MEK inhibitors in maintenance of a postoperative patient with vasculopathy due to neurofibromatosis type I

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.