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Thoracoabdominal and coronary arterial aneurysms in a young man with a history of Kawasaki disease
Thoracoabdominal and coronary arterial aneurysms in a young man with a history of Kawasaki disease Mladen Petrunic´, MD, PhD,a Nikša Drinkovic´, MD, PhD,b Ranka Štern-Padovan, MD, PhD,c Tomislav Meštrovic´, MD,a and Danijel Lovric´, MD,b Zagreb, Croatia We report a case of a large symptomatic thoracoabdominal aortic aneurysm in a 22-year-old man with a history of Kawasaki disease in childhood. According to multislice computed tomography scan findings, the aneurysm was classified as Crawford type III. Coronary angiography revealed a giant aneurysm of the left coronary artery and aneurysm of the circumflex artery. Functional tests for myocardial perfusion and function revealed no significant ischemic territories. Because of symptoms of imminent rupture, aneurysm resection and aortic reconstruction with a 26-mm zero porosity Dacron graft was performed and was successful. Cardiovascular consequences of Kawasaki disease are discussed with attention to the late sequelae. Indications for surgical treatment and importance of follow-up into adulthood are emphasized. ( J Vasc Surg 2009;50:1173-6.)
Kawasaki disease (KD) is a mucocutaneous lymph node syndrome first reported in Japan more than four decades ago1 and since then has been found worldwide. Most cases are reported in children aged ⬍5 years old, although KD may occur at any age, including in adults.2 The etiology remains unknown, and there is still no specific diagnostic test. The diagnosis is made using a constellation of classical clinical criteria that in isolation have poor sensitivity and specificity.3 The major pathologic feature of KD is generalized vasculitis with a predilection to coronary arteries that leads to aneurysm formation in approximately 20% to 30% of all patients.4 Coronary arteries are the predominant, but not exclusive, site for aneurysm formation. The incidence of peripheral arterial aneurysms is uncertain, and aortic aneurysms are extremely rare. About one-half to two-thirds of coronary aneurysms regress within a few years. Chronic aneurysms carry the risk for thrombosis, stenosis or occlusion, and myocardial infarction. Death from myocardial infarction occurs in about 1% to 2% of patients.5 Peripheral arterial and aortic aneurysms occur rarely but may lead to severe complications like rupture or acute ischemia. Although most KD aneurysms are diagnosed within the first few weeks after onset of the disease, some may become apparent months and years later, even in adulthood.6 CASE REPORT A 22-year-old man was referred due to a painful, large pulsatile abdominal mass. The pain had lasted for several days with spreadFrom the Department of Vascular Surgery,a the Clinic for Cardiovascular Diseases,b and the Department of Diagnostic and Interventional Radiology,c University Hospital Center Zagreb. Competition of interest: none. Reprint requests: Tomislav Meštrovic´, University Hospital Center, Kišpatic´eva 12, 10000 Zagreb, Croatia (e-mail: [email protected]). 0741-5214/$36.00 Copyright © 2009 by the Society for Vascular Surgery. doi:10.1016/j.jvs.2009.05.025
ing toward the back and groin. His medical history indicated that he was treated in the Pediatric Department at the age of 10 years because of prolonged fever, enlarged neck lymph nodes, oral mucositis, and conjunctivitis. Laboratory tests showed anemia, thrombocytosis, leucocytosis, and elevated erythrocyte sedimentation rate. Immunoglobulin (Ig) G, IgM, and IgE values were raised. Echocardiography revealed pericardial effusion. Renal insufficiency also occurred. On the basis of clinical and laboratory findings, KD was suspected and corticosteroid therapy was administered. After prednisolone administration, the patient’s condition improved and he was discharged from the hospital after 21 days of treatment. At the 4-month follow-up, the patient was well and without symptoms. His pericardial effusion regressed and laboratory findings returned to normal values. During childhood and adolescence he occasionally felt a pulsatile abdominal mass but did not seek medical attention. However, he practiced free climbing, remained well, and did not undergo further follow-up until the current referral. At the present admission, he was conscious, with normal breathing and without fever. Physical examination showed normal findings in the heart and lungs. Blood pressure was 95/60 mm Hg. A large pulsatile mass, moderately painful on palpation, was present in the umbilical and epigastric area. The spleen was slightly enlarged but not painful on palpation. No peripheral edema was seen. Peripheral pulses were present. Results of the routine laboratory tests were normal. Abdominal and chest x-ray imaging showed peculiarly formed mineral shadows in the left abdomen. Furthermore, ring-shaped mineral shadows were seen next to the left heart margin and within projection in the cranial segment of the right scapula. To clarify those findings, native and contrast-enhanced multislice computerized tomography (MSCT) of the thorax and abdomen was performed. It showed a large Crawford type III thoracoabdominal aneurysm (Fig 1). The maximum diameter of the aneurysm was 9 cm and the width of the aortic wall was 2 cm (Fig 1, lower right). A giant aneurysm of the left coronary artery was also revealed (measuring 3.2 ⫻ 2.5 cm), as well as a heavily calcified and occluded right coronary artery (Fig 1, upper right).
