Screening for intracranial aneurysm
- Robert J Singer, MD
Robert J Singer, MD
- Associate Professor of Neurosurgery
- Neurovascular Therapeutics
- Geisel School of Medicine at Dartmouth
- Christopher S Ogilvy, MD
Christopher S Ogilvy, MD
- Professor of Surgery
- Harvard Medical School
- Guy Rordorf, MD
Guy Rordorf, MD
- Associate Professor of Neurology
- Harvard Medical School
Subarachnoid hemorrhage (SAH) is often a devastating event. Approximately 10 percent of patients die prior to reaching the hospital and, of those who make it in time, only one-third will have a "good result" after treatment .
Most SAHs are caused by ruptured saccular aneurysms. Recommendations for screening for aneurysms and methods of screening are discussed here. The epidemiology and pathogenesis of intracranial aneurysms and management of unruptured aneurysms, and screening for new aneurysms after treatment for SAH are discussed separately. (See "Unruptured intracranial aneurysms" and "Treatment of cerebral aneurysms", section on 'Early rebleeding'.)
The prevalence of intracranial saccular aneurysms by radiographic and autopsy series is approximately 0.4 to 6.0 percent , or between 1 to 18 million people in the United States. In adult patients without risk factors, the best estimate is that approximately 2 percent harbor asymptomatic cerebral aneurysms . Of patients with cerebral aneurysms, 20 to 30 percent have multiple aneurysms . Aneurysmal SAH occurs at an estimated rate of 6 to 16 per 100,000 population . In North America, this translates into approximately 30,000 affected persons per year. Thus, most aneurysms do not rupture.
The probability of rupture is related to the size of the aneurysm. Small aneurysms (less than 6 mm in diameter) are most commonly identified with screening, and these are at low risk for rupture (figure 1) . In addition, patients with smaller aneurysms (<10 mm) that have ruptured have a better prognosis than larger aneurysm rupture .
Aneurysm surgery is associated with significant morbidity and mortality. In an international multicenter report of 1449 patients with unruptured intracranial aneurysms, the rate of surgery-related morbidity and mortality in those without a prior history of bleeding from a different aneurysm was 18 percent at 30 days and 16 percent at one year . The one-year rates were affected by age: 6.5 percent under age 45; 14 percent between the ages of 45 and 64; and 32 percent over the age of 64. (See "Treatment of aneurysmal subarachnoid hemorrhage".)
- Tidswell P, Dias PS, Sagar HJ, et al. Cognitive outcome after aneurysm rupture: relationship to aneurysm site and perioperative complications. Neurology 1995; 45:875.
- Rinkel GJ, Djibuti M, Algra A, van Gijn J. Prevalence and risk of rupture of intracranial aneurysms: a systematic review. Stroke 1998; 29:251.
- Vernooij MW, Ikram MA, Tanghe HL, et al. Incidental findings on brain MRI in the general population. N Engl J Med 2007; 357:1821.
- STEHBENS WE. ANEURYSMS AND ANATOMICAL VARIATION OF CEREBRAL ARTERIES. Arch Pathol 1963; 75:45.
- Unruptured intracranial aneurysms--risk of rupture and risks of surgical intervention. International Study of Unruptured Intracranial Aneurysms Investigators. N Engl J Med 1998; 339:1725.
- Roos EJ, Rinkel GJ, Velthuis BK, Algra A. The relation between aneurysm size and outcome in patients with subarachnoid hemorrhage. Neurology 2000; 54:2334.
- Bederson JB, Awad IA, Wiebers DO, et al. Recommendations for the management of patients with unruptured intracranial aneurysms: A statement for healthcare professionals from the Stroke Council of the American Heart Association. Circulation 2000; 102:2300.
- Bederson JB, Connolly ES Jr, Batjer HH, et al. Guidelines for the management of aneurysmal subarachnoid hemorrhage: a statement for healthcare professionals from a special writing group of the Stroke Council, American Heart Association. Stroke 2009; 40:994.
- Neil-Dwyer G, Bartlett JR, Nicholls AC, et al. Collagen deficiency and ruptured cerebral aneurysms. A clinical and biochemical study. J Neurosurg 1983; 59:16.
- Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med 2000; 342:673.
- Ronkainen A, Hernesniemi J, Puranen M, et al. Familial intracranial aneurysms. Lancet 1997; 349:380.
- Raaymakers TW. Aneurysms in relatives of patients with subarachnoid hemorrhage: frequency and risk factors. MARS Study Group. Magnetic Resonance Angiography in Relatives of patients with Subarachnoid hemorrhage. Neurology 1999; 53:982.
- Bromberg JE, Rinkel GJ, Algra A, et al. Familial subarachnoid hemorrhage: distinctive features and patterns of inheritance. Ann Neurol 1995; 38:929.
- Teasdale GM, Wardlaw JM, White PM, et al. The familial risk of subarachnoid haemorrhage. Brain 2005; 128:1677.
- Linn FH, Rinkel GJ, Algra A, van Gijn J. Incidence of subarachnoid hemorrhage: role of region, year, and rate of computed tomography: a meta-analysis. Stroke 1996; 27:625.
- Rasing I, Nieuwkamp DJ, Algra A, Rinkel GJ. Additional risk of hypertension and smoking for aneurysms in people with a family history of subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry 2012; 83:541.
