Non-Marfan idiopathic medionecrosis (cystic medial necrosis) presenting with multiple visceral artery aneurysms and diffuse connective tissue fragility: two brothers.
Two brothers with multiple visceral artery aneurysms or dilatations and diffuse connective tissue fragility who did not have clinical features of Marfan syndrome are reported. One presented with retroperitoneal hemorrhage during angiography, and idiopathic medionecrosis was proved by resection of the aneurysms. These cases belong to the heterogeneous group of Marfan syndrome. The angiographical features (multiple dilation of visceral arteries) suggests fragility of connective tissue and is predictive of hazards during and after a catheterization and operation.
Kubota
J
J
Department of Nuclear Medicine, Gunma University School of Medicine, Japan.
Tsunemura
M
M
Amano
S
S
Tokizawa
S
S
Oowada
S
S
Shinkai
H
H
Maehara
Y
Y
Endo
K
K
D000328:Adult; D000783:Aneurysm / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D000792:Angiography / Q000009:adverse effects; D003240:Connective Tissue Diseases / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology; D004064:Digestive System / Q000098:blood supply*; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000235:genetics; D009336:Necrosis; D012187:Retroperitoneal Space; D017540:Tunica Media / Q000473:pathology*