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Fig 1. Left, Sagittal multiplanar reconstruction (MPR) of an average contrast-enhanced computed tomography (CT) scan through the thoracoabdominal aorta. Upper right, Oblique MPR average contrast-enhanced CT at the level of the coronary arteries shows calcified walls of both coronary arteries (arrowheads), with a giant aneurysm of the left coronary artery (arrows). Lower right, a transverse nonenhanced CT at the infrarenal level shows thickened and calcified aneurysmal wall. Echocardiographic examination showed a slightly enlarged left ventricle (diameter, 5.8 cm) with preserved systolic function (ejection fraction, 70%), normal diastolic function, and no valvular disease. Coronary angiography showed the giant aneurysm of the distal left main coronary artery and the occlusion of proximal left anterior descending artery. The circumflex artery, which originated from the aneurysm, was also aneurysmatically changed (2.1 ⫻ 1.2 cm). The right coronary artery was proximally occluded with its own collateralization and collateralization from circumflex to the posterolateral branch, which gave collateral branches to the distal part of left anterior descending artery by way of the posterior descending artery (Fig 2). Myocardial perfusion was assessed by treadmill testing and by a 2-day stress test with a technetium-99m sestamibi single-photon emission computed tomography scan. Neither reversible nor irreversible accumulation defects were found. Because of the progression of the abdominal symptoms, the patient was scheduled for surgery. Intratracheal anesthesia was initiated and catheters were placed for cerebrospinal fluid drainage and for pressure measurement and urine output. A left thoracoretroperitoneal approach was used, entering the seventh intercostal space and the pararectal line. The large aneurysm involved all visceral and renal branches and extended to the aortic bifurcation. The underlying inflammatory disease, the periaortic scar formation, and severe calcification of the aneurysm wall made the exposure difficult. Having isolated the normal aorta at the level of pulmonary hilum, under anticoagulation we clamped the short segment of the descending thoracic aorta and created an oblique proximal anastomosis with a 26-mm collagen-coated Dacron graft. The anastomosis included two pairs of intercostal arteries.
Fig 2. A, Coronary angiography shows a giant partially thrombosed aneurysm of the distal left main trunk (arrows) and an aneurysm of the circumflex artery (arrowheads). B, A proximally occluded right coronary artery (arrow) and collateral branches to the distal right coronary and distal left anterior descending arteries are noted (arrowheads).
Clamps were moved distally after completion of the proximal anastomosis and the enormous aneurysmal sac was opened. A large amount of debris was removed, and visceral and renal orifices were identified. Celiac trunk, superior mesenteric artery (SMA), and right renal artery were implanted into one window of the graft by use of running 3-0 Prolene suture (Ethicon, Somerville, NJ). This segment was released after 34 minutes. The left renal artery was attached 4 cm distally by button technique. The infrarenal placement of the clamp was followed by removal of rough calcified lamellae at the aortic bifurcation and by an end-to-end anastomosis. Unidentified
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bleeding caused temporary hypotension. Further exploration showed injury of the left kidney, which had to be removed. After anticoagulation was reversed, the wound was closed by layers. The patient remained in the intensive care unit for 5 days, and gradually all parameters returned to normal. He was transferred to the ward and discharged from the hospital on postoperative day 15. A control MSCT angiography revealed normal position of the graft with all replanted arteries (Fig 3). At the 1-year follow-up, the patient was well and asymptomatic. Annual myocardial scintigraphy is planned to follow continuously his coronary status.
DISCUSSION The initial clinical presentation of KD is not unique. Some patients with prolonged fever and less than four of the classical clinical features have been diagnosed as having incomplete KD. For patients with a problem generally unseen in KD, such as renal impairment, the phrase “atypical Kawasaki disease” should be reserved.7 Nonspecific clinical features and evolving and incomplete or atypical presentation make early diagnosis and timely treatment difficult. Delays in diagnosis and treatment, which occur more frequently in older children, are associated with an increased risk of aneurysm formation. A study from Joffe et al8 showed that coronary aneurysms developed in 100% of infants. However, aneurysms developed in only 4% to 8% of patients who received intravenous immuno-␥-globulin in the early phase of KD.7 Coronary aneurysms in KD are predominantly in the proximal segments and at bifurcations and are often multiple. Lesions in coronary arteries appear to progress differently. Aneurysms in the right coronary artery (RCA) are prone to massive thrombosis, whereas those in the left coronary artery (LCA) are prone to progressive localized stenosis.9 About one-half to two-thirds of all coronary aneurysms regress over time, and that is a characteristic phenomenon in KD. This usually happens within 1 to 2 years.10 Aneurysms with inner diameter of ⬎8 mm are classified as giant.11 Giant aneurysms usually do not regress, but may become calcified, thrombosed, and stenosed or occluded, resulting in ischemic heart disease.12 However, the study from Hijazi et al13 showed that most of the chronic coronary aneurysms are associated with normal perfusion and ventricular function. Although the incidence and natural course of coronary aneurysms after KD are well documented in studies,10,11 related reports on peripheral arterial and aortic aneurysms are scarce. In the long-term follow-up study on the consequences of KD, 13 of 594 patients (2.2%) exhibited systemic artery aneurysms. Among these, there were 11 axillary, 9 common iliac, 7 internal iliac, and 4 subclavian artery aneurysms.6 Peripheral KD aneurysms also have a tendency to regress, similar to coronary aneurysms, but sometimes serious and life-threatening complications may occur. Deaths secondary to ruptured hepatic and femoral arterial aneurysms were reported.14,15 Progressive and severe leg ischemia occurred in a middle-aged man with a history
Fig 3. A postoperative contrast-enhanced computed tomography scan of the aorta shows the position of the graft with replanted arteries. CA, Celiac artery; SMA, superior mesenteric artery; RRA, right renal artery.
of KD due to a thrombosed popliteal aneurysm.16 Aortic aneurysms are rarely reported, although aortitis has occasionally been found in KD patients at autopsy.17 Most of the KD-related aortic aneurysms were found in children.18 Pathologic studies revealed thickened and calcified aneurysm wall, with atrophy of elastic media and the presence of the intraluminal thrombus.19 Such aneurysms are extremely rare in adults; therefore, pathologic studies in these patients are not available. Our patient had a large thoracoabdominal aortic aneurysm (TAAA). He was very young and free of atherosclerotic risk factors. His family medical history was negative for any aneurysmal disease. No clinical signs of connective tissue disorders such as Marfan or Ehlers-Danlos syndrome were present. Other causes for such uncommon pathology in young adults consist of noninfectious aortitis with predominant aortic involvement such as Takayasu disease and other aortitises with incidental involvement of the aorta. These include many of the autoimmune diseases, such as giant cell arteritis, rheumatoid arthritis, systemic lupus erythematosus; Sjögren, Reiter, Cogan, Kawasaki, and Be-
1176 Petrunic´ et al
hçet syndromes; and ankylosing spondilitis.20 Tuberous sclerosis has also been reported as a cause of thoracic aortic aneurysm in young adults.21 We want to emphasize that our patient did not receive immuno-␥-globulin in the early phase of the disease. Location and morphology (shape) of his coronary aneurysms were characteristic for KD. Intraoperative finding during resection of his TAAA and his medical history made us strongly believe that KD was the underlying pathology causing the late cardiovascular sequelae. Clinical signs of imminent TAAA rupture and aneurysm diameter made the surgery imperative. We considered options for a simultaneous surgical procedure of the patient’s TAAA and coronary pathology, then staged operation, and finally TAAA resection alone. Favorable long-term clinical results of coronary artery bypass grafting (CABG) using arterial conduits have been reported in children with KD.22 However, surgical revascularization for adult survivors of childhood KD has rarely been reported. Coronary revascularization in adults is recommended in patients with multiple giant coronary aneurysms with severe stenotic or occlusive lesions associated with territories of reversible ischemia.23 Surgery should also be considered in patients with recurrent myocardial infarction because the prognosis is unfavorable. Reports on aortic aneurysm surgery in adults due to KD are extremely rare. Staged AAA resection was reported in a man who previously underwent CABG for a coronary artery aneurysm complicated with stenosis and myocardial ischemia.24 Because KD aortic aneurysms are extremely rare, standard treatment protocols are not established. In our patient, tests for myocardial perfusion and function showed no significant abnormalities. His TAAA was 9 cm in diameter, and he had severe abdominal and back pain; therefore, we decided to treat his TAAA alone. Such a late appearance of cardiovascular sequelae long after childhood KD, as it happened in this patient as well as in published reports, suggests the need for prolonged followup of patients with KD, not only through childhood but also into adulthood. Indications for surgery should be based on individual approach and clinical judgment. REFERENCES 1. Kawasaki T. Acute febrile mucocutaneous lymph node syndrome with lymphoid involvement, with specific desquamation of the fingers and toes. Jpn J Allergy 1967;16:178-222. 2. Jackson JL, Kunkel MR, Libow L, Gates RH. Adult Kawasaki disease: report of two cases treated with intravenous gamma globulin. Arch Intern Med 1994;154:1398-405. 3. Gersony WM. Diagnosis and management of Kawasaki disease. JAMA 1991;265:2999-703. 4. Kusakawa S, Toshio A. Cardiovascular lesions in Kawasaki disease. In: Shiokaw Y, editor. Vascular lesions of collagen disease and related conditions. Baltimore: University Park Press; 1977. p. 273. 5. Kato H, Koike S, Tanaka C, Yokchi K, Yashioka F, Takeuchi S, et al. Coronary heart disease in children with Kawasaki disease. Jpn Circ J 1979;43:469-75.
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6. Kato H, Sugimura T, Akagi T, Sato N, Hashino K, Maeno Y, et al. Long-term consequences of Kawasaki disease. A 10- to 21- year follow up study of 594 patients. Circulation 1996;94:1379-85. 7. Newburger JW, Takahashi M, Gerber MA, Gevitz MH, Tami LY, Burns JC, et al. Diagnosis, treatment, and long term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young. American Heart Association. Circulation 2004;110;2747-71. 8. Joffe A, Kabani A, Jadaviji T. Atypical and complicated Kawasaki disease in infants. Do we need criteria? West J Med 1995;162:322-7. 9. Suzuki A, Kamiya T, Yasuo O, Kuroe K. Extended long term follow-up study of coronary arterial lesions in Kawasaki disease. J Am Coll Cardiol 1991;17:33A. 10. Kato H, Ichinose E, Yoshida F, Takechi T, Matsuaga S, Suzuki K, et al. Fate of coronary aneurysms in Kawasaki disease. Serial coronary angiography and long-term follow-up study. Am J Cardiol 1982;49:1758-66. 11. Akagi T, Rose V, Benson LN, Newman A, Freedom RM. Outcome of coronary artery aneurysm after Kawasaki disease. J Pediatr 1992;121: 689-94. 12. Inoue O, Akagi T, Kato H. Fate of giant coronary artery aneurysms in Kawasaki disease: long-term follow-up study. Circulation 1989; 80(suppl II):262. 13. Hijazi ZM, Udelson JE, Snapper H, Rhodes J, Marx GR, Schwartz SL, et al. Physiologic significance and chronic coronary aneurysms in patients with Kawasaki disease. J Am Coll Cardiol 1994;24:1633-8. 14. Lipson M, Amant M, Fonkalsrud E. Ruptured hepatic artery aneurysm and coronary artery aneurysm with myocardial infarction in a 14-yearold boy: new manifestation of the mucocutaneous lymph node syndrome. J Pediatr 1985;98:933-6. 15. Vizcaíno-Alarcón A, Arévalo-Salas A, Rodríguez-López AM, SadowinskiPine S. Kawasaki disease in Mexican children. Bol Med Hosp Infant Mex 1991;48:398-408. 16. Bradway MW, Drezner AD. Popliteal aneurysm presenting as acute thrombosis and ischemia in a middle-aged man with a history of Kawasaki disease. J Vasc Surg 1997;26:884-7. 17. Tanaka N, Sekimoto K, Fukushima T, Tokita H, Ueno Y, Naoe S. Pathological study of fatal MCLS cases of Kawasaki disease: relationship with infantile polyarteritis nodosa. In: Shiokawa Y, editor. Vascular lesions of collagen diseases and related conditions. Baltimore: University Park Press; 1977. p. 269. 18. Fuyama Y, Hamada R, Uehara R, Yano I, Fujiwara M, Matoba M, et al. Long-term follow up of abdominal aortic aneurysm complicating Kawasaki disease. Comparison of the effectiveness of different imaging methods. Acta Pediatr Jpn 1996;38:252-5. 19. Amano S, Hazama F, Hamashima Y. Pathology of Kawasaki disease: I. Pathology and morphogenesis of the vascular changes. Jpn Circ J 1979;43:633-43. 20. Scully RE, Mark EJ, McNeely WF, Ebeling SH. Case records of the Massachusetts general hospital. N Engl J Med 1999;340:635-41. 21. Jost CJ, Gloviczki P, Edwards WD, Stanson AW, Joyce JW, Pairolero PC. Aortic aneurysm in children and young adults with tuberous sclerosis: report of two cases and review of the literature. J Vasc Surg 2001;33:639-42. 22. Kitamura S, Kaneda Y, Seki T, Kenochi K, Endo M, Takeuchi Y. Long term outcome of myocardial revascularisation in patients with Kawasaki coronary artery disease. A multicenter cooperative study. J Thorac Cardiovasc Surg 1994;107:663-74. 23. Rozo JC, Jefferies JL, Eidem BW, Cook PJ. Kawasaki disease in the adult. A case report and review of the literature. Tex Heart Inst J 2004;31:160-4. 24. Masashi H, Etsuko T, Kenichi K, Shigeyuki E, Tagusari O, Kobayashi J, et al. Multiple giant coronary aneurysm with calcification: Atherosclerosis and coronary artery lesions due to Kawasaki disease compared by electron beam computed tomography. A case report. Prog Med 2004; 24:1689-93. Submitted Mar 11, 2009; accepted May 14, 2009.
Kawasaki disease (KD) is a mucocutaneous lymph node syndrome first reported in Japan more than four decades ago1 and since then has been found worldwide. Most cases are reported in children aged ⬍5 years old, although KD may occur at any age, including in adults.2 The etiology remains unknown, and there is still no specific diagnostic test. The diagnosis is made using a constellation of classical clinical criteria that in isolation have poor sensitivity and specificity.3 The major pathologic feature of KD is generalized vasculitis with a predilection to coronary arteries that leads to aneurysm formation in approximately 20% to 30% of all patients.4 Coronary arteries are the predominant, but not exclusive, site for aneurysm formation. The incidence of peripheral arterial aneurysms is uncertain, and aortic aneurysms are extremely rare. About one-half to two-thirds of coronary aneurysms regress within a few years. Chronic aneurysms carry the risk for thrombosis, stenosis or occlusion, and myocardial infarction. Death from myocardial infarction occurs in about 1% to 2% of patients.5 Peripheral arterial and aortic aneurysms occur rarely but may lead to severe complications like rupture or acute ischemia. Although most KD aneurysms are diagnosed within the first few weeks after onset of the disease, some may become apparent months and years later, even in adulthood.6 CASE REPORT A 22-year-old man was referred due to a painful, large pulsatile abdominal mass. The pain had lasted for several days with spreadFrom the Department of Vascular Surgery,a the Clinic for Cardiovascular Diseases,b and the Department of Diagnostic and Interventional Radiology,c University Hospital Center Zagreb. Competition of interest: none. Reprint requests: Tomislav Meštrovic´, University Hospital Center, Kišpatic´eva 12, 10000 Zagreb, Croatia (e-mail: [email protected]). 0741-5214/$36.00 Copyright © 2009 by the Society for Vascular Surgery. doi:10.1016/j.jvs.2009.05.025
ing toward the back and groin. His medical history indicated that he was treated in the Pediatric Department at the age of 10 years because of prolonged fever, enlarged neck lymph nodes, oral mucositis, and conjunctivitis. Laboratory tests showed anemia, thrombocytosis, leucocytosis, and elevated erythrocyte sedimentation rate. Immunoglobulin (Ig) G, IgM, and IgE values were raised. Echocardiography revealed pericardial effusion. Renal insufficiency also occurred. On the basis of clinical and laboratory findings, KD was suspected and corticosteroid therapy was administered. After prednisolone administration, the patient’s condition improved and he was discharged from the hospital after 21 days of treatment. At the 4-month follow-up, the patient was well and without symptoms. His pericardial effusion regressed and laboratory findings returned to normal values. During childhood and adolescence he occasionally felt a pulsatile abdominal mass but did not seek medical attention. However, he practiced free climbing, remained well, and did not undergo further follow-up until the current referral. At the present admission, he was conscious, with normal breathing and without fever. Physical examination showed normal findings in the heart and lungs. Blood pressure was 95/60 mm Hg. A large pulsatile mass, moderately painful on palpation, was present in the umbilical and epigastric area. The spleen was slightly enlarged but not painful on palpation. No peripheral edema was seen. Peripheral pulses were present. Results of the routine laboratory tests were normal. Abdominal and chest x-ray imaging showed peculiarly formed mineral shadows in the left abdomen. Furthermore, ring-shaped mineral shadows were seen next to the left heart margin and within projection in the cranial segment of the right scapula. To clarify those findings, native and contrast-enhanced multislice computerized tomography (MSCT) of the thorax and abdomen was performed. It showed a large Crawford type III thoracoabdominal aneurysm (Fig 1). The maximum diameter of the aneurysm was 9 cm and the width of the aortic wall was 2 cm (Fig 1, lower right). A giant aneurysm of the left coronary artery was also revealed (measuring 3.2 ⫻ 2.5 cm), as well as a heavily calcified and occluded right coronary artery (Fig 1, upper right).
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Fig 1. Left, Sagittal multiplanar reconstruction (MPR) of an average contrast-enhanced computed tomography (CT) scan through the thoracoabdominal aorta. Upper right, Oblique MPR average contrast-enhanced CT at the level of the coronary arteries shows calcified walls of both coronary arteries (arrowheads), with a giant aneurysm of the left coronary artery (arrows). Lower right, a transverse nonenhanced CT at the infrarenal level shows thickened and calcified aneurysmal wall. Echocardiographic examination showed a slightly enlarged left ventricle (diameter, 5.8 cm) with preserved systolic function (ejection fraction, 70%), normal diastolic function, and no valvular disease. Coronary angiography showed the giant aneurysm of the distal left main coronary artery and the occlusion of proximal left anterior descending artery. The circumflex artery, which originated from the aneurysm, was also aneurysmatically changed (2.1 ⫻ 1.2 cm). The right coronary artery was proximally occluded with its own collateralization and collateralization from circumflex to the posterolateral branch, which gave collateral branches to the distal part of left anterior descending artery by way of the posterior descending artery (Fig 2). Myocardial perfusion was assessed by treadmill testing and by a 2-day stress test with a technetium-99m sestamibi single-photon emission computed tomography scan. Neither reversible nor irreversible accumulation defects were found. Because of the progression of the abdominal symptoms, the patient was scheduled for surgery. Intratracheal anesthesia was initiated and catheters were placed for cerebrospinal fluid drainage and for pressure measurement and urine output. A left thoracoretroperitoneal approach was used, entering the seventh intercostal space and the pararectal line. The large aneurysm involved all visceral and renal branches and extended to the aortic bifurcation. The underlying inflammatory disease, the periaortic scar formation, and severe calcification of the aneurysm wall made the exposure difficult. Having isolated the normal aorta at the level of pulmonary hilum, under anticoagulation we clamped the short segment of the descending thoracic aorta and created an oblique proximal anastomosis with a 26-mm collagen-coated Dacron graft. The anastomosis included two pairs of intercostal arteries.
Fig 2. A, Coronary angiography shows a giant partially thrombosed aneurysm of the distal left main trunk (arrows) and an aneurysm of the circumflex artery (arrowheads). B, A proximally occluded right coronary artery (arrow) and collateral branches to the distal right coronary and distal left anterior descending arteries are noted (arrowheads).
Clamps were moved distally after completion of the proximal anastomosis and the enormous aneurysmal sac was opened. A large amount of debris was removed, and visceral and renal orifices were identified. Celiac trunk, superior mesenteric artery (SMA), and right renal artery were implanted into one window of the graft by use of running 3-0 Prolene suture (Ethicon, Somerville, NJ). This segment was released after 34 minutes. The left renal artery was attached 4 cm distally by button technique. The infrarenal placement of the clamp was followed by removal of rough calcified lamellae at the aortic bifurcation and by an end-to-end anastomosis. Unidentified
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bleeding caused temporary hypotension. Further exploration showed injury of the left kidney, which had to be removed. After anticoagulation was reversed, the wound was closed by layers. The patient remained in the intensive care unit for 5 days, and gradually all parameters returned to normal. He was transferred to the ward and discharged from the hospital on postoperative day 15. A control MSCT angiography revealed normal position of the graft with all replanted arteries (Fig 3). At the 1-year follow-up, the patient was well and asymptomatic. Annual myocardial scintigraphy is planned to follow continuously his coronary status.
DISCUSSION The initial clinical presentation of KD is not unique. Some patients with prolonged fever and less than four of the classical clinical features have been diagnosed as having incomplete KD. For patients with a problem generally unseen in KD, such as renal impairment, the phrase “atypical Kawasaki disease” should be reserved.7 Nonspecific clinical features and evolving and incomplete or atypical presentation make early diagnosis and timely treatment difficult. Delays in diagnosis and treatment, which occur more frequently in older children, are associated with an increased risk of aneurysm formation. A study from Joffe et al8 showed that coronary aneurysms developed in 100% of infants. However, aneurysms developed in only 4% to 8% of patients who received intravenous immuno-␥-globulin in the early phase of KD.7 Coronary aneurysms in KD are predominantly in the proximal segments and at bifurcations and are often multiple. Lesions in coronary arteries appear to progress differently. Aneurysms in the right coronary artery (RCA) are prone to massive thrombosis, whereas those in the left coronary artery (LCA) are prone to progressive localized stenosis.9 About one-half to two-thirds of all coronary aneurysms regress over time, and that is a characteristic phenomenon in KD. This usually happens within 1 to 2 years.10 Aneurysms with inner diameter of ⬎8 mm are classified as giant.11 Giant aneurysms usually do not regress, but may become calcified, thrombosed, and stenosed or occluded, resulting in ischemic heart disease.12 However, the study from Hijazi et al13 showed that most of the chronic coronary aneurysms are associated with normal perfusion and ventricular function. Although the incidence and natural course of coronary aneurysms after KD are well documented in studies,10,11 related reports on peripheral arterial and aortic aneurysms are scarce. In the long-term follow-up study on the consequences of KD, 13 of 594 patients (2.2%) exhibited systemic artery aneurysms. Among these, there were 11 axillary, 9 common iliac, 7 internal iliac, and 4 subclavian artery aneurysms.6 Peripheral KD aneurysms also have a tendency to regress, similar to coronary aneurysms, but sometimes serious and life-threatening complications may occur. Deaths secondary to ruptured hepatic and femoral arterial aneurysms were reported.14,15 Progressive and severe leg ischemia occurred in a middle-aged man with a history
Fig 3. A postoperative contrast-enhanced computed tomography scan of the aorta shows the position of the graft with replanted arteries. CA, Celiac artery; SMA, superior mesenteric artery; RRA, right renal artery.
of KD due to a thrombosed popliteal aneurysm.16 Aortic aneurysms are rarely reported, although aortitis has occasionally been found in KD patients at autopsy.17 Most of the KD-related aortic aneurysms were found in children.18 Pathologic studies revealed thickened and calcified aneurysm wall, with atrophy of elastic media and the presence of the intraluminal thrombus.19 Such aneurysms are extremely rare in adults; therefore, pathologic studies in these patients are not available. Our patient had a large thoracoabdominal aortic aneurysm (TAAA). He was very young and free of atherosclerotic risk factors. His family medical history was negative for any aneurysmal disease. No clinical signs of connective tissue disorders such as Marfan or Ehlers-Danlos syndrome were present. Other causes for such uncommon pathology in young adults consist of noninfectious aortitis with predominant aortic involvement such as Takayasu disease and other aortitises with incidental involvement of the aorta. These include many of the autoimmune diseases, such as giant cell arteritis, rheumatoid arthritis, systemic lupus erythematosus; Sjögren, Reiter, Cogan, Kawasaki, and Be-
1176 Petrunic´ et al
hçet syndromes; and ankylosing spondilitis.20 Tuberous sclerosis has also been reported as a cause of thoracic aortic aneurysm in young adults.21 We want to emphasize that our patient did not receive immuno-␥-globulin in the early phase of the disease. Location and morphology (shape) of his coronary aneurysms were characteristic for KD. Intraoperative finding during resection of his TAAA and his medical history made us strongly believe that KD was the underlying pathology causing the late cardiovascular sequelae. Clinical signs of imminent TAAA rupture and aneurysm diameter made the surgery imperative. We considered options for a simultaneous surgical procedure of the patient’s TAAA and coronary pathology, then staged operation, and finally TAAA resection alone. Favorable long-term clinical results of coronary artery bypass grafting (CABG) using arterial conduits have been reported in children with KD.22 However, surgical revascularization for adult survivors of childhood KD has rarely been reported. Coronary revascularization in adults is recommended in patients with multiple giant coronary aneurysms with severe stenotic or occlusive lesions associated with territories of reversible ischemia.23 Surgery should also be considered in patients with recurrent myocardial infarction because the prognosis is unfavorable. Reports on aortic aneurysm surgery in adults due to KD are extremely rare. Staged AAA resection was reported in a man who previously underwent CABG for a coronary artery aneurysm complicated with stenosis and myocardial ischemia.24 Because KD aortic aneurysms are extremely rare, standard treatment protocols are not established. In our patient, tests for myocardial perfusion and function showed no significant abnormalities. His TAAA was 9 cm in diameter, and he had severe abdominal and back pain; therefore, we decided to treat his TAAA alone. Such a late appearance of cardiovascular sequelae long after childhood KD, as it happened in this patient as well as in published reports, suggests the need for prolonged followup of patients with KD, not only through childhood but also into adulthood. Indications for surgery should be based on individual approach and clinical judgment. REFERENCES 1. Kawasaki T. Acute febrile mucocutaneous lymph node syndrome with lymphoid involvement, with specific desquamation of the fingers and toes. Jpn J Allergy 1967;16:178-222. 2. Jackson JL, Kunkel MR, Libow L, Gates RH. Adult Kawasaki disease: report of two cases treated with intravenous gamma globulin. Arch Intern Med 1994;154:1398-405. 3. Gersony WM. Diagnosis and management of Kawasaki disease. JAMA 1991;265:2999-703. 4. Kusakawa S, Toshio A. Cardiovascular lesions in Kawasaki disease. In: Shiokaw Y, editor. Vascular lesions of collagen disease and related conditions. Baltimore: University Park Press; 1977. p. 273. 5. Kato H, Koike S, Tanaka C, Yokchi K, Yashioka F, Takeuchi S, et al. Coronary heart disease in children with Kawasaki disease. Jpn Circ J 1979;43:469-75.
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