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Eur J Cardiothorac Surg 2001;19:96-98
© 2001 Elsevier Science NL


Case report

Cholesterol crystal embolization after cardiac operations.

Report of two cases

Jaime F. Vazquez-Jimeneza, Alberto Pérez-Bouzab, Oliver J. Liakopoulosa, Bruno J. Messmera

a Thoracic and Cardiovascular Surgery, Universitätsklinikum RWTH Aachen, Germany
b Institute of Pathology, Universitätsklinikum RWTH Aachen, Germany

Received 27 June 2000; received in revised form 9 October 2000; accepted 3 November 2000.

Corresponding author. Klinik für Thorax, Herz und Gefäßchirurgie, Universitätsklinikum RWTH Aachen, Pauwelsstraße 30, 52074 Aachen, Germany Tel.: +49-241-8089-961, fax: +49-241-8888-454
e-mail: jvazquez-jimenez{at}post.klinikum.rwth-aachen.de


    Abstract
 Top
 Abstract
 1. Introduction
 2. Discussion
 References
 
We report two cases of systemic cholesterol embolization after coronary bypass surgery under extracorporeal circulation. First clinical symptoms starting at 48–72 h after cardiac operation were severe abdominal pain and elevated ischemia associated blood parameters. In both patients a laparotomy was performed but fatal outcome due to multi-organ failure could not be avoided. The awareness of this often fatal complication is of great importance for the surgeon since prevention is the only effective treatment.

Key Words: Cholesterol emboli syndrome • Multiple cholesterol emboli syndrome • Cholesterol clefts • Cutaneous emboli • Blue toe syndrome • Cardiac surgery complications


    1. Introduction
 Top
 Abstract
 1. Introduction
 2. Discussion
 References
 
Cholesterol crystal embolization (CCE), a complication of atherosclerosis, is found in 2–8% of necropsies [1]. It occurs due to dislodgement of cholesterol crystals from atherosclerotic plaques lining the walls of the major arteries with embolization in arteries of multiple organs with 100–200 µm diameter [2].

Although CCE may appear spontaneously [1], it often follows invasive procedures like cardiovascular operations [2,3] or angiography [4,5]. Organs most frequently involved are the skin and kidneys but any other organ can be affected [4]. Cutaneous manifestation show a wide range starting from livedo reticularis of the lower extremities to acrocyanosis, know as the ‘blue toe syndrome’, and less frequently nodules, ulcerations, gangrene of the toes and purpura. Renal failure occurs in 50% of cases and gastrointestinal bleeding in 10% [4]. Less common features include ischemic pancreatitis [6], involvement of the central nervous system and coronary arteries as well as the multiple cholesterol emboli syndrome (MCES) [4,6,7].

We describe two cases of severe cholesterol crystal embolization with multiple organ failure after coronary artery bypass grafting surgery.

1.1. Case 1
A 69-year-old woman with a history of severe dyspnea and unstable angina because of two vessel coronary artery disease underwent coronary artery bypass operation. The first postoperative day at the intensive care unit (ICU) was uneventful, so that the patient was extubated. At the second postoperative day, severe abdominal pain, together with increasing ischemia-associated blood parameters (lactate dehydrogenase, creatinine phosphokinase) led to the suspicion of acute gastrointestinal ischemia. Laparatomy revealed an acute ischemic insult of the large bowel distal to the left colon flexure, so that a left hemicolectomy with an artificial anus was performed. At this time a massive decline in cardiovascular and renal function was noted. One day later, a second-look laparotomy showed complete necrosis of the bowel. The patient died the same day.

Autopsy showed a pan-necrosis of the bowel, a widespread, superficial and sharp delimited necrosis of the cortex of both kidneys (Fig. 1A) and multiple wedge-shaped anemic infarcts of the spleen, liver and left kidney. The abdominal aorta displayed large ulcerated atherosclerotic plaques of soft consistence. Aorto-coronary vein graft and mammary artery were not occluded and no thrombotic material was found in either atria or ventricles.



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Fig. 1. (A) Macroscopic aspect of an autopsied kidney (case 1). Note the superficial, widespread ‘map-like’ cortical anaemic necrosis (pallor yellow) with a sharp border to non anaemic cortex (arrows). These necroses were only superficial and not wedge-shaped. Scale bar: 2 cm. (B) Micrograph of an hematoxylin-eosin stained histological section of the vessels from the infarcted small bowel. The arterioles contain in their lumina blood which demarcate needle-shaped cholesterol crystal clefts. A necrosis-related granulocyte extravasation is already present. Scale bar: 200 µm.

 
Microscopically, the end-arterioles of the infarcted organs (intestine, kidneys, spleen and liver) showed needle-shaped cholesterol crystal clefts (Fig. 1B). In the intestine, granulocyte infiltration around the vessels was already present. No intraluminal foreign body giant cells with intracytoplasmic crystal inclusions could be detected, suggesting that the cholesterol embolisms happened shortly before death.

1.2. Case 2
A 73-year-old woman with history of hypertension and severe coronary artery disease. Coronary angiography revealed severe three vessel disease and coronary artery bypass surgery was performed. Because of low cardiac output, an intra-aortic balloon pump was inserted via the left femoral artery. Cardiac performance was stabilized with maximum inotropic therapy at the ICU. Three days later the clinical status worsened with severe abdominal pain and increased serum potassium levels. The patient developed multiple skin necrosis on the face, thorax and abdominal wall. An acute mesenterial infarction was excluded by explorative laparotomy but the patient died the same day with the clinical signs of low cardiac output.

