Pseudoartrose é uma séria complicação de uma fratura óssea na qual o osso nunca se regenera. Uma cicatriz de tecido fibroso se forma no meio do osso. Sem tratamento resulta em deformidade e prejuízo permanente da função, como por exemplo uma perna torta e incapaz de suportar peso se a pseudoartrose é no fêmur ou na tíbia. O tratamento é cirúrgico e a recuperação demora muitos meses.
Introdução
O que você precisa saber de cara
Condição rara que afeta o desenvolvimento ósseo, caracterizada pela ausência de continuidade na fíbula desde o nascimento. Resulta em uma falha na formação óssea, levando a instabilidade e deformidade no tornozelo e perna.
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Pseudoartrose congênita da fíbula
Centros de Referência SUS
24 centros habilitados pelo SUS para Pseudoartrose congênita da fíbula
Centros para Pseudoartrose congênita da fíbula
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Do Fibular Growth Patterns Change After Tibial-Fibular Fusion in Children With Congenital Pseudarthrosis of the Tibia?
In order to reduce the incidence of congenital pseudarthrosis of the tibia (CPT) refracture, current tibial-fibular surgery is quite popular. The fibular growth pattern of CPT after tibial-fibular fusion is unclear. To explore the changes in fibular growth patterns in CPT patients with 3-in-1 osteosynthesis. A retrospective analysis of 33 children with CPT who were admitted to the hospital from April 2013 to June 2020 and who underwent combined surgery with 3-in-1 osteosynthesis was performed. Among them, 19 were males, and 14 were females; 16 cases were on the left, and 17 cases were on the right. Twenty-two children with neurofibromatosis type I (NF-1) were present. The average age at the time of surgery was 33.3 months (13 to 72 mo). In this study, self-control was used, and the study group underwent combined surgery with 3-in-1 osteosynthesis on the tibia and fibula on the affected side of the children with CPT. The control group included healthy fibula and tibia from children with CPT. The patients' ankle valgus, proximal tibial-fibular epiphyseal plate distance, and ankle pain were recorded. In this study, with an average follow-up of 94.5 months, 18 patients developed ankle valgus, and 22 patients have proximal displacement of the fibula. No children experienced ankle pain. 3-in-1 osteosynthesis with CPT may change the growth pattern of the fibula, leading to a relative proximal shift of the fibula in relation to the tibia, but this still requires validation through a prospective multicenter large-sample investigation.
Cross-union surgery for tibiofibular fractures in McCune-Albright syndrome: a case report and literature review.
McCune-Albright syndrome (MAS) is a rare disease. MAS manifests in the skeletal system as Fibrous dysplasia (FD). In MAS patients, bone lesions often result in fractures under weight-bearing forces during childhood, and pseudarthrosis formation is highly likely after fractures. There is currently no ideal treatment for fractures in children with MAS. This case study presents the innovative application of the cross-union technique in treating a 14-year-old female MAS patient with a distal tibial and fibular fracture nonunion. The patient had a history of conservative treatment with persistent symptoms, including an inability to bear weight and walk independently. A "cross-union" procedure was performed using a Fassier-Duval rod, locking plate, and K-wire fixation. At the final follow-up 12 months postoperatively, x-rays revealed a stable "cross-union" bone connection at the distal ends of the tibia and fibula, with no occurrence of refracture or other complications. The Radiographic Union Score for Tibial Fractures (RUST) was 12, and the Olerud Molander Ankle Score was 60. We successfully applied the "cross-union" technique to a MAS child with nonunion after a distal tibiofibular fracture. We look forward to further improvement and promotion of the "cross-union" technology in the future, bringing new hope for treating fractures in MAS/FD children.
Mother to Child Non-Vascularized Fibula Transfer in Congenital Pseudoarthrosis of Tibia.
