The Role of Extraanatomic Exclusion Bypass in the Treatment of Disseminated Atheroembolism Syndrome Jeffrey L. Kaufman, MD, Javid Saifi, MD, Benjamin B. Chang, MD, Dhiraj M. Shah, MD, Robert P. Leather, MD, Albany, N e w York
We treated six patients with disseminated atheroembolism complicated by severe and unremitting pain from bilateral foot lesions. All patients had multiple and severe medical risk factors. One patient had a recent myocardial infarction, six patients had renal failure, and three were undergoing hemodialysis. Definitive aortic reconstruction was therefore precluded. After failing a course of medical therapy, each patient was treated with axillobifemoral bypass with exclusion-ligation of the external lilac arteries. Healing of foot wounds occurred in 11 of 12 limbs at risk, with one below-knee amputation required for progressive forefoot necrosis. In 12 patients with severe cardiopulmonary disease and limited life expectancy, exclusion-ligation bypass is an effective and safe palliative procedure for severe disseminated atheroembolism. (Ann Vasc Surg 1990;4:260-263). KEY WORDS: Disseminated atheroembolism syndrome; axillobifemoral bypass; forefoot necrosis; extraanatomic exclusion bypass.
Disseminated atheroembolism is a syndrome of multiple organ damage caused by microthrombi and cholesterol emboli from a suprarenal aortic source [1]. Patients with this syndrome have ulcerated and degenerative atherosclerotic plaque extending the whole length of the aorta. Theoretically, the optimal treatment of disseminated atheroembolism is total replacement of the offending thoracoabdominal arterial segment, the typical patient with this syndrome is severely debilitated and cannot safely undergo an arterial reconstruction extending from the suprarenal aorta. In a subset of patients with disseminated atheroembotism, continued showers of microparticulate material to the lower extremities are associated with severe pain and From the Departments o f Surgel~, Albany Medical College, and the Albany Veterans Administration Medical Center, Albany, New York. Presented at the Annual Meeting of the Peripheral Vascular Surgeo' Society, New York, New York, June 17, 1989. Reprints requests: Jeffrey L. Kaufman, MD, Department of Surgery. (112), Albany Veterans Administration Medical Center, Albany, New York 12208. 260
impending or frank gangrene of the toes. We report on operative procedures which alter the inflow of blood to the legs in hope of achieving palliation of this disease.
METHODS The patients were treated at the Robert Wood Johnson University Hospital, (New Brunswick, N J), and at the Albany Veterans Administration Medical Center, (Albany, NY), from July 1982 to May 1989. The presence of disseminated atheroembolism was determined on the basis of clinical, pathological, and arteriographic studies [1]. Atheroembolism to the lower extremities was defined by the sudden onset of painful, asymmetrical, patchy, blue-purple lesions on the toes and feet in an extremity with either clearly palpable pedal pulses or where vascular laboratory studies indicated absence of critical ischemia (an ankle blood pressure greater than 100 torr with a Class 1, 2, or 3 pulse volume recording at the ankle) [1--4]. Atheroembolism to the viscera was defined by either (a) serial evidence for recent and significant deterioration
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of renal function, (b) cholesterol embolism demonstrated on a renal biopsy specimen, or (c) signs and symptoms of ischemic colitis or enteritis with cholesterol emboli present within intestinal biopsy or resected bowel specimens. Biplanar arteriograms were obtained from the mid-thoracic aorta to at least the femoral arteries. Each patient had evidence of severe aortoiliac atherosclerosis with marked irregularity of the flow lumen, indicative of plaque degeneration. Each patient also had unmistakable involvement of the suprarenal aorta. The six patients in this series complained of severe and unremitting pain in their toes and feet at the sites of clinical atheroembolism. They also had at least one area of cutaneous gangrene or impending gangrene to a toe from atheroembolism. Ongoing atheroembolism was defined by clinical observation of either new foot or toe lesions or by failure of existing lesions either to heal or to cease being painful. Initially, each patient had a trial of analgesics, aspirin and dipyridamole for pain, which in each case failed to relieve the symptoms. The methods for performing axillobifemoral reconstruction have been described elsewhere [5]. Exclusion of aortoiliac inflow into the legs was accomplished by ligation of the external iliac arteries before performing the femoral anastomoses (Fig. !). The femoral sheathtransversalis fascia was broadly dissected free from the inguinal ligament, which was lifted to provide exposure of the external itiac artery. Proximal dissection was facilitated by division of the one large vein crossing at the level of the inferior epigastric artery. Care was taken to preserve this external iliac arterial branch and the circumflex iliac branches. If the artery was found to be soft, it was doubly ligated with an umbilical tape or Number 2 silk tie as far proximally as possible through this exposure. If the artery was found firm or calcified, it was suture-ligated with polypropylene material. This series excludes patients with atheroembolism from an infrarenal aortic source [2] and those without evidence for atheroembolism to the visceral or pelvic structures. Those patients with less advanced disease were treated with conventional aortoiliac or aortofemoral reconstruction or with aortoiliac endarterectomy. In addition, each patient underwent careful cardiac, pulmonary, and renal function studies to determine whether total aortic repair was a surgical option. Based on previously described criteria [6-8], each patient was evaluated to determine his suitability for thoracoabdominal aortic reconstruction.
