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 de nascimento onde o fêmur não se forma corretamente, resultando em uma fratura persistente e não consolidada (pseudoartrose) na diáfise. Frequentemente associada a outras anomalias e pode levar a deformidades e encurtamento do membro.
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
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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 do fêmur
Centros de Referência SUS
24 centros habilitados pelo SUS para Pseudoartrose congênita do fêmur
Centros para Pseudoartrose congênita do fêmur
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
Vascularized Deep Femoral Periosteal Flap: Anatomical Study and Clinical Application in Congenital Pseudarthrosis of the Tibia in Two Cases.
Vascularized periosteal transplants have proven to be highly effective and rapid in promoting bone healing in biologically complex nonunions in children, with several reported donor sites. This study aimed to assess the feasibility of harvesting a vascularized diaphyseal femoral periosteal flap (VDFPF) from cadavers and evaluate its clinical application in two cases of bilateral congenital pseudarthrosis of the tibia. This study investigated the periosteal branches of the deep femoral vessels (DFV) supplying the femoral diaphysis in 19 previously latex-injected cadavers. The femur was divided into four segments. The distance from the midpoint of the inguinal ligament to the origin of the DFV and the number of periosteal branches were recorded. The vascularized diaphyseal femoral periosteal flap (VDFPF) was used to treat one side in two patients with bilateral congenital pseudarthrosis of the tibia (aged 14 and 2 years). The DFV's origin was located at a mean of 4.2 cm (range 2.8-8.5 cm) distal to the midpoint of the inguinal ligament. In 15 cases, the DFV coursed adjacent to the second and third quarters of the femoral shaft, providing a mean number of 5 (range 4-8) periosteal branches, allowing for the harvesting of a new pure vascularized periosteal flap. Healing of congenital pseudarthrosis of the tibia occurred within 3 months in both patients, with no donor or recipient site complications at the final follow-up of 13 months. The VDFPF may be an effective periosteal transplant and a viable alternative to other large free periosteal transplants used in children for treating complex non-unions, especially when tibial and fibular donors are not available. Bone union was fast compared to other techniques used to treat these challenging cases.
Congenital Pseudoarthrosis of the Femur Managed with Masquelet Technique.
Congenital pseudoarthrosis is a rare disorder, and mostly occurs in the radius, ulna, tibia, and fibula, but is not reported in the femur. Congenital cases that were reported in the literature, most were associated with neurofibromatosis, cleidocranial dysostosis, congenital constriction band syndrome and fibrous dysplasia and the acquired cases followed fractures of bones or tuberculosis. Since the life span of the patient is affected rarely, clinical examination of these patients and serial radiographs can give us plenty of information about the limb abnormality, its clinical course, and the nature of the underlying disturbance of growth. A 3 year female child presents to the outpatient department of a tertiary care hospital with complaints of pain, swelling and deformity thigh region right side for 5 months. On examination, Swelling was present [2x2 cm] at the junction of the mid and distal thigh, smooth surface, ill defined edges, soft and non tender. There was abnormal mobility which was noted at the right thigh at the junction of the middle and distal 1/3 right femurMRI reported circumferential periosteal reaction with cortical surface irregularity of the right femoral shaft was also noted. The findings were reported to be concerning for chronic osteomyelitis with non-united fracture & cortical resorption/chronic bone loss. Histopathological sections showed multiple bits of viable bony trabeculae with surrounding fibro-collagenous tissue displaying significant proliferation of fibroblasts, slit-like blood vessels, arterioles and moderate mixed inflammatory infiltrate of plasma cells, few neutrophils, lymphocytes, histiocytes and few multinucleated histiocytes.She was managed with resection of pseudoarthrosis and masquelet procedure stage 1 and later masquelet stage 2 (cement spacer removal, ipsilateral ileac crest bone grafting with fixation with 7-hole distal fibular anatomical plate and bone graft substitute placement [ChronOs]). Idiopathic or primary congenital pseudoarthrosis can involve the femur as well as other long bones. It may or may not be associated with hip dysplasia and the outcome may not be as bad as previously reported in the pseudoarthrosis of other long bones.
Vascularized Femoral Myo-Periosteal Graft for Congenital Pseudarthrosis of the Tibia: A Case Report.
Pure vascularized periosteal transplants have been shown to be extremely effective at achieving rapid bone healing in children with biologically complex non-union. Free tibial and fibular periosteal transplants are generally indicated when large periosteal flaps are necessary. We report using a vascularized femoral myo-periosteal graft (VFMPG) to treat distal tibial osteotomy non-union in a six-year-old boy with congenital pseudarthrosis of the tibia. The graft consisted of a 9 cm myo-periosteal flap (after 50% of elastic retraction) that incorporated the vastus intermedius muscle and diaphyseal femoral periosteum nourished by the descending branch of the lateral circumflex femoral vessels. Plantaris medialis was used as a recipient vessel. Healing occurred 10 weeks after surgery. The patient resumed gait and sports activity without orthosis. No donor or recipient site complications occurred 17 months after surgery. Employing a VFMPG might be an alternative to other free or large vascularized periosteal flaps currently in use for complex pediatric non-unions.
