Clinical Radiology (1981) 32, 461-466 © t981 Royal College of Radiologists
0009-9260/81/00830461502.00
Tuberculous Aortoarteritis ARCOT GAJARAJ* and SOLOMAN VICTOR
Barnard Institute o f Radiology and Cancer and Cardiovascular and Thoracic Surgery Department, Madras Medical College, Madras 60003, lndia Tuberculous aortoarteritis is a distinct entity. Despite the still wide prevalence of active tuberculosis in developing countries, tuberculous aort oarteritis appears to be rare. The vessel is often involved by a direct extension of the disease from adjacent tuberculous tissue. Occasionally it may result from blood-borne seedlings from an active distant focus. True and false aneurysms are the common manifestations. Stenosing and/or constricting types of lesions and perivascular fibrosis have been encountered by us. The probable pathogenesis is discussed with illustrative cases. Despite the wide prevalence of active tuberculosis in developing countries there is a low incidence of tuberculous aortoarteritis. Almost all the cases reviewed in the literature are from the West. The earliest report dates back to 1882 by Weigert quoted by Silbergleit et al. (1965). Other early reports are those of Haythorn (1913) and Dafoe (1925). The literature is punctuated since then by periodic case reports, the most recent being that of Efremedis et al. (1976). Further, all the cases are either true or false aneurysms of the aorta or its large branches. The purpose of this paper is to present illustrative cases from India and draw attention to a stenosing and/or constricting type of pathology and perivascular fibrosis.
stenosing types of lesions. In one of the patients, a mate aged 18 with spinal tuberculosis, there was extensive mediastinal fibrosis (Fig. 1) in addition to the paravertebral abscess (Fig. 2a). The patient presented with dysphagia. Barium swallow studies showed obstruction of the oesophagus (Fig. 2b, c). Angiography of the arch and descending aorta demonstrated a segment showing an irregular outline. The major branches arising from the arch were compressed and stretched (Fig. 3). A lymphangiogram demonstrated periaortic nodes in the abdomen and medistinum. Surgical exploration conftrmed
METHODS Over the course of about five years amongst the cases referred to the institute for investigation of vascular disorders, we encountered 6 cases of tuberculous aortoarteritis. All the patients were evaluated with angiography besides other pertinent radiological investigations as considered useful. The series comprised of five males and one female. The youngest was 18 and the oldest was 35 years of age. In three of the six cases there was evidence of para-aortic tuberculous abscesses two of which were secondary to spinal tuberculosis and the third to caseating paravascular tuberculous nodes. In the other three cases, the aortoarteritis was associated with renal tuberculosis. The Manteaux test was strongly positive in all and the smears from the cold abscesses and/or caseating, lymphnodes demonstrated acid fast bacilli. An interesting observation in all the cases was the demonstration of prodominently constricting or * Present address: Department of Radiology, PresbyterianUniversity Hospital, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA.
Fig. 1 - X-ray of the chest of a male patient aged 18 showing mediastinal widening due to fibrosis.
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the above findings. Decompression by release of fibrous strands and drainage of the abscess gave dramatic symptomatic relief. Another patient, a female aged 30, with spinal tuberculosis was referred with signs and symptoms of peripheral vascular disease, hypertension and cardiac decompensation. She had a dorsolumbar kyphosis and a tender pointing abscess. X-rays of the spine showed destruction and anterior wedging of the dorso-lumbar vertebral bodies (Fig. 4a, b). Angiography revealed irregularity of the outlines of the thoracic and abdominal aorta with segments of aneurysmal dilatation. The abdominal aorta and the left common iliac artery were narrowed with almost total stenosis. The left renal artery and the right common iliae arteries were completely occluded (Fig. 5a, b). A third patient, a young male age 24, was again evaluated for a history of intermittent claudication. The abdominal aortogram showed constriction of the lumen of the abdominal aorta with ill-defined outlines. Both kidneys were rotated on their axis with lateral displacement of the ureters. Laparotomy Fig. 2(a) - X-rays o f the dorsal spine o f the case shown in
Fig. 1. Demonstrating tuberculosis of the spine (-t>) and paravertebral abscess (white arrows). (b) (c) Barium swallow studies in the same patient showing obstruction of the oesophagus with proximal dilatation and irregularity.
(b)
(c)
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(a)
Fig. 3 - Aortogram o f patient shown in Figs 1 and 2 demonstrating the arch and descending aorta with a segment showing irregular outline (---~). The branches from the arch are compressed and stretched out. A few lymph nodes can also be identified (-4-~).
revealed the presence of caseating paravascular tuberculous nodes with fibrous adhesions and a cold abscess. Decompression by release of adhesions and drainage of the abscess gave symptomatic relief. In three patients the aortitis was associated with renal tuberculosis. The abdominal aorta was narrowed in all the cases, with almost total occlusion in one case (Fig. 6a). Two patients had a non-functioning kidney on one side. Selective arteriography of the autonephrectomised kidneys revealed marked attenuation of the intrarenal vessels (Fig. 6b). Both the patients underwent nephrectomy.
(b) Fig. 4 a , b - X-rays o f the spine of a female patient aged 30 showing tuberculosis o f the spine with destruction and anterior wedging o f the dorsolumbar vertebral bodies.
