Asian Cardiovasc Thorac Ann 2006;14:150-152
© 2006 Asia Publishing EXchange Ltd
Surgical Repair of a Syphilitic Aneurysm of the Distal Arch and Descending Aorta
Masayoshi Umesue, MD,
Manoj Durairaj, MCh,
George Matalanis, FRACS,
Sarah Parsons, MBBS1
Department of Cardiac Surgery
1 Department of Anatomical Pathology, Austin Hospital, Melbourne, Australia
For reprint information contact: Masayoshi Umesue, MD Tel: 61 3 9496 5453 Fax: 61 3 9459 6220 Email: umesue{at}xa2.so-net.ne.jp, Department of Cardiac Surgery, Austin Hospital, HSB-5, Studley Road, Heidelberg, Melbourne, Victoria 3084, Australia.
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ABSTRACT
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Cardiovascular syphilis, which used to be a well-recognized manifestation of tertiary syphilis, has become a rarity. In this report we describe a 47-year-old man presenting with an aneurysm of the distal arch and proximal descending aorta, a somewhat unusual presentation for a syphilitic aneurysm, and discuss the clinical features of cardiovascular syphilis.
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INTRODUCTION
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The decreased incidence of luetic infection and the early use of antibiotics to treat it have reduced the observed cases of late-stage syphilis in Western society to the extent that tertiary manifestations may be misdiagnosed. Major cardiovascular features of tertiary syphilis are asymptomatic aortitis, aortic insufficiency, coronary ostial stenosis, and aortic aneurysm.1 We describe the surgical treatment of a syphilitic aneurysm of the distal arch and proximal descending thoracic aorta, which is a relatively rare manifestation of syphilitic aortic aneurysm.
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CASE REPORT
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A 47-year-old man presented to his local doctor complaining of bilateral leg weakness, blurred vision and forgetfulness. He was diagnosed with syphilis and treated with intravenous benzyl penicillin for 2 weeks. The visual symptoms almost completely resolved following the treatment, but the leg weakness showed no improvement. He was referred to us for the treatment of thoracic aneurysm, which was found incidentally on chest X-Ray. There was no significant medical history, in particular, he was not hypertensive, diabetic or hyperlipidemic, and was not a smoker. The patient did not complain of hoarseness or dysphagia. On examination he had a waddling gait. Cranial nerve and upper limbs were normal but there was mild bilateral lower limb weakness with normal sensation. His blood pressure was 115/88 mm Hg, heart sounds were normal and there were no features of Marfan’s syndrome. A treponemal serologic test revealed a positive rapid plasma reagin (RPR). Positive treponemal hemagglutination (TPHA) and syphilis total antibody were detected. An aortograph, magnetic resonance (MR) angiogram and computed tomography (CT) scan showed a fusiform aneurysm of the thoracic aorta extending from immediately after the left subclavian artery to the middle of the descending aorta (Figure 1A
). The maximum diameter was 8 cm and it contained thick mural thrombus (Figure 1B). The ascending aorta was slightly dilated. Coronary angiogram showed normal coronary arteries including normal coronary ostia.
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Figure 1. (A) MR angiogram shows the thoracic aneurysm starting immediately after the left subclavian artery and extending through to the middle of the descending aorta. The arrow points to the left subclavian artery, (B) CT scan with intravenous contrast shows the distal arch aneurysm with markedly thick mural thrombus (Arrows).
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Surgical repair of the thoracic aneurysm was performed via a left thoracotomy using cardiopulmonary bypass. Aprotinin was given during the surgery. Cardiopulmonary bypass was established using an arterial cannula inserted into the left femoral artery and a long venous cannula inserted via the left femoral vein with its tip positioned in the right atrium. The chest was opened through a left posterolateral thoracotomy through the 4th intercostal space. There was a large aneurysm 8 cm in diameter extending from the distal arch to the junction of the upper and the middle third of the descending thoracic aorta. Extensive adventitial adhesions were seen between the aneurysm and the surrounding structures including the left vagus nerve. Leaving it attached to a ring of the aortic aneurysm sack preserved the nerve. The aneurysm contained thick laminated thrombus. The intercostal arteries originating from the aneurysm were chronically occluded. Upon reaching a core temperature of 20°C the distal descending aorta was clamped and the aneurysm was incised longitudinally. Antegrade cerebral perfusion was established via three separate 12 French Foley catheters inserted into the innominate artery, the left carotid artery and the left subclavian artery. At completion of the anastomosis of a 30 mm gelatin impregnated woven vascular prosthesis (GelweaveTM, Sulzer Vascutec, Renfreshire, Scotland) to the distal arch, cerebral and coronary perfusion was started through a perfusion side graft which had been pre-attached to the prosthesis. This graft was anastomosed to the distal descending aorta, and rewarming was initiated. Coronary and cerebral exclusion time, cardiopulmonary bypass time, and operation time were 14 minutes, 3 hours 52 minutes, and 6 hours, respectively.