Special Report: The Moore Pediatric Surgery Center: An Evolving Model for Pediatric Orthopaedic Surgical Care in a Limited Resource Environment.
The authors describe their 11-year experience with 1 model for providing short-term (about 1 wk/y in country) pediatric orthopaedic surgical care in a limited resource environment. This paper provides a detailed narrative of 1 team's pediatric orthopaedic work at the Moore Pediatric Surgery Center in Guatemala City, how it has evolved over these 11 years, financial aspects of the model, and examines patient follow-up data for a consecutive 8-year period. The authors have reviewed financial records, case lists, patient charts from 2014 to 2022, and patient photographic records from The Moore Center and as provided via internet by a local contracted Guatemalan pediatric orthopaedic fellowship-trained surgeon to present a complete picture of how the service functions. Specific follow-up data included: last follow-up date, date discharged from follow-up, and major complications including infection, surgical wound dehiscence, return for unplanned surgery, major nerve injury, and recurrent hip dislocation for cases of closed or open reduction of developmental hip dislocation. A total of 297 consecutive pediatric orthopaedic surgical patients were identified from 2014 to 2022. Of these, charts were found for 235 patients (135 female, 110 male), of which 43% were from the urban Guatemala City region. Two hundred sixteen (72%) had at least 1 follow-up clinic visit, and 87 (37%) had at least 1-year follow-up or were discharged. All complications identified by this retrospective chart review included 4 recurrent hip dislocations (3 after closed reduction), 1 femur fracture after implant removal, 1 superficial infection requiring antibiotics, 1 partial dehiscence treated only with dressings, 1 thumb subluxation, and 1 failed graft with internal fixation for congenital pseudoarthrosis of tibia. The Moore Pediatric Surgery Center is a financially viable, sustainable, safe, and effective model for delivering short-term surgical care for many pediatric orthopaedic conditions in a limited resource environment. None (descriptive).
Failure of tension band plating: a case series.
Growth modulation with tension band plates (TBP) has been shown to be a very useful method for the treatment of angular deformities in growing children. Recently, we have observed cases of failure where the epiphyseal screw was drawn through the physis into the metaphysis. This study describes a series of children who developed this complication. Patients who developed TBP failure after operative treatment of lower limb angular deformities were identified from the databases at four institutions over a 5-year period. The medical records were reviewed to record demographics, primary diagnoses, details of the operative procedure, development of physeal arrest, and recurrence of the original deformity. Six patients (five girls) with nine implant failures were identified. The mean age of the children at the time of implant insertion was 7.2 years (range, 4-10 years). The primary diagnoses included hypophosphatemic rickets (n=7), congenital pseudoarthrosis of the tibia associated with neurofibromatosis 1 (n=1), and post-traumatic malunion after distal tibial fracture (n=1). Of the nine TBP that presented with the complication, four were inserted into the medial distal femur (one bilateral case), two into the medial proximal tibia (one bilateral case), two into the lateral distal tibia, and one into the medial distal tibia. None of these patients developed physeal growth arrest at the last follow-up as assessed on the latest radiographs. The use of TBP for guided growth in patients younger than 10 years old with rickets, neurofibromatosis, or other conditions that produce osteopenia leads to an increased risk for implant failure. In these cases, it is important to confirm that the epiphyseal screw has good purchase. Patients with these features should be monitored closely for early detection of this complication.
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.
📚 EuropePMC4 artigos no totalmostrando 8
Vascularized Deep Femoral Periosteal Flap: Anatomical Study and Clinical Application in Congenital Pseudarthrosis of the Tibia in Two Cases.
MicrosurgeryCongenital Pseudoarthrosis of the Femur Managed with Masquelet Technique.
Journal of orthopaedic case reportsVascularized Femoral Myo-Periosteal Graft for Congenital Pseudarthrosis of the Tibia: A Case Report.
MicrosurgerySpecial Report: The Moore Pediatric Surgery Center: An Evolving Model for Pediatric Orthopaedic Surgical Care in a Limited Resource Environment.
Journal of pediatric orthopedicsFemoral overgrowth in children with congenital pseudarthrosis of the Tibia.
BMC musculoskeletal disordersFailure of tension band plating: a case series.
Journal of pediatric orthopedics. Part BSurgical technique and indications of the induced membrane procedure in children.
Orthopaedics & traumatology, surgery & research : OTSR[Versatility of the microvascular fibular flap in limb reconstruction].
Cirugia y cirujanosAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Pseudoartrose congênita do fêmur.
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Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Pseudoartrose congênita do fêmur
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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.
- Vascularized Deep Femoral Periosteal Flap: Anatomical Study and Clinical Application in Congenital Pseudarthrosis of the Tibia in Two Cases.
- Congenital Pseudoarthrosis of the Femur Managed with Masquelet Technique.
- Vascularized Femoral Myo-Periosteal Graft for Congenital Pseudarthrosis of the Tibia: A Case Report.
- Special Report: The Moore Pediatric Surgery Center: An Evolving Model for Pediatric Orthopaedic Surgical Care in a Limited Resource Environment.
- Failure of tension band plating: a case series.
- 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.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:295020(Orphanet)
- MONDO:0017463(MONDO)
- GARD:21204(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55787072(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
