Greater Rupture Risk for Familial as Compared to Sporadic Unruptured Intracranial Aneurysms

Broderick, Joseph P.; Brown, Robert D.; Sauerbeck, Laura; Hornung, Richard; Huston, John; Woo, Daniel; Anderson, Craig S.; Rouleau, Guy; Kleindorfer, Dawn; Flaherty, Matthew L.; Meissner, Irene; Foroud, Tatiana; Charles, E. Leonard; Moomaw, J.; Connolly, E. Sander

doi:10.1161/strokeaha.108.542571

Cited by 187 publications

(111 citation statements)

References 11 publications

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“…In the Swedish Inpatient Registry Study, the risk of SAH in individuals with one or two or more affected relatives was 2.15 and 51 times higher, respectively, than that of individuals with no affected relatives (30). In the Familial Intracranial Aneurysm Study, the yearly risk of rupture of patients with at least two siblings or three relatives with known history of IA or SAH who were also smokers or had hypertension was 1.2%, approximately 17 times higher than the rupture rate for subjects with an UIA in the ISUIA with a matched distribution of IA size and location (31). In the study presented here, 50% of the patients had a family history of IA or SAH, a high frequency because a strong family history of IA or SAH is an indication for presymptomatic screening at our center.…”

Section: Discussionmentioning

confidence: 90%

Extended Follow-Up of Unruptured Intracranial Aneurysms Detected by Presymptomatic Screening in Patients with Autosomal Dominant Polycystic Kidney Disease

Irazabal

Huston²,

Kubly

et al. 2011

Clinical Journal of the American Society of Nephrology

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117

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SummaryBackground and objectives Autosomal dominant polycystic kidney disease (ADPKD) patients have an increased risk for intracranial aneurysms (IAs). The importance of screening for unruptured IAs (UIAs) depends on their risks for growth and rupture.Design, setting, participants, & measurements ADPKD patients with UIAs found by presymptomatic screening with magnetic resonance angiography (MRA) during 1989 to 2009 were followed initially at 6 months and annually, and less frequently after demonstration of stability.Results Forty-five saccular aneurysms were detected in 38 patients from 36 families. Most were small (median diameter 3.5 mm) and in the anterior circulation (84%). Median age at diagnosis was 49 years. During cumulative imaging follow-up of 243 years, one de novo UIA was detected and increased in size from 2 to 4.4 mm over 144 months and two UIAs grew from 4.5 to 5.9 mm and 4.7 to 6.2 mm after 69 and 184 months, respectively. Seven patients did not have imaging follow-up. No change was detected in the remaining 28 patients. During cumulative clinical follow-up of 316 years, no aneurysm ruptured. Five patients died from unrelated causes and two were lost to follow-up after 8 and 120 months. Three patients underwent surgical clipping. ConclusionsMost UIAs detected by presymptomatic screening in ADPKD patients are small and in the anterior circulation. Growth and rupture risks are not higher than those of UIAs in the general population. These data support very selective screening for UIAs in ADPKD patients, and widespread screening is not indicated.

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Section: Discussionmentioning

confidence: 90%

Extended Follow-Up of Unruptured Intracranial Aneurysms Detected by Presymptomatic Screening in Patients with Autosomal Dominant Polycystic Kidney Disease

Irazabal

Huston²,

Kubly

et al. 2011

Clinical Journal of the American Society of Nephrology

Self Cite

117

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“…1 There is some evidence that familial intracranial aneurysms have a greater rupture risk compared with sporadic aneurysms. 3 In a Markov model based on observational data from screening 626 first-degree relatives of a consecutive series of patients with aSAH between 1995 and 1997, we found that a single magnetic resonance angiographic (MRA) screening for intracranial aneurysms increased life expectancy slightly. However, this increase was at the cost of a reduced number of life years in good health because of complications of preventive treatment and thus screening was not effective.…”

mentioning

confidence: 92%

Long-term risk of aneurysmal subarachnoid hemorrhage after a negative aneurysm screen

