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Cerebellum (London, England) Jun 2024We describe a male patient presenting with cerebellar ataxia and behavioural frontotemporal dementia in whom imaging showed cerebellar atrophy. He had significantly low...
We describe a male patient presenting with cerebellar ataxia and behavioural frontotemporal dementia in whom imaging showed cerebellar atrophy. He had significantly low N-acetyl aspartate to creatine (NAA/Cr) area ratio on MR spectroscopy of the cerebellum, primarily affecting the vermis. CT body scan showed extensive abnormal tissue within the mesentery, the retroperitoneum and perinephric areas. PET-CT showed increased tracer uptake within the wall of the aorta suggestive of an aortitis and within the perinephric tissue bilaterally. Biopsy of the perinephric tissue confirmed IgG4 disease. Treatment with steroids and mycophenolate improved his clinical state, but he developed symptoms attributed to pericardiac effusion that necessitated treatment initially with drainage and subsequently with pericardial window. After a course of rituximab, he had an episode of sepsis that did not respond to appropriate treatment and died as a result. Both the imaging findings and neurological presentation with cerebellar ataxia and behavioural frontotemporal dementia are novel in the context of IgG4 disease.
Topics: Humans; Male; Cerebellar Ataxia; Immunoglobulin G4-Related Disease; Middle Aged; Frontotemporal Dementia
PubMed: 37558930
DOI: 10.1007/s12311-023-01592-8 -
Annals of Vascular Surgery Nov 2023Endovascular repair of inflammatory abdominal aortic aneurysms (IAAAs) has emerged as an alternative to open surgery, but direct comparisons are limited. The aim of the...
BACKGROUND
Endovascular repair of inflammatory abdominal aortic aneurysms (IAAAs) has emerged as an alternative to open surgery, but direct comparisons are limited. The aim of the study was to compare clinical outcomes of endovascular and open repair for IAAA according with specific clinical characteristics.
METHODS
We performed a literature review of reports describing patients who had open or endovascular repair for IAAA. A literature search was performed in June 2022 by 2 investigators who conducted a review of papers reported in PubMed, Embase, MEDLINE, and Cochrane Database. The strings "Inflammatory aneurysm" and "Abdominal Aortic Aneurysms" were used. There was no language restriction and screened reports were published from March 1972 to December 2021. We identified 2,062 patients who had open (1,586) or endovascular repair (476) for IAAA. Primary outcomes were operative mortality and morbidity. Secondary outcomes were complications during follow-up (mean follow-up: 48 months). Propensity score matching was performed between patients who had open or endovascular surgery.
RESULTS
In Western countries, propensity-weighted postoperative mortality (in-hospital) (1.5% endovascular vs. 6% open) and morbidity rates (6% vs. 18%) were significantly lower in patients who had endovascular repair (P < 0.0001); patients with larger aneurysm (more than 7 cm diameter), signs of active inflammation, and retroperitoneal rupture of the aneurysm had better outcomes after endovascular repair than after open surgery. Hydronephrosis was present in 20% of the patients. Hydronephrosis regressed in most patients when signs of active inflammation were present suggesting an acute onset of the hydronephrosis itself (fever, elevated serum C Reactive Protein) either after endovascular or open surgery. Long-standing hydronephrosis as suggested by the absence of signs of active inflammation rarely regressed after endovascular surgery despite associated steroid therapy. During a mean follow-up of 48 months, propensity-weighted graft-related complications were more common in patients who had endovascular repair (20% vs. 8%). For patients from Asia, short-term and medium-term results were similar after open and endovascular repair. IAAAs related with aortitis were more common in Asia. In Western countries, IAAAs were commonly associated with atherosclerosis.
CONCLUSIONS
Patients with IAAA represent a heterogeneous population, suggesting biological differences from continent to continent; conservative therapy and endovascular or open surgery should be chosen according to the patient clinical condition. Endovascular repair presents advantages in patients with signs of active inflammation and contained rupture of the IAAA and larger aneurysms. Hydronephrosis, without signs of active inflammation, rarely regresses after endovascular repair associated with steroid therapy. Further studies are needed to establish the long-term results of endovascular repair.
Topics: Humans; Aortic Aneurysm, Abdominal; Aortitis; Endovascular Procedures; Hydronephrosis; Inflammation; Postoperative Complications; Retrospective Studies; Risk Factors; Steroids; Treatment Outcome
PubMed: 37454897
DOI: 10.1016/j.avsg.2023.06.036 -
Journal of Cardiothoracic Surgery Jul 2023Salmonella spp. cause infectious aortitis through the hematogenous spread of an intestinal Salmonella infection. Salmonella aortitis can result in extensive tissue...
BACKGROUND
Salmonella spp. cause infectious aortitis through the hematogenous spread of an intestinal Salmonella infection. Salmonella aortitis can result in extensive tissue damage in the aorta leading to complications including dissection, abscess formation, pseudoaneurysms, and rupture, which require early diagnosis and treatment with both surgery and antibiotic therapy.
CASE PRESENTATION
We report a case of Salmonella aortitis complicated by Stanford type A aortic dissection. A 62-year-old man with a history of heroin use presented with chest pain, epigastric pain and vomiting. The computed tomography scan showed Stanford type A aortic dissection without malperfusion. At the time of surgery, an aortic dissection with purulent fluid and contained rupture was noted in the ascending aorta. Fluid culture was consistent with Salmonella. A composite valve-graft conduit aortic root replacement with ascending aorta and hemiarch replacement was performed. The patient recovered well and was discharged on long-term antibiotics.
