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Aortic Aneurysm in a Patient with Syphilis-Related Spinal Pain and Paraplegia

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Aortic Aneurysm in a Patient with Syphilis-Related Spinal Pain and Paraplegia Case report Reumatologia 2017; 55, 3: 151–153 DOI: https://doi.org/10.5114/reum.2017.68916 Aortic aneurysm in a patient with syphilis-related spinal pain and paraplegia Daniel Brito de Araujo1, Danise Senna Oliveira1, Rodrigo Kraft Rovere2, Umberto Lopes de Oliveira Filho1 1Universidade Federal de Pelotas, Brazil 2Oncology Department – Santo Antonio Hospital, Blumenau, Brazil Abstract The tertiary stage of syphilis is nowadays extremely rare, showing predilection for the cardiovascu- lar and nervous systems. A 57-year-old Caucasian man sought medical assistance due to back pain that evolved to paraple- gia of the lower limbs. A thoracic CT scan demonstrated an important aneurysmatic lesion of the descending thoracic aorta causing erosion of the vertebral bodies and VDRL and FTA-abs positivity. Although rare, syphilitic aortitis, the hallmark of cardiovascular syphilis, should be considered in the differential diagnosis in patients with thoracic aneurysm when in the absence of classic risk factors for atherosclerosis, especially in cases that progress with erosion of vertebral bodies. Key words: back pain, tertiary syphilis, syphilitic aneurysm. Introduction In non-treated syphilis, aortitis occurs in over 70% Back pain is one of the most common reasons for of cases, with such symptoms as aortic regurgitation, visiting a clinician, but less than 5% will have a serious stenosis, and aortic aneurysm occurring in up to 15% of underlying systemic pathology such as infection or ma- patients [2, 5–7]. Classically, syphilitic aneurysms occur lignancy [1]. in 90% of cases on the thoracic aorta, and in 10% in the Syphilis is a disease known for a long time, which abdominal aorta [3, 7–9]. Infection of aortic wall devel- peaked during the Second World War, with a swift de- ops during the secondary or bacteraemic phase of syphi- cline after the arising of penicillin, but it remains one of lis, having a latent period from infection until the clinical the most prevalent sexually transmitted diseases [2]. It presentation ranging from 5 to 50 years [5, 8]. is a chronic systemic affection originated by Treponema For unknown reasons T. pallidum, after infecting the pallidum infection, which may be either congenitally or body and spreading through vascular dissemination, sexually transmitted, alternating activity and latency pe- may cause an inflammatory process in the vasa vaso- riods during the time span of the infection. rum in the inner layer of the aorta leading to strictures. Due to widespread use of antibiotics, the tertiary There is also obstruction due to granuloma formation stage of syphilis is currently extremely rare, being the and focal necrosis of the elastic and muscular layers ad cardiovascular and central nervous systems the most substitution for fibrotic tissue that afterwards turns into commonly affected sites, even if asymptomatic [2–4]. calcification (syphilitic aorta) [10, 11]. This process may Among the cardiovascular complications caused by carry on and generate extreme strictures with saccular tertiary syphilis, aortitis is the most common, in which or fusiform aneurysms [8–10]. Due to intense scarring 10% of patients develop meaningful critical complica- of the media layer, rupture resulting from a dissection tions [5]. is highly unlikely to occur, as opposed as non-syphilitic Address for correspondence: Daniel Brito de Araujo, Universidade Federal de Pelotas, Praca Coronel Pedro Osorio 158, AP 32, 96015-010 Pelotas, Brazil, e-mail: [email protected] Submitted: 28.04.2017; Accepted: 28.06.2017 Reumatologia 2017; 55/3 152 Daniel Brito de Araujo, Danise Senna Oliveira, Rodrigo Kraft Rovere, Umberto Lopes de Oliveira Filho aortic aneurysms, in which the rupture is the main cause During the first half of the 20th century, thoracic aor- of death. tic aneurysms were much more common than their ab- dominal counterparts, due to the prevalence of syphilitic Case report thoracic aortic aneurysms [3, 11]. In 1952 the thoracic/ abdominal aneurysms ratio was 2 : 1, until 1964 when A 57-year-old Caucasian man, living in the country- this ratio equalised, making the syphilitic cardiovascular side, sought medical assistance referring to back pain involvement very rare in current days [8]. that had started 6 months before, with progressive During the pre-antibiotic era, about one third of un- worsening in the last 45 days, until it became excruci- treated syphilitic patients would develop recognisable ating, disabling the patient from performing minimal tertiary syphilis clinically. Sclerosis initialis or ulcus du- tasks. The clinical picture had evolved in a 7-day time rum, typical for first period of syphilis, may not occur or span with symmetrical paraesthesia and subsequent- be unusual in appearance, or may be localised to a site ly paraplegia on lower limbs. He denied weight loss or that is not available for medical examination, but they other constitutional symptoms. As past history, he re- also