Aortic rupture following acute aortitis in a patient with head and neck carcinoma treated with nivolumab: a rare but severe immune-related adverse event.

INTRODUCTION: Immune-related adverse events (irAEs) due to immune checkpoint inhibitors may lead to discontinuation and treatment-related death. Acute aortitis is a rare but severe irAE. CASE PRESENTATION: A 67-year-old man with recurrent lower gingival carcinoma received nivolumab therapy. Twenty-three months later, he experienced chest compression, which resulted in syncope. Following a whole-body computed tomography (CT) scanning, which revealed diffuse thickening of the aorta, and systemic assessments of the causes of aortitis, he was diagnosed with acute aortitis due to irAE. Nivolumab discontinuation and oral steroids improved CT findings. However, 11 months after nivolumab discontinuation, he developed an aortic aneurysmal rupture. Endovascular aortic repair rescued him. A durable anti-cancer response was still observed 4 months after the aortic rupture. CONCLUSION: Although severe irAE, such as acute aortitis, occurred, the patient may still achieve a durable response. A broad examination and prompt treatment of irAE can help improve the patient's survival.

[A Case of Granulocyte Colony-Stimulating Factor-Related Aortitis that Developed during the Treatment of Advanced Prostate Cancer with Neuroendocrine Differentiation].

An 81-year-old man with prostate cancer (cT3aN0M0), who had been undergoing hormonal therapy for 4 years and had maintained low prostate specific antigen levels, developed metastasized pelvic lymph nodes. A tissue biopsy revealed neuroendocrine differentiation of prostate cancer in the metastatic lymph nodes. Consequently, chemotherapy with carboplatin＋etoposide was initiated. During the first course, filgrastim was administered for 2 days due to a drop in his neutrophil count to 230/µl. During the second course, pegfilgrastim was administered as prophylaxis on day 4. However, on day 10 of the second course, he started to develop a fever and fatigue. Suspecting infection, antibiotics were administered, but failed to ameliorate his symptoms. On day 14, plain computed tomography revealed signs of aortic inflammation. Given the lack of improvement even after one week of antibiotic therapy, steroid treatment was initiated on the suspicion of granulocyte colony-stimulating factor (G-CSF) -induced aortitis, which rapidly improved his symptoms. Therefore, when encountering a case in which a fever remains unresponsive to antibiotics during chemotherapy with G-CSF agents, a differential diagnosis of aortic inflammation caused by G-CSF agents needs to be considered.

Multidisciplinary diagnosis and management of inflammatory aortic aneurysms.

BACKGROUND: Inflammatory abdominal aortic aneurysms (IAAAs) are a variant involving a distinct immunoinflammatory process, with nearly one half believed to be associated with IgG4-related disease (IgG4-RD). METHODS: MEDLINE and Google Scholar searches were conducted for English-language publications relevant to inflammatory aortic aneurysms from January 1970 onward. The search terms included inflammatory aortic aneurysms, aortitis, periaortitis, IgG4-related disease, and retroperitoneal fibrosis. Relevant studies were selected for review based on their relevance. RESULTS: Morphologically, IAAAs are characterized by a thickened aneurysm wall often displaying contrast enhancement and elevated metabolic activity on fluorine-18 fluorodeoxyglucose-positron emission tomography imaging. A strong association exists with perianeurysmal and retroperitoneal fibrosis. Although the rupture risk appears lower with IAAAs than with noninflammatory abdominal aortic aneurysms (AAAs), the currently recommended diameter threshold for operative management is the same. Open repair has been associated with increased morbidity compared with noninflammatory AAAs, and a retroperitoneal approach or minimal dissection transperitoneal approach has been recommended to avoid duodenal and retroperitoneal structural injuries. Endovascular aneurysm repair has been increasingly used, especially for patients unfit for open surgery. It is important to exclude an infectious etiology before the initiation of immunosuppressive therapy or operative repair. Multimodality imaging follow-up is critical to monitor disease activity and secondary involvement of retroperitoneal structures by the associated fibrotic process. Maintenance of immunosuppressive therapy will be needed postoperatively for most patients with active systemic disease, especially those with IgG4-RD and those with persistent symptoms. Additional interventions aimed at ureteral decompression could also be required, and lifelong follow-up is mandatory. CONCLUSIONS: Preoperative multimodality imaging is a diagnostic cornerstone for assessment of the disease extent and activity. IgG4-RD is an increasingly recognized category of IAAAs, with implications for tailoring adjunctive medical therapy. Open surgical repair remains the procedure of choice, although endovascular aneurysm repair is increasingly being offered. Maintenance immunosuppressive therapy can be offered according to the disease activity as assessed by follow-up imaging studies.

Management of Inflammatory Aortic Aneurysms - A Scoping Review.