Congenital pseudoarthrosis tibia (CPT) is a relatively rare disease, characterized by anterolateral bowing of the tibia, non-union, and limb length discrepancy. Various surgical treatments have been described in literature for its management with differing favorable outcomes. In this report, we present a case of 3-year-old child with CPT of Crawford type IV, with associated fibular dysplasia. Maternal fibula was harvested and used as a bone graft and was stabilized by intra-medullary fixation. Complete union was achieved at 1 year after the primary surgery. No re-fractures were seen in a follow-up period of 2 years. Using maternal fibula as an alternative to use as a bone graft in the management of congenital pseudoarthrosis tibia may prove beneficial. Moreover, it is cheaper and readily available and needs less surgical expertise when compared to its alternatives such as use of bone morphogenetic protein 7, allogenic cadaveric grafting, or use of vascularized fibular graft.
Vascularized Periosteal Grafts for Bone Union in Children: A Systematic Review.
The periosteum is the main organ responsible for bone regeneration. Vascularized Periosteal Grafts (VPG) have demonstrated exceptional efficacy and speed in facilitating bone union among children with challenging bone healing conditions. Despite their promising results, the overall impact of these interventions has yet to be comprehensively evaluated through systematic review. This systematic review aimed to provide comprehensive insights into bone union outcomes and complications related to the use of VPG in children. MEDLINE/PubMed, Embase, Web of Science, Clinicaltrials.gov, and Cochrane Library were searched from database inception to September 2023 and screened for relevant studies. Data were collected regarding patient demographics, disease, treatment, anatomical location, graft used, donor and receptor vessels, skin paddle monitoring, follow-up duration, time to union, consolidation, and complications. The correlation between age and bone union was assessed using Pearson and Spearman correlation coefficients, as appropriate. Study quality was assessed using the Methodological Index for Non-randomized Studies Criteria. A total of 15 studies involving 135 patients were included. All the studies were classified as Level 4 evidence. The mean MINORS score was 5.1 ± 1. The aim of the VPG was nonunion treatment in 90 patients (67%), nonunion prevention in 35 patients (26%), and bone union acceleration in 10 patients (7%). The origin of the bone union problem was traumatic in 59 cases (44%), congenital pseudoarthrosis of the tibia or fibula in 48 patients (35%), oncologic in 23 patients (17%), and infectious in 5 patients (4%). Nine different sources of periosteal flaps were used to enhance bone union. Bone union rate was 96% with a mean time of 4.2 months (range 1-18 months). Spearman test showed a non-statistically significant negative correlation between age and bone union time (r = -0.3, p = 0.759). VPGs are a safe and reliable treatment for promoting bone union, especially in the context of complex pediatric bone-healing challenges.
Efficacy of the cross-union protocol in the treatment of congenital tibial pseudarthrosis: a comparative study.
Congenital Pseudarthrosis of the Tibia (CPT) is a rare pediatric condition presenting substantial challenges for orthopedic surgeons. Aiming to achieve bone union, with subsequent complications such as refractures being common. The aim of the present study is to evaluate the results of our intentional cross-union protocol and to compare these outcomes with those obtained from our previously used techniques. Sixteen patients, with a mean age of 4.8 years (range: 1-13.3 years), who were treated with the intentional cross-union protocol were included in Group (A) Eleven patients, with a mean age of 3.5 years (range: 1.3-7.6 years), who primarily underwent intramedullary rodding with bone graft, were included in Group (B) The intentional cross-union protocol involved cross-union of the tibia and fibula, autogenous iliac bone grafting, and the insertion of bone morphogenetic protein 2 (BMP-2) aimed at achieving tibia-fibula cross-union. Retrospective evaluation of serial radiographs was conducted, and the outcomes regarding union and any subsequent complications were analyzed. A total of 27 patients with CPT were included in the study. All 16 patients (100%) in Group A successfully achieved primary union. Among the 11 patients in Group B, only 4 cases achieved primary union, with a primary healing rate of 36.4%. Seven cases with delayed healing required 2-4 revision surgeries for final healing. The time to union was significantly shorter in Group A (3.37 ± 0.64 months) compared to Group B (8.67 ± 3.0 months). The cross-sectional area of union was also significantly larger in Group A (6.71 ± 0.58 cm²) compared to Group B (1.18 ± 0.19 cm²). In Group A, there were no cases of refracture, whereas in Group B, 7 cases (77.8%) experienced refracture, indicating a statistically significant difference. Our study demonstrates that the Cross-union protocol is highly effective for achieving union and preventing refracture in CPT. With such significant improvement, it has notably altered the prognosis of this challenging condition. IV.
