Images in… Martin D Weaver,1,2 Hayan Dayoub,3 Emily Damuth,4 Tapan Kavi5 1 Department of Internal Medicine, Cooper University Hospital, Camden, New Jersey, USA 2 Department of Physical Medicine and Rehabilitation, University of Miami Miller School of Medicine / Jackson Memorial Hospital, Miami, Florida, USA 3 Department of Neurosurgery, Cooper University Hospital, Camden, New Jersey, USA 4 Department of Critical Care, Cooper University Hospital, Camden, New Jersey, USA 5 Department of Neurology and Neurosurgery, Cooper University Hospital, Camden, New Jersey, USA Correspondence to Dr Martin D Weaver, ​martin.​weaver@​jhsmiami.​org Accepted 28 July 2017 Description A middle-aged African-American male with homozygous sickle cell disease presented with vaso-occlusive crisis and suffered a generalised tonic-clonic seizure while in the emergency department. He had been seizure-free for more than a decade, thus was not taking antiepileptic medications. CT head revealed diffuse cerebral oedema, effacement of the fourth ventricle and obstructive hydrocephalus (figure 1). An external ventricular drain was placed with improvement of hydrocephalus, and a repeat CT head revealed right parietal hypodensity (figure 1). MRI brain and conventional cerebral angiogram showed right parietal cerebral oedema, a large arteriovenous malformation, right internal carotid artery occlusion, moyamoya disease and basilar artery aneurysm (figure 2). The patient’s cerebral oedema and neurological examination initially improved after exchange transfusion; however, he developed acute subarachnoid haemorrhage from basilar artery aneurysm rupture and brainstem strokes causing coma. He underwent palliative extubation after discussion with family and is now deceased. Ischaemic strokes, haemorrhagic strokes and seizures are the most common neurological complications in patients with sickle cell disease.1 Vascular abnormalities such as arteriovenous malformations Figure 2 MRI brain fluid-attenuated inversion recovery (FLAIR) sequence (A) shows right parietal hyperintensity from venous hypertension-induced oedema and large arteriovenous malformation. Four vessel angiogram shows right terminal internal carotid artery occlusion and moyomoya disease distally (B and C) and basilar artery aneurysm (D). and moyomoya pattern have been described before2; however, to our knowledge, this is the first case reporting diffuse cerebral oedema complicating vaso-occlusive crisis. Vaso-occlusive crisis can precipitate venous hypertension, especially in the presence of arteriovenous malformation by obstructing the high-pressured venous system. Venous hypertension can then lead to development of cerebral oedema and hydrocephalus from obstruction of ventricular drainage. Recognising cerebral oedema as a neurological complication in patients with sickle cell disease is important, as seen Learning points ►► Chronic sickle cell disease can lead to To cite: Weaver MD, Dayoub H, Damuth E, et al. BMJ Case Rep Published Online First: [please include Day Month Year]. doi:10.1136/bcr-2017221345 Figure 1 CT head shows effacement of fourth ventricle (A), diffuse cerebral oedema (B), mild obstructive hydrocephalus (C) and right parietal hypodensity (D). intracranial vascular stenosis, moyamoya disease, arteriovenous malformation and aneurysm formation. ►► Venous hypertension and cerebral oedema can be a complication of sickle cell vaso-occlusive sickle cell crisis, especially in the presence of cerebral arteriovenous malformation. Weaver MD, et al. BMJ Case Rep 2017. doi:10.1136/bcr-2017-221345 1 BMJ Case Reports: first published as 10.1136/bcr-2017-221345 on 10 August 2017. Downloaded from http://casereports.bmj.com/ on 12 April 2024 by guest. Protected by copyright. Diffuse cerebral oedema from sickle cell vasoocclusive crisis Images in… Contributors MDW, TK and ED contributed to conception, planning, conduct, design and reporting. HD contributed to planning, conception and design. Competing interests None declared. Patient consent Detail has been removed from this case description/these case descriptions to ensure anonymity. The editors and reviewers have seen the detailed information available and are satisfied that the information backs up the case the authors are making. Provenance and peer review Not commissioned; externally peer reviewed. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted. References 1 Prengler M, Pavlakis SG, Prohovnik I, et al. Sickle cell disease: the neurological complications. Ann Neurol 2002;51:543–52. 2 Fasano RM, Meier ER, Hulbert ML. Cerebral vasculopathy in children with sickle cell anemia. Blood Cells Mol Dis 2015;54:17–25. Copyright 2017 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ►► Submit as many cases as you like ►► Enjoy fast sympathetic peer review and rapid publication of accepted articles ►► Access all the published articles ►► Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact consortiasales@bmjgroup.com Visit casereports.bmj.com for more articles like this and to become a Fellow 2 Weaver MD, et al. BMJ Case Rep 2017. doi:10.1136/bcr-2017-221345 BMJ Case Reports: first published as 10.1136/bcr-2017-221345 on 10 August 2017. Downloaded from http://casereports.bmj.com/ on 12 April 2024 by guest. Protected by copyright. in this case, and should prompt investigation for vascular abnormality.