Journal of the Accident and Medical Practitioners Association (JAMPA) 2004; Vol. 1 (No. 1) Accident and Medical Practitioners Association, New Zealand ___________________________________________________________________________ Intracranial Bleeding/Subarachnoid Haemorrhage Secondary to a Ruptured Aneurysm: Recognition, Investigation and Management Elaine Jeffrey, MBChB Locum MOSS/GP About the author Dr Elaine Jeffrey, MBChB, works as a locum Medical Office Special Scale (MOSS)/GP throughout New Zealand. Her principal areas of interest are emergency department work, general medicine and paediatrics. Address for correspondence: Dr Elaine Jeffrey, MBChB, P.O. Box 678 Rangiora, New Zealand. E-mail: pjem@xtra.co.nz Issues this article will address Aetiology and presenting symptoms of subarachnoid haemorrhage The concept of the ‘warning leak’ Current guidelines for investigation and management of severe headache Familial links and screening in subarachnoid haemorrhage 2 Salient Points ___________________________________________________________________________ Aetiology of Subarachnoid Haemorrage Many patients present to general practitioners or emergency departments with Warning Leak headaches of Headaches varying severity and a broad spectrum of associated symptoms. Investigation and Management of Headaches The annual incidence of subarachnoid haemorrhage (SAH) is 6 to 16 per 100,000 population, being attributable to ruptured aneurysms. Familial Links15% in Subarachnoid Haemorrhage The spectrum ofmembers symptoms SAH iswith vast,subarachnoid ranging fromhaemorrhage. new/unusual headaches to Screening family ofof patients loss of consciousness. ___________________________________________________________________________ ‘Warning leak’ (prodromal) symptoms occur in 20-40% of SAH cases. Improved recognition of these warning symptoms would improve the outcome for patients. All patients presenting with severe headaches require an accurate history and full examination. If the diagnosis is unclear, computed tomography (CT) should be undertaken promptly, particularly if abnormal neurology is present. Delays in diagnosis of SAH and referral of patients to neurosurgical centres lead to poorer overall outcomes. Familial links in SAH exist. First-degree relatives of patients with SAH are 3 to 7 times more likely to have the condition. Screening of first-degree relatives of SAH patients has been recommended. Family members should be advised to seek medical advice if they develop new-onset headaches. Key words: Headache · Subarachnoid haemorrhage · Warning leak headaches · Thunderclap headaches 3 Introduction The ‘thunderclap’ headache is something all clinicians have been taught to be wary of in case the patient is experiencing a subarachnoid haemorrhage (SAH). Although secondary intracranial haemorrhage (due to vascular malformations or aneurysms) is rare when considered alongside the overall incidence of intracranial bleeding, it is, tragically, the most common cause in the younger age group. However, such patients do not always present in ‘typical text book’ fashion. Illustrative Case Histories The following two cases histories involve young males, both of whom suffered SAH but had vastly different outcomes. CASE DESCRIPTIONS The first patient, a 53-year-old male, woke from sleep with a sudden severe, frontal headache, vomiting, photophobia and feeling disoriented. A known migraine sufferer, he had noticed more frequent headaches recently but none this severe. There was a history of hypertension and hypercholesterolaemia for which he was taking celiprolol and simvastatin, respectively. He was a non-smoker. The only positive finding on initial examination was neck pain but no stiffness. Morphine and antiemetics had little effect, so the next morning transfer was arranged for computed tomography (CT). This revealed a SAH involving the 4th ventricle and right occipital horn. He underwent clipping of a right middle cerebral artery aneurysm and progressed well with no neurological deficit. The second patient was 46 years of age. He suddenly collapsed while at a party. His eyes rolled back and he seemed not to be breathing, so mouth-to-mouth resuscitation was performed briefly. No seizure activity was noted. He had had a “few rums and some red wine” but no other drugs. He had no past medical history but smoked 30g tobacco per week. 4 On examination, he was