Macroscopic examination showed a roughened and hemorrhagic intimal surface. A thrombus 7 to 25 mm thick was identified, containing dark areas of lamination alternating with pale areas. The external aspect was pale with hemorrhagic areas. Microscopic examination of the aortic wall revealed features consistent with an aneurysm secondary to syphilis, including adventitial lymphocytic infiltrate, obliterative endarteritis and fibrosis. Atherosclerotic changes were superimposed. No bacteria, fungi or spirochetes were seen with special stains (Figure 2). The postoperative course was uneventful. Chest drains were removed 41 hours after the operation and postoperative bleeding was 730 mL in total. The patient was extubated at 17 hours, with a normal voice and cough reflex. He was discharged from hospital 5 days after the operation. The patient will be followed-up closely, with special care to detect development of aortic insufficiency or coronary ischemia due to coronary ostial stenosis.
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Figure 2. Photomicrograph of aortic wall showing perivascular infiltrate of plasma cells and lymphocytes, obliterate endarteritis and fibrosis (hematoxylin and eosin stain original magnification x 100).
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DISCUSSION
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Cardiovascular syphilis, a common disorder in the pre-antibiotic era, has dramatically reduced in incidence owing to the introduction of penicillin and improvement in public hygiene. The reported incidence of syphilis was 10.4 per 100,000 in the Australian population according to the "Nationwide surveillance of communicable disease" in 1999.2 Given the decreased number of luetic patients, early recognition of the disease and the efficacy of antibiotic therapy, tertiary syphilis may be very rare in most doctors’ experiences, especially in developed countries. The underlying cardiovascular pathology of tertiary syphilis is the consequence of aortitis. The most common complication of syphilitic aortitis is aortic valvular insufficiency, the second is coronary ostial stenosis and the least common is aortic aneurysm.1 Of the syphilitic aneurysms, the most frequently involved segment is the ascending thoracic aorta (50%), next, the aortic arch (30–40%), and last, the descending aorta (10–15%).1 Syphilitic aneurysms of the abdominal aorta are quite uncommon.3 The tendency of proximal involvement is considered to be secondary to the lodgement of the spirochetes within vasa vasora, which are more frequent in the ascending aorta and aortic arch.1 Saccular aneurysms are more frequent than fusiform among syphilitic aneurysms.4 The wall of the aneurysm is thick as a result of extensive destruction and fibrosis and whilst this is considered to be a protection against dissection,1 it may still occur.5
When patients present with symptoms related to a syphilitic aneurysm, the progression tends to be rapid with a two-year mortality of 80%,1 hence surgical repair must not be delayed. The surgical strategy, including endovascular stenting for the syphilitic aneurysm, is basically similar to that of the atherosclerotic aneurysm. In our case, there was no distance between the left subclavian artery and the aneurysm which contained substantial mural thrombus, hence endovascular stenting was not considered safe. Consequently, operative repair was conducted under deep hypothermia and circulatory arrest. Nevertheless, endovascular stent-grafting covering the origin of the left subclavian artery with or without a left carotid-to-subclavian bypass might have been a possible alternative treatment.
In our patient, symptoms were not related to the thoracic aneurysm, and the huge aneurysm was found coincidentally on X-Ray. Interestingly, linear calcification of the ascending aorta, which is considered a useful diagnostic sign of syphilitic aortitis,4 was not seen on the X-Ray. The aneurysm was surgically treated after the completion of the penicillin treatment and the histopathological test of the aortic wall confirmed the syphilitic aneurysm. Other preoperative investigations, including serological tests, were consistent with past syphilitic infection. The thoracic aneurysm was thought to be a syphilitic aneurysm even though the location and shape of the aneurysm were relatively unusual. In particular, cardiovascular investigations did not show aortic insufficiency or coronary ostial stenosis, which are the more common manifestations of cardiovascular syphilis. Although syphilic aneurysms are rare, syphilis should still be considered as a differential diagnosis of aortic aneurysm, especially when the patient does not have manifestations of Marfan’s syndrome, and has no risk factors for atherosclerosis, as in this case.
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REFERENCES
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- Jackman JD Jr, Radolf JD. Cardiovascular syphilis. Am J Med 1989;87:425–33.[Medline]
- Communicable Diseases – Australia. Australia’s notifiable disease status, 1999: Annual report of the National Notifiable Diseases Surveillance System. Roche P, Spencer J, Lin M, et al. Communicable Diseases Intelligence 25:4;2001.
- Cina G, Ferrante AM, Carbone A, Tulli A, Casale S, Viola G. Syphilitic aneurysm of the abdominal aorta. Eur J Dermatol 1999;9:399–401.[Medline]
- Dinsmore RE, Jang GC. Roentgen diagnosis of aortic disease. Prog Cardiovasc Dis 1973;16:151–85.[Medline]
- Kellett MW, Young GR, Fletcher NA. Paraparesis due to syphilitic aortic dissection. Neurology 1997;48:221–3.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
