Introduction
Background
Cholesterol embolism syndrome should be suspected in a patient who develops worsening renal function, hypertension, distal ischemia, or acute multisystem dysfunction after an invasive arterial procedure. Atheroemboli may also occur spontaneously. The protean manifestations of this syndrome make the diagnosis challenging. As the population ages, the incidence of cholesterol embolism syndrome will increase.
Cholesterol crystal embolization from upstream coronary artery plaque after percutaneous transluminal coronary angioplasty.
Pathophysiology
Any organ system, with the exception of the lungs, may be directly affected. Cholesterol embolism syndrome has 2 mechanisms of action.
In the first, cholesterol crystals spontaneously break off from severely atherosclerotic plaques and shower into downstream organs, occluding arterioles 100-200 micrometers in diameter. The crystals induce an inflammatory body reaction and adventitial fibrosis, which eventually obliterate the vessel lumen. Local vasospastic mediators compound tissue ischemia and produce progressive, irreversible organ damage.
With the second mechanism, larger cholesterol plaques break off and occlude larger arteries, causing tissue infarction with acute organ dysfunction. This can occur after local trauma to the atherosclerotic plaque, such as that caused by angiography or aortic trauma, or it can occur after destabilization of the protective clot overlying the plaque, which can occur as a result of anticoagulation.
Cholesterol crystal embolization occurs from the arterial system, and crystals are trapped in the arterioles where they either immediately occlude the vessels or induce an intense inflammatory response that leads to tissue ischemia. Crystals do not travel to the lungs; however, inflammatory mediators released by ischemic tissue may result in acute lung injury.
Frequency
United States
See Internationally below.
International
Estimates of the incidence of cholesterol embolic disease are usually based on autopsy data. Tissue sections from patients with the following diseases or indicate the incidence of atheroembolic events: aortic aneurysms (31%), abdominal aortic aneurysm repair (up to 77%), severe aortic disease (13-16%), and mild aortic disease (1-2%). Of patients undergoing angiography, 25-30% may have atheroembolic events, while 2.5-3% of patients who receive percutaneous coronary transluminal angioplasty vein grafts and 1.4-3% of patients undergoing renal artery angioplasty or cardiac catheterization have been reported to have clinical signs of atheroemboli.
Mortality/Morbidity
- The mortality rate of acute multisystem organ failure resulting from cholesterol embolism syndrome is 58-90%. Jucgla found an overall incidence of 58% at 15 months, increasing to 65% if visceral organs were involved.1 Pre-existing chronic renal insufficiency had a relative risk of death of 4.54.
- The mortality rate of severe cholesterol embolism is 90% at 3 months.
- Mild cases with renal dysfunction with or without skin findings had a mortality of 16%.
Sex
Men have a higher risk than women.
Age
Cholesterol embolism is a disease of persons ranging from middle-aged to elderly, with a minimum age of 50 years.
Clinical
History
The diagnosis of cholesterol embolism must be considered in patients older than 50 years who have atherosclerotic disease presenting with multisystem dysfunction after undergoing an invasive vascular procedure or receiving an anticoagulant or thrombolytic agent within the past several months. All patients with the classic triad of livedo reticularis, acute renal failure, and eosinophilia should be evaluated for cholesterol embolism, including a funduscopic examination.
Physical
- Constitutional
- Fever
- Weight loss
- Hypermetabolic state
- Cardiovascular
- Tachycardia
- Uncontrolled or accelerating hypertension
- Congestive heart failure
- Myocardial infarction
- Intact peripheral pulses with livedo reticularis and tissue ischemia: These findings suggest small-vessel occlusion, such as cholesterol embolization, in a patient at risk.
- Neurologic
- Hollenhorst plaques in retinal arteries
- Hemispheric ischemic stroke
- Paraplegia
- Confusion
- Delirium
- Renal - Oliguria, acute renal failure
- Dermatologic
- Gangrene, nodules, purpura, cyanosis, ulcerations (in 35-90% of patients)
- Livedo reticularis
- Infarction of perineal area
- Ischemic patches involving lower extremities more often than upper
- Blue toe syndrome and splinter hemorrhages2
- Gastrointestinal
- Minor or major bleeding
- Abdominal pain
- Bowel infarction
- Pancreatitis
- Acalculous cholecystitis
- Musculoskeletal - Myalgias
- Endocrine -Adrenal insufficiency
- Pulmonary -Acute respiratory distress syndrome (ARDS)
Causes
Any risk factor for atherosclerotic disease is a risk factor for cholesterol embolism.
Preoperative risk factors for cholesterol embolism syndrome after coronary artery bypass surgery include being older than 60 years, hypertension, cerebrovascular disease, aortoiliac disease, and mitral annular calcification. Although the other factors are well known, the association between mitral annular calcification and aortic atherosclerosis was identified only recently.
Identifying patients at risk and making efforts to minimize aortic wall trauma help reduce the chance of cholesterol embolism. The risk for a patient developing cholesterol embolism may be reduced by using a brachial or axillary approach in patients known to have severely ulcerated aortic plaque, using soft flexible catheters, and avoiding high-pressure jets of contrast material.
