CASE REPORT
Introdução: Os neurofibromas intra e extradurais da raiz C2 são uma patologia desafiadora, geralmente com evolução indolente, por isso os sintomas de início são déficit neurológico assim como sintomas da mielopatia cervical alta e explica o diagnóstico tardio de tumores gigantes com compressão grave da medula espinhal com envolvimento e distorção de anatomia dos grandes vasos. Esses factos dificultam as cirurgias. O acompanhamento desse tipo de cirurgia deve ser muito próximo, pois muitas vezes pode ocorrer fístula liquórica. Relatos de Caso: O presente artigo relata uma série de três casos de neurofibromas, dois deles são neurofibromas C2 gigantes em forma de haltere e o terceiro é um caso de neurofibromatose tipo 1 de raízes C2 bilateralmente, raiz C3 unilateral com evolução pós-operatória incomum. A reconstrução dural foi necessária em todos os casos. Conclusão: Os pacientes desta série foram bem sintomáticos e com boa recuperação. Nos procedimentos cirúrgicos, embora desafiadores, foi possível a remoção total do tumor, sendo necessária a reconstrução dos defeitos durais em todos os casos. No caso da NF1, o pós-operatório foi praticamente apenas o acompanhamento das complicações e a sua abordagem. Este fato reforça a importância de um planejamento pré-operatório meticuloso e de um acompanhamento próximo. A qualidade de vida pós-operatória foi melhor do que a pré-operatória em todos os casos. O objetivo do tratamento cirúrgico foi a descompressão da medula espinhal, remoção do tumor e profilaxia da fístula do líquor através do fechamento dos defeitos durais. Em geral, a ressecção cirúrgica de neurofibromas da raiz C2 é um procedimento seguro e associado a bons resultados clínicos.
Background: Intra and extradural neurofibromas of the C2 root is a challenging pathology, usually with indolent evolution. That is why the onset symptoms are neurologic deficit, such as upper cervical myelopathy symptoms, which explains the late diagnosis of giant tumors with severe spinal cord compression and the involvement and distortion of great vessel anatomy. These facts make these surgeries more difficult. The follow-up of this type of surgery must be very closely observed because CSF fistula can often occur. Case Presentation: The present article reports a series of three cases of neurofibromas; two of them were giant dumbbell-shaped C2 neurofibromas, and the third was a case of type 1 neurofibromatosis of C2 roots bilaterally, and a unilateral C3 root with an unusual postoperative course. Dural reconstruction was necessary in all cases. Conclusions: The patients in this series were very symptomatic with good recovery. The surgical procedures were challenging; total tumor removal was possible; the reconstruction of dural defects was needed in all cases. In the case of NF1, the postoperative period was practically just the follow up of complications and addressing them. This fact stresses the importance of meticulous preoperative planning and close follow up. Postoperative quality of life was better than preoperative in all cases. The purpose of the surgical treatment is the decompression of the spinal cord, tumor removal and prophylaxis of CSF fistula by closing the dural defects. Overall, the surgical C2 root neurofibromas resection is a safe procedure associated with good clinical outcomes.
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1Neurosurgery Department, Centro Hospitalar Tondela Viseu, Tondela, Viseu, Portugal.
2Neurosurgery Department, Hospital Prenda, Luanda, Angola.
3Orthopedic Department, Centro Hospitalar Tondela Viseu, Tondela, Viseu, Portugal.
4Faculty of Medicine, University of Coimbra, Coimbra, Portugal.
Received July 27, 2023
Corrected Oct 27, 2023
Accepted July 20, 2024