Review
Aneurysmal subarachnoid haemorrhage in pregnancy

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Abstract

Although uncommon, aneurysmal subarachnoid haemorrhage (SAH) in pregnancy can be devastating for both mother and baby. It is the leading cause of other indirect maternal death in England and Wales accounting for 60 deaths in the decade 1988–1999. No single obstetric or neurosurgical unit has sufficiently large database or experience in managing this condition in pregnancy. With significant improvements in antenatal care and management of deliveries, non-obstetric causes of maternal death such as aneurysmal subarachnoid haemorrhage are likely to become increasingly significant. The clinical features of aneurysmal subarachnoid haemorrhage closely resemble those of other commoner conditions seen in pregnancy. It is therefore imperative that awareness by obstetricians and other frontline staff is increased so that a high index of suspicion is maintained when pregnant women present with unique headaches. Prompt neurosurgical referral is vital and early involvement of an experienced neuroradiologist essential. It is only when an early diagnosis is made and an aggressive treatment instituted that the bleak case-fatality figure associated with aneurysmal subarachnoid haemorrhage in pregnancy can be improved.

This review, by a multidisciplinary and multicenter team, provides a comprehensive update on the epidemiology, aetiology, clinical presentation, diagnosis and the complexities of the multidisciplinary management of this serious and potentially fatal condition when it occurs in pregnancy.

Introduction

Although aneurysmal subarachnoid haemorrhage (SAH) is an uncommon event in pregnancy, its consequences can be devastating for both mother and baby as it is associated with significant mortality and serious maternal morbidity [1]. In the UK, aneurysmal SAH is the leading cause of other indirect maternal death [2]. During the period from 1988 to 1999, there were 60 maternal deaths from aneurysmal SAH in England and Wales [2], [3], [4], [5]. In 1990, Dias and Sekhar [1] found that of 154 pregnant patients with a verified intracranial haemorrhage, 77% were caused by a ruptured intracranial aneurysm.

In general, despite considerable advances in the diagnosis and treatment of SAH, the case fatality rate continues to be grave [6], [7]. Furthermore, SAH continues to be associated with a high incidence of misdiagnosis [8]. While about 12% of patients die before receiving medical attention [9], 40% of those hospitalised die within 1 month of the event, and of those who survive, more than one-third develop major neurologic deficits [10].

Since it is uncommon, not many obstetric and neurosurgical units have significant experience in the management of pregnancy-related SAH. Indeed, the literature on the subject is sparse, and consists mostly of small case series and isolated case reports [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21]. Nevertheless, as we continue to make significant improvements in antenatal care and management of deliveries, non-obstetric causes of maternal death such as aneurysmal SAH are likely to become increasingly significant causes of maternal mortality and morbidity. Thus, it becomes imperative that obstetricians and other frontline staff have a high index of suspicion so that the bleak case-fatality figures of aneurysmal SAH in pregnancy might be potentially ameliorated, through early diagnosis and timely intervention.

This article, by a multidisciplinary and multicenter team, aims to provide an up to date review of the aetiology, clinical features, diagnosis and the intricacies of the multidisciplinary management of aneurysmal SAH in pregnancy.

Section snippets

Methodology

A MEDLINE search via PubMed from 1 January 1966 to July 2003 using the search terms ‘pregnancy’, ‘subarachnoid haemorrhage’, ‘aneurysm’, non-traumatic/atraumatic, ‘endovascular embolisation’, and ‘anaesthesia’ was performed.

Only articles in the English language were considered. All published case reports and case series on pregnancy related SAH, review articles on SAH as well as cerebral haemorrhages in pregnancy along with other articles that illuminate the subject in general were considered

Epidemiology

In general, there is a wide variation in the incidence of SAH reported in different countries. For example, it is high in the United States, Finland and Japan [6] but low in New Zealand and the Middle East. Typically, the incidence varies with age and race, being more common in African Americans than in white Americans [22]. A review of 13 epidemiological studies has suggested an approximate incidence of 10 per 100,000 population (with a range of 6.5–26.4 per 100,000). SAH is consistently more

Aetiology

Whilst the most frequent cause of SAH is a ruptured cerebral aneurysm (and the only cause discussed in this review) it is important to note that numerous other causes abound, as listed in Table 1. The underlying pathology in intracranial aneurysmal formation and rupture is a weakness of the vessel wall. It has been noted that aneurysms arising from intracranial arteries are considerably more common than those arising from extracranial arteries of similar size. This fact probably relates to the

Clinical features

The clinical features of an aneurysmal SAH in pregnancy do not differ from those in the general population. The initial haemorrhage may be fatal, result in devastating neurological outcomes, or may produce relatively minor symptoms.