More on Cholesterol Embolism |
 Overview: Cholesterol Embolism |
| Differential Diagnoses & Workup: Cholesterol Embolism |
| Treatment & Medication: Cholesterol Embolism |
| Follow-up: Cholesterol Embolism |
| Multimedia: Cholesterol Embolism |
| References |
| Next Page » |
References
Jucgla A, Moreso F, Muniesa C. Cholesterol Embolism: Still an Unrecognized Entity with a High Mortality. J Am Acad Derm. 2006;55:786-793.
Willens HJ, Kramer HJ, Kessler KM. Transesophageal echocardiographic findings in blue toe syndrome exacerbated by anticoagulation. J Am Soc Echocardiogr. Nov-Dec 1996;9(6):882-4. [Medline].
Ling G, Ovbiagele B. Oral antiplatelet therapy in the secondary prevention of atherothrombotic events. Am J Cardiovasc Drugs. 2009;9(3):197-209. [Medline].
Acarturk E, Ozeren A, Sarica Y. Detection of aortic plaques by transesophageal echocardiography in patients with ischemic stroke. Acta Neurol Scand. Aug 1995;92(2):170-2. [Medline].
Adler Y, Shohat-Zabarski R, Vaturi M, Shapira Y, Ehrlich S, Jortner R. Association between mitral annular calcium and aortic atheroma as detected by transesophageal echocardiographic study. Am J Cardiol. Mar 15 1998;81(6):784-6. [Medline].
Belenfant X, Meyrier A, Jacquot C. Supportive treatment improves survival in multivisceral cholesterol crystal embolism. Am J Kidney Dis. May 1999;33(5):840-50. [Medline].
Carroccio A, Olin JW, Ellozy SH, Lookstein RA, Valenzuela R, Minor ME. The role of aortic stent grafting in the treatment of atheromatous embolization syndrome: results after a mean of 15 months follow-up. J Vasc Surg. Sep 2004;40(3):424-9. [Medline].
Fukumoto Y, Tsutsui H, Tsuchihashi M, Masumoto A, Takeshita A,. The incidence and risk factors of cholesterol embolization syndrome, a complication of cardiac catheterization: a prospective study. J Am Coll Cardiol. Jul 16 2003;42(2):211-6. [Medline].
Hasegawa M, Sugiyama S. Apheresis in the treatment of cholesterol embolic disease. Therap Apher Dial. Aug 2003;7(4):435-8. [Medline].
Hirano Y, Ishikaw K. Cholesterol Embolization Syndrome: How to Recognize and Prevent This Potentially Catastrophic Iatrogenic Disease. Int Med. 2005;44:1209-1210.
Katz ES, Tunick PA, Rusinek H, Ribakove G, Spencer FC, Kronzon I. Protruding aortic atheromas predict stroke in elderly patients undergoing cardiopulmonary bypass: experience with intraoperative transesophageal echocardiography. J Am Coll Cardiol. Jul 1992;20(1):70-7. [Medline].
Keen RR, McCarthy WJ, Shireman PK, Feinglass J, Pearce WH, Durham JR. Surgical management of atheroembolization. J Vasc Surg. May 1995;21(5):773-80; discussion 780-1. [Medline].
Kirkland L. Cholesterol embolism in intensive care patients. J Intensive Care Med. 1993;7(3):12-21.
Kolh PH, Torchiana DF, Buckley MJ. Atheroembolization in cardiac surgery. The need for preoperative diagnosis. J Cardiovasc Surg (Torino). Feb 1999;40(1):77-81. [Medline].
Lowe HC, Houser SL, Aretz T, MacNeill BD, Oesterle SN, Palacios IF. Significant atheromatous debris following uncomplicated vein graft direct stenting: evidence supporting routine use of distal protection devices. J Invasive Cardiol. Oct 2002;14(10):636-9. [Medline].
Manganoni AM, Venturini M, Scolari F, Tucci G, Facchetti F, Graifemberghi S. The importance of skin biopsy in the diverse clinical manifestations of cholesterol embolism. Br J Dermatol. Jun 2004;150(6):1230-1. [Medline].
Resnik KS. Intravascular cholesterol clefts as an incidental finding: cholesterol embolism or not?. Am J Dermatopathol. Dec 2003;25(6):497-9. [Medline].
Ribakove GH, Katz ES, Galloway AC, Grossi EA, Esposito RA, Baumann FG. Surgical implications of transesophageal echocardiography to grade the atheromatous aortic arch. Ann Thorac Surg. May 1992;53(5):758-61; discussion 762-3. [Medline].
Tenenbaum A, Garniek A, Shemesh J, Fisman EZ, Stroh CI, Itzchak Y. Dual-helical CT for detecting aortic atheromas as a source of stroke: comparison with transesophageal echocardiography. Radiology. Jul 1998;208(1):153-8. [Medline].
Vayssairat M, Chakkour K, Gouny P, Nussaume O. Atheromatous embolisms and cholesterol embolisms: medical treatment [French]. J Mal Vasc. 1996;21 Suppl A:97-9. [Medline].
Verneuil L, Bekolo R, Dompmartin A. Efficiency of colchicine and corticosteroids in a leg ulceration with cholesterol embolism in a woman with rheumatoid arthritis. Rheumatology. 2003;42:1014-1016.
Further Reading
Keywords
cholesterol, embolism, cholesterol embolism, cholesterol embolism syndrome, CES, atheroembolism, trash foot syndrome, hypertension, HT, distal ischemia, cholesterol crystals, cholesterol plaques, atherosclerotic plaque, atheroembolic events