Publicações recentes
Tibial lengthening in congenital pseudoarthrosis of the tibia: a scoping review.
Development of the Extended Lane and Sandu Score to Assess Osseous Repair.
Unraveling the molecular landscape of congenital pseudoarthrosis of the tibia: insights from a comprehensive analysis of 159 probands.
Vascularized Periosteal Grafts for Bone Union in Children: A Systematic Review.
Paley cross union protocol for congenital pseudoarthrosis of the tibia.
📚 EuropePMC2 artigos no totalmostrando 36
Do Fibular Growth Patterns Change After Tibial-Fibular Fusion in Children With Congenital Pseudarthrosis of the Tibia?
Journal of pediatric orthopedicsCross-union surgery for tibiofibular fractures in McCune-Albright syndrome: a case report and literature review.
Frontiers in pediatricsMother to Child Non-Vascularized Fibula Transfer in Congenital Pseudoarthrosis of Tibia.
Journal of orthopaedic case reportsVascularized Periosteal Grafts for Bone Union in Children: A Systematic Review.
MicrosurgeryEfficacy of the cross-union protocol in the treatment of congenital tibial pseudarthrosis: a comparative study.
BMC musculoskeletal disordersPaley cross union protocol for congenital pseudoarthrosis of the tibia.
Medicinski glasnik : official publication of the Medical Association of Zenica-Doboj Canton, Bosnia and HerzegovinaCongenital Pseudoarthrosis of the Femur Managed with Masquelet Technique.
Journal of orthopaedic case reportsOsteoperiosteal fibular strut grafting - A technique to improve union rates.
International orthopaedicsFree fibula flap to achieve bone union after congenital pseudoarthrosis of the forearm in neurofibromatosis: Technical report based on 3 cases, and literature review.
Journal of plastic, reconstructive & aesthetic surgery : JPRASFree Fibula Flap for Congenital Pseudarthrosis of the Tibia: Indications and Challenges.
JPRAS openIs Using a Ring External Fixator in the Treatment of Congenital Pseudarthrosis of the Tibia Associated With Better Results or Using a Locking Plate?
Journal of pediatric orthopedicsTreatment of Concomitant Congenital Pseudarthrosis of the Fibula With Propeller Periosteal Flap.
Annals of plastic surgeryRefracture, a Potential Complication of the Periosteal Flap Technique Without Bone Graft for Isolated Congenital Pseudarthrosis of the Fibula: A Report of Two Cases and Literature Review.
Journal of the American Academy of Orthopaedic Surgeons. Global research & reviewsManagement of congenital pseudoarthrosis using Ilizarov Device-Delsuth, oghara experience.
Nigerian journal of clinical practiceCongenital Pseudarthrosis of the Tibia: Long-term Outcome of Treatment With Intramedullary Vascularized Fibular Graft Combined With Ilizarov Distraction.
Journal of pediatric orthopedicsTraumatic Radius Pseudoarthrosis in Neurofibromatosis Type 1: All Treatment Options in One Patient.
CureusEXPERIMENTAL STUDY OF BONE DENSITY IN PATIENTS WITH CONGENITAL PSEUDOARTHROSIS OF THE TIBIA BEFORE AND AFTER SURGERY.
Wiadomosci lekarskie (Warsaw, Poland : 1960)Combination Treatment by Cross-Union of the Tibia and Fibula, Autogenic Iliac Bone Grafting, Reliable Fixation and Bone Morphogenetic Proteins for the Treatment of Refractory Congenital Pseudarthrosis of the Tibia.
