記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Neurofibromatosis 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 PRESENTATION: We 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. CONCLUSIONS: Although 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.

DOI： 10.1186/s40792-019-0570-4

PubMed

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記述言語：日本語   出版者・発行元：(NPO)日本血管外科学会  

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記述言語：日本語   出版者・発行元：血管外科症例検討会  

下行大動脈にentryを有する逆行A型大動脈解離に対するTEVARの留意点として、1)deviceを選択するための大動脈計測、2)術前planning、3)術中手技と術後観察などについて概説した。特に真腔に対して過度のオーバーサイズなdeviceを選択しないこと、中枢landing部位に対してballooningは極力避けるなど大動脈壁に対して愛護的に取り扱うことが大切である。本治療の目的は上行大動脈の偽腔圧低減であるため、術後早期からCT(Computed Tomography)などで上行大動脈の状態を評価して必要があれば積極的に追加治療の介入を考慮する必要がある。(著者抄録)

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J-GLOBAL

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J-GLOBAL

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