Re-bleeding

The primary goal in the management of aneurysmal SAH is the prevention of re-bleeding which is the most fatal complication with a mortality rate in the region of 50–70% [63], [64]. The risk of re-bleeding is 4% over the first 24 h following aneurysmal haemorrhage and rises to 10–20% in the first month [65], [66], and, mortality is progressively increased with each successive re-rupture. Re-bleeding occurs in 33–50% of untreated ruptured intracranial aneurysms within 4–6 weeks [64], [65], [66],

Diagnosis

It is important that an accurate early diagnosis of an SAH is made as early definitive treatment reduces short-term complication and improves clinical outcome. However, it is occasionally difficult to distinguish SAH from either severe pre-eclampsia, or from eclampsia [14]. There may be transient hypertension following aneurysm rupture due to raised intracranial pressure or an increase in catecholamine release. Proteinuria may be detected in up to 30% of cases. The manifestations of

Unruptured aneurysm

The management of unruptured cerebral aneurysms is currently marred by controversy. The results of the International Study of Unruptured Aneurysms (ISUA) [29] suggested that all aneurysms smaller than 10 mm in size in patients without a prior SAH ought to be managed conservatively, since the risk of rupture was estimated at only 0.05% per year. However, various biases (in particular, selection biases) undermined this study, questioning its general applicability [90]. Notwithstanding, the more

Conclusion

A reduction in the high maternal mortality and morbidity associated with aneurysmal SAH can only be achieved through early diagnosis and timely neurosurgical intervention. It is therefore imperative that frontline obstetric staffs maintain a high index of suspicion when a pregnant woman presents with an unusual headache so that prompt neurosurgical consultation can be made. This will reduce the incidence of misdiagnosis with its associated severe consequences.

Key points

  • Ruptured SAH was responsible for 60 maternal deaths in England and Wales between 1988 and 1999.

  • Predominant presenting symptom is a uniquely severe headache.

  • Urgent neurovascular consultation is vital once suspected.

  • The principles of management are similar to those in a non-pregnant woman.

  • Endovascular embolisation is a useful alternative to clipping.

  • There is no contraindication to vaginal delivery following successful treatment remote from term.

References (108)

  • R.M. El Gawly

    Ruptured intracranial aneurysm in pregnancy: a case report and review of the literature

    Eur J Obstet Gynaecol

    (1992)
  • N.W.C. Dorsch et al.

    A Review of cerebral vasospasm in aneurysmal subarachnoid haemorrhage

    J Clin Neurosci

    (1994)
  • D.O. Wiebers

    Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment

    Lancet

    (2003)
  • P.M. White et al.

    Unruptured intracranial aneurysms: prospective data have arrived. Commentary

    Lancet

    (2003)
  • A. Molyneux

    International Subarachnoid Aneurysm Trial (ISAT) of neurosurgical clipping versus endovascular coiling in 2143 patients with ruptured intracranial aneurysms: a randomised trial

    Lancet

    (2002)
  • D.A. Nichols et al.

    Coils or clips in subarachnoid haemorrhage?

    Lancet

    (2002)
  • M.S. Dias et al.

    Intracranial haemorrhage from aneurysms and arteriovenous malformations during pregnancy and the puerperium

    Neurosurgery

    (1990)
  • De Swiet M. Other indirect deaths. In: Gwyneth L, editor. Why mothers die. Report on confidential enquiries into...
  • Hibbard BM, editor. Report on confidential enquiries into maternal deaths in the United Kingdom 1988–1990. London:...
  • Hibbard BM, editor. Report on confidential enquiries into maternal deaths in the United Kingdom 1991–1993. London:...
  • Gwyneth L, editor. Why mothers die. Report on confidential enquiries into maternal deaths in the United Kingdom...
  • T. Inagawa et al.

    Study of aneurysmal subarachnoid haemorrhage in Izumo City, Japan

    Stroke

    (1995)
  • W.I. Schievink et al.

