved.…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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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“…11 ). Then, the next-generation telescopic rods that facilitate antegrade insertion of the obturator without arthrotomy of the distal joint and without damage to the distal articular cartilage of the long bone were introduced, including the interlocking telescopic rod, 35 36) Fassier-Duval rod, 37) and cork-screw tipped telescopic rod 38) ( Figs. 12 and 13 ).…”

Section: Fixation Options For Fracture And/or Osteotomymentioning

confidence: 99%

Management of Osteogenesis Imperfecta: A Multidisciplinary Comprehensive Approach

Cho

Kim

et al. 2020

Clin Orthop Surg

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Osteogenesis imperfecta (OI) is a heterogeneous group of genetic skeletal disorders characterized by bone fragility and low bone mass, which manifests as multiple fractures and deformities of the spine and extremities. GENETICS OI is mostly inherited as an autosomal dominant trait; however, some cases are autosomal recessive. Mutations in type I collagen genes (COL1A1 and COL1A2) were found to be the most common causes of OI in 70%-80% of all cases, characterized by an autosomal dominant inheritance. Type V is the other autosomal dominant OI subtype, which is caused by a single recurrent mutation of IFITM5 (~10%). 1,2) To date, more than 10 OI genes of autosomal recessive trait have been identified (10%-15%). The 2019 version of the nosology and classification system of genetic skeletal disorders listed 17 genes under the name of OI. 3) Most of them are involved in the biosynthesis of type I collagen (COL1A1, COL1A2, CRTAP, LEPR1, PPIB, SERPINH1, FKBP10, and BMP1) or differentiation and function of osteoblasts (SP7, TMEM38B, WNT1, CRE-B3L1, and SPARC). However, the function of some genes responsible for the OI phenotype (IFITM5, SERPINF1, and TENT5A) has yet to be elucidated. Genetic testing is indicated when a clinical diagnosis is not obvious or when molecular confirmation is legally required such as differentiation from child abuse, exemption of military service, and so on. Because those having mutation of WNT1 or SERPINF1 are known to be less responsive to bisphosphonate treatment than other subtypes, 4,5) genotyping could be helpful in determining medical treatment. Considering numerous causative genes and their phenotypic similarity, it is more appropriate to test all the known OI genes simultaneously. Many of the OI genes such as COL1A1 and COL1A2 are huge in size and expensive to analyze via Sanger sequencing. Therefore, exome sequencing or target sequencing using panels of known OI genes via next-generation sequencing technology is the diagnostic test of choice for OI mutations. However, these methods cannot be used to detect large copy number variations including intragenic exonal deletions or duplications, which is an important limitation. The authors have conducted molecular genetic analysis using next-generation sequencing and/or Sanger sequencing in 177 Korean patients with OI and found that 96 patients (54%) carried COL1A1 mutations, 39 (22%)

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“…In the current study, Shin and colleagues [6] took this concept further, advancing from the single-to a dual interlocking telescopic rod (D-ITR), by substituting the proximal fixed T-piece with an interlocking pin. The authors found no differences between these two systems in terms of mean surgery-free survival time, mean rod survival time, cessation of elongation, or elongated length.…”

mentioning

confidence: 99%

“…Surgeons should consider, in terms of epiphyseal damage and/or radiation exposure, the long distance that the interlocking pin must travel within the tibial epiphysis before it reaches the small hole in the rod. Although the authors state that "the procedure is less challenging than it may appear" [6], they also mention that "because of osteopenia of the typical OI epiphysis, the interlocking pin can be manipulated easily during insertion" [6]. Future studies should explore either the various specific design elements of the commercially available rods or the instruments for implanting these rods (such as guiding systems to help ensure correct placement of the interlocking pins), with the goal of mitigating complications.…”

mentioning

confidence: 99%

CORR Insights®: Dual Interlocking Telescopic Rod Provides Effective Tibial Stabilization in Children With Osteogenesis Imperfecta

Liebs

2018

Clin Orthop Relat Res

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Dual Interlocking Telescopic Rod Provides Effective Tibial Stabilization in Children With Osteogenesis Imperfecta

Abstract: Level III, therapeutic study.

Cited by 12 publications

References 19 publications

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

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

Management of Osteogenesis Imperfecta: A Multidisciplinary Comprehensive Approach

CORR Insights®: Dual Interlocking Telescopic Rod Provides Effective Tibial Stabilization in Children With Osteogenesis Imperfecta

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