Forensic Science International 244 (2014) e38–e41

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Case Report

Acute aortic dissection caused by Clostridium septicum aortitis Franziska Eplinius *, Carsten Ha¨drich 1 Institute for Legal Medicine, University of Leipzig, Germany Johannisallee 28, 04103 Leipzig, Germany

A R T I C L E I N F O

A B S T R A C T

Article history: Received 17 June 2014 Received in revised form 25 August 2014 Accepted 26 August 2014 Available online 6 September 2014

Clostridium septicum aortitis is a rare cause of aortic dissection. So far, only 28 cases have been described in literature before. Most of these cases occurred in elderly patients and an association to colonic neoplasms and/or atherosclerosis has been witnessed frequently. Here we report the case of a 32-yearold man with fatal aortic dissection due to aortic infection with C. septicum. Beside a case of a 22-year-old man who died of aortic dissection due to C. septicum aortitis this is the second case of C. septicum aortitis in a young individual with no signs of colonic neoplasms or atherosclerosis. ß 2014 Elsevier Ireland Ltd. All rights reserved.

Keywords: Clostridium septicum Aortitis Infectious vasculitis Aortic dissection

1. Introduction Infectious aortitis is an unusual cause of aortic aneurysms or dissections. The best known etiology of infectious aortic diseases is mesaortitis luica, which is caused by Treponema pallidum – however, it is rarely seen nowadays due to decreasing incidence, earlier diagnosis and therapy. The most common bacterial infections causing aortitis these days are Staphylococcus aureus and Salmonella [1]. Throughout literature, have been only 28 cases of infectious aortitis due to Clostridium spp. described earlier [2,3]. This disease is predominantly found in elderly patients (mean age 75 years) and is often associated with colonic malignancies. In younger patients aortic aneurysms or aortic dissections are predominantly caused by hereditary connective tissue diseases such as Marfan’s syndrome. Clostridum septicum aortitis of a young individual is described in only one case (22 years of age [2]). Here we report the case of a 32-year-old man with fatal nonaneurysmatic aortic dissection caused by Clostridium septicum aortitis. 2. Case report In June 2013, a 32-year-old man suffered from sudden chestand back pain and a tingling, numb feeling in his right arm during

* Corresponding author. Tel.: +49 17621908349. E-mail addresses: [email protected] (F. Eplinius), [email protected] (C. Ha¨drich). 1 Tel.: +49 3419715105. http://dx.doi.org/10.1016/j.forsciint.2014.08.032 0379-0738/ß 2014 Elsevier Ireland Ltd. All rights reserved.

physical exercise in a local training studio. An accompanying friend drove him to the emergency room of a nearby hospital. The doctor annotated chest pain and radiating headaches – while auscultating the heart, he further noticed a cardiac murmur. Clinical chemistry showed leucocytosis with 15 Gpt/l, and heart enzymes were in a normal range. The patient received an infusion with painkiller, an ECG was written, and he was discharged under suspicion of muscle tension after sports. The next few days, the symptoms showed no improvement. Six days after his meeting with the medical doctor he was found dead in his apartment by a friend. There were no signs of a traumatic cause of death. The patient had not suffered from chronic diseases, especially hereditary connective tissue diseases; he sometimes had a cold, which he tried to cure with natural medicine. 3. Autopsy findings On external examination the body showed early signs of putrefaction. With body height of 179 cm and a body weight of 98 kg there was no evidence of Marfan’s syndrome. On his right knee, a ca. 5  3.5 cm measuring skin scratch was found. On internal examination the pericardial sac was filled with about 400 ml of partly liquid and clotted blood. The thoracic aorta showed a dissection reaching from the ascending aorta, ca. 1 cm above the valvular line, to the descending aorta shortly above the aortic hiatus, without aneurysmatic widening of the aortic trunk (Fig. 1A). On the right side, the A. subclavia dextra was included by the dissection with stenosis of the vascular lumen for approximately 50%. In the false lumen, liquid blood without signs of thrombosis was found. About 1 cm above the valvular line the

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Fig. 1. Autopsy findings (A): descending thoracic aorta with dissection of the aortic wall (FL: false lumen, TL: true lumen) and (B): rupture of the dissection membrane of the ascending aorta.