    The poor prognosis of ruptured intracranial aneurysms of the posterior circulation

    J Neurosurg

    (1995)
  • J.A. Edlow et al.

    Avoiding pitfalls in the diagnosis of subarachnoid haemorrhage

    N Engl J Med

    (2000)
  • W.I. Schievink et al.

    Sudden death from aneurysmal subarachnoid haemorrhage

    Neurology

    (1995)
  • W.T. Longstreth et al.

    Clinical course of spontaneous subarachnoid haemorrhage: a population-based study in King County, Washington

    Neurology

    (1993)
  • O. Kizilkilic et al.

    Endovascular treatment of ruptured intracranial aneurysms during pregnancy: report of three cases

    Arch Gynecol Obstet

    (2003)
  • F.M.S. Basama et al.

    Subarachnoid haemorrhage from ruptured aneurysm during pregnancy

    J Obstet Gynaecol

    (2000)
  • V.A. Catanzarite et al.

    Management of pregnancy subsequent to rupture of an intracranial aneurysm

    Am J Perinatol

    (1984)
  • S.L. Giannotta et al.

    Ruptured intracranial aneurysms during pregnancy: a report of four cases

    J Reprod Med

    (1986)
  • T.E. Gill et al.

    Anaesthetic management of cerebral aneurysm clipping during pregnancy: a case report

    AANA J.

    (1993)
  • P. Hubert

    Acute subdural haematoma of the convexity caused by rupture of an aneurysm in the anterior communicating artery. A propos of a case in a pregnant woman

    Neurochirurgie

    (1994)
  • P.M. Meyers et al.

    Endovascular treatment of cerebral artery aneurysms during pregnancy: report of three cases

    Am J Neuroradiol

    (2000)
  • C. Georgantopoulou et al.

    Intracranial aneurysm in pregnancy presenting as hyperemesis gravidarum

    J Obstet Gynecol

    (2003)
  • K.J. Nedd et al.

    Subarachnoid haemorrhage during pregnancy and puerperium: report of 3 cases and review of the literature

    J Am Osteopath Assoc.

    (1986)
  • J.P. Broderick et al.

    The risk of subarachnoid haemorrhage and intracerebral haemorrhages in blacks as compared with whites

    N Engl J Med

    (1992)
  • J.L. Mas et al.

    Stroke in pregnancy and the puerperium

    J Neurol

    (1998)
  • H.J. Miller et al.

    Berry aneurysms in pregnancy: a 10-year report

    S Med J

    (1970)
  • J.M. Barrett et al.

    Pregnancy-related rupture of arterial aneurysms

    Obstet Gynecol Surv

    (1982)
  • G.A. Siwolke et al.

    Cerebrovascular accidents complicating pregnancy and the puerperium

    Obstet Gynecol

    (1991)
  • Yaşargil MG. Clinical considerations, surgery of the intracranial aneurysms and results. Stuttgart: Georg Thieme...
  • D.O. Wiebers

    Unruptured intracranial aneurysms: risk of rupture and risks of surgical intervention. The international study of unruptured intracranial aneurysms investigators

    N Engl J Med

    (1998)
  • G. Austin et al.

    The significance of the extracellular matrix in intracranial aneurysms

    Ann Clin Lab Sci

    (1993)
  • M.C. Wong et al.

    Cerebrovascular disease and stroke in women

    Cardiology

    (1990)
  • S.J. Kittner et al.

    Pregnancy and the risk of stroke

    N Engl J Med

    (1996)
  • S.M. de la Monty et al.

    Risk factors for the development and rupture of intracranial berry aneurysms

    Am J Med

    (1985)
  • B.K. Weir et al.

    Rapid growth of residual aneurysmal neck during pregnancy: a case report

    J Neurosurg

    (1991)
  • W.I. Schievink et al.

    Neurovascular manifestations of heritable connective tissue disorders: a review

    Stroke

    (1994)
  • D. Gaist et al.

    Risk of subarachnoid haemorrhage in first-degree relatives of patients with subarachnoid haemorrhage: follow up study based on national registries in Denmark

    BMJ

    (2000)
  • D.B. Petitti et al.

    Use of oral contraceptives, cigarette smoking, and risk of subarachnoid haemorrhage

    Lancet

    (1978)
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