need to be differentiated from changes such as ferred to unprotected sexual activity during his youth. herpes simplex or Haemophilus ducreyi-induced ulcer, In the physical examination, we found pain to minimal and the detection of diseases can occur in its subse- movement and light touch of vertebral bodies and aor- quent stages. Confirmation of syphilis in patients with tic holosystolic murmur without fremitus. During the VDRL-positive results, with or without typical symp- investigation, a thoracic CT scan was performed, which toms, is performed by FTA-ABS or TPHA. demonstrated an important aneurysmatic lesion of the Today, in industrialised countries, specific treatment descending thoracic aorta causing erosion of the ver- and disseminated use of antibiotics in the treatment of tebral bodies corresponding to T4 to T7 (Fig. 1). It also other infections has almost wiped out tertiary syphilis, showed an important cardiomegaly and bilateral pleu- except for cases of neurologic involvement in HIV-infect- ral effusion. The haemodynamic study diagnosed a se- ed patients [4]. vere aortic valve insufficiency and a descending aorta The tertiary stage of syphilis is a non-contagious aneurysm of the thoracoabdominal portion. The serum form of the disease, occurring any time after the sec- marker Venereal Disease Research Laboratory (VDRL) ondary stage, affecting about 35% of untreated patients test was 1 : 64 and FTA-abs was positive. Patient was [12]. The tissue reactivity characterised by vasculitis and referred for surgical treatment; unfortunately, the lesion necrosis is severe and highly suggestive of hypersensi- had a spontaneous rupture before any action was tak- tivity phenomena. In the past, the most common form en. Due to the known cause of death, autopsy was not of tertiary stage was the gumma, normally a benign performed. granulomatous lesion, extremely rare nowadays. Cardiovascular syphilis is characterised by obliter- Discussion ating endarteritis, which may affect small-calibre vases We describe here a rare case of a huge pulsatile irrespective of location, but it is much more devastat- syphilitic aortic aneurysm presenting with back pain as ing whenever it affects the aorta’s vasa vasorum, jeop- the first manifestation and evolving with neurological ardising the blood supply of the aortic wall, leading to dysfunction due to osteolytic vertebral erosion. destruction of the elastic tissue and weakening of the Fig. 1. Thorax CT showing an aneurysmatic lesion of the descending thoracic aorta causing erosion of the vertebral bodies corresponding to T4 to T7. Reumatologia 2017; 55/3 Syphilitic thoracic aorta aneurysm 153 medial aortic tunic, particularly in the ascendant and References transverse segments of the thoracic aortic arc [3, 8–10, 1. Deyo RA, Rainville J, Kent DL. What can the history and physi- 12]. The macroscopic aspect is classically described as cal examination tell us about low back pain? JAMA 1992; 268: “tree bark” due to inner layer alterations and parietal 760-765. calcification [7, 10, 11]. 2. Hofmann-Wellenhof R, Domej W, Schmid C, et al. Mediastinal The cardiovascular syphilitic lesions become symp- mass caused by syphilitic aortitis. Thorax 1993; 48: 568-569. tomatic in only 10% of untreated patients. Among the 3. Kampmeier RH, Morgan HJ. The specific treatment of syphilitic cardiovascular lesions, 85% of patients present with aortitis. Circulation 1952; 5: 771-778. aortic insufficiency, coronary ostium stenosis in 25 to 4. Varela P, Alves R, Velho G, et al. Two recent cases of tertiary syphilis. Eur J Dermatol 1999; 9: 300-302. 30%, and the presence of aneurysms in 5 to 10%, 75% of 5. Jackman JD, Radolf JD. Cardiovascular syphilis. Am J Med these being of saccular morphology [3]. 1989; 87: 425-433. As documented in our case, a special particularity, 6. Tong SY, Haqqani H, Street AC. A pox on the heart: five cases even though rare, of the aortic aneurysms is the pulsa- of cardiovascular syphilis. Med J Aust 2006; 184: 241-243. tile destruction of nearby structures such as sternum, 7. Heggtveit HA. Syphilitic aortitis. A clinicopathologic autop- ribs, vertebrae, and skin [9]. Heggtveit [7] analysed 100 sy study of 100 cases, 1950 to 1960. Circulation 1964; 29: syphilitic aortitis cases, and in only two were there verte- 346-355. bral bodies erosions. Leung et al. found the same lesion 8. Waikittipong S. Syphilitic aortic aneurysm presenting with up- in 20% of syphilitic aneurysm patients [13]. per airway obstruction. Asian Cardiovasc Thorac Ann 2012; 20: 575-577. The tertiary syphilis diagnosis may be difficult be- 9. Panday S, Hishikar A, Karbhase J. Rupture of syphilitic aneu- cause clinical features may be deceptive, having strong rysm of ascending aorta into main pulmonary artery: suc- similarities with other granulomatous diseases, and the cessful emergency repair. J Thorac Cardiovasc Surg 1982; 83: serologic titles can be low or negative [4]. Diagnostic ex- 470-471. ams include treponaemic (FTA-abs) and non-treponaemic 10. Kumar V, Robbins SL. Robbins basic pathology. 8th ed. Saun- tests (VDRL). The VDRL sensibility depends on the level of ders/Elsevier, Philadelphia 2007.
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