PATIENTS AND PROCEDURES During the study period, 18 patients were found to meet the clinical criteria for disseminated atheroembolism. Mean age of the patients was 68.7 years, equal to that of a larger cohort with disseminated atheroembolism. Six patients (all male, age range 61-72) had
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Fig. 1. Exclusion-ligation procedure with axillobifemoral reconstruction. Ligatures are placed as far proximally as possible on external lilac arteries using infrainguinal exposure.
severe, unremitting pain in the feet and toes from atheroembolism, to the degree that a Class II narcotic was necessary to achieve even minimal relief of discomfort. All were tobacco users, three of the six were diabetic, and all six had hypertension. All had clinical or electrocardiographic evidence for coronary artery disease; one suffered a myocardial infarction one month prior to the onset of atheroembolism. All had renal impairment with a serum creatinine greater than 2 mg/dl, and three were undergoing hemodialysis at the time of their bypass. E v e r y patient had bilateral lesions. Aspirin, dipyridamole, and oral or parenteral analgesics were administered to patients for an average of 6.8 weeks (range two to 12 weeks) before we determined that
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the pain was chronic and unremitting. During this period of observation, one patient developed progressive gangrene extending to the forefoot, despite palpable pedal pulses in his foot. The remaining five patients manifested no clinical healing of their toe lesions during this same period. After we determined that a suprarenal source was the etiology for the patient's disseminated atheroembolism, using criteria for simultaneous visceral and peripheral atheroembolism as noted above, each of the six patients underwent careful medical evaluation for surgical risk, in hopes that a definitive thoracic aortic repair could be performed. Five of the six were within Goldman Class IV [6]. One patient within Goldman Class III refused a definitive repair but accepted the lesser procedure of an exclusion bypass operation. Each patient underwent axillobifemoral bypass with ligation of the external iliac arteries. The procedures were performed under general anesthesia. The patient with a recent myocardial infarction had pulmonary artery balloon catheterization. Length of operation was minimized by using two surgical teams. There were no operative complications. Pulse volume recordings remained stable in all patients. No patient had a decrease in measurable perfusion to the feet from this procedure. Pain from the toe decreased significantly over the first postoperative week, and by the second week all narcotics were eliminated. The six patients were maintained on aspirin and dipyridamole after the exclusion bypass. The treatment for small areas of cutaneous gangrene or of pregangrenous areas over the toes was expectant autoamputation or sloughing of necrotic tissue in five patients with formal toe amputation. The one patient with gangrene extending to the forefoot required a complete transmetatarsal amputation, which failed to heal. He subsequently healed a below-knee amputation, and lesions on the toes of the other foot healed spontaneously. That patient nevertheless suffered the only significant morbidity of the group: During the month after bypass, he developed a painful nonhealing ulcer over the glans penis. This required penectomy, and atheroembolic debris was found in the penile arteries. His renal failure was treated with maintenance hemodialysis through a PTFE graft fistula. This became infected and was the source of fatal metastatic sepsis in the axiUobifemoral bypass. Overall, four of the six patients died within six months of the exclusion-bypass procedure. One patient underwent postmortem examination which confirmed friable degenerative atherosclerosis of the suprarenal aorta (Fig. 2). Of the two survivors, one was lost to follow-up at one year. The other was alive with chronic renal failure, but not requiting dialysis, at one year after the procedure. DISCUSSION The pathophysiology of organ damage from atheroembolism is not yet understood, despite recognition of
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Fig. 2. Visceral and lower suprarenal segment of aorta of patient with disseminated atheroembolism. Friable character of plaque is clearly demonstrated and renders the ostia of bilaterally paired renal arteries difficult to see. Ostia of celiac trunk and superior mesenteric arteries are noted with arrows.