restless, agitated, vomiting, had a Glasgow Coma Score (GCS) of 9/15, heart rate 60 beats/min, BP 140/80 mm Hg, HS dual, abdomen soft, respiratory rate 20/min with deep sighing respirations and widespread crackles throughout his chest. His right side was not moving and had decreased tone and reflexes. His pupils, though equal, were sluggish. After intubation, he was transferred for CT which confirmed extensive left frontal haemorrhage with intraventricular blood, moderate hydrocephalus, and involvement of the subarachnoid space, basal cisterns and sylvian fissures. An aneurysmal site was noted in the left anterior communicating artery. Unfortunately, he never regained consciousness and died later that night. Incidence, Aetiology and Presenting Symptoms of Subarachnoid Haemorrhage The annual incidence of SAH is 6 to16 per 100,000 population, 15% being attributable to ruptured aneurysms and young adults accounting for around 18% of cases.[1-5] It is almost twice as common in females. Hypertension increases the risk and is associated with the presence of multiple aneurysms. Smoking also significantly increases the risk. The prevalence of SAH is greater in patients with polycystic kidney disease, and in those with a family history of SAH and atherosclerosis.[3,4,6,7] Headaches account for 1 to 2% of all emergency department presentations and up to 4% of all general practice presentations. The spectrum of symptoms of SAH is vast, ranging from little more than a new/unusual headache to loss of consciousness. Those presenting to emergency departments tend to have more severe symptoms. All patients presenting with new/unusual headaches should undergo careful evaluation for more serious causes of headache.[8,9] The concept of ‘warning leak’ symptoms is well recognised. A prospective study by Jakobsson et al.[10] found that 19.9% of patients with SAH had symptoms suggesting a ‘warning leak’ but 40.5% did not have their symptoms recognised as such. The authors suggested that improved recognition of ‘warning leaks’ would improve outcome.[10] In other studies, Fridriksson et al.[11] noted that 40% of patients had prodromal symptoms with 12% 5 not being recognised initially, while Tolias et al.[5] found that 20% of patients had ‘warning leak’ symptoms when seen by a general practitioner but were not referred for investigation at the time. On the basis of these findings, it was suggested that closely linked teaching between GPs and neurosurgeons may improve recognition of warning symptoms,[11] and that the threshold for investigating these patients needs to be lowered.[5] In a review of the pitfalls that can be encountered when diagnosing SAH, Edlow et al.[8] concluded that most warning headaches signal SAH, and not recognising this leads to diagnostic delays and a worsening of the clinical state when it is finally diagnosed. However, Linn et al.[12,13] consider that the concept of ‘warning leaks/headaches’ is not as significant as others have suggested, and that the clinical and radiological features of SAH are the same regardless of preceding headaches. Current Guidelines for Investigation and Management of Severe Headache Patients presenting with severe headache require an accurate history and full examination, including head/eyes/ears/nose/throat (HEENT) and neurology signs. If no clear diagnosis is evident, they should then undergo CT and/or lumbar puncture for cerebrospinal fluid (CSF) analysis.[14] In a study that compared the efficiency of CT and CSF analysis for excluding SAH in patients with ‘worst headaches’, CT-confirmed SAH was found in 17% of patients but among those with a negative imaging result, CSF analysis detected SAH in only 2.5% of cases.[15] It was concluded, therefore, that CT is sufficient to exclude SAH in 97.5% of patients presenting to emergency departments with ‘worst headaches’.[14,15] However, a clinical policy statement by the American College of Emergency Physicians recommends CSF analysis in the presence of a normal CT but ongoing high clinical suspicion of SAH. They suggest that the response to analgesia does not provide a guide to the aetiology of severe headache, and recommend that all patients with headache and any abnormal neurology proceed to CT. In particular, all patients over 50 years of age with new-onset headache require urgent CT. Those with headache but normal neurology should also be considered for CT. The guidelines further recommend that all patients with normal CT, normal opening pressure on lumbar puncture, and normal CSF findings can be discharged from the emergency department to GP follow-up. In the absence of abnormal neurology, a lumbar puncture can be performed without prior CT, but it should be noted that lumbar puncture alone is not sufficient to assess all causes of sudden severe headache.