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Paediatric and adult vascular intracranial complications of sickle-cell disease

  • Clinical Article
  • Published:
Acta Neurochirurgica Aims and scope

Abstract

Background

To enumerate possible intracranial vascular sequelae of sickle-cell disease, to identify risk factors and outline management strategies.

Method

Retrospective review of a single unit experience managing vascular intracranial complications of sickle-cell disease from 1995 until 2005. Information such as homozygosity/heterozygosity, duration of disease, disease control as indicated by haematology follow-up, concurrent sickle-cell disease (SCD)-related health problems and neurosurgical management was recorded. The pattern of vascular disease was analysed to reveal possible contributory/risk factors towards development of vascular intracranial complications.

Subjects

All patients presenting with vascular intracranial complications of sickle-cell disease from 1995 to 2005 were evaluated.

Outcome measures

Classification of vascular intracranial complications into one or more of the following categories: aneurysmal subarachnoid haemorrhage, non-aneurysmal subarachnoid/intraventricular haemorrhage and vasculitis.

Findings

There were ten patients in the study. All symptomatic vascular intracranial complications of SCD requiring neurosurgical intervention were homozygous for SCD. Aneurysms were likely to be multiple. Ruptured aneursyms in SCD were small (average 4 mm). There was a propensity for aneurysms to occur in the posterior circulation, in particular the posterior cerebral artery was frequently involved. Patients with aneurysms and Moyamoya-type vasculitis were likely to have occlusive disease of the internal carotid arteries.

Conclusions

The vascular intracranial complications of sickle-cell disease have an aggressive natural history. Tight control of SCD may reduce the possibility of complications. Complications that arise should be managed in the context of the disease entity rather than in isolation. Consideration should be given to bypass procedures, parent vessel ligations and revascularization techniques. Transcranial Doppler may be used to identify SCD patients with cerebrovascular occlusive disease, who may have increased risk of aneurysmal rupture.

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Conflicts of interests

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Authors and Affiliations

  1. Department of Neurosurgery, Southern General Hospital, Glasgow, UK

    Imran Liaquat

  2. Department of Neurosurgery, Royal Free Hospital, London, UK

    Mary Murphy

  3. Department of Neurosurgery, Kings College Hospital, London, UK

    Sanjeev Bassi & Peter R. Bullock

  4. Flat 3/3, 339, Glasgow Harbour Terrace, Glasgow, G11 6BH, UK

    Imran Liaquat

Authors
  1. Imran Liaquat
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  2. Mary Murphy
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  3. Sanjeev Bassi
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  4. Peter R. Bullock
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Corresponding author

Correspondence to Imran Liaquat.

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Comments

Naturally, we only very rarely see patients with SCD in general in Finland where the prevalence of aneurysms in is the same as elsewhere but the incidence of SAH is twice higher together with Japan for unknown probably genetic reasons [1]. SCD patients are known to have intracranial vascular complications, in the present study in 2.7%. SCD patients with SAH were as the authors note, younger with smaller aneurysms that were more often multiple and in the posterior circulation than in the normal SAH population. An adjunct to the mechanisms postulated for the development of aneurysms in SCD patients, one could also add the role of inflammation [2, 3] after the arterial walls have been weakened by endothelial damage and increased hemodynamic stress. To screen or follow-up homozygous SCD patients’ cerebral vessels, preferably MRA could be performed and when needed CTA or DSA to keep the adverse effects of radiation after repeated scans minimal.

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2. Tulamo R, Frösen J, Junnikkala S, Paetau A, Kangasniemi M, Niemelä M, Jääskeläinen J, Jokitalo E, Karatas A, Hernesniemi J, Meri S. Complement activation associates with saccular aneurysm wall degeneration and rupture. Neurosurgery 59(5):1069-1076, 2006

3. Tulamo R, Frösen J, Junnikkala S, Paetau A, Kangasniemi M, Pelaez J, Hernesniemi J, Niemelä M, Meri S. Complement system becomes activated by the classical pathway in intracranial aneurysm walls. Lab Invest 90(2):168-179, 2010

Mika Niemelä

Juha Hernesniemi

Helsinki, Finland

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Liaquat, I., Murphy, M., Bassi, S. et al. Paediatric and adult vascular intracranial complications of sickle-cell disease. Acta Neurochir 152, 1175–1179 (2010). https://doi.org/10.1007/s00701-010-0628-3

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  • DOI: https://doi.org/10.1007/s00701-010-0628-3

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