Uma doença óssea rara que, em adultos, se manifesta pela morte espontânea (osteonecrose) do osso navicular, um dos ossos do pé. Os pacientes apresentam dor crônica na parte média e traseira do pé, inchaço e sensibilidade ao toque na parte superior e interna do meio do pé. Além disso, pode haver um achatamento do arco interno do pé e uma deformidade chamada pé plano varo (um tipo de pé chato em que o calcanhar vira para dentro). Os exames de imagem revelam uma deformidade em forma de vírgula, causada pelo desabamento da parte lateral do osso navicular, e um deslocamento para dentro ou para cima de uma parte ou do osso inteiro. A condição pode ser bilateral (afetando os dois pés) ou assimétrica. Também pode estar associada a fraturas patológicas, que são quebras no osso que acontecem por causa da doença, e não por um impacto ou acidente.
Introdução
O que você precisa saber de cara
Uma doença óssea rara que, em adultos, se manifesta pela morte espontânea (osteonecrose) do osso navicular, um dos ossos do pé. Os pacientes apresentam dor crônica na parte média e traseira do pé, inchaço e sensibilidade ao toque na parte superior e interna do meio do pé. Além disso, pode haver um achatamento do arco interno do pé e uma deformidade chamada pé plano varo (um tipo de pé chato em que o calcanhar vira para dentro). Os exames de imagem revelam uma deformidade em forma de vírgula, causada pelo desabamento da parte lateral do osso navicular, e um deslocamento para dentro ou para cima de uma parte ou do osso inteiro. A condição pode ser bilateral (afetando os dois pés) ou assimétrica. Também pode estar associada a fraturas patológicas, que são quebras no osso que acontecem por causa da doença, e não por um impacto ou acidente.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 12 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 21 características clínicas mais associadas, ordenadas por frequência.
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 — Síndrome Mueller-Weiss
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Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
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Publicações mais relevantes
Inflammatory Arthritis Uncovered Through Imaging of Osteonecrosis: A Case Series.
Osteonecrosis (ON) is typically viewed as an isolated condition stemming from vascular compromise. We present a three-case series demonstrating that MRI, performed to confirm the diagnosis of ON, incidentally revealed features consistent with underlying, previously undiagnosed inflammatory arthritis (IA). Even in the absence of typical clinical features, the presence of imaging findings such as synovitis, tenosynovitis, and erosions should prompt a thorough clinical and serological evaluation for coexisting IA. An interesting point to note is that none of the three patients reported a history of steroid use. This manuscript aims to highlight a possible pathophysiological link between IA and ON.
Utility of Quantitative and Semi-Quantitative SPECT/CT Metrics in Differentiating Mueller-Weiss Syndrome.
Background/Objectives: Mueller-Weiss syndrome (MWS) is a rare condition characterized by spontaneous adult-onset osteonecrosis of the navicular bone. This study aimed to assess the diagnostic value of quantitative and semi-quantitative standardized uptake value (SUV) measurements on Tc-99m MDP SPECT/CT for differentiating MWS from other foot pathologies. Methods: We retrospectively reviewed 21 MWS patients who underwent SPECT/CT and compared them with 10 feet from 5 non-MWS patients as controls. MWS severity was staged using the Maceira classification. Volumes of interest (VOIs) were defined in the lateral navicular and distal tibia. SUVmax values were measured for the navicular bone (N), tibial metaphysis (Tm), and diaphysis (Td). Uptake ratios (N/Tm and N/Td) were calculated for semi-quantitative comparison. Results: MWS patients showed significantly higher SUVmax in the navicular compared with controls (9.2 vs. 1.5, p < 0.001). Both N/Tm and N/Td ratios were also significantly elevated (p < 0.001). SUVmax and uptake ratios positively correlated with Maceira stage and visual navicular uptake intensity. Diagnostic thresholds of N SUVmax > 3.77 (AUC = 0.93), N/Tm > 1.139 (AUC = 0.95), and N/Td > 0.93 (AUC = 0.93) effectively distinguished MWS from non-MWS cases. Conclusions: Quantitative and semi-quantitative SUV analysis on SPECT/CT offers a reliable tool for diagnosing MWS and evaluating disease severity. Semi-quantitative ratios, by normalizing metabolic variability, provide a practical and reproducible alternative to absolute SUV measurements for early detection and treatment planning in MWS.
Chronic Midfoot Pain Due to Idiopathic Osteonecrosis of Tarsal Navicular Bone in Adult - Mueller-Weiss Syndrome: A Case Report.
Mueller-Weiss syndrome is a rare condition seen in adults due to spontaneous osteonecrosis of tarsal navicular bone unlike Koehler's disease which is quite common in paediatric population. We report a single case of Mueller-Weiss syndrome in an adult female. After a trail of conservative treatment, operative management was done with excision of necrotic fragment and arthrodesis of mid-foot joints augmented with tricortical iliac crest graft. Good fusion and significant reduction of pain on weight-bearing was observed after a duration of 6 weeks. Mueller-Weiss syndrome is relatively rare differential for mid-foot pain in adult population. Early recognition and management of the condition prevents arthritic changes and disability.