this syndrome for more than 40 years [9]. It is remarkable that the clinical findings of blue toe syndrome, livido reticularis, renal dysfunction, intestinal ischemia, and lower extremity pain may be severe, yet the amount of cholesterol and atheromatous debris found in affected tissues may be very small. Once there is clinical evidence for atheroembolism, surgical treatment becomes an option, as no carefully controlled study has yet demonstrated continued efficacy for any medical therapy, such as administration of antiplatelet drugs. The surgical approach is to remove the offending arterial segment from the inflow blood stream serving the previously damaged organ or limb. If the source is a simple iliac or femoral plaque, local procedures such as endarterectomy, with or without patch angioplasty, may be effective. If the source is the aorta, with disease limited to a short arterial segment, aortoiliac endarterectomy may be indicated. Otherwise, treatment of extensive atherosclerosis with atheroembolism from the infrarenal segment involves conventional aortofemoral bypass techniques performed with an end-to-end aortic anastomosis as close to the level of the renal arteries as possible [2]. The vast majority of patients with infrarenal disease only can undergo primary arterial repair safely with a retroperitoneal aortic exposure, even if multiple medical risk factors are present [ 10]. Treating the patient with extensive degenerative aortic atherosclerosis poses a difficult problem when disseminated atheroembolism occurs. Extensive atherosclerosis in this population carries a grave prognosis. The majority of these patients have severe cardiopulmonary dysfunction. It is therefore virtually impossible in the majority of these patients to offer safe performance of suprarenal or thoracoabdominal aortic replacement, which is the optimal procedure for treatment of their disease. Instead, they may require a
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palliative procedure to treat ongoing painful atheroembolism to the lower extremities, with recognition that there is no safe way to prevent further atheroembolism to the viscera. An axillobifemoral bypass is therefore a reasonable choice of treatment for its ease of placement and safety, Ligation of the external iliac arteries is performed in order to obviate the need for secondary abdominal incisions to reach the common iliac arteries, which were severely diseased in all patients. Clearly, this treatment allows continued atheroembolism to the pelvis, but in only one patient has this become a problem of clinical significance, with development of a non-healing ulcer of the glans penis. Given the overall poor prognosis of disseminated atheroembolism, this is a minor problem in comparison with the discomfort these patients experienced in their feet. The cohort of patients in this paper was highly selected among those who were seen in our clinical practice with atheroembolism. They constituted only those with progressive bilateral foot and leg pain and clinical evidence for ongoing atheroembolism. All had failed to achieve relief with a course of antiplatelet drugs, and all required significant doses of narcotic analgesics for pain relief. The etiology of pain in atheroembolism is not understood, particularly the poor correlation of the degree of pain with the amount of atheromatous debris found in histological studies of affected tissue. Nevertheless, the clinical effectiveness of techniques which remove the offending source of atherosclerotic plaque is remarkable, and that beneficial effect was likewise found in the six patients we treated. Alter exclusion-bypass, reduction in pain in the legs and feet was noted by all patients within a few days. Sympathectomy may be an alternative or adjunctive therapy for continued pain from atheroembolism atler definitive arterial repair, but such a procedure was not needed by any patient in this series. Continued therapy for damaged toes consisted entirely of protective dressings in the case of diffusely blue toes, povidone-iodine ointment dressings to areas of wet gangrene, and dry dressings for dry gangrenous patches of skin. The foot lesions were noted to stabilize within one week of the bypass graft; gangrenous toe tips were allowed to autoamputate. One patient with massive forefoot gangrene eventually required below-knee amputation on one side, but he healed multiply damaged toes of the other foot. This treatment plan is based on an impression that most damaged skin from atheroembolism will spontaneously heal if the source of atheroembolism is eliminated and if the skin is protected. The most distant level of amputation is achieved by allowing this spontaneous healing to occur.
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CONCLUSION The six patients we treated with an axillobifemoral bypass were selected among those seen in our clinical practice with atheroembolism. They constituted only those with progressive bilateral foot and leg pain and clinical evidence for ongoing atheroembolism. All had failed to achieve relief with a course of antiplatelet drugs, and all required significant doses of narcotic analgesics for pain relief. The principle of exclusion and ligation may be used to achieve effective palliation of the painful and recurrent symptoms of atheroembolism of the lower extremities in the disseminated atheroembolism syndrome. The procedure is safe and carries little risk in patients with extensive cardiopulmonary disease and with a short life expectancy.
ACKNOWLEDGMENT We would like to thank Donna Youmans, Rosamond Burnham, and Rosemary Phillips of the Medical Media Department at the Albany Veterans Administration Medical Center for their expert assistance in the preparation of this manuscript.
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