DISCUSSION Tuberculosis is still widely prevalent in the developing countries. A positive PPD reaction of about 66% in the Mexican population has been reported
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(a) (b) Fig. 5 - (a) Thoracic aortogram of patient shown in Fig. 4 revealing segments of constrictions, dilatations and irregularity of the outlines of the walls. (b) Abdominal aortogram of the same patient showing again segments of constriction, dilatation and irregularity - total occlusion of the left renal and right common iliac arteries. (Lupi et al., 1977). Eighty per cent of the adult population above 20 years of age is Mantoux positive in south India. Yet tuberculous aortoarteritis is rare and has not been reported to our knowledge from these areas. The vessel is often involved by a direct extension of the disease from adjacent tuberculous lymph nodes (Scott et al., 1949; Rob and Eastcott, 1955; German and Green, 1956). Volini et al. (1962) reported a case which spread from a tuberculous empyema. Cases of aortic involvement by seeding from active distant foci, or as a manifestation of a systemic disease have also been reviewed (Haythorn, 1913; Dafoe, 1925; Meehan et al., 1957). An analysis of our material shows that in three cases, the aorta appears to have been involved by a direct extension of the disease process from adjacent tuberculous abscesses, two of which are due to spinal tuberculosis and one to caseating tuberculous lymph nodes, and three to renal tuberculosis. Dafoe (1925), while
discussing his cases, postulated that in the aortoarteritis associated with renal tuberculosis, the paraaortic nodes below the coeliac axis and draining the kidneys get affected first and there is 'subsequent spread to the aorta. Alternatively the aorta may be involved by direct bacterial seeding in the wall. The disease process in the aorta in our cases is predominantly a stenosing or constricting type of pathology with segments of aneurysmal dilatations. Stenosing or constricting lesions with arterial and periaortic fibrosis are known to occur in small vessels coursing through tuberculosis cavities-in the lungs, tuberculous meningitis and tuberculous kidneys (Fig. 6b). It is conjectured that the obliterative arteritis at these sites are the result of a protective mechanism by the host, against dissemination of the disease (Volini et aL, 1962). In certain circumstances the protective mechanism may be incomplete in large vessels such as the aorta,
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(a)
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(b)
Fig. 6 - (a) Abdominal aortogram o f patient aged 35 years. Constriction and stenosis o f t h e abdominal aorta, the right renal and the left c o m m o n iliac arteries, a n d total occlusion of the right c o m m o n iliac artery. (b) Selective study of left renal artery reveals m a r k e d a t t e n u a t i o n o f the calibre of left main renal artery and the intrarenal vessels o f the autonephrectomised kidney.
particularly in persons who do not have immunity against the disease. In countries like India where tuberculosis is still rampant, the population is subject to repeated infections from childhood and development of natural immunity. This immune mechanism and the consequent vascular and perivascular response contribute perhaps to the stenosing type of lesions encountered by us as opposed to aneurysm formation in the cases reported in Western literature. The occurrence of perivascular fibrosis in aortic aneurysms and in specific and non-specific perivascular inflammatory processes has been well documented. Wieder and Robinowitz (1977)have demonstrated extensive fibrosis in cases of mediastinal histop!asmosis. Gajaraj et al. (1977) have reported stenosing aortoarteritis and perivascular fibrosis in a series of cases of arteritis associated with perivascular nonspecific lymphadenitis. Timely excision of the nodes and decompression of vessels by release of fibrous strands prevent the development of stenosing aortoarteritis. It is possible that in tuberculous aortoarteritis as well, irreversible changes can be prevented by similar management of the paravascular tuberculous process. Perivascular fibrosis in cases of aortic 33
aneurysms is not considered to be due to leakage of blood since haem pigment has not been demonstrated in the perivascular space (Abbott et al., 1973). The stenosing type of pathology draws into discussion the probable tuberculous aetiology attributed by some authors to Takayasu arteritis (Sen et al., 1962; Lupi et al., 1977). These authors have observed telltale evidence of previous tuberculotis infections, such as healed tuberculous pulmonary or extrapulmonary lesions or positive PPD reactions, in a high proportion of their cases. They consider that the stenosing aortoarteritis in Takayasu disease is an allergic or hypersensitive reaction to tuberculosis antigen from distant healed foci. Danaraj and Wong (1959, 1960) do not consider that the coincidence Of distant healed tuberculous lesions in cases of Takayasu arteritis has aetiological significance, particularly in patients from countries where a high percentage of the general population have positive Mantoux reaction. We ascribe to a similar view. In a series of 91 cases of the Takayasu type of aortoarteritis observed by us, only 23 had positive Mantoux reaction. None had active tuberculosis. Further the Takayasu type of aortoarteritis has a wide global distribution. It is increasingly
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r e p o r t e d f r o m countries where tuberculosis has been eradicated (Lande, 1976; Lande and Berkmen, 1976). It is, therefore, unlikely that tuberculosis has aetiological significance in Takayasu disease. We consider that tuberculous aortoarteritis is a distinct e n t i t y and w h e n manifest, it is either due to a direct spread f r o m an active adjacent t u b e r c u l o u s process or due to deposition of bacilli in the wall o f vessels f r o m an active distant focus, via t h e vasa vasorum.
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