Syphilitic aortitis: still a current common cause of aneurysm of the tubular portion of ascending aorta.

Aortic syphilis today is infrequently diagnosed clinically. Described herein are findings in 5 women who had resection of a fusiform aneurysm of the tubular portion of ascending aorta, and examination of the wall of the aneurysm disclosed classic features of aortic syphilis. The 5 patients were among 36 who had ascending aortic operations at Baylor University Medical Center in Dallas in 2018 and early 2019. Syphilitic aneurysm in each spared the sinus portion and involved diffusely the tubular portion of ascending aorta, beginning at the sinotubular junction. The aneurysmal wall was thicker than normal because of thickening of both intima and adventitia. The latter contained foci of lymphocytes and plasmacytes and thickened and narrowed vasa vasora. The media was disrupted by fibrous scars, which weakened the integrity of the aorta. Aortitis of the tubular portion of ascending aorta in syphilis is a diffuse process, but often is mistakenly called "atherosclerosis" which, when present in this portion of aorta, can be extensive but is focal. Aortic syphilis is important to diagnose so that patients can receive antibiotic therapy to delay, prevent, or treat neurosyphilis, a common accompaniment of aortic syphilis.

Classical syphilitic lesions from the museum.

Syphilis is an increasingly diagnosed venereal disease which has four distinctive stages that may last over decades if appropriate treatment is not given. Review of the files of the Pathology Museum in the Faculty of Medicine at The University of Adelaide revealed three cases with classical cardiovascular and neurological findings. Case 1: An 80-year-old man with a large syphilitic aneurysm of the ascending aorta with a smaller aneurysm of the proximal descending aorta. Case 2: A 56-year-old man with chronic syphilitic meningoencephalitis with cerebral atrophy. Case 3: A 77-year-old man with tabes dorsalis. Given the increase in cases coming to medical attention in recent years due to high-risk sexual activity, migration, travel and reduced access to medical treatment, an awareness of the classical features of syphilis is appropriate as some cases will undoubtedly require medicolegal evaluation.

Syphilis-Cardiovascular Manifestations of the Great Imitator.

Rates of syphilis are beginning to once again increase, with the World Health Organization estimating that in recent years there were 12 million new cases of syphilis each year; in 2002, syphilis was responsible for 0.3% of deaths globally. At-risk groups include young males (20-29 years), prisoners, and sex workers. Increased rates in young females have elevated the numbers of congenital cases. Review of the University of Adelaide Pathology Archive revealed four cases with significant pathology, which included cardiac gummas and aortic arch aneurysms. These cases demonstrate the cardiovascular characteristics of untreated syphilis in the tertiary stage. Cases with such advanced pathology will only occur where diagnoses have not been made, and/or standard antibiotic therapy has not been implemented in the early stage of disease.

A case report of CT-diagnosed renal infarct secondary to syphilitic aortitis.

BACKGROUND: Even though reported cases of syphilis have been increasing, cases of tertiary syphilis remain extremely rare. The majority of our knowledge with regard to complications of syphilis such as aortitis was acquired before the advent of relatively modern technologies such as CT, MRI and PET. This case report presents a rare case of syphilitic aortitis associated with a renal infarct caused by a peripheral arterial embolism diagnosed by CT. CASE PRESENTATION: We present a young man with sudden abdominal pain and flank tenderness without fever. Blood tests showed acute kidney failure. Computed tomography showed a right renal infarct and a non-circular thickening of the descending thoracic aortic wall with intra-luminal thrombus. Serology confirmed the diagnosis of syphilis. Treatment with anticoagulant and penicillin resulted in a good outcome. Follow-up PET-MRI showed resolution of the thrombus with a metabolically inactive atheromatous plaque. CONCLUSION: Technologies, such as CT, PET-CT and PET-MRI, that were not present during the pre-antibiotic era, can provide new insights into rare presentations of tertiary syphilis such as aortitis. These imaging modalities show promise for early radiological diagnosis of aortitis in syphilis and may be useful for determining the response to treatment in specific cases.

Computed Tomographic and Morphologic Features of Syphilis of the Aorta.

This report describes certain computed tomographic and morphologic features of syphilitic aortitis in 2 patients in whom the process involved the entire thoracic aorta.

Usefulness of (18)F-FDG PET/CT in disease extent and treatment response assessment in a patient with syphilitic aortitis.