Rasing

Ruigrok

Greebe³

et al. 2015

Neurology

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Objective: The objective was to assess the risk of aneurysmal subarachnoid hemorrhage (aSAH) in the initial 15 years after negative aneurysm screening in persons with one first-degree relative with aSAH.Methods: From a cohort of first-degree relatives of patients with aSAH who underwent screening between 1995 and 1997 (n 5 626), we included those with a negative screening (n 5 601). We retrieved all causes of death and sent a questionnaire to screenees who were still alive. If aSAH was reported, we reviewed all medical data. We assessed the incidence of aSAH in this cohort with survival analysis and calculated an incidence ratio by dividing the observed incidence with the age-and sex-adjusted incidence in the general population.Results: Of the 601 screenees, 3 had aSAH during 8,938 follow-up patient-years (mean 14.9 years). After 15 years, the cumulative incidence was 0.50% (95% confidence interval: 0.00%-1.06%) with an incidence rate of 33.6 per 100,000 person-years; the incidence rate ratio was 1.7 (95% confidence interval: 0.3-5.7).Conclusions: In the first 15 years after a negative screening, the risk of aSAH in persons with one first-degree relative with aSAH is not nil, but in the range of that in the general population, or even higher. Whether this finding justifies serial aneurysm screening in this population requires further study. A familial preponderance is a strong risk factor for the development of intracranial aneurysms. Persons with 2 or more affected first-degree relatives have a 50 times higher risk of having an aneurysmal subarachnoid hemorrhage (aSAH) during life, 1 and in such relatives, repeated screening is cost-effective.2 Persons with only one affected first-degree relative have a 2-timeshigher risk of aSAH compared with the general population.1 There is some evidence that familial intracranial aneurysms have a greater rupture risk compared with sporadic aneurysms.3 In a Markov model based on observational data from screening 626 first-degree relatives of a consecutive series of patients with aSAH between 1995 and 1997, we found that a single magnetic resonance angiographic (MRA) screening for intracranial aneurysms increased life expectancy slightly. However, this increase was at the cost of a reduced number of life years in good health because of complications of preventive treatment and thus screening was not effective. On average, screening and preventive treatment increased estimated life expectancy by 0.9 months per person screened, at the expense of 19 years of impaired functional health per person. 4 Also, screening was not considered to be efficient because 149 relatives would need to be screened to prevent a subarachnoid hemorrhage in one person on a lifetime basis.However, in the model, it was assumed that persons with a negative screen were no longer at risk of aSAH, but empirical data supporting this assumption are lacking.

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“…The Familial Unruptured Aneurysm Study 15 showed that, of 313 subjects with unruptured aneurysms and a family history of intracranial aneurysms, 2 subjects with 3-mm and 4-mm AC aneurysms bled at 17 and 16 months after enrollment, with an overall 1.2%/yr rupture rate, 17-fold greater than reported in ISUIA (0.069%/yr with a matched distribution of intracranial aneurysms size and location). This analysis even excluded 2 patients with known unruptured intracranial aneurysms who bled before enrollment (one patient with a 4-mm AC aneurysm and the other with 8-mm MC artery aneurysm and 5 mm posterior communicating artery aneurysms), as well as 1 patient who was enrolled and bled before the MR angiography was completed, who was shown to have a 4-mm MC aneurysm on angiography.…”

Section: S98 Strokementioning

confidence: 93%

Controversy: Clipping of Asymptomatic Intracranial Aneurysm That is <7 mm

Steinberg

2013

Stroke

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Greater Rupture Risk for Familial as Compared to Sporadic Unruptured Intracranial Aneurysms

Cited by 187 publications

References 11 publications

Extended Follow-Up of Unruptured Intracranial Aneurysms Detected by Presymptomatic Screening in Patients with Autosomal Dominant Polycystic Kidney Disease

Extended Follow-Up of Unruptured Intracranial Aneurysms Detected by Presymptomatic Screening in Patients with Autosomal Dominant Polycystic Kidney Disease

Long-term risk of aneurysmal subarachnoid hemorrhage after a negative aneurysm screen

Controversy: Clipping of Asymptomatic Intracranial Aneurysm That is <7 mm

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