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Casuística do tratamento de craniossinostoses e disostoses craniofaciais no CHULN-HSM no período 2002-2021
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Introdução: As craniossinostoses definem-se como a fusão prematura de uma ou mais suturas cranianas (craniostenose). Nas disostoses craniofaciais, para além da fusão prematura das suturas da calote craniana, verifica-se a fusão prematura das sincondroses da base do crânio, com compromisso do crescimento facial. O Hospital de Santa Maria (CHULN-HSM) é um centro de referência português para esta intervenção desde 2001.
Objetivos: Descrever a casuística do tratamento das craniossinostoses realizadas no CHULN-HSM no período 2002-2021. Realizar um estudo retrospetivo com análise do follow-up dos doentes e posterior estudo de correlações entre os antecedentes pessoais, as técnicas cirúrgicas implementadas e complicações precoces e tardias descritas. Comparar os resultados deste estudo com estudos a nível internacional.
Metodologia: Revisão da literatura mais recente relativa a craniossinostoses e o seu tratamento, seguido de estudo retrospetivo com levantamento de casuística e análise estatística dos dados.
Resultados: Os 283 processos clínicos analisados incluíram 376 cirurgias. A média da idade na 1ª cirurgia, excluindo cirurgias ortognáticas, foi de 11 meses, com predomínio do sexo masculino (66%). As craniossinostoses simples e múltipla mais comuns foram a escafocefalia (45%) e a pansinostose (2,5%), respetivamente. 40 doentes apresentavam síndrome genético associado, sendo o mais comum o Crouzon (5,3%). A cranioplastia foi a técnica mais comum (215 vs 68 distrações ósseas), sendo de destacar o predomínio da pi-plastia modificada (30%). A complicação precoce e tardia mais comum foi síndrome febril (5%) e presença de irregularidades na calote (29%), respetivamente. Apresentar síndrome genético e craniossinostose múltipla está associado a complicações precoces e tardias. Foi visualizada associação entre complicações e a sutura afetada. A cranioplastia demonstrou levar a 2x mais complicações tardias que a distração óssea.
Conclusão: A casuística analisada demonstra compatibilidade com estudos semelhantes publicados na literatura. Foram observadas correlações significativas entre os antecedentes do doente e a presença de complicações precoces ou tardias.
Introduction: Craniosynostosis are defined as the premature fusion of one or more cranial sutures, which result in craniostenosis. In craniofacial dysostosis syndromes, both the cranial vault sutures and the cranial base synchondroses are prematurely fused, resulting in compromised midfacial growth. CHULN-HSM is a reference centre in Portugal for this intervention since 2001. Aims: Describe the casuistic of the craniosynostosis treatment in the CHULN-HSM, between 2002-2021. Perform a retrospective study and analysis of the patient’s follow-up and consequent study of plausible correlations between patients’ personal history, surgical techniques used, and complications described. Compare the results of this Portuguese study with similar studies worldwide. Methods: Literature review of craniosynostosis and its treatment, followed by a retrospective study of the CHULN-HSM casuistic and statistical data analysis. Results: There were 283 clinical processes analysed, which included 376 surgeries. The median age of the first surgery was 11 months (excluding orthognathic surgery), and male patients were more prevalent (66%). The most common simple and multiple craniosynostosis were scaphocephaly (44,9%) and pansynostosis (2,5%), respectively. Forty patients had a genetic syndrome associated, being Crouzon the most common (5,3%). Cranioplasty was the most performed technique (215 vs 68 bone-distractions), with modified pi-plasty being the most common (30%). The most common early complication was febrile syndrome and late complication was irregularity of the cranial vault (29%). There is an association between having a genetical syndrome or multiple craniosynostosis and early/late complications. Furthermore, the type of suture affected showed association with the presence of complications. Cranioplasty demonstrated 2x more probability of leading to late complications than bone-distraction. Conclusion: The casuistic analysed showed similarity with worldwide studies published. There were important correlations proved between patients’ history and the presence of early and late complications.
Introduction: Craniosynostosis are defined as the premature fusion of one or more cranial sutures, which result in craniostenosis. In craniofacial dysostosis syndromes, both the cranial vault sutures and the cranial base synchondroses are prematurely fused, resulting in compromised midfacial growth. CHULN-HSM is a reference centre in Portugal for this intervention since 2001. Aims: Describe the casuistic of the craniosynostosis treatment in the CHULN-HSM, between 2002-2021. Perform a retrospective study and analysis of the patient’s follow-up and consequent study of plausible correlations between patients’ personal history, surgical techniques used, and complications described. Compare the results of this Portuguese study with similar studies worldwide. Methods: Literature review of craniosynostosis and its treatment, followed by a retrospective study of the CHULN-HSM casuistic and statistical data analysis. Results: There were 283 clinical processes analysed, which included 376 surgeries. The median age of the first surgery was 11 months (excluding orthognathic surgery), and male patients were more prevalent (66%). The most common simple and multiple craniosynostosis were scaphocephaly (44,9%) and pansynostosis (2,5%), respectively. Forty patients had a genetic syndrome associated, being Crouzon the most common (5,3%). Cranioplasty was the most performed technique (215 vs 68 bone-distractions), with modified pi-plasty being the most common (30%). The most common early complication was febrile syndrome and late complication was irregularity of the cranial vault (29%). There is an association between having a genetical syndrome or multiple craniosynostosis and early/late complications. Furthermore, the type of suture affected showed association with the presence of complications. Cranioplasty demonstrated 2x more probability of leading to late complications than bone-distraction. Conclusion: The casuistic analysed showed similarity with worldwide studies published. There were important correlations proved between patients’ history and the presence of early and late complications.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2022
Palavras-chave
Craniossinostose Complicações Cranioplastia Distração óssea Cirurgia plástica e reconstrutiva