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inner and outer dissection membrane each presented with an about 1.5 cm long rupture (Fig. 1B). The aortic valve showed no malformation; the body showed no signs of infection and/or gastrointestinal neoplasms. The cause of death was pericardial tamponade by rupture of aortic dissection. Under suspicion of a hereditary connective tissue disease, histological examinations were imposed. 4. Histology Along the aortic wall, a dense lymphogranulocytic infiltration of the outer parts of the media and adventitia was found. The layers of the media appeared loosened and fragmented with focal bleedings (Fig. 2). In Elastica van Gieson staining of the aortic wall, the elastic fibers showed no rarefaction or other abnormalities indicating a connective tissue disease. The corresponding intimal layer showed clusters of mild gram-positive, rod-shaped microorganisms (Fig. 3). Regarding a possible infection caused by T. pallidum, no increased number of plasma cells was found. Initially suggesting an aortic infection caused by fungi, Periodic acid–Schiff stain, however, gave no evidence of fungal infection. These findings led us to include microbiological diagnostics. 5. Microbiology Anaerobic blood cultures examined at the Institute for Medical Microbiology showed growth of C. septicum. 6. Discussion Aortic dissection is altogether a rather rare cause of death. According to the Federal Statistical Office, Public Health Report 2013, in 2012, about 4.4 of 100,000 people died from aortic dissection in Germany; nevertheless it is a well-known cause of sudden death in forensic medicine and is frequently seen in autopsies. In 2013, 4 non-traumatic aortic dissections out of 233 cases of natural deaths investigated were found in autopsies at the Institute for Legal Medicine in Leipzig; 2 out of them, including the case presented, had consulted a medical practitioner in advance, but were not diagnosed with aortic dissection. Symptoms of aortic dissection are known for being misinterpreted or diagnosed too late [4]. Reasons for frequent false diagnoses are the unspecific symptoms, which can vary depending on the dissection site and its dynamics due to secondary growth of the false lumen caused by

Fig. 2. (A) Overall view of the dissected aortic wall shows a loosened media with focal bleeding (yellow arrow). The outer layers of the media and the adventitia (lower part of the picture) show dense infiltration with granulocytes and lymphocytes (black arrow and (B)). The intimal layer is detached (staining: HE) (A: magnification 4; B: magnification 10). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

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Fig. 3. (A) shows the swollen and loosened intima with clusters of bacteria as marked with the yellow arrow. Occasional macrophages surrounded by vacuoles are found (black arrows). (B) shows the area marked with the yellow arrow in (A) in higher magnification. Clusters of rod-shaped bacteria infiltrate the intimal layer and transmigrate into the aortic lumen (staining: HE). (A: magnification 40; B: magnification 100). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

