The etiology and the pathogenesis of congenital pseudarthrosis of the tibia (CPT) are still unknown. The affected tibia exhibits insufficient mechanical strength and osteogenetic capability. CPT is frequently associated with neurofibromatosis type 1 (NF1; von Recklinghausen's disease); however, both diseases have not yet been linked pathogenetically. This study presents the pathomorphologic findings of CPT under special consideration of NF1. Therefore, samples from patients operated on for CPT (n = 4) with (n = 3) and without (n = 1) neurofibromatosis were investigated by light microscopy, immunohistochemistry, and electron microscopy. The most striking finding in all patients was thickened periosteum with accumulation of nerval cells surrounding small arteries, causing subtotal or complete obliteration. In conclusion, impaired vascularization can result in decreased osteogenic capabilities. The similarity of ultrastructural findings in the abnormal periosteum and in skin neurofibromas of neurofibromatosis patients may indicate a pathogenetic association of both diseases.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.prp.2004.09.013 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Paley cross union protocol for congenital pseudoarthrosis of the tibia.
Med Glas (Zenica)
September 2024
Orthopaedics and Traumatology Department, Faculty of Medicine Universitas Brawijaya - Saiful Anwar General Hospital, Malang, East Java, Indonesia.
Aim: Congenital pseudarthrosis of the tibia (CPT) is a rare, dysplastic condition causing significant disability in the lower limbs. A treatment aims to achieve long-lasting union, prevent limb length discrepancies, and prevent soft tissue lesions. Traditional surgery often yields satisfactory results, but amputation may be proposed in failed cases.
View Article and Find Full Text PDFCongenital Pseudoarthrosis of the Clavicle in a Softball Player.
J Am Acad Orthop Surg Glob Res Rev
November 2024
From the College of Medicine, University of Florida, Gainesville, FL (Mr. Root), and the Department of Orthopaedics and Sports Medicine, University of Florida, Gainesville, FL (Dr. Hones and Dr. Farmer).
Article Synopsis
- Congenital pseudarthrosis of the clavicle is a rare condition with an unclear cause, but orthopedic surgeons should recognize and effectively treat it.
- A 15-year-old female softball player experienced shoulder pain with no prior injury and was diagnosed with this condition.
- After conservative treatment failed, she underwent surgery involving open reduction and internal fixation using a bone graft for better outcomes.
A good index surgery for congenital pseudarthrosis of the tibia minimizes complications following surgery.
Bone Jt Open
November 2024
Pediatric Orthopedics, Children's Hospital of Fudan University, Shanghai, China.
Aims: Congenital pseudarthrosis of the tibia (CPT) has traditionally been a difficult condition to treat, with high complication rates, including nonunion, refractures, malalignment, and leg length discrepancy. Surgical approaches to treatment of CPT include intramedullary rodding, external fixation, combined intramedullary rodding and external fixation, vascularized fibular graft, and most recently cross-union. The current study aims to compare the outcomes and complication rates of cross-union versus other surgical approaches as an index surgery for the management of CPT.
View Article and Find Full Text PDFReconstruction of bone defects around the ankle with retrograde gentamicin-coated tibial intramedullary nail (ETN PROtect™) for tibiotalocalcaneal arthrodesis: a case series.
Eur J Orthop Surg Traumatol
October 2024
Limb of Lengthening and Reconstruction Unit, Clínica Imbanaco Grupo QuirónSalud, Carrera 38A #5ª-100, Tower A, Office 803, Cali, Colombia.
Article Synopsis
- The study aimed to evaluate the effectiveness of a gentamicin-coated tibial nail (PROtect™) in patients needing tibiotalocalcaneal arthrodesis due to tibial defects.
- It involved a review of seven male patients treated between January 2018 and December 2023, focusing on fracture healing, complications, and quality of life outcomes.
- Results showed that most patients achieved fracture union, though a few experienced complications; overall, PROtect™ was deemed a promising option for enhancing bone healing and reducing infection risks in complex cases.
Surgical Technique for Management of Congenital Pseudarthrosis of the Clavicle.
Arthrosc Tech
September 2024
Department of Orthopaedics, University of Utah, Salt Lake City, Utah, U.S.A.
Congenital pseudarthrosis of the clavicle is a rare phenomenon with multiple proposed causes. Surgical management is typically indicated either because of the cosmetic appearance of the irregularity or, less commonly, for pain, particularly in adolescent patients. Multiple surgical methods have previously described in the literature, which have raised concern owing to the persistently high rate of nonunion postoperatively.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!