Spontaneous rupture of the pancreatic arcade artery caused by neurofibromatosis type 1 successfully treated using emergency transcatheter arterial embolization, partial intra-aortic balloon occlusion, and stent graft placement: a case report and review of the literature

CVIR Endovascular

article

Morita, Ryo¹  Abo, Daisuke¹  Soyama, Takeshi¹  Yoshino, Yuki¹  Yoshikawa, Toru¹  Kimura, Tasuku³  Kudo, Kohsuke¹ 

[1] Department of Diagnostic and Interventional Radiology, Hokkaido University Hospital;Department of Diagnostic Imaging, Hokkaido University Graduate School Medicine, Hokkaido University;Department of Diagnostic Radiology, National Hospital Organization Hokkaido Cancer Center

关键词: Neurofibromatosis type 1;    Stent-graft placement;    Endovascular therapy;    Pancreatic arcade artery;    Spontaneous rupture;    Vasculopathy;    Intra-aortic balloon occlusion;   

DOI  :  10.1186/s42155-020-00129-y

学科分类：计算机科学（综合）

来源: Springer

【 摘 要 】

Vascular abnormalities in neurofibromatosis type 1 (NF1) are rare, but are the second leading cause of death in persons with NF1. In NF1 vasculopathy (NF-V), fatal bleeding due to a spontaneous arterial rupture sometimes occurs. Ruptured extracranial arteries in patients with NF1 often involve thoracic vessels, such as the intercostal and subclavian arteries; very few reports exist regarding the abdominal region. Herein, we present the first case of intraperitoneal bleeding due to spontaneous pancreatic arcade artery (PAA) rupture associated with NF1, successfully treated by transcatheter arterial embolization (TAE) combined with stent-graft placement and partial intra-aortic balloon occlusion (IABO). A 40-year-old woman complained of back and abdominal pain. Upon admission, her blood pressure was 85/41 mmHg and heart rate was 129 beats/min. Computed tomography (CT) showed large intraperitoneal bleeding due to PAA rupture. After CT scanning, her systolic blood pressure decreased to 50 mmHg. Therefore, we performed emergency TAE with partial IABO. She was treated by TAE of the anterior superior pancreaticoduodenal artery, anterior inferior pancreaticoduodenal artery, and inferior pancreaticoduodenal artery. However, even after TAE, minor extravasation around the superior mesenteric artery continued, and her vital signs remained unstable. Stent-graft placement was selected to stop the haemorrhage, preserving normal blood flow of the superior mesenteric artery trunk. Excellent patency of the stent graft was confirmed on follow-up CT, and she was discharged on postoperative day 56. PAA rupture associated with NF1 can be successfully treated by TAE combined with partial intra-aortic balloon occlusion, and stent-graft placement.

【 授权许可】

CC BY