Case Report Sudden death in a case of sickle cell anemia: Post-mortem computed tomography and autopsy correlation from a radiologist’s perspective S.N. Abdul Rashid a,b,c,⇑ , A. Krauskopf a , B. Vonlanthen a , M.J. Thali a , T.D. Ruder a a Center of Forensic Imaging, Institute of Forensic Medicine, University of Zurich, Switzerland b Department of Radiology, Faculty of Medicine & Health Sciences, Universiti Putra Malaysia c National Institute of Forensic Medicine, Hospital Kuala Lumpur, Malaysia article info Article history: Received 24 September 2012 Received in revised form 20 January 2013 Accepted 21 January 2013 Available online xxxx Keywords: Sickle cell anemia Acute hemolytic crisis Post-mortem computed tomography Autopsy abstract Sickle cell anemia (SCA) is a hemolytic disease characterized by the production of abnormal hemoglobin chains and distorted red blood cell morphology or sickling. ‘‘Sickle cell crisis’’ includes vaso-occlusive cri- sis, a plastic crisis, sequestration crisis, haemolytic crisis and often culminating in serious complications, organ damage and even sudden death. Post-mortem computed tomography (PMCT) findings of sickle cell disease have never been reported in literature. This case of sudden death from acute hemolytic crisis in SCA where post-mortem computed tomography (PMCT) and autopsy findings complemented each other, both revealing findings invisible to the other and both crucial to the case. Ó 2013 Elsevier Ireland Ltd. All rights reserved. 1. Introduction Sickle cell anemia (SCA) is a hemolytic disease characterized by the production of abnormal hemoglobin chains that tend to poly- merize when deoxygenated, causing distortion of the red blood cell (RBC) morphology (sickling) leading to vascular occlusion and ischemia with multi-organs involvement [1]. The term ‘‘sickle cell crisis’’ is used to describe several acute conditions occurring in pa- tients with sickle cell disease which includes vaso-occlusive crisis, a plastic crisis, sequestration crisis, haemolytic crisis and others. Each acute painful episode is associated with inflammation that worsens with recurrent episodes, often culminating in serious complications and organ damage such as acute chest syndrome, multi-organ failure and sudden death [2]. The most common cause of death for all sickle variants and for all age groups was infection (33–48%). The terminal infection was heralded by upper respiratory tract syndromes in 72.6% and by gastroenteritis in 1.7%. Other causes of death included stroke 9.8%, therapy complications 7.0%, splenic sequestration 6.6%, pul- monary emboli/thrombi 4.9%, renal failure 4.1%, pulmonary hyper- tension 2.9%, hepatic failure 0.8%, massive haemolysis/red cel laplasia 0.4% and left ventricular failure 0.4%. Death was frequently sudden and unexpected (40.8%) or occurred within 24 h after pre- sentation (28.4%), and was usually associated with acute events (63.3%) [3]. The utilization of imaging modalities in the clinical diagnosis of SCA and detection of complications is well reported and estab- lished in literatures [4]. However, post-mortem imaging utilizing computed tomography (CT) or magnetic resonance imaging (MRI) findings of sickle cell disease have never been reported in litera- ture. Autopsy is usually required for the definitive diagnosis of cause of death in suspected sickle cell disease cases. Here we would like to report a case of sudden death from acute hemolytic crisis in SCA where post-mortem computed tomography (PMCT) findings and autopsy findings complemented each other, both revealing findings invisible to the other and both crucial to the case. This report also emphasizes the good collaboration be- tween radiologists and pathologists involved in medico-legal investigation as well as the importance of having radiologists with clinical radiology background in reading radiological images in forensic medicine as an adjunct to conventional autopsy. 2. Case circumstances A 39 years-old, Italian male with underlying SCA presented to the emergency department with acute abdominal pain. He was admitted to the ward for pain management and further investiga- tions. However, he deteriorated clinically and succumbed to his death despite rigorous resuscitation. The body was transported to the Institute of Forensic Medicine (IRM), Zurich, Switzerland. A whole body PMCT was performed using a dual source helical 1344-6223/$ - see front matter Ó 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.legalmed.2013.01.003 ⇑ Corresponding author. Address: Department of Radiology, Faculty of Medicine & Health Sciences, Universiti Putra Malaysia, Serdang 43400, Selangor Darul Ehsan, Malaysia. Tel.: +603 89472511 (Off), +6019 3809380 (h/p); fax: +603 89426957. E-mail address: drsnar72@gmail.com (S.N. Abdul Rashid). Legal Medicine xxx (2013) xxx–xxx Contents lists available at SciVerse ScienceDirect Legal Medicine journal homepage: www.elsevier.com/locate/legalmed Please cite this article in press as: Abdul Rashid SN et al. Sudden death in a case of sickle cell anemia: Post-mortem computed tomography and autopsy correlation from a radiologist’s perspective. Leg Med (2013), http://dx.doi.org/10.1016/j.legalmed.2013.01.003