Autopsy showed an acute myocardial infarction after thrombotic obliteration of the bypass to the intermediate branch. This was considered to be the cause of death. No thrombi were found in the atria or ventricles. The abdominal aorta displayed, as in case one, large ulcerated atherosclerotic plaques of soft consistence, and the spleen showed small, wedge-shaped subcapsular anaemic infarcts. The kidneys were atypically located in the pelvis, just above the bladder and between both common iliac arteries. Both kidneys were fused forming a single horseshoe-shaped kidney with two functional ureters and a ventrally located hilus. Macroscopically, the surface of the singular kidney displayed similar morphological features as the kidneys in the former case: a widespread, superficial and sharp delimited cortex necrosis. Microscopically, cholesterol crystal clefts were found in the small diameter arterioles of the skin, spleen and kidney. In this particular case, cholesterol embolism in the abnormal located horseshoe-shaped kidney was interpreted retrospectively as the origin of the acute low abdominal pain and high levels of potassium in serum.

In both cases an advanced atherosclerosis of the large vessels was found. The soft ulcerating plaques in the abdominal aorta, that were easily removable, were considered the source of the cholesterol crystals found in the infarcted organs.


    2. Discussion
 Top
 Abstract
 1. Introduction
 2. Discussion
 References
 
CCE occurs when atheromatous plaques rupture and release their contents, either spontaneously or as a result of iatrogenic trauma [1]. Risk factors for CCE are old age, peripheral vascular disease and severe atherosclerosis of the ascending aorta [8]. Approximately one third of the patients have medical history of previous vascular surgery, angiography or angioplasty hours to weeks before CCE onset [1].The treatment is usually symptomatic, including renal and hemodynamic support [9]. Morbidity is considerable and the reported mortality is 64–81% [1].

Prevention of this often fatal disease is striking for the results of invasive vascular interventions. Palpation of the ascending aorta before cannulation identified the atheromatous disease in only 38% of the patients and underestimate its severity. The use of intraoperative echocardiography of the ascending aorta and aortic arch has been useful to assess the degree of atherosclerosis. Therefore, in case of severe atherosclerosis it might be necessary to modify the standard cannulation and clamping techniques, to use filtration devices of the ascending aorta or even perform off-pump surgery [10].

Clinicians, surgeons and pathologists should be aware of the risk of CCE after cardiac surgery. The diagnosis can only be confirmed histologically, thus in organ biopsies or autopsies of patients, which are suspected to suffer or have suffered CCE. The real incidence of CCE remains to be documented. Adequate future strategies to prevent the disease are necessary, since no proven effective treatment of this dangerous complication is known.


    References
 Top
 Abstract
 1. Introduction
 2. Discussion
 References
 

  1. Rhodes J.M. Cholesterol crystal embolism: an important ‘new’ diagnosis for the general physician. Lancet 1996;347:1641.[Medline]
  2. Piriou V., Claudel J.P., Bastien O., Ross S., Lehot J.J. Severe systemic cholesterol embolization after open heart surgery. Br J Anaesth 1996;77:277-280.[Abstract/Free Full Text]
  3. Price D.L., Harris J. Cholesterol emboli in cerebral arteries as a complication of retrograde aortic perfusion during cardiac surgery. Neurology 1970;20:1209-1214.
  4. Fine M.J., Kapoor W., Falanga V. Cholesterol crystal embolization: a review of 221 cases in the English literature. Angiology 1987:769-784.
  5. Lye W.C., Cheah J.S., Sinniah R. Renal cholesterol embolic disease. Case report and review of the literature. Am J Nephrol 1993;13:489-493.[Medline]
  6. Moolenaar W., Lamers C.B. Cholesterol crystal embolization to liver, gallbladder, and pancreas. Dig Dis Sci 1996;41:1819-1822.[Medline]
  7. Fernandez-Samos R., Suarez D., Ortega J.M., Zorita A., Vazquez J., Moran C.F., Moran O., Arias R., Barrios A., Vaquero F. Multiple cholesterol embolization syndrome: a lethal complication of vascular procedures. Report of two histologically proven cases. J Cardiovasc Surg 1995;36:87-91.[Medline]
  8. Blauth C.I., Cosgrove D.M., Webb B.W., Ratliff N.B., Boylan M., Piedmonte M.R., Lytle B.W., Loop F.D. Atheroembolism from the ascending aorta. An emerging problem in cardiac surgery. J Thorac Cardiovasc Surg 1992;103:1104-1111.[Abstract]
  9. Belenfant X., Meyrier A., Jacquot C. Supportive treatment improves survival in multivisceral cholesterol crystal embolism. Am J Kidney Dis 1999;33:840-850.[Medline]
  10. Wareing T.H., Davila-Roman V.G., Barzilai B., Murphy S.F., Kouchoukos N.T. Management of the severely atherosclerotic ascending aorta during cardiac operations. A strategy for detection and treatment. J Thorac Cardiovasc Surg 1992;103:453-462.[Abstract]



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