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Congenital pseudarthrosis of the tibia: biological and biomechanical considerations to achieve union and prevent refracture.

ABSTRACT: Congenital pseudarthrosis of the tibia (CPT) is likely to be a primary periosteal disease and secondary bone disease. The primary goal of treatment is to obtain union, correct the diaphyseal deformity, correct any proximal fibular migration and prevent refracture. The pathobiology demonstrates increased osteoclasis by the surrounding fibrous hamartoma and reduced osteogenesis and bone morphogenic protein production by the bone. This leads to a loss of remodelling potential and gradual bowing and atrophy of the bone with eventual fracture of the tibia and or fibula. This recommends the synergistic use of bisphosphonates and bone morphogenic protein. The pathomechanics of CPT implicate the anterolateral bowing, narrow diameter of the atrophic bone ends and proximal fibular migration. These biomechanical factors can be addressed by means of straightening of the deformity, intramedullary support of both bones, stable fixation and reduction of proximal migration of the fibula. A summary of the literature on CPT shows that the mean probability of achieving primary union without refracture, by most treatments is 50% (12% to 80%). Two recent studies have shown a much higher success rate approaching 100%, by creating a cross-union between the tibia and fibula. The cross-union with intramedullary reinforcement of the bone makes refracture unlikely due to the cross-sectional area of union with its two-bar linkage. A new classification to guide such treatment is also proposed. Level of Evidence:V - expert opinion.

SUBMITTER: Paley D

PROVIDER: S-EPMC6442511 | biostudies-other | 2019 Apr

REPOSITORIES: biostudies-other

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Congenital pseudarthrosis of the tibia: biological and biomechanical considerations to achieve union and prevent refracture.

Paley D D

Journal of children's orthopaedics 20190401 2

Congenital pseudarthrosis of the tibia (CPT) is likely to be a primary periosteal disease and secondary bone disease. The primary goal of treatment is to obtain union, correct the diaphyseal deformity, correct any proximal fibular migration and prevent refracture. The pathobiology demonstrates increased osteoclasis by the surrounding fibrous hamartoma and reduced osteogenesis and bone morphogenic protein production by the bone. This leads to a loss of remodelling potential and gradual bowing and ...[more]

PMID: 30996736

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Project description:PurposeThe goal of this study is to evaluate the treatment outcomes of anterolateral bowing and residual deformities of distal tibia in patients with CPT using circular external fixation and hydroxyapatite coated flexible intramedullary nailing without excision of affected part of tibia.Patients and methodsSix patients (4 boys and 2 girls, mean age 12.4 ± 4.1 years) were included in the study. Mean follow-up is 2.1 years. In 4 patients with early onset of disease initial surgical treatment (at age of 5-8 years) was dysplastic zone or pseudarthrosis resection with proximal metaphyseal osteotomy for bone transport. Children with unbroken bowed tibia (2 cases of type II according to Crawford classification) had no previous surgery. Neurofibromatosis type I was diagnosed in 4 cases. Surgical technique for residual deformity correction consisted of percutaneous osteotomy, application of circular external frame and composite hydroxyapatite-coated intramedullary nailing.ResultsMean external fixation time was 95.3 ± 17.5 days. All patients never get fractured after frame removal. At the present time, they are considered to be healed, in 2.1 years, in average, without fractures or deformity recurrence. Mean lower limb length discrepancy varied from 2 to 10 mm at the latest follow-up control. After realignment procedure, patients didn't require additional surgery but one. Intramedullary nails were removed in two years after deformity correction for individual reason.ConclusionCorrection of anterolateral bowing or residual deformity in children with CPT is indicated. Association of external fixation with intramedullary nailing/rodding left in situ after frame removal ensure stability and accuracy of deformity correction. Biological methods of stimulation of bone formation in dysplastic zone are obligatory to ensure bone union. Intramedullary nailing with composite hydroxyapatite-coated surface provides mechanical and biological advantages in patients with CPT.

Project description:The purpose of this study was to investigate the initial union rate, refracture rate and residual deformities of congenital pseudarthrosis of the tibia (CPT), using combined surgery including pseudarthrosis resection, intramedullary rodding, autogenous iliac bone grafting and Ilizarov's fixator, with a mean 5.2 years follow-up.We retrospectively reviewed the records and diagrams of patients with Crawford type IV congenital pseudarthrosis of the tibia between February 2007 and March 2010. Patients managed by pseudarthrosis resection, intramedullary rod of the tibia, wrapping autogenous iliac bone grafting and Ilizarov's fixator were enrolled. We evaluated the bone union rate, tibial alignment, limb length discrepancy (LLD), valgus deformity of the ankle and the frequencies of refracture during period of follow-up.There were 56 cases enrolled in the study, with a mean follow-up 5.2 years (range, 3 to 6.7 years). The mean age of the patients at surgery was 3.5 years (range, 1.5 to 12.4 years). Fifty (89.2 %) of the 56 patients had primary bone union at site of pseudarthrosis, while 5 obtained union after second surgery and 1 failed. The average time spent to obtain pseudarthrosis initial union was 4.5 months (range, 3.0 to 10.0 months) and mean duration of Ilizarov treatment was 4.7 months (range, 3.2 to 10.4 months). Eleven (19.6 %) patients had proximal tibial valgus with a mean angle of 9.5° (range, 5 to 24°), while 10 (17.9 %) patients had ankle valgus deformities with a mean of 12.3° (range, 6 to 21°). Sixteen (28.6 %) patients had an average 2.2 cm LLD (range, 1.5-4.2 cm). Of the 50 cases who obtained initial bone union of pseudarthrosis, 13 (26.0 %) had refracture which need cast immobilization or secondary surgery.This combined surgery obtained initial union rate of 89.2 % at primary surgery while the refracture rate is 26.0 %. However, residual deformities such as proximal tibial valgus, LLD and ankle valgus were also existed which should be pay more attention to and dealt with.This study was registered in ClinicalTrials.gov under the name "The Effect of Combined Surgery in Management of Congenital Pseudarthrosis of Tibia" ( NCT02640040 ), which was released on August 31, 2015.

Dataset Information

Congenital pseudarthrosis of the tibia: biological and biomechanical considerations to achieve union and prevent refracture.

Publications

Congenital pseudarthrosis of the tibia: biological and biomechanical considerations to achieve union and prevent refracture.

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