Mueller-Weiss Syndrome, a Rare Cause of Flatfoot: Case Report.
There are numerous causes of flatfoot, among them osteonecrosis of the navicular bone, a rare disease known as Mueller-Weiss syndrome, also leads to deformity. A 53-year-old male presented to our Outpatient Department with pain and swelling over bilateral foot for 3 years with increased severity in the left. Physical examination as well as footprint revealed a bilateral flat foot, and there was tenderness as well as swelling over the dorsomedial aspect of bilateral foot. After a detailed physical examination and radiological assessment, the case was diagnosed as Mueller-Weiss syndrome. He was treated with non-steroidal anti-inflammatory drugs, custom ankle-foot orthotic brace for 6 weeks, and activity modification and symptoms were relieved and he was able to do daily activities without any discomfort. MW syndrome is a rare condition, where conservative treatment provides better results if it is diagnosed and treated early, whereas late cases require surgical intervention.
Talonavicular-cuneiform arthrodesis in the management of Mueller-Weiss Syndrome: a retrospective case series.
Mueller-Weiss Syndrome (MWS), characterized by spontaneous adult-onset tarsal navicular osteonecrosis, is an uncommon cause of chronic midfoot pain that can lead to functional impairment and progressive deformities. This study aimed to present clinical and radiological outcomes of talonavicular-cuneiform (TNC) arthrodesis in the treatment of patients with MWS. A retrospective study was performed on 8 consecutive patients (6 female, 2 male; mean age = 50 years; range = 33-64) who underwent TNC arthrodesis using plate fixation with autologous bone grafting for the treatment of MWS. To evaluate clinical status, the American Orthopaedic Foot and Ankle Society (AOFAS) Ankle-Midfoot Score was performed immediately preoperatively and at the final follow-up. In radiographic evaluation, talus-first metatarsal angle (Meary's angle) was measured preoperatively and at the final follow-up. Solid fusion was also examined on postoperative radiographs and computerised tomography. The mean follow-up was 35 months (range = 24-52). The mean AOFAS improved from 37 (range = 24-53) preoperatively to 85 (range = 80-93) at the final follow-up (p < 0.001). No major intra- operative complications were observed in any of the patients. According to the Maceira and Rochera radiological staging system, 5 feet was stage 3, and 3 feet was stage 4. The mean union time was 10 months (range = 5-15). Radiographic solid fusion was achieved in all but one foot that developed talonavicular non-union. TNC arthrodesis using plate fixation with autologous bone grafting seems to be an effective surgical method for reconstruction of MWS.
Publicações recentes
Inflammatory Arthritis Uncovered Through Imaging of Osteonecrosis: A Case Series.
Utility of Quantitative and Semi-Quantitative SPECT/CT Metrics in Differentiating Mueller-Weiss Syndrome.
Chronic Midfoot Pain Due to Idiopathic Osteonecrosis of Tarsal Navicular Bone in Adult - Mueller-Weiss Syndrome: A Case Report.
Mueller-Weiss Syndrome, a Rare Cause of Flatfoot: Case Report.
Talonavicular-cuneiform arthrodesis in the management of Mueller-Weiss Syndrome: a retrospective case series.
📚 EuropePMC14 artigos no totalmostrando 8
Inflammatory Arthritis Uncovered Through Imaging of Osteonecrosis: A Case Series.
CureusUtility of Quantitative and Semi-Quantitative SPECT/CT Metrics in Differentiating Mueller-Weiss Syndrome.
Diagnostics (Basel, Switzerland)Chronic Midfoot Pain Due to Idiopathic Osteonecrosis of Tarsal Navicular Bone in Adult - Mueller-Weiss Syndrome: A Case Report.
Journal of orthopaedic case reportsMueller-Weiss Syndrome, a Rare Cause of Flatfoot: Case Report.
Journal of orthopaedic case reportsTalonavicular-cuneiform arthrodesis in the management of Mueller-Weiss Syndrome: a retrospective case series.
Acta orthopaedica BelgicaThree-phase bone quantitative-SPECT of navicular bones with a high-sensitivity whole-body CZT-camera in a Müeller-Weiss syndrome.
European journal of nuclear medicine and molecular imagingSpontaneous Osteonecrosis of the Tarsal Navicular: A Report of Two Cases.
Case reports in orthopedicsImaging of Mueller-Weiss Syndrome: A Review of Clinical Presentations and Imaging Spectrum.
AJR. American journal of roentgenologyAssociações
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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.
- Inflammatory Arthritis Uncovered Through Imaging of Osteonecrosis: A Case Series.
- Utility of Quantitative and Semi-Quantitative SPECT/CT Metrics in Differentiating Mueller-Weiss Syndrome.
- Chronic Midfoot Pain Due to Idiopathic Osteonecrosis of Tarsal Navicular Bone in Adult - Mueller-Weiss Syndrome: A Case Report.
- Mueller-Weiss Syndrome, a Rare Cause of Flatfoot: Case Report.
- Talonavicular-cuneiform arthrodesis in the management of Mueller-Weiss Syndrome: a retrospective case series.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:566943(Orphanet)
- MONDO:0035452(MONDO)
- GARD:22283(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q18629322(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.
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