Background: Neurofibromatosis type 1 (NF1), also known as von Recklinghausen disease, is a rare congenital disorder with an autosomal dominant inheritance. Severe perioperative complications owing to the congenital fragility of the vascular wall in NF1 patients have been reported. The optimal vascular surgical approach remains controversial. We describe endovascular aortic repair of an abdominal aortic aneurysm rupture in a patient with NF1.
Case presentation: A 78-year-old woman with NF1 was transferred to our institution with a diagnosis of ruptured abdominal aortic aneurysm. The patient showed multiple café-au-lait spots, numerous neurofibromatosis, and severe scoliosis. Emergency endovascular aortic repair was performed, without technical difficulty. Despite an uneventful postoperative course, she developed an idiopathic left cervical hematoma caused by hemorrhage, and required tracheostomy due to severe airway obstruction. In addition, postoperative CT showed a newly developed saccular aneurysm at the proximal end of the stent graft. On postoperative day 40, she was transferred to a rehabilitation hospital, without recurrent bleeding and saccular aneurysm enlargement.
Conclusion: In patients with NF1 who require a vascular surgical procedure, surgeons should consider the vascular wall fragility in selecting the optimal treatment strategy and the possible complications.
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