Abstract
BackgroundNeurofibromatosis type 1 (NF1) is an autosomal dominant disease of the skin and soft tissue. Aneurysms associated with NF1 can occur, but a secondary aneurysm rupture is very rare, with very few cases reported in literature.Case presentationWe describe the case of a 67-year-old female with NF1 who underwent endovascular aneurysm repair (EVAR) for an abdominal aortic aneurysm (AAA) rupture. She developed a type Ib endoleak requiring a redo-EVAR. Eighteen days after her primary operation, she was found to have two new left colic artery aneurysms. She required emergency surgery consisting of a left hemicolectomy and transverse colon colostomy. Pathology showed neurofibromatous changes to the peri-vasculature tissue, consistent with her underlying disease.ConclusionsAlthough rare, secondary aneurysms can occur following AAA repair. Patients with soft tissue connective tissue disorders, like NF1, may be at an increased risk for development of these secondary aneurysms. Endovascular repair appears to be a safe approach for NF1 patients with AAA, but endovascular management can be challenging in the setting of NF1. Surgeons should be ready to convert to open surgery if the patient displays persistent signs of bleeding or structural changes related to connective tissue disorders like NF1.
Highlights
Neurofibromatosis type 1 (NF1) is an autosomal dominant disease of the skin and soft tissue
Endovascular repair appears to be a safe approach for NF1 patients with abdominal aortic aneurysm (AAA), but endovascular management can be challenging in the setting of NF1
Surgeons should be ready to convert to open surgery if the patient displays persistent signs of bleeding or structural changes related to connective tissue disorders like NF1
Summary
EVAR appears to be a safe approach for NF1 patients with AAA due to the extreme fragility of the vessel wall. Endovascular management can be challenging at times, such as the case with our patient. The surgeon should be ready to consider open surgery in the settings of underlying connective tissue disease or emergency circumstances. Abbreviations 3D-CT: Three-dimensional-CT; AAA: Abdominal aortic aneurysm; CT: Computed tomography; EVAR: Endovascular aortic repair; ICG: Indocyanine green; IIA: Internal iliac artery; IMA: Inferior mesenteric artery; IVC: Inferior vena cava; LCA: Left colic artery; MCA: Middle colic artery; NF1: Neurofibromatosis type 1; SMA: Superior mesenteric artery; SRA: Superior rectal artery
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