Journal of pediatric orthopedicsEvaluation of the efficacy of ipsilateral fibular transfer for reconstruction of large tibial defects in children: a retrospective study.
Journal of orthopaedic surgery and researchCross-Union Surgery for Congenital Pseudarthrosis of the Tibia.
Children (Basel, Switzerland)Crawford Type IV Congenital Pseudarthrosis of the Tibia: Treatment With Vascularized Fibular Grafting and Outcome at Skeletal Maturity.
Journal of pediatric orthopedicsDiagnostic and treatment challenge in adult presentation of congenital pseudoarthrosis of the tibia: A case report.
Annals of medicine and surgery (2012)Combined massive allograft and intramedullary vascularized fibula transfer: the Capanna technique for treatment of congenital pseudarthrosis of the tibia.
Acta orthopaedicaCongenital Pseudarthrosis of the Fibula: Controlling Ankle Valgus in a Heterogenous Condition.
Journal of pediatric orthopedicsSurgical Outcomes, Complications, and Long-Term Functionality for Free Vascularized Fibula Grafts in the Pediatric Population: A 17-Year Experience and Systematic Review of the Literature.
Journal of reconstructive microsurgerySurgical treatment of congenital pseudarthrosis of the forearm: Review and quantitative analysis of individual patient data.
Hand surgery & rehabilitationManagement of Congenital Pseudoarthrosis of the Tibia and Fibula.
Current reviews in musculoskeletal medicineWhat Factors Influence Union and Refracture of Congenital Pseudarthrosis of the Tibia? A Multicenter Long-term Study.
Journal of pediatric orthopedicsVascularised fibula or induced membrane to treat congenital pseudarthrosis of the Tibia: A multicentre study of 18 patients with a mean 9.5-year follow-up.
Orthopaedics & traumatology, surgery & research : OTSRCongenital Pseudoarthrosis of the Distal Radius Treated With Physeal-Sparing Double-Barrel Vascularized Free Fibula Transfer: A Case Report.
Hand (New York, N.Y.)Relationship between postoperative complications and fibular integrity in congenital pseudarthrosis of the tibia in children.
World journal of pediatrics : WJPRetrograde Intramedullary Nailing for Recurrent Fracture in Congenital Pseudarthrosis of the Tibia.
The Journal of foot and ankle surgery : official publication of the American College of Foot and Ankle SurgeonsFoot and Ankle Function at Maturity After Ilizarov Treatment for Atrophic-Type Congenital Pseudarthrosis of the Tibia: A Comprehensive Outcome Comparison with Normal Controls.
The Journal of bone and joint surgery. American volume[Versatility of the microvascular fibular flap in limb reconstruction].
Cirugia y cirujanosCongenital pseudoarthrosis in a child with neurofibromatosis.
BMJ case reportsPreliminary Results of the Induced Membrane Technique for the Reconstruction of Large Bone Defects.
Journal of pediatric orthopedicsAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Pseudoartrose congênita da fíbula.
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Comunidades
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Do Fibular Growth Patterns Change After Tibial-Fibular Fusion in Children With Congenital Pseudarthrosis of the Tibia?
- Cross-union surgery for tibiofibular fractures in McCune-Albright syndrome: a case report and literature review.
- Mother to Child Non-Vascularized Fibula Transfer in Congenital Pseudoarthrosis of Tibia.
- Vascularized Periosteal Grafts for Bone Union in Children: A Systematic Review.
- Efficacy of the cross-union protocol in the treatment of congenital tibial pseudarthrosis: a comparative study.
- Tibial lengthening in congenital pseudoarthrosis of the tibia: a scoping review.
- Development of the Extended Lane and Sandu Score to Assess Osseous Repair.
- Unraveling the molecular landscape of congenital pseudoarthrosis of the tibia: insights from a comprehensive analysis of 159 probands.
- Paley cross union protocol for congenital pseudoarthrosis of the tibia.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:295022(Orphanet)
- MONDO:0017464(MONDO)
- GARD:21205(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55787073(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
