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Congenital pseudarthrosis tibia

Guang-Hui Zhu, Hai-Bo Mei, Rong-Guo He, Yao-Xi Liu, Kun Liu, Jin Tang, Jiang-Yan Wu
BACKGROUND: 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. METHODS: We retrospectively reviewed the records and diagrams of patients with Crawford type IV congenital pseudarthrosis of the tibia between February 2007 and March 2010...
October 22, 2016: BMC Musculoskeletal Disorders
Van Royen Kjell, Brems Hilde, Legius Eric, Lammens Johan, Laumen Armand
UNLABELLED: A strong relationship between congenital pseudarthrosis of the tibia (CPT) and neurofibromatosis type 1 (NF1) has been suggested, but prevalence varies widely throughout the literature and the criteria used for diagnosis are very heterogeneous. Literature focus is mainly on treatment and no specific review on the prevalence of NF1 has been published. Based on our own observations, we hypothesized the prevalence of NF1 in patients with CPT to be higher than what is previously accepted...
September 2016: European Journal of Pediatrics
Mi Hyun Song, Moon Seok Park, Won Joon Yoo, Tae-Joon Cho, In Ho Choi
BACKGROUND: Having observed a tendency towards femoral overgrowth (FO) of the affected limb in children with atrophic-type congenital pseudarthrosis of the tibia (CPT), we aimed to identify the incidence of, contributors to, and patterns of FO among such children. METHODS: We retrospectively evaluated 55 children with CPT, 22 with prepseudarthrosis and 33 with atrophic-type CPT from 1989 to 2012. FO was defined as an affected femoral segment ≥10 mm longer than the contralateral segment...
2016: BMC Musculoskeletal Disorders
B Stephens Richards, Terrence D Anderson
BACKGROUND: In congenital pseudarthrosis of the tibia, use of intramedullary (IM) fixation and autogenous bone graft has long been the standard of care. This study was undertaken to determine whether the addition of rhBMP-2 to this treatment method further enhances healing potential. METHODS: Twenty-one patients with congenital pseudarthrosis of the tibia were evaluated. Fifteen of these patients had neurofibromatosis type 1 (NF1). All had IM fixation and autogenous bone graft, followed by a BMP-soaked collagen sponge wrapped around both the fracture site and bone graft...
June 2, 2016: Journal of Pediatric Orthopedics
A Chalopin, S Pesenti, E Peltier, K Bin, F Launay, J-L Jouve
The treatment objectives in congenital pseudarthrosis of the tibia are bone consolidation and a restored lower-limb axis. They are difficult to achieve, and various surgical techniques have been described, with varying results in terms of bone consolidation and complications. The present study reports clinical and radiographic results in 3 patients managed by the same original technique of transplantar intramedullary nailing using a custom-made proximal locking nail, without surgical approach or resection of the pseudarthrosis site...
June 2016: Orthopaedics & Traumatology, Surgery & Research: OTSR
G Ian Taylor, Russell J Corlett, Mark W Ashton
BACKGROUND: The first successful free vascularized bone flap was performed on June 1, 1974 (and reported in 1975), using the fibula. This was followed by the iliac crest based on the superficial circumflex iliac artery in 1975 and then the deep circumflex iliac artery in 1978. METHODS: A total of 384 transfers using fibula (n = 198), iliac crest (n = 180), radius (n = 4), rib (n = 1), and metatarsal (n = 1) were used between June of 1974 and June of 2014 for reconstruction of the mandible (n = 267), maxilla (n = 20), clavicle (n = 1), humerus (n = 8), radius and ulna (n = 21), carpus (n = 3), pelvis (n = 2), femur (n = 11), tibia (n = 47), and foot bones (n = 4)...
April 2016: Plastic and Reconstructive Surgery
Sang Gyo Seo, Dong Yeon Lee, Yeon Soo Kim, Won Joon Yoo, Tae-Joon Cho, In Ho Choi
BACKGROUND: The purpose of this study was to evaluate clinical outcomes and the biomechanical function of the foot and ankle at skeletal maturity of patients treated for atrophic-type congenital pseudarthrosis of the tibia (CPT) compared with healthy young adult controls. METHODS: Twenty-four patients (mean age of 19.1 years) who had undergone Ilizarov treatment for unilateral atrophic-type CPT were compared with twenty-four controls (mean age of 19.6 years). All participants were evaluated using validated outcome questionnaires, radiographs, physical examination, instrumented motion analysis including a multisegmental foot model, and pedobarographic measurement...
