Does pamidronate enhance the osteogenesis in mesenchymal stem cells derived from fibrous hamartoma in congenital pseudarthrosis of the tibia?

Madhuri, Vrisha; Mathew, Smitha; Rajagopal, Karthikeyan; Ramesh, Sowmya; Antonisamy, B.

doi:10.1016/j.bonr.2016.10.003

Cited by 8 publications

(7 citation statements)

References 22 publications

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“…Madhuri et al 19 2016 studied stem cells harvested from hamartoma tissue in three CPT patients compared with healthy bone marrow stem cells in controls. They found that the mesenchymal stem cells in the CPT patients had a higher proliferation rate than in controls, but that these stem cells showed less differentiation potential and less osteogenic potential than control stem cells.…”

Section: Pathology Of Cptmentioning

confidence: 99%

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

Paley¹

2019

Journal of Children's Orthopaedics

149

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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

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Section: Pathology Of Cptmentioning

confidence: 99%

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

Paley¹

2019

Journal of Children's Orthopaedics

149

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“…Important steps with any of these techniques include complete resection of the hamartoma, preoperative bisphosphonate treatment, and application of bone morphogenic protein-2 (BMP-2). [15][16][17] Surgical treatment of CPT frequently results in limb length discrepancy and deformity. 18,19 Surgical treatment of limb length discrepancy and deformity in CPT often necessitates osteotomy.…”

mentioning

confidence: 99%

“…The increased surface area of this union in combination with intramedullary fixation produces a stable construct with decreased risk for refracture. Important steps with any of these techniques include complete resection of the hamartoma, preoperative bisphosphonate treatment, and application of bone morphogenic protein-2 (BMP-2) 15–17…”

mentioning

confidence: 99%

Does An Osteotomy Performed in Congenital Pseudarthrosis of the Tibia Heal?

Nahm

Makarewich

Rosenwasser

et al. 2022

Journal of Pediatric Orthopaedics

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Background: Shortening and deformity of the tibia commonly occur during the treatment of congenital pseudarthrosis of the tibia (CPT). The role of osteotomies in lengthening and deformity correction remains controversial in CPT. This study evaluates the approach to and outcome after osteotomy performed in CPT. Methods: We performed an IRB approved retrospective review of consecutive patients with CPT treated at our institution from 2010 through 2019. Patients who underwent osteotomies were included in this study. Results: Nine patients (10 osteotomies—5 proximal metaphyseal and 5 diaphyseal) with a median age at osteotomy of 8.9 years (range: 4 to 21 y) were included. Six patients had neurofibromatosis-1, 1 had cleidocranial dysplasia, and 2 patients had idiopathic CPT. Four osteotomies were performed for deformity correction, 3 osteotomies to allow intramedullary instrumentation, and 3 osteotomies for lengthening. Five osteotomies were preceded by zolendronate treatment before surgery. Nine were fixed with a rod supplemented with external fixation (7) or locking plates (2). One osteotomy was stabilized with locked intramedullary nailing alone. Four osteotomies were supplemented with autologous bone graft, and bone morphogenic protein-2 was utilized in 3 osteotomies. Median time to healing was 222.5 days (range: 124 to 323 d). One osteotomy (locked intramedullary nailing) required grafting at 5.5 months and then healed uneventfully. Median healing index for patients undergoing lengthening was 57.9 days/cm (range: 35 to 81 d/cm). All 3 osteotomies performed for lengthening required a second osteotomy for preconsolidation at a mean of 34 days. Other complications included compartment syndrome requiring fasciotomy (n=2), tibial osteomyelitis (n=1), and fracture distal to cross-union (n=1). Conclusions: Contrary to much of the established practice, osteotomies may be safely performed in CPT for various indications. All osteotomies healed with only 1 osteotomy requiring secondary bone grafting. Although time to healing of the osteotomy was generally prolonged, this study suggests, somewhat surprisingly, that preconsolidation can occur frequently in lengthening procedures. Level of Evidence: Level IV—case series.

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“…Stem cells harvested from the hamartoma tissue of CPT patients, have less osteogenic potential. We are using fibrin clot tissue, derived from the blood of the patients, to improve the union in surgically treated patients [31][32][33].…”

Section: Osseous Involvement Of Long Bones 31 Tibiamentioning

confidence: 99%

Bone Lesions in Children with Neurofibromatosis

Laliotis¹

2022

Clinical and Basic Aspects of Neurofibromatosis Type 1

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Neurofibromatosis is often related with severe orthopaedic disorders in children. Bone lesions are rare but pose severe difficulties in management. It affects the spine and long bones. Lesions are associated either from enlargement of neurofibromas that affect the normal growth or from primary neurofibromatosis of long bones. Dystrophic scoliosis appears with short curves, with kyphosis and rotation of the apical vertebrae. Usually affect the thoracic spine, with penciling of the ribs. Surgical treatment is challenging in cases of rapid progression. Scoliosis may appear with curvatures similar to those in idiopathic scoliosis, without dysplastic changes of the vertebrae. Anterior bowing of the tibia is manifestation of NF and is distinguished from the benign posterolateral bowing. Evaluation of the medullary canal and presence of cystic lesions in the tibia is essential. Progression to pseudoarthrosis or pathologic fracture is common. Surgical management of tibial pseudoarthrosis remains a difficult procedure. Pseudoarthrosis may appear in fibula, radius or ulna but are extremely rare. Irregular eccentric bone cysts in long bones that are commonly diagnosed after a pathologic fracture, must be differentiated for NF. Malignant transformation of neurofibromas must be considered when there is rapid progression of the lesion.

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Does pamidronate enhance the osteogenesis in mesenchymal stem cells derived from fibrous hamartoma in congenital pseudarthrosis of the tibia?

Cited by 8 publications

References 22 publications

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

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

Does An Osteotomy Performed in Congenital Pseudarthrosis of the Tibia Heal?

Bone Lesions in Children with Neurofibromatosis

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