A 40-year-old man was admitted to our hospital for surgical treatment of aortic insufficiency and coronary ostial stenosis. Histopathology and serological tests revealed a syphilitic aortitis. F-FDG PET/CT was performed to assess the extent of aortitis, showing increased radiopharmaceutical uptake along the ascending aortic wall. A repeated FDG PET/CT after antibiotic therapy showed a markedly reduced uptake in the aortic wall, suggesting resolution of the infection according to clinical and serological data. This case highlights the usefulness of FDG PET/CT for the assessment of disease extent and treatment response in patients with syphilitic aortitis.

Syphilitic aortitis: rearing of the ugly head.

CONTEXT: Syphilitic aortitis has been relegated to the category of rare cardiovascular disease or a "medical curiosity" in the west. The same situation may not exist in developing countries due to the stigmata that continue to remain attached to sexually-transmitted diseases in general. AIMS: To study the prevalence of syphilitic aortitis among autopsied non-atherosclerotic aortic diseases encountered in a span of 15 years. SETTINGS AND DESIGN: Retrospective, autopsy-based study. MATERIALS AND METHODS: Among 187 cases of non-atherosclerotic diseases of the aorta, 44 had been diagnosed as syphilitic aortitis on the basis of the pathological features and serology. The demographic details and modes of clinical presentation were retrieved from the health records. Depending on the presence of complicating lesions, the cases were classified as uncomplicated or complicated aortitis. RESULTS: The 44 cases of syphilitic aortitis formed 23.5 % of the non-atherosclerotic aortic diseases. They were predominantly seen in males in the fifth decade, who often presented with valvular regurgitation, aneurysmal disease or myocardial ischemia; 13.6 % of patients were asymptomatic. Blood VDRL results were available in 19 patients; 84.2 % were positive. Concomitant involvement of the ascending, transverse and descending thoracic was seen in 45.5 % of cases. None had uncomplicated aortitis. Complications in the form of aortic regurgitation (72.7 %), coronary ostial stenosis (59 %) and aneurysms (59 %) frequently coexisted. Thirty-five aneurysms were present in 59 %, chiefly involving the aorta. CONCLUSIONS: We found syphilitic aortitis to be a common cause of aortitis at autopsy. Diagnosis should be made with the help of characteristic pathological features correlated to the clinical context and appropriate serological tests.

Syphilitic arteritis involving the origin of the cervical internal carotid artery.

We report a case of meningovascular syphilis in a young adult woman presenting with left hemiparesis due to near occlusion of proximal cervical internal carotid with subacute middle cerebral artery territory infarction. Diagnosis was made on the basis of positive serum, and spinal fluid serology for syphilis, carotid Doppler, and magnetic resonance angiography, as well as improvement after intravenous penicillin therapy. In this case report, the imaging findings were described and related literature was reviewed.

Natural history of syphilitic aortitis.

No large studies of cardiovascular syphilis at necropsy have been reported since 1964. We examined at necropsy 90 patients who had characteristic morphologic findings of syphilitic aortitis. None had ever undergone cardiovascular surgery. With the exception of 2 cases seen more recently, the hearts and aortas of the 90 patients were examined and categorized by one of us (W.C.R.) from 1966 to 1990. All 90 had extensive involvement of the tubular portion of the ascending aorta by the syphilitic process, which spared the sinuses of Valsalva in all but 4 patients. The aortic arch was also involved in 49 (91%) of 54 patients and the descending thoracic aorta in 47 (90%) of 52 patients. Syphilis was the cause of death in 23 (26%) of the 90 patients. It was secondary to rupture of the ascending or descending thoracic aorta in 12, severe aortic regurgitation leading to heart failure in 10, and severe narrowing of the aortic ostium of the right coronary artery in 1 patient. Of the 40 patients who had undergone serologic testing for syphilis, 28 (70%) had a positive (reactive) finding. Those patients with a negative or nonreactive test or who did not undergo a serologic test for syphilis had morphologic and histologic findings in the aorta at necropsy similar to the findings of those patients who had had a positive serologic test for syphilis. In conclusion, cardiovascular syphilis has not disappeared. In patients with dilated ascending aortas, with or without aortic regurgitation, a serologic test for syphilis is recommended. If the findings are positive or if characteristic morphologic features of cardiovascular syphilis are suspected, irrespective of the results of the serologic tests, antibiotic therapy appears desirable.

Identifying cardiovascular syphilis at operation.