March 16, 2016: Journal of Bone and Joint Surgery. American Volume
A Carlier, H Brems, J M A Ashbourn, I Nica, E Legius, L Geris
Congenital pseudarthrosis of the tibia (CPT) is a rare disease which normally presents itself during early childhood by anterolateral bowing of the tibia and spontaneous tibial fractures. Although the exact etiology of CPT is highly debated, 40-80% of CPT patients are carriers of a mutation in the Neurofibromatosis Type 1 (NF1) gene, which can potentially result in an altered phenotype of the skeletal cells and impaired bone healing. In this study we use a computational model of bone regeneration to examine the effect of the Nf1 mutation on bone fracture healing by altering the parameter values of eight key factors which describe the aberrant cellular behaviour of Nf1 haploinsufficient and Nf1 bi-allelically inactivated cells...
2016: Scientific Reports
Dmitry Y Borzunov, Alexander Y Chevardin, Alexander I Mitrofanov
PURPOSE: Our study compared the rates of union achieved with the Ilizarov method in congenital pseudarthrosis of the tibia (CPT) associated with neurofibromatosis type 1 (NF1) or CPT of idiopathic origin in paediatric patients. METHODS: We studied the outcomes of 28 children that were treated for CPT between 2005 and 2013. Group 1 included children (n = 14, mean age = 9.7 years) with CPT associated with NF1 while group 2 were CPT cases that had radiographic confirmation of dysplastic lesions in the tibia but lacked clinical NF1 manifestations (n = 14, mean age = 8...
February 2016: International Orthopaedics
Toni M Mansour, Ismat B Ghanem
BACKGROUND: During past decades, gradual bone transplant using external fixators and vascularized fibular or rib transplants widely contributed to solve difficult problems related to the reconstruction of large bone defects in children. However, these surgeries are time consuming and require specialized surgical skills, not always available in a general hospital setting, mainly in rural areas. PURPOSE: To report the preliminary results of the more recently described induced membrane technique in a consecutive series of 8 children and to identify some factors related to the procedure's success...
October 13, 2015: Journal of Pediatric Orthopedics
Kaushik Bhowmick, Viju Daniel Varghese
We present the case of a 23-year-old male with congenital pseudarthrosis of the tibia, who had undergone treatment with Ilizarov ring fixation and had experienced 4 episodes of repeat fracture. He had associated type 1 neurofibromatosis, and his radiographs confirmed a type 6 Boyd's congenital pseudarthrosis of the left tibia, with concomitant arthritic ankle and subtalar joints. He was treated successfully with retrograde intramedullary nailing of the tibia and autologous bone grafting. At his final follow-up visit at 3 years postoperatively, he displayed complete union with no repeat fractures...
September 2, 2015: Journal of Foot and Ankle Surgery: Official Publication of the American College of Foot and Ankle Surgeons
Guang-hui Zhu, Hai-bo Mei, Rong-guo He, Kun Liu, Jin Tang, Jiang-yan Wu
BACKGROUND: The purpose of our retrospective study was to evaluate the preliminary result of distraction osteogenesis in patient with tibial shortening after initial union of Congenital Pseudarthrosis of the Tibia (CPT). METHODS: All the CPT cases with tibial shortening after initial union managed by proximal tibial lengthening using Ilizarov technique were identified. All the patient charts and radiograms were reviewed. RESULTS: Between March 2007 and January 2012, 11 CPT cases were included with an average follow-up of 41 months (range, 34-51 months)...
2015: BMC Musculoskeletal Disorders
Jan Vanderstappen, Johan Lammens, Pieter Berger, Armand Laumen
PURPOSE: Most studies on congenital pseudarthrosis of the tibia (CPT) report on the short-term union rate and refracture rate but do not take into account the long-term outcome. This review includes patients treated with an Ilizarov bone transport, who all reached skeletal maturity. It describes long-term results and highlights any prognostic factors that could predict the final outcome. METHODS: The records of patients with CPT treated with an Ilizarov bone transport in our institution were retrospectively evaluated...