[16] 6 The Need to Avoid Delays in Diagnosis of SAH In a study of the timing of presentation to hospital in relation to outcome, the severity of the clinical condition was found to be worse in those presenting within 6 hours of onset of their symptoms, and this group of patients had a poorer outcome overall.[17] Neil-Dwyer et al.[18] found that 51% of patients who had been diagnosed with SAH experienced diagnostic delays in either the prehospital or in-hospital settings, and these 65% of these patients had further bleeding before finally being diagnosed. These delays contributed to poorer overall outcomes.[18] Importance of Rapid Referral for Treatment Avoiding delays in referral to neurosurgical centres is important with respect to treatment and outcome. In recent years, the outcome for patients with aneurysmal SAH has improved, especially for those with less severe symptoms, and this improvement has been attributed to better referral guidelines and surgical management.[19] More recently, there has been a move towards medical management of SAH, but at present, studies have shown that this contributes to considerable worsening of morbidity and mortality.[20,21] Familial Links and Screening for Subarachnoid Haemorrhage Although there is a familial link in SAH, screening is a contentious issue. As first-degree relatives of patients with SAH have been found to be 3 to 7 times more likely to have the condition, Bromberg et al.[22] recommend screening of family members. In other studies, an increased risk has been noted where two or more relatives have had SAH, with siblings more at risk than children.[23] A greater risk from a positive maternal versus paternal history has been noted by Okamoto et al.[24] and these authors strongly recommend screening of firstdegree relatives. Others are less supportive of screening, as the overall prevalence of aneurysms is only about 2%, most being small and less likely to rupture.[7] One meta-analysis found that clipping of unruptured aneurysms carries a significant morbidity and mortality risk,[25] while another 7 study found considerable evidence of decreased functioning/disablement following elective clipping of aneurysms and that surgery only increased life expectancy by 2.5 years.[26] Lessons from the Case Histories Case one of the illustrative histories presented above highlights some important issues. This patient is representative of many patients with a past history of migraines seen in either emergency departments or general practice. Often this new headache is treated as a more severe form of the previous headaches. It is important to remain vigilant and look closely at the patient’s history. If the headache or history differs in any way from the ‘usual’ headache for that patient, it is crucial to consider the need for early referral for investigation and management in order to maximise the outcome. The second patient presented immediately after the onset of his symptoms. His clinical status at the time pointed strongly to a poor outcome and it is unlikely anything would have changed this. However, many peripheral hospitals transfer patients to the closest tertiary hospital for CT, regardless of whether a neurosurgical team is available at that hospital or not. This results in a further delay if the CT is positive while the transfer is being made. When time is of the essence, this is certainly not in the best interests of the patient. In such cases, direct transfer to a neurosurgical unit would seem more appropriate. With both these cases, each family had only one relative with SAH. Given the risks and limited benefits of surgery, screening would not be advised. However, all first-degree relatives of these men should be advised to seek medical advice if they develop new-onset headaches. References 1. Bederson JB, Awad IA, Wiebers DO, Piepgras D, Haley EC Jr, Brott T, et al. Recommendations for the management of patients with unruptured intracranial aneurysms. Stroke 2000;31(11):2742-50. 