the blood flow. The mortality rate in untreated patients is high, and 25% die within the first 24 h [5]. In elderly patients, the main cause of aortic dissections is chronic hypertension; whereas in younger patients (35 years and younger), hereditary diseases such as connective tissue diseases or autoimmune inflammatory diseases are the dominating causes. Therefore, especially in younger people, an autopsy is necessary to evaluate the risk for first-degree relatives of the deceased to evolve aortic aneurysms as well [6]. Infectious diseases of the aorta are very rare, especially in younger patients. The so-called mycotic aneurysm of the aorta is most commonly caused by aerobic, gram-positive bacteria such as Staphylococcus and Salmonella spp. [1,6]. Only 28 cases are present in literature these days that describe infectious aortitis related to C. septicum. C. septicum is an obligatory anaerobic, gram-positive, rod-shaped bacterium, in which pathogenetic effects are caused by formation of alpha-toxine, a cytolytic enzyme that leads to vast necrosis of the implemented tissues. The infection can be caused by external factors, for example, wounds in which the spores can breed, or by endogen factors, such as a compromised immune system or gastrointestinal malignancies [7]. C. septicum aortitis is an infection of the elderly (range 55–91 years) and 19 out of 27 patients had colonic carcinoma whereas 2 patients showed colonic polyps [2,8]. Prognosis of C. septicum aortitis is poor: 14 out of the reported 28 patients died from the infection or its complications [2]. Within C. septicum infections, an increased association to gastrointestinal neoplasms is witnessed by many authors [8–12], probably due to anaerobic metabolism and pH changes in the intestinal wall, which offer a suitable habitat and a ‘‘weak’’ area to entry the bloodstream for anaerobic bacteria [8]. In addition, atherosclerotic plaques seem to be a preferential spot for bacterial aortitis caused by the vascular lesion [13]. Altogether, this can be an explanation for the increased incidence of Clostridium associated with aortitis in elderly patients. Out of these 28 cases, this case is the second case of C. septicum aortitis in a young individual and, similar to the case described by Yang [2], there were no signs for gastrointestinal neoplasms suitable as a predisposing condition. In autopsy, a skin scratch on the right knee of the deceased was found, which could be a possible entrance for bacterial spores. It is possible that in other fatal cases of C. septicum aortitis, where no endogen predisposing factors were

detected in autopsy, small skin lesions existed, which have not been noticed or considered relevant. The first clinical indications of an anaerobic infectious aortitis can be elevated leukocytes and humoral inflammation markers, but those are unspecific for the infection site. The elevated leukocytes in this case were interpreted as early symptoms of a cold or, otherwise, infection. Subsequent aortic aneurysm or aortic dissection can lead to corresponding symptoms such as chest or back pain, numbness of upper or lower extremities, differences in blood pressure, or cardiac murmur (if the ascending aorta is affected). As frequently described in lethal aortic dissection, in this individual, the symptoms were wrongly interpreted as muscle tension by the medical practitioner. The documented cardiac murmur was not medically clarified. In CT scan, gas formations and oedematous thickening of the aortic wall and periaortic tissues may lead to the diagnosis [3,13]. In a short postmortal interval, a postmortem CT scan might show these signs of an anaerobic infectious aortitis as well and, therefore, might be useful; in our case, one can assume that the specific gas formations described for clinical CT would have been impaired by putrefaction gas and would have been non-specific. In autopsy, the aortic dissection was found, but there were no macroscopical signs of an infection. In this case, the remarkable histopathological findings gave first evidence of an infectious etiology and the subsequent blood cultures secured the diagnosis of C. septicum aortitis. Microbiological cultures from cardiac blood and vessel parts are essential to diagnose infectious aortitis as a cause of aortic dissection found in autopsy as well as in clinical cases. Since infectious aortitis in general and C. septicum aortitis especially is a very rare autopsy finding, it is useful to wait for histopathological results from the affected and non-affected vessel sites first and add a microbiological examination if microscopically signs for an infection are found. 7. Summary Infections of the aorta, especially anaerobic infections caused by C. septicum similar to the case described, are a rare cause of aortic aneurysms and/or dissections and, therefore, are clinically and macroscopically difficult to differentiate from other etiologies; for example, connective tissue disorders or secondary lesions

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caused by hypertension. To acquire a secure diagnosis of infectious aortitis through autopsy in cases of aortic dissections with a lethal course, histological examinations of the affected and non-affected vessel parts are obligatory. We recommend that blood samples and/or vessel parts should be taken as evidence for further microbiological examinations in case there are signs for an infectious inflammation of the aorta. Acknowledgments The authors would like to thank Prof. Dr. med. Rodloff of the Institute of Medical Microbiology, University of Leipzig for processing the microbiological cultures.

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Acute aortic dissection caused by Clostridium septicum aortitis.

Clostridium septicum aortitis is a rare cause of aortic dissection. So far, only 28 cases have been described in literature before. Most of these case...
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