August 2015: Journal of Children's Orthopaedics
Lior Shabtai, Eli Ezra, Shlomo Wientroub, Eitan Segev
Ten children with congenital pseudarthrosis of the tibia were treated with resection of pathologic bone, bone grafting, intramedullary rodding, compression with circular frame, simultaneous proximal tibia lengthening, and bone morphologic proteins. Thirteen operations were performed to achieve union. Four patients underwent simultaneous lengthening and four patients received recombinant human bone morphologic protein. Six children required complementary operations for residual ankle and knee valgus, and shortening...
September 2015: Journal of Pediatric Orthopedics. Part B
B Stephens Richards, David Wilkes, Molly Dempsey, Pamela Nurenberg
The aim of this study was to determine whether an established radiographic union scoring system for tibial fracture healing (RUST) is reliable when used in neurofibromatosis (NF1) patients with congenital pseudarthrosis of the tibia (CPT) treated by intramedullary fixation. Four individuals reviewed 36 sets of radiographs from 12 NF1 patients with CPT (preoperative, and 6 months and 1 year postoperative). Intraobserver reliability (κ-value 0.89) and interobserver reliability (κ-value 0.76) were high. The modified RUST scoring system is a useful tool when faced with the challenge of postoperative radiographic evaluation of the tibia in NF1 patients with CPT...
March 2015: Journal of Pediatric Orthopedics. Part B
C E Johnston
No abstract text is available yet for this article.
March 1, 1983: Orthopedics
A Vilks, D Ozols, V Boka, M Murovska, B Mamaia
Neurofibromatosis type-1 (NF-1)--is a common genetic disease effecting the skin, subcutaneous tissue peripheral nerves and bones (tibia pseudarthrosis). Immunomodulatory viruses HHV-6 and HHV-7 are classifying as a genus of roseoloviruses of subfamily beta-herpesviruses. Reactivation of HHV-6 and HHV-7 inhibits immune system and indirectly promote to other infectious agents. The article deals with a unique case repot of two repeated transplantations of fibula due to congenital tibia pseudarthrosis caused by NF-1...
January 2014: Anesteziologiia i Reanimatologiia
Zoran Vukasinović, Dusko Spasovski, Igor Seslija, Ismet Gavrankapetanović, Elvir Bazdar, Zorica Zivković
INTRODUCTION: Congenital pseudarthrosis of tibia is a rare congenital deformity with progressive evolution. Treatment is vague and difficult, and many methods have been used--from once mandatory early amputation to contemporary operative (Ilizarov method, free microvascular fibular graft) and adjuvant methods (electrostimulation, biphosphonates, bone morphogenetic protein). We present the usage of once popular method of homologous graft insertion and intramedullary fixation. CASE OUTLINE: This is a case report of male patient with pseudarthrosis involving both crural bones (Boyd type 5), diagnosed in neonatal age...
January 2014: Srpski Arhiv za Celokupno Lekarstvo
Husam Alrumaih, Imran Ilyas, Syed Kashif
PATIENT: Male, 43 FINAL DIAGNOSIS: Neurofibromatosis Symptoms: Hip pain Medication: - Clinical Procedure: - Specialty: Orthopedics and Traumatology. OBJECTIVE: Rare disease. BACKGROUND: Neurofibromatosis type 1 is a disease known for orthopedic manifestations such as spine deformities, congenital pseudarthrosis of the tibia and other bony dysplasias; joint dislocations are rare. Joint arthritis caused by neurofibromatosis, with a stable hip, has never been reported in the English literature before...
2014: American Journal of Case Reports
Stéphanie Pannier, Zagorka Pejin, Caroline Dana, Alain Charles Masquelet, Christophe Glorion
PURPOSE: The purpose of this study was to evaluate the two-stage surgical technique combining induced membrane, spongy autograft and intramedullary fixation for the treatment of congenital pseudarthrosis of the tibia (CPT). METHODS: Three boys and two girls were treated by this technique between 2003 and 2008. All patients had type IV CPT in Crawford's classification. Four of them had a limited dystrophic form, whereas one case presented an extensive tibia bone dystrophy...
December 2013: Journal of Children's Orthopaedics
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