To describe the morphologic features so the process can be easily identified during surgery, we studied 34 patients with cardiovascular syphilis, 32 of whom underwent excision and replacement of the ascending aorta or aortic valve or both. Of the 34 patients, 22 were treated at Baylor University Medical Center from 1998 to 2008 and 12 at non-Baylor University Medical Center hospitals from 1958 to 1987. In all 34 patients, the tubular portion of the aorta was diffusely thickened and the sinus portion of the aorta was apparently uninvolved. The process involved all 3 layers of the aorta, with thickening of the adventitia, mainly by fibrous tissue. Within the fibrous tissue were collections of plasma cells and lymphocytes, focal destruction of the media without thickening, and marked thickening of the intima by atherosclerotic-appearing lesions. Serologic tests for syphilis were done in only 14 patients (41%) and were positive (reactive) in 6 (43%) of them. The ascending aorta, however, was similar in all 34 patients. In conclusion, cardiovascular syphilis has not disappeared. Its identification during surgery can prompt appropriate antibiotic therapy postoperatively. Although the serologic test results for syphilis might be negative, antibiotic therapy is recommended for patients with panaortitis requiring resection of the ascending aorta with or without aortic regurgitation.

Occlusion of the left coronary trunk secondary to tertiary syphilis.

A 27-year-old patient with tertiary syphilis, manifested as myocardial ischemia, presenting unstable angina, secondary to left coronary trunk occlusion. The diagnosis was confirmed by the serological findings and the pathological assessment of the aorta fragment.

Tronco de coronária esquerda ocluso secundário a Lues terciária / Tronco de coronaria izquierda ocluido secundario a Lúes terciaria / Occlusion of the left coronary trunk secondary to tertiary syphilis

Paciente de 27 anos, portador de sífilis terciária, manifestando isquemia miocárdica, com angina instável, secundária à oclusão do tronco da coronária esquerda. O diagnóstico foi confirmado pelos achados da sorologia e da patologia do fragmento da aorta.

A 27-year-old patient with tertiary syphilis, manifested as myocardial ischemia, presenting unstable angina, secondary to left coronary trunk occlusion. The diagnosis was confirmed by the serological findings and the pathological assessment of the aorta fragment.

Paciente de 27 años, portador de sífilis terciaria, manifestando isquemia miocárdica, con angina inestable, secundaria a la oclusión del tronco de la coronaria izquierda. El diagnóstico fue confirmado por los resultados de la serología y de la patología del fragmento de la aorta.

Sífilis cardiovascular: diagnóstico y tratamiento / Cardiovascular syphilis: diagnosis, treatment

Cardiovascular tertiary syphilis may lead to aortitis, aortic aneurism, coronary stenosis, aortic insufficiency and, rarely, to myocarditis. The physician must be familiar with the clinical presentations of this process, including the asymptomatic variety and must be able to have an organized plan for the diagnosis and evaluation to establish or exclude the presence of cardiovascular pathology and the differential diagnosis with other entities. Once the etiologic and topographic diagnosis is established, the patient should be treated with penicillin, doxicycline and other antibiotics, and the consequences of the disorder, both actual and potential, should be considered before deciding weather to recommend surgical intervention. Although late syphilis can be prevented by appropriate therapy of early syphilis, this is a cardiovascular disease that most likely will continue to be diagnosed lately. Understanding of the pathology and pathophysiology of the disease, is most important for its prompt recognition and subsequent management. This paper reviews the natural history, diagnosis and therapy of cardiovascular syphilis.

Review of isolated ascending aortitis: differential diagnosis, including syphilitic, Takayasu's and giant cell aortitis.

The image of tree-barking and proximal aortic root dilatation is firmly entrenched in the minds of practising pathologists as representing syphilis until proven otherwise. We discuss the differential diagnosis of syphilitic aortitis, Takayasu's disease, and giant cell aortitis, with a review of the literature and brief overview of other types of aortitis. As a starting point, we report a case of non-specific, or idiopathic, aortitis with aneurysm that was initially misdiagnosed as syphilitic aortitis. We then review the literature and emphasise the lack of histological data and histopathological criteria for the diagnosis of non-infectious aortitis and the implications for treatment in cases of isolated aortitis. Tree-barking is a non-specific finding in aortitis of any aetiology, and syphilitic aortitis in developed countries is rare. It is still unclear if there are histological features that separate Takayasu's disease and giant cell arteritis. In the majority of patients presenting with aortic root aneurysms, aortitis is an isolated finding not associated with autoimmune disease. Despite a plethora of literature, a histological classification of aortitis has yet to be attempted.