BACKGROUND: Ehlers Danios syndrome (type IV) and kidney and liver cyst dise
ase can present a common factor: anomalous biosynthesis of structural colla
gen and elastic tissue, We present an exceptional case,
CASE REPORT: A 62-year-old man complained of pain in the upper left quadran
t of the abdomen. Ultrasonography, magnetic resonance imaging and arteriogr
aphy evidenced an aneurysm of the splenic artery, an aneurysm of the hypoga
stric artery, multiple cysts in the kidney and liver, and mitral valvulopat
hy. Treatment was splenectomy with resection of the splenic aneurysm and re
section of the iliac aneurysm and iliac-iliac bypass.
DISCUSSION: The association of kidney and liver cyst disease with venous ga
strointestinal and cardiovascular complications is well known. The simultan
eous presence of cysts and peripheral and visceral aneurysms with anomalies
of the arterial wall resembling Ehlers Danlos syndrome (type IV) would sug
gest that these two diseases might result from a common connective tissue a
nomaly, The underlying mechanism(s) remain unknown. (C) 2000, Masson, Paris
.