TY - JOUR
T1 - Aneurysm-osteoarthritis syndrome with visceral and iliac artery aneurysms
AU - Linde, Denise
AU - Verhagen, Hence
AU - Moelker, Adriaan
AU - De Graaf - van de Laar, Ingrid
AU - Van Herzeele, I
AU - Backer, J
AU - Dietz, HC
AU - Roos - Hesselink, Jolien
PY - 2013
Y1 - 2013
N2 - Objective: Aneurysms-osteoarthritis syndrome (AOS), caused by SMAD3 mutations, is a recently described autosomal-dominant syndrome characterized by arterial aneurysms, tortuosity, and aortic dissections in combination with osteoarthritis. Our objective was to evaluate the AOS-related vascular consequences in the visceral and iliac arteries and raise awareness for this aggressive syndrome among vascular specialists. Methods: AllAOSpatients were monitored regularly according to our clinical AOS protocol. The study included those with one or more visceral aneurysms or tortuosity, or both. Clinical and surgical data were obtained from record abstraction. Results: The study included 17 AOS patients (47% men) aged 47 +/- 13 years. A total of 73 aneurysms were encountered, of which 46 were located in the abdomen. The common iliac artery was most commonly affected (37%), followed by the superior mesenteric artery (15%), celiac trunk (11%), and splenic artery (9%). Rapid aneurysm growth <= 1 year was found in three arteries (gastric, hepatic, and vertebral artery). Furthermore, arterial tortuosity was noted in 94% of patients. Four patients underwent Conclusions: AOS predisposes patients to widespread visceral and iliac artery aneurysms and extreme arterial tortuosity. Early elective aneurysm repair should be considered because the risk of aneurysm rupture is estimated to be very high and elective (endo) vascular interventions were not complicated by fragility of arterial tissue. Given the aggressive behavior of AOS, it is of utmost importance that vascular specialists are aware of this new syndrome. (J Vasc Surg 2013;57:96-102.)
AB - Objective: Aneurysms-osteoarthritis syndrome (AOS), caused by SMAD3 mutations, is a recently described autosomal-dominant syndrome characterized by arterial aneurysms, tortuosity, and aortic dissections in combination with osteoarthritis. Our objective was to evaluate the AOS-related vascular consequences in the visceral and iliac arteries and raise awareness for this aggressive syndrome among vascular specialists. Methods: AllAOSpatients were monitored regularly according to our clinical AOS protocol. The study included those with one or more visceral aneurysms or tortuosity, or both. Clinical and surgical data were obtained from record abstraction. Results: The study included 17 AOS patients (47% men) aged 47 +/- 13 years. A total of 73 aneurysms were encountered, of which 46 were located in the abdomen. The common iliac artery was most commonly affected (37%), followed by the superior mesenteric artery (15%), celiac trunk (11%), and splenic artery (9%). Rapid aneurysm growth <= 1 year was found in three arteries (gastric, hepatic, and vertebral artery). Furthermore, arterial tortuosity was noted in 94% of patients. Four patients underwent Conclusions: AOS predisposes patients to widespread visceral and iliac artery aneurysms and extreme arterial tortuosity. Early elective aneurysm repair should be considered because the risk of aneurysm rupture is estimated to be very high and elective (endo) vascular interventions were not complicated by fragility of arterial tissue. Given the aggressive behavior of AOS, it is of utmost importance that vascular specialists are aware of this new syndrome. (J Vasc Surg 2013;57:96-102.)
U2 - 10.1016/j.jvs.2012.06.107
DO - 10.1016/j.jvs.2012.06.107
M3 - Article
C2 - 22975338
SN - 0741-5214
VL - 57
SP - 96
EP - 102
JO - Journal of Vascular Surgery
JF - Journal of Vascular Surgery
IS - 1
ER -