2. International Subarachnoid Aneurysm Trial (ISAT) Collaborative Group. International subarachnoid aneurysm trial (ISAT) of neurological clipping versus endovascular coiling 8 in 2143 patients with ruptured intracranial aneurysms: a randomised trial. Lancet 2002;360:1267-74. 3. Ogungbo B, Gregson B, Blackburn A, Barnes J, Vivar R, Sengupta R, et al. Aneurysmal subarachnoid haemorrhage in young adults. J Neurosurg 2003;98(1):43-9. 4. Isaksen J, Egge A, Waterloo K, Romner B, Ingebrigtsen T. Risk factors for aneurysmal subarachnoid haemorrhage: the Tromso study. J Neurol Neurosurg Psychiatry 2002;73(2):185-7. 5. Tolias CM, Choksey MS. Will increased awareness among physicians of the significance of sudden agonising headache affect the outcome of subarachnoid hemorrhage? Coventry and Warwickshire study: audit of subarachnoid hemorrhage (establishing historical control), hypothesis, campaign layout and cost estimation. Stroke 1996;27(5):807-12. 6. Macdonald RL, Stoodley M, Weir B. Intracranial aneurysms. Neurosurgery Quarterly 2001;11:181-98. 7. Rinkel GJ, Djibuti M, Algra A, van Gijn J. Prevalence and risk of rupture of intracranial aneurysms: a systematic review. Stroke 1998;29:251-6. 8. Edlow JA, Caplan LR. Avoiding pitfalls in the diagnosis of subarachnoid hemorrhage. N Engl J Med 2000;342:29-36. 9. Raskin NH, Peroutka SJ. Clinical evaluation of acute, new-onset headache. Chapter 15. Harrison’s Online. Available from: http://harrisons.accessmedicine.com. 10. Jakobsson KE, Saveland H, Hillman J, Edner G, Zygmunt S, Brandt L, et al. Warning leak and management outcome in aneurysmal subarachnoid hemorrhage. J Neurosurg 1996;85(6):995-9. 11. Fridriksson S, Hillman J, Landtblom AM, Boive J. Education of referring doctors about sudden onset headache in subarachnoid hemorrhage. A prospective study. Acta Neurol Scand 2001;103(4)238-42. 12. Linn FH, Wijdicks EF, van der Graaf Y, Weerdesteyn-van Vliet FA, Bartelds AI, van Gijn J. Prospective study of sentinel headache in aneurysmal subarachnoid haemorrhage. Lancet 1994;344:590-3. 13. Linn FH, Rinkel GJ, Algra A, van Gijn J. The notion of “warning leaks” in subarachnoid haemorrhage: are such patients in fact admitted with a rebleed? J Neurol Neurosurg Psychiatry 2000;68(3):332-6. 14. Edlow JA. Diagnosis of subarachnoid hemorrhage in the emergency department. Emerg Med Clin North Am 2003;1(1):73-87. 9 15. Morgenstern LB, Luna-Gonzales H, Huber JC Jr, Wong SS, Uthman MO, Gurian JH, et al. Worst headache and subarachnoid hemorrhage: prospective, modern computed tomography and spinal fluid analysis. Ann Emerg Med 1998;32(3 Pt 1):297-304. 16. American College of Emergency Physicians. Clinical policy: critical issues in the evaluation and management of patients presenting to the emergency department with acute headache. Ann Emerg Med 2002;39(1)108-22. 17. Inagawa T. Timing of admission and management outcome in patients with subarachnoid hemorrhage. Surg Neurol 1994;41(4):268-76. 18. Neil-Dwyer G, Lang D. “Brain attack” – aneurysmal subarachnoid haemorrhage: death due to delayed diagnosis. J Roy Coll Physicians Lond 1997;31(1):49-52. 19. Cesarini KG, Hardemark HG, Persson L. Improved survival after aneurysmal subarachnoid haemorrhage: review of case management during a 12-year period. J Neurosurg 1999;90(4):664-72. 20. Hanley D, Ulatowski JA. Medical management of aneurysmal subarachnoid hemorrhage. Crit Care Med 1995;23(6):992-3. 21. Ogungbo B, Gregson BA, Blackburn A, Mendelow AD; Newcastle Subarachnoid Study Group. Trends over time in the management of subarachnoid haemorrhage in Newcastle: review of 1609 patients. Br J Neurosurg 2001;15(5):388-95. 22. Bromberg JE, Rinkel GJ, Algra A, Greebe P, van Duyn CM, Hasan D, et al. Subarachnoid haemorrhage in first and second-degree relatives of patients with subarachnoid haemorrhage. BMJ 1995;311:288-9. 23. Raaymakers TW, Rinkel GJ, Ramos LM. Initial and follow up screening for aneurysms in families with familial subarachnoid hemorrhage. Neurology 1998;51(4):1125-30. 24. Okamoto K, Horisawa R, Kawamura T, Asai A, Ogino M, Takagi T, et al. Family history and risk of subarachnoid hemorrhage: a case-controlled study in Nagoya, Japan. Stroke 2003;34(2):422-6. 25. Raaymakers TW, Rinkel GJ, Limburg M, Algra A. Mortality and morbidity of surgery for unruptured intracranial aneurysms – a meta-analysis. Stroke 1998;29(8):1531-8. 26. The 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;341:1344-50.