Abstract
BackgroundSplenic artery aneurysm (SAA) is a rare but potentially fatal condition. Rupture results in 25% mortality up to 75% in pregnant women with 95% fetal mortality. Brief reports suggest an increased risk of developing SAA in patients with HHT.MethodsWe analyzed enhanced multidetector CT data in 186 HHT patients matched (gender and ± 5 year old) with 186 controls. We screened for SAA and recorded diameter of splenic and hepatic arteries and hepatic, pancreatic and splenic parenchymal involvements. We determined by univariate and multivariate analysis, the relationship with age, sex, genetic status, cardiovascular risk factors (CVRF) and visceral involvement.ResultsSAA concerned 24.7% of HHT patients and 5.4% of controls, p<0.001. Factors associated with increased risk of SAA in HHT were female gender (p = 0.04, OR = 2.12, IC 95% = 1.03–4.50), age (p = 0.0003, OR = 1.04, 95% CI = 1.02–1.06) and pancreatic parenchymal involvement (p = 0.04, OR = 2.13, 95% CI = 1.01–4.49), but not type of mutation, hepatic or splenic parenchymal involvements, splenic size or splenic artery diameter or CVRF.ConclusionsWe found a 4.57 higher rate of SAA in HHT patients without evidence of splenic high output related disease or increased CVRF. These results suggest the presence of a vascular intrinsic involvement. It should lead to screening all HHT patients for SAA. The vasculopathy hypothesis could require a change in management as screening of all systemic arteries and even the aorta and to further research in the field.
Highlights
Hereditary hemorrhagic telangiectasia (HHT), known as Rendu-Osler-Weber disease is a rare autosomal dominant genetic disease, with an incidence of 1/8.000 to 1/5.000
Factors associated with increased risk of Splenic artery aneurysm (SAA) in HHT were female gender (p = 0.04, OR = 2.12, IC 95% = 1.03– 4.50), age (p = 0.0003, OR = 1.04, 95% CI = 1.02–1.06) and pancreatic parenchymal involvement (p = 0.04, OR = 2.13, 95% CI = 1.01–4.49), but not type of mutation, hepatic or splenic parenchymal involvements, splenic size or splenic artery diameter or cardiovascular risk factors (CVRF)
We found a 4.57 higher rate of SAA in HHT patients without evidence of splenic high output related disease or increased CVRF
Summary
Hereditary hemorrhagic telangiectasia (HHT), known as Rendu-Osler-Weber disease is a rare autosomal dominant genetic disease, with an incidence of 1/8.000 to 1/5.000. It is characterized by the development of muco-cutaneous or visceral telangiectasias and arteriovenous malformations (AVMs). Visceral manifestations include hepatic (32 to 78% of patients), pulmonary (15–50%), cerebro-spinal (23%), gastrointestinal (80%) and pancreatic (26%) involvements [2,3]. They are responsible for serious complications, especially bleeding and systemic embolism. Brief reports suggest an increased risk of developing SAA in patients with HHT
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