CONCLUSIONS
This rare case of a Stanford type A aortic dissection with contained rupture due to Salmonella aortitis was successfully treated with emergent surgery and antibiotic therapy.
Topics: Male; Humans; Middle Aged; Aortitis; Aortic Dissection; Aorta; Salmonella; Anti-Bacterial Agents
PubMed: 37452382
DOI: 10.1186/s13019-023-02318-x -
International Journal of Infectious... Sep 2023Fusarium (F.) species are ubiquitous filamentous fungi that may cause various opportunistic infections, especially in patients who are immunocompromised. A rare...
Fusarium (F.) species are ubiquitous filamentous fungi that may cause various opportunistic infections, especially in patients who are immunocompromised. A rare manifestation of disseminated fusariosis affects the aortic valve and results in invasive aortitis, which poses a significant challenge for clinicians in diagnosis and treatment. Here, we report a case of a patient, aged 54 years, who is immunocompromised, presenting initially with Fusarium keratitis and chorioretinitis in both eyes and a new endovascular aortic mass. Positron emission tomography/computed tomography was performed, suggesting aortitis. Transoesophageal echocardiography and electrocardiogram-guided computed tomography-angiography confirmed a large intraluminal mass in the ascending aorta. The aortic mass and a part of the ascending aorta were resected surgically, and a filamentous fungus with the microscopic features of the genus Fusarium was isolated and later identified molecularly as F. petroliphilum. The course of the treatment was complicated by perioperative cerebral embolization and mesenteric ischemia. These complications could be attributed to a preoperatively existing occlusion of the superior and inferior mesenteric artery and a subtotal stenosis of the celiac trunk. This case report describes a rare manifestation of disseminated fusariosis, frequently characterized by protracted clinical courses with poor prognosis. Fusariosis may manifest at different sites at different times or persist as a long-lasting disease with reactivation. This case highlights the importance of the interdisciplinary approach for effectively treating invasive mycoses.
Topics: Humans; Fusarium; Fusariosis; Aortitis; Immunocompromised Host; Tomography, X-Ray Computed; Antifungal Agents
PubMed: 37279826
DOI: 10.1016/j.ijid.2023.05.069 -
Annals of Thoracic and Cardiovascular... Jan 2024A syphilitic aortitis is a late cardiovascular lesion of tertiary syphilis that has become exceptionally rare in the antibiotic era but not eradicated completely....
A syphilitic aortitis is a late cardiovascular lesion of tertiary syphilis that has become exceptionally rare in the antibiotic era but not eradicated completely. Syphilitic aortitis of ascending aorta complicates in ascending aortic aneurysm formation and aortic valve regurgitation, both requiring surgical treatment. After surgery, lifelong surveillance of the remainder of the aorta is recommended because of a priori supposed high incidence of delayed involvement of noninvolved aortic segments. A 3-year follow-up result of surgery of syphilitic ascending aortic aneurysm with aortic valve regurgitation in condition of active ongoing syphilitic aortitis and valvulitis is described with addressing the dimensions of remaining aortic segments. This case demonstrates that the dilatation of the remainder of the aorta does not occur during 3 years, at least when anti-syphilitic course of antibiotic is used just after operation without additional treatment during the follow-up period. A few reports on surgical treatment of syphilitic aneurysms of the ascending aorta are discussed.
Topics: Humans; Syphilis, Cardiovascular; Aortic Valve Insufficiency; Aneurysm, Ascending Aorta; Treatment Outcome; Aortic Aneurysm; Anti-Bacterial Agents; Aortic Valve
PubMed: 36967122
DOI: 10.5761/atcs.cr.22-00236 -
Internal Medicine (Tokyo, Japan) Nov 2023
Topics: Humans; Aortitis; Granulocyte Colony-Stimulating Factor; Filgrastim
PubMed: 36948622
DOI: 10.2169/internalmedicine.1357-22 -
Internal Medicine (Tokyo, Japan) Nov 2023Aortitis is a rare adverse event associated with granulocyte colony-stimulating factor (G-CSF). Contrast-enhanced computed tomography (CECT) is widely used to diagnose...
Aortitis is a rare adverse event associated with granulocyte colony-stimulating factor (G-CSF). Contrast-enhanced computed tomography (CECT) is widely used to diagnose G-CSF-associated aortitis. However, the usefulness of gallium scintigraphy for the diagnosis of G-CSF-associated aortitis is unknown. We herein report a set of pre- and post-treatment gallium scintigrams of a patient with G-CSF-associated aortitis. During the diagnosis, gallium scintigraphy revealed hot spots on the arterial walls that appeared inflamed on CECT. Both the CECT and gallium scintigraphy findings disappeared. Gallium scintigraphy can be a supportive diagnostic tool for G-CSF-associated aortitis, especially in patients with an impaired renal function or allergy to iodine contrast.
Topics: Humans; Aortitis; Granulocyte Colony-Stimulating Factor; Radionuclide Imaging; Tomography, X-Ray Computed; Gallium
PubMed: 36948620
DOI: 10.2169/internalmedicine.1453-22