Fratura da coluna torácica na síndrome de gorham-stout com cifose progressiva: relato de caso e revisão da literatura / Thoracic spine fracture in gorham-stout syndrome with progressive kyphosis: case report and literature review
DOI:
https://doi.org/10.34117/bjdv8n3-152Keywords:
gorham-stout, síndrome do osso fantasma, “gorham-stout syndrome in spine”, “gorham-stout disease in spine”, “vanishing bone disease in spine” e “disappearing bone disease in spine”.Abstract
A Síndrome de Gorham-Stout (SGS), também denominada de osteólise maciça é uma patologia rara, que pode se dispor a importante morbidade e mortalidade, caracterizada pela proliferação intraóssea idiopática de estruturas angiomatosas ou dos vasos linfáticos resultando em destruição progressiva e reabsorção óssea. Ela afeta principalmente ossos das áreas maxilofaciais, dos membros superiores (incluindo a escápula) e do tronco (incluindo as costelas, clavículas e cinturas pélvicas), e, menos comumente, ocorre o envolvimento primário da coluna.Cerca de 300 casos de GSD foram descritos na literatura até o momento. Os sinais e sintomas iniciais costumam ser fraturas patológicas, mas podem ser inespecíficos, como dor, inchaço local, restrição de movimento e parestesia. A SGS costuma evoluir para deformidade óssea, fraturas espontâneas, derrame pericárdico e quilotórax.Atualmente, não existe um consenso sobre plano terapêutico, podendo variar desde um manejo conservador e medicamentoso, com o uso de radioterapia e medicamentos como o INF-α ou bisfosfonatos, até o cirúrgico, realizado ao envolver a caixa torácica, com a ressecção do osso afetado ou artrodese. Destarte, descrevemos a abordagem terapêutica realizada pela equipe médica neste relato de caso para contribuir futuro consenso sobre o tratamento.Devido à escassez de estudos publicados sobre essa síndrome, foi abordado, no presente artigo, um relato de caso de um paciente do sexo masculino de 43 anos com a Síndrome de Gorham-Stour, aprovado para publicação pelo paciente, bem como uma revisão completa da literatura sobre a doença para melhor análise da patologia. As palavras-chaves foram utilizadas na base de dados Pubmed, com obtenção de 38 artigos publicados no período de 1944 e 2021. Dentre esses, foram selecionados todos aqueles que abordam o acometimento da coluna espinhal, sendo posteriormente confeccionada uma tabela para analisar os principais aspectos do quadro da Síndrome Gorham-Stout.A revisão é de extrema importância devido ao tratamento incerto e à má responsividade dessa doença às terapias habituais. Pode-se, assim, ajudar a formular um consenso sobre a melhor abordagem e o mais adequado plano terapêutico, respaldado nos relatos de caso publicados até o momento da confecção e publicação deste presente artigo.
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