Medical Care
Medical treatment of CCE has largely been unsuccessful, with the exception of a few anecdotal reports. The early goals of treatment are to augment the circulation and to try to prevent occlusion. Some believe this can be achieved with drugs that inhibit coagulation, platelet aggregation, and RBC sludging. Vasodilators and corticosteroids have also been used. Variable results have been reported with the use of heparin, streptokinase, urokinase, tissue plasminogen activator, warfarin, bishydroxycoumarin, aspirin, pentoxifylline, dipyridamole, prostaglandins, prostacyclin, intra-arterial papaverine, sulfinpyrazone, low–molecular-weight dextran, nifedipine, prednisone, and methylprednisolone. Some have also recently reported success with hemostatic and lipid-lowering agents (eg, vitamin K, carbazochrome, tranexamic acid, reptilase, lovastatin, cholestyramine, probucol).
Although anticoagulants have been observed to cause CCE and many reports indicate the cessation of symptoms upon discontinuation, in 2 patients, heparin resulted in resolution of their myalgias, tenderness, and pregangrene. Furthermore, they did not have any recurrence of symptoms or signs. Another group reported clinical and radiographic improvement following therapy with intra-arterial streptokinase, heparin, and prostacyclin. Iloprost, a prostacyclin analog, has also been reported to improve renal function and peripheral symptoms in patients with CE. Subcutaneous heparin, which is intermittently administered through the tissue to provide trough periods, may allow more effective healing of plaques than the intravenous form.
Some recommend a trial of corticosteroids for their anti-inflammatory effect to limit arteritis and the subsequent fibrotic occlusion of vessels; however, in 9 patients with CCE thought to have vasculitis who were treated with corticosteroids, 7 died.
Lovastatin may effect healing by inducing the regression of atherosclerosis and by decreasing plaque cholesterol content, which is linked to an increased incidence of emboli.
One group used the combination of hemostatic (ie, vitamin K, carbazochrome, tranexamic acid, reptilase) and antihyperlipidemic (ie, cholestyramine, probucol) drugs and had positive results.
The clinical effect of circulator boot therapy in patients with cholesterol embolization syndrome of the lower extremities in patients following cardiac or vascular procedures may be an effective noninvasive therapeutic option. [37]
Surgical Care
Removal of the source of the emboli using thromboendarterectomy or excision and replacement of the prosthesis has resulted in resolution of CCE. Because gangrene is more likely to occur in persons whose circulation is already compromised, reconstruction of the stenotic proximal artery, which may or may not be the source of emboli, is also advised. Other forms of surgical treatment include embolectomy, sympathectomy, and primary excision of necrotic tissue, possibly involving amputation.
Thoracoabdominal repair is the criterion standard of treatment. Bypass without vessel ligation is contraindicated because it does not remove the source of emboli. Improvement and healing of cutaneous lesions was observed in 2 of 3 patients after resection of abdominal aortic aneurysms. Another group reported that 4 of 5 patients with ischemic lesions from toe gangrene or necrosis benefited from arterial reconstruction. For multilevel occlusive disease, proximal reconstructions are performed prior to distal ones, but the reverse may be appropriate for blue toe syndrome.
If the entire aorta is diffusely ulcerative, the source of emboli is inaccessible, or the patient is a poor surgical candidate, then thoracoabdominal repair may not be possible. Palliative treatment for such patients is axillobifemoral bypass with external iliac ligation. Four patients with peripheral emboli who underwent this procedure had cessation of new lesions, healing, and pain relief. Embolization to the pelvic circulation may be controlled by iliac ligation at the aortic bifurcation or by individual interruption of the internal iliac arteries. Another group had similar success with this technique and was able to salvage 12 limbs in 6 patients, apart from the loss of a fifth toe.
Embolectomy may be effective in cases of the larger atheroemboli.
Peripheral nerve blockade or lumbar sympathectomy has been used to deter cutaneous breakdown and promote healing. They are advocated for patients with persistent areas of pain, cyanosis, or cutaneous gangrene in the involved limb. Sympathetic blockade influences the microcirculation of the skin through a direct effect on arteriovenous communications that are almost entirely made of smooth muscle.
If peripheral circulation is intact, hard eschars from infarcted skin and/or muscle on the legs should be excised primarily.
If circulation is inadequate, amputation may be the only way to stop the advance of ascending gangrene.
Consultations
CE can cause severe dysfunction of practically any organ, but renal emboli resulting in hypertension and renal insufficiency or failure is the most common complication. Management by a nephrologist, with possible referral to a dialysis service, may be necessary.
Prevention
During aortic surgery, gentle clamping, no reclamping, and minimal handling of the aorta are recommended. Smaller, less rigid catheters and less manipulation of them is important, as is the avoidance of high-pressure injectors. For aortography, consider a transaxillary approach instead of a translumbar approach if aortic plaques are present; however, the transaxillary approach is technically more difficult, with an increased risk of thrombosis, hematoma, and possible brachial plexus injury.
Avoid anticoagulants, which can cause hemorrhage within the walls of sclerotic arteries, with subsequent thrombosis and elevation of an atheromatous plaque.
During aortoiliac reconstruction, the downward flush is a maneuver in which, following the proximal and one distal anastomosis, a gush of blood is allowed to escape through the unanastomosed limb of the prosthesis, while the other artery is clamped, to remove debris. In a variation on this technique, one group was able to prevent peripheral emboli during aortoiliac reconstruction with the placement of a blood filter and perfusion cannula between the unanastomosed limb and unattached distal artery, allowing a larger gush of blood and more atheromatous debris to be flushed out.
Some authorities have suggested that CABG surgery should be contraindicated in patients with a known history of CE.
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Chronic leg ulcer due to cutaneous cholesterol emboli on the leg of a 79-year-old woman.
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Skin biopsy specimen demonstrating ulceration and an occluded vessel at the right border of the specimen within the fat. Hematoxylin and eosin stain at 22X magnification.
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Higher magnification of the same biopsy specimen, demonstrating cholesterol clefts within an occluded arteriole. Hematoxylin and eosin stain at 297X magnification.
