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Congenital + pseudarthrosis

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
Ricardo Horta, Ricardo Nascimento, Alvaro Silva, Rui Pinto, Pedro Negrão, Ricardo São-Simão, Jorge Carvalho, Marta Santos Silva, Jose Amarante
Radial club hand may be congenital or acquired; radial deviation of the hand is usually found, associated with palmar flexion-pronation and treatment of severe forms of radial club hand is often difficult. Here we present a case of reconstruction of a severe postraumatic radial club hand with a free fibular osteoseptocutaneous flap and Sauve-Kapandji procedure in a 28-year-old man. The patient had a radial deviation of the wrist and right upper limb shortening as a result of an infected pseudarthrosis of the radius...
July 28, 2016: Microsurgery
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
I F Lagas, J W Colaris
A 26-year-old male complained of painful clavicles. His medical history included multiple fractures of both clavicles, all treated conservatively. Conventional radiographs revealed a typical shape of both clavicles, suggesting non-union. His extraordinary medical history and non-union of both clavicles led to the diagnosis: 'congenital pseudarthrosis of both clavicles'.
2016: Nederlands Tijdschrift Voor Geneeskunde
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
Marina Juliana Pita Sassioto Silveira de Figueiredo, Susana Dos Reis Braga, Miguel Akkari, José Carlos Lopes Prado, Cláudio Santili
Congenital pseudarthrosis of the clavicle (PCC) is a rare affection, that can be diagnosed at birth and represent a disturbance of union of the ossification centers. It's more common in girls and in the right side. This study objectives to proceed a revision about the subject, that was searched in online database of LILACS and MEDLINE. We found 56 articles till present data. Besides be a bit infrequent, the PCC must not be missed or even forgotten, especially as differential diagnosis with acute fracture of the clavicle at birth by trauma in the childbirth...
January 2012: Revista Brasileira de Ortopedia
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
C R Soria-Sánchez, A López-Durán, A Isunza-Ramirez
OBJECTIVE: To report the ability of bone morphogenetic protein to achieve bone healing in patients with congenital pseudoarthrosis of the tibia. PATIENTS AND METHODS: Three patients with a diagnosis of congenital pseudoarthrosis of the tibia were followed-up for six months. Bone healing and functional capacity of the limb were observed. RESULTS: Bone healing occurred in all cases at a mean of three and half months of treatment. CONCLUSIONS: Morphogenetic protein is useful as an adjuvant to achieve bone regeneration and healing in patients with congenital pseudoarthrosis of the tibia...
May 2015: Acta Ortopédica Mexicana
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
R Gouron
The induced membrane technique is now quite adaptable to segmental bone reconstruction in children. This technique is much the same as the technique used in adults. A cement spacer is interposed, and in a second operating phase, occurring 6 weeks after the interposition of the spacer, the cement is removed and a morselized corticocancellous graft is installed in the induced membrane that had formed around the cement. Graft expansion using allograft chips should not exceed 30% of the total volume. An additional autograft strut is useful in the reconstruction of long femoral or metaphyseal-diaphyseal tibial defects...
February 2016: Orthopaedics & Traumatology, Surgery & Research: OTSR
Akihiro Hirakawa, Yoshiyuki Ohno, Haruhiko Akiyama
No abstract text is available yet for this article.
November 18, 2015: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
Denis Dufrane, Pierre-Louis Docquier, Christian Delloye, Hélène A Poirel, Wivine André, Najima Aouassar
Long bone nonunion in the context of congenital pseudarthrosis or carcinologic resection (with intercalary bone allograft implantation) is one of the most challenging pathologies in pediatric orthopedics. Autologous cancellous bone remains the gold standard in this context of long bone nonunion reconstruction, but with several clinical limitations. We then assessed the feasibility and safety of human autologous scaffold-free osteogenic 3-dimensional (3D) graft (derived from autologous adipose-derived stem cells [ASCs]) to cure a bone nonunion in extreme clinical and pathophysiological conditions...
December 2015: Medicine (Baltimore)
Shengru Wang, Kahaer Aikenmu, Jianguo Zhang, Guixing Qiu, Jianwei Guo, Yanbin Zhang, Xisheng Weng
PURPOSE: The aim of this retrospective study is to evaluate the efficacy and safety of posterior-only vertebral column resection (PVCR) for the treatment of angular and isolated congenital kyphosis. METHODS: 24 patients with isolated angular congenital kyphosis treated by PVCR in our hospital were retrospectively studied. The patients' radiographs and hospital records were reviewed. Deformity in sagittal planes and global sagittal alignment were analyzed for correction and maintenance of the correction in preoperative, postoperative, and follow-up radiographs...
December 11, 2015: European Spine Journal
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
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