MARTINEZ-TUREGANO, Beatriz et al. MEK inhibitors in maintenance of a postoperative patient with vasculopathy due to neurofibromatosis type I. []. , 52, 3, pp.108-113.   21--2025. ISSN 2696-130X.  https://doi.org/10.24875/rma.23000007.

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Vasculopathy in the context of neurofibromatosis type I is challenging, and its management requires a multidisciplinary approach. We describe the case of a 24-year-old girl with NFI and vascular complications who was successfully managed with MEK inhibitors, obtaining clinical stabilization. After left occipital artery embolization prior to surgical resection of an occipital neurofibrom, a left internal carotid artery pseudoaneurysm was detected, requiring open repair that failed, leading to direct ligation due to profuse bleeding. In the immediate postoperative period, during a coughing spell, rupture of the left subclavian artery occurred, which was successfully treated by implanting a 7 × 10 mm Viabahan®. Trametinib (0.5 mg/24 h) was administered, replaced by selumetinib (45 mg every 12 h), both therapies in compassionate use. Selumetinib was effective in stabilizing the development of vascular complications, achieving good control over symptomatology of the patient. Three months after starting treatment with MEK inhibitors, no new events have occurred.

La neurofibromatosis tipo I (NFI) es un trastorno que conduce a una sobreactivación de la vía RAS, asociando anomalías vasculares de mediano y gran vaso, espectro denominado vasculopatía de la NFI. Presentamos el caso de una paciente de 24 años con NFI que tras el intento fallido de reparación de un pseudoaneurisma de la arteria carótida interna, requirió ligadura por sangrado profuso. Durante un acceso de tos, presentó ruptura espontánea de la arteria subclavia izquierda, tratada con éxito implantando un Viabahn® de 7 x 10 mm. En el postoperatorio se inicia trametinib (0.5 mg/24 h), reemplazándose por selumetinib 45 mg cada 12 h por dos meses. Tras el alta hospitalaria, el selumetinib fue eficaz para estabilizar el desarrollo de complicaciones vasculares. A tres meses de iniciado el tratamiento farmacológico, no ha presentado nuevas complicaciones.

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