- St Jean P, Hart B, Webster M, et al. Alpha-1-antitrypsin deficiency in aneurysmal disease. Hum Hered 1996; 46:92.
- Broderick JP, Brown RD Jr, Sauerbeck L, et al. Greater rupture risk for familial as compared to sporadic unruptured intracranial aneurysms. Stroke 2009; 40:1952.
- Woo D, Hornung R, Sauerbeck L, et al. Age at intracranial aneurysm rupture among generations: Familial Intracranial Aneurysm Study. Neurology 2009; 72:695.
- Raaymakers TW, Rinkel GJ, Ramos LM. Initial and follow-up screening for aneurysms in families with familial subarachnoid hemorrhage. Neurology 1998; 51:1125.
- Schievink WI. Intracranial aneurysms. N Engl J Med 1997; 336:28.
- Ronkainen A, Puranen MI, Hernesniemi JA, et al. Intracranial aneurysms: MR angiographic screening in 400 asymptomatic individuals with increased familial risk. Radiology 1995; 195:35.
- Crawley F, Clifton A, Brown MM. Should we screen for familial intracranial aneurysm? Stroke 1999; 30:312.
- Bor AS, Koffijberg H, Wermer MJ, Rinkel GJ. Optimal screening strategy for familial intracranial aneurysms: a cost-effectiveness analysis. Neurology 2010; 74:1671.
- Wermer MJ, Rinkel GJ, van Gijn J. Repeated screening for intracranial aneurysms in familial subarachnoid hemorrhage. Stroke 2003; 34:2788.
- Connolly ES Jr, Rabinstein AA, Carhuapoma JR, et al. Guidelines for the management of aneurysmal subarachnoid hemorrhage: a guideline for healthcare professionals from the American Heart Association/american Stroke Association. Stroke 2012; 43:1711.
- Magnetic Resonance Angiography in Relatives of Patients with Subarachnoid Hemorrhage Study Group. Risks and benefits of screening for intracranial aneurysms in first-degree relatives of patients with sporadic subarachnoid hemorrhage. N Engl J Med 1999; 341:1344.
- Raaymakers TW. Functional outcome and quality of life after angiography and operation for unruptured intracranial aneurysms. On behalf of the MARS Study Group. J Neurol Neurosurg Psychiatry 2000; 68:571.
- Fick GM, Gabow PA. Hereditary and acquired cystic disease of the kidney. Kidney Int 1994; 46:951.
- Watson ML. Complications of polycystic kidney disease. Kidney Int 1997; 51:353.
- Chapman AB, Rubinstein D, Hughes R, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. N Engl J Med 1992; 327:916.
- Schievink WI, Torres VE, Piepgras DG, Wiebers DO. Saccular intracranial aneurysms in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1992; 3:88.
- Huston J 3rd, Torres VE, Sulivan PP, et al. Value of magnetic resonance angiography for the detection of intracranial aneurysms in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1993; 3:1871.
- Chauveau D, Pirson Y, Verellen-Dumoulin C, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. Kidney Int 1994; 45:1140.
- Chauveau D, Sirieix ME, Schillinger F, et al. Recurrent rupture of intracranial aneurysms in autosomal dominant polycystic kidney disease. BMJ 1990; 301:966.
- Litchfield WR, Anderson BF, Weiss RJ, et al. Intracranial aneurysm and hemorrhagic stroke in glucocorticoid-remediable aldosteronism. Hypertension 1998; 31:445.
- Schievink WI, Raissi SS, Maya MM, Velebir A. Screening for intracranial aneurysms in patients with bicuspid aortic valve. Neurology 2010; 74:1430.
- Raaymakers TW, Buys PC, Verbeeten B Jr, et al. MR angiography as a screening tool for intracranial aneurysms: feasibility, test characteristics, and interobserver agreement. AJR Am J Roentgenol 1999; 173:1469.
- Wiebers DO, Torres VE. Screening for unruptured intracranial aneurysms in autosomal dominant polycystic kidney disease. N Engl J Med 1992; 327:953.
- Huston J 3rd, Nichols DA, Luetmer PH, et al. Blinded prospective evaluation of sensitivity of MR angiography to known intracranial aneurysms: importance of aneurysm size. AJNR Am J Neuroradiol 1994; 15:1607.
- Li MH, Cheng YS, Li YD, et al. Large-cohort comparison between three-dimensional time-of-flight magnetic resonance and rotational digital subtraction angiographies in intracranial aneurysm detection. Stroke 2009; 40:3127.
- Schwartz RB, Tice HM, Hooten SM, et al. Evaluation of cerebral aneurysms with helical CT: correlation with conventional angiography and MR angiography. Radiology 1994; 192:717.
- van Gelder JM. Computed tomographic angiography for detecting cerebral aneurysms: implications of aneurysm size distribution for the sensitivity, specificity, and likelihood ratios. Neurosurgery 2003; 53:597.
- RELATIVES OF PATIENTS WITH CEREBRAL ANEURYSM
- Two or more affected first-degree relatives
- One affected first-degree relative
- HEREDITARY SYNDROMES ASSOCIATED WITH ANEURYSM FORMATION
- Autosomal dominant polycystic kidney disease
- Other hereditary disorders
- CHOICE OF SCREENING TEST
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS