Thoracoabdominal aortic aneurysm in Cogan’s syndrome☆☆☆★
Article Outline
Abstract
Cogan’s syndrome is an autoimmune disease of unknown etiology, clinically manifested as non-syphilitic interstitial keratitis and audiovestibular symptoms. Increasing evidence suggests that Cogan’s may be a systemic vasculitis. In this report, we review the vascular manifestations of Cogan’s syndrome and report two cases of thoracoabdominal aortic aneurysm in patients with this disorder. (J Vasc Surg 1999;30:565-8.)
Cogan’s syndrome is an autoimmune disease characterized by non-syphilitic interstitial keratitis and bilateral nerve deafness. The ophthalmologic signs and symptoms include keratoconjunctivitis, decreased acuity, photophobia, and lacrimation. The vestibuloauditory features include vertigo, tinnitus, and deafness. The syndrome is found in young adults of either sex, and its etiology remains unknown.1 Cogan’s syndrome has also been associated with vasculitis of medium and large vessels, suggesting that the ophthalmic and auditory components might be local manifestations of a systemic vasculitis.2, 3 Previously described cardiovascular complications associated with Cogan’s syndrome include aortic valvular disease, stenotic lesions of visceral vessels, and polyarteritis nodosa.
We describe two cases of thoracoabdominal aortic aneurysm in association with Cogan’s syndrome.
CASE 1
A 38-year-old white man was referred in May 1989 with Cogan’s syndrome and bilateral lower-extremity claudication. In 1973, the patient had bilateral hearing loss and keratoconjunctivitis. Cogan’s syndrome was diagnosed, and therapy with systemic corticosteroids was initiated. His medical history was otherwise significant for hypertension. In 1974, at the age of 23 years, he underwent mechanical aortic valve replacement, and anticoagulation with warfarin was begun. At the time of referral, the patient had lower-extremity claudication at 100 feet, but no rest pain. The patient’s systemic vasculitis was inactive at the time of referral, with normal erythrocyte sedimentation rate while on steroid therapy.
On physical examination, the patient had a palpable abdominal aortic aneurysm. His right femoral artery pulse was 2+, with no left femoral pulse. He had a faintly palpable right popliteal pulse and no pedal pulses. Aneurysmal disease extending to the level of the celiac axis was suggested by means of a magnetic resonance imaging scan. The ascending aorta was dilated to 4.5 cm, but the arch and descending thoracic aorta appeared unremarkable. Type IV thoracoabdominal aortic aneurysm was confirmed by means of angiography (Fig 1).
Fig. 1.
Arteriogram demonstrating aneurysmal visceral aortic segment and associated critical bilateral renal artery stenoses (arrows ).
In June 1989, the patient underwent a staged reconstruction. Initially, a right hepatorenal bypass grafting procedure was performed (the modest celiac stenosis would be corrected at a second stage) with a greater saphenous vein graft and an end-to-end anastomosis to the distal right renal artery. Concern about dealing with the right renal artery reconstruction from a left flank approach, particularly because the pathology was not atherosclerosis (possibly obviating endarterectomy), led to this management plan. Four days later, the patient underwent type IV thoracoabdominal aneurysm repair. A 20-mm tube graft was anastomosed end-to-side to the distal thoracic aorta, interrupting the aorta below this anastomosis. An 18- by 9-mm bifurcation graft was sutured to the caudal tube graft, and distal anastomoses were performed to the profunda femoris arteries bilaterally. Side-arm grafts were then taken sequentially to the celiac axis (8 mm Dacron) and to the left renal artery (saphenous vein). Finally, the aorta was interrupted distal to the proximal thoracic aortic anastomosis.
The postoperative course was complicated by a lower-extremity deep venous thrombosis and subsequent anticoagulation, followed by a rectus sheath hematoma that required evacuation. The patient was discharged home 22 days after his thoracoabdominal aortic aneurysm repair. The patient declined to resume anticoagulation. In 1991, he had a minor stroke, thought to be related to his aortic valve, and warfarin was reinstituted. The patient recovered neurologically. At the time of this report, the patient was doing well. His Cogan’s syndrome remained clinically quiescent on oral steroid therapy.
CASE 2
A 17-year-old Greek woman was referred in January 1997 for treatment of a thoracoabdominal aneurysm. She was a previously healthy student in whom eye inflammation and deafness developed when she was 13 years old. She had been treated with steroid injections, and her vision improved. A diagnosis of Cogan’s disease was made in Germany. On further evaluation, an aortic aneurysm was discovered. A progressive enlargement of a type III thoracoabdominal aortic aneurysm was shown by means of aortography, computed tomography scanning, and magnetic resonance angiography (Fig 2).
Fig. 2.
A, Non-contrast computed tomography scan of type III thoracoabdominal aneurysm. B, Corresponding arteriogram demonstrates focal nature of the largest part of the aneurysm in the distal descending thoracic aorta. C, Abdominal aortic phase of arteriogram demonstrating severe proximal left renal artery stenosis (arrow ) and diffusely aneurysmal infrarenal aorta.
On examination, the patient had a blood pressure of 140/95 mm Hg. She had a II/VI diastolic murmur. No abdominal aneurysm was palpable. Peripheral pulses were intact. Her serum urea nitrogen concentration was 12, her creatinine level was 1.0 mg/dL, and her erythrocyte sedimentation rate was 62.
The patient was admitted with the diagnosis of Cogan’s syndrome, thoracoabdominal aneurysm, and severe left renal artery stenosis. She underwent Dacron graft replacement of the aneurysm with continuous epidural cooling.4 Findings at the time of operation included a 6.5-cm aneurysm of the descending thoracic aorta at the T8-9 level. The smaller aneurysmal abdominal aorta had marked surrounding inflammation; the aortic wall was composed of dense inflammatory tissue. A 16-mm collagen-impregnated Dacron graft with a 5-mm polytetrafluoroethylene side-arm left renal artery graft was implanted. Separate inclusion button anastomoses were performed to the intercostal vessels at T10-11; the celiac, superior mesenteric, and right renal arteries were reconstructed with a single inclusion button.
Severe, active arteritis with prominent lymphocytic infiltration was revealed by means of pathology (Fig 3).
Fig. 3.
A, Low-power photomicrograph of aorta, showing marked destruction of medial elastic tissue (original magnification 25×; elastic van Gieson). B, Medium-power photomicrograph of the medial-adventitial interface of the arterial specimen showing a dense inflammatory infiltrate consisting of mostly lymphocytes. Fibrosis and neovascularization is also present (original magnification 100×; hematoxylin and eosin).
DISCUSSION
Aortitis is an uncommon but recognized etiology of aortic aneurysms. In acute aortitis, a striking inflammatory cell reaction ensues, with thickening of the intima and a marked transmural inflammatory reaction (Fig 3). The media undergoes elastin destruction, with subsequent necrosis and scarring. The inflammation and necrosis of the aortic media predisposes it to elastin fiber degradation, an important mechanical defect in experimental aneurysms.5 Although in the past syphilis and tuberculosis were common causes of aortitis, the Takayasu variant and nonspecific giant cell aortitis are more familiar in contemporary practice. In our experience with thoracoabdominal aneurysms, 2% of cases are associated with aortitis documented by means of pathology, often with no known or suspected association with other vasculitides. 6 However, as exemplified in our cases, aortitis may predominate as the etiology of complex aneurysms in very young patients, after exclusion of patients with Marfan’s syndrome.
Our review of the English-language literature finds an increasing recognition that Cogan’s syndrome is not limited to ocular and auditory involvement, but is a systemic vasculitis. The initial reports of vascular involvement in Cogan’s syndrome concerned polyarteritis nodosa.1, 2 More recent reviews have confirmed the presence of vasculitis as a major component of the disease.7 In 1980, Haynes differentiated typical Cogan’s syndrome (acute interstitial keratitis, Menière-like vestibuloauditory dysfunction) from atypical Cogan’s syndrome (other significant inflammatory eye lesions, non-Menière-like or remote vestibuloauditory symptoms). Aortic insufficiency was seen largely in typical Cogan’s syndrome, whereas biopsy-proven vasculitis occurred in atypical Cogan’s syndrome. Atypical Cogan’s syndrome frequently overlapped other rheumatologic syndromes, was associated with vasculitis in 21% of cases, and presented a less favorable prognosis than typical Cogan’s syndrome.8
In 1986, Vollerstein reviewed 78 cases of Cogan’s syndrome.9 Aortic insufficiency, the most severe cardiac lesion, developed in 11 patients. Twelve patients had vasculitis documented by means of angiography (n = 5) or biopsy (n = 7). The median length of time from syndrome onset to vasculitis diagnosis was 7 months. One of the cases documented by means of angiogram, previously reported by La Raja, 10 described Cogan’s syndrome associated with mesenteric vascular insufficiency.9 The authors briefly mention that the autopsy of one patient, several years after the onset of Cogan’s syndrome, demonstrated generalized aortic dilatation. Also, fatal renal artery rupture in a patient with aneurysmal endothelial plaques in the aorta and carotid arteries has been reported in a patient with Cogan’s syndrome.11
Although the cause and pathogenesis of Cogan’s syndrome remain unknown, the inflammatory findings and associated vasculitis suggest either infection or an autoimmune process. The present report of thoracoabdominal aneurysm in Cogan’s syndrome adds aortitis with subsequent aneurysm formation to the vascular pathology attributed to this entity. Our second case provided pathologic verification of the presumed pathogenesis of aneurysm in patients afflicted with aortitis. The detection of extensive aneurysm in young patients should lead to investigation for vasculitis and aortitis, and an important treatment issue in these patients is the requirement for corticosteroid therapy. Such therapy, although generally necessary in the acute phase of aortitis, may lead to expansion and rupture in patients with established aneurysms.12 The management of thoracoabdominal aortic aneurysm resulting from aortitis is graft replacement, dictated by (1) the frequent requirement for chronic steroid therapy, (2) afflicted patients often being young and otherwise healthy, and (3) the desirability of correcting renovascular and mesenteric occlusive lesions that often occur when the aortitis affects the visceral aortic segment.
References
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☆ From the Division of Vascular Surgery, Department of Surgery, Massachusetts General Hospital, Harvard Medical School.
☆☆ Reprint requests: Richard P. Cambria, MD, Massachusetts General Hospital, Boston, MA 02114.
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© 1999 Society for Vascular Surgery and International Society for Cardiovascular Surgery, North American Chapter. Published by Elsevier Inc. All rights reserved.