OBJECTIVE: Inflammatory abdominal aortic aneurysms (InflAAAs) account for 5 - 10% of aortic aneurysms and are characterised by retroperitoneal fibrosis. Diagnosis is often delayed, and doubts remain about the optimal management strategy. This scoping review describes the current state of knowledge on InflAAAs. METHODS: Medline, PubMed, EMBASE, and Scopus were searched for relevant studies that evaluated the diagnosis and treatment of InflAAAs. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol was followed. RESULTS: Fifty-seven papers were selected (low level of evidence), which included 1 554 patients, who were mostly male and heavy smokers. A triad of chronic abdominal or back pain, weight loss, and elevated inflammatory markers was highly suggestive of the diagnosis but rarely present, and fever was noted only randomly. A mantle sign was seen on computed tomography angiography (CTA) in 73 - 100% of patients. Open surgical repair (OSR) and endovascular aortic aneurysm repair (EVAR) was reported in 1 376 and 178 patients, respectively. OSR was associated with significant iatrogenic bowel (n = 22), urinary tract system (n = 7), venous (n = 30), pancreatic (n = 6), and splenic (n = 5) injuries, while EVAR was associated with lower 30 day mortality (0 - 5% vs. 0 - 32%). One and two year mortality rates were similar between the two treatment modalities (0 - 20% and 0 - 36%, respectively). EVAR was more often associated with post-operative progression of inflammation (17% vs. 0.4%), and a higher frequency of persistent hydronephrosis (> 50%) and limb occlusion (20%). Used in < 10% of patients, corticosteroids led to complete pain relief and a reduction in peri-aortic inflammation within 6 - 18 months. CONCLUSION: InflAAAs are characterised by non-specific symptoms, with the mantle sign on CTA being pathognomonic. Corticosteroids may be considered a basic treatment that all patients should receive initially. Low quality data indicate that EVAR (vs. OSR) is associated with fewer intra-operative complications and lower peri-operative mortality but more late fibrosis related adverse events. International multicentre registries are required to gather more insights into this challenging pathology.

Relationship between histopathological features of non-infectious aortitis and the results of pre-operative 18F-FDG-PET/CT: a retrospective study of 16 patients.

OBJECTIVES: To describe the characteristics of 18F-fluorodeoxyglucose positron-emission tomography/computed-tomography (18FDG-PET/CT) findings before surgery in patients with active, histologically confirmed aortitis, and to correlate the degree of arterial wall inflammation with PETVAS score. METHODS: This was a multiple-centre retrospective study including cases with histologically proven active, non-infectious aortitis who had a 18FDG-PET/CT performed within one year before surgery for aneurysm repair. PETVAS score was determined by radiologists blinded to the pathology findings. Cardiovascular pathologists reviewed aortic tissue samples and graded the degree of inflammation in the vessel wall. RESULTS: Sixteen patients were included (8 giant cell arteritis, 4 clinically isolated aortitis, 2 Takayasu's arteritis, 1 relapsing polychondritis, and 1 rheumatoid arthritis). In 5/16 (31%) patients, 18FDG-PET/CT did not detect the presence of aortic inflammation; two of whom were being treated with glucocorticoids at the time of procedure. Ascending thoracic and abdominal aorta had the highest FDG uptake among the affected territories. Patients without active aortitis on 18FDG-PET/CT were significantly older (p=0.027), had a lower PETVAS score (p=0.007), and had a lower degree of adventitial inflammation (p=0.035). In contrast, there was no difference between 18FDG-PET/CT active and inactive aortitis patients as regards the timing between PET/CT and surgery, serum CRP level (during 18FDG-PET/CT) and, FDG uptake per study site. CONCLUSIONS: In histologically proved aortitis, 18FDG-PET/CT before surgery did not detect vascular inflammation in 31% patients, and PETVAS score correlated with the degree of adventitial histopathologic inflammation.

Brucella aortitis managed with debridement, extra-anatomical bypass, and long-term antimicrobial therapy.

OBJECTIVES: This report aims to review the management and outcomes of Brucella-associated mycotic aortic aneurysms. METHODS: This is a retrospective chart review at a tertiary-level healthcare system. IRB approval was waived per policy. RESULTS: We describe a case of Brucella aortitis acquired from habitual contact with wild hogs. Clinical presentation included lower back pain and elevated white blood cell count. Diagnosis was confirmed with imaging showing an infrarenal abdominal aortic aneurysm and serology revealing elevated Brucella antibodies titers. The patient was initially managed with endovascular aortic repair and combined oral and intravenous antibiotics therapy. He then underwent explanation and extra-anatomical bypass due to symptomatic periaortic infection and interval development of type I endoleak. The patient was asymptomatic after his final operation at 24 months of follow-up and remained on suppressive oral antibiotic therapy. CONCLUSIONS: An aortic aneurysm secondary to Brucella is a rare entity. A detailed history of long-term exposure to animals may be a clue to obtain serologic testing. Operative debridement and re-establishing of reliable blood flow combined with long-term antibiotic suppression are the mainstay of treatment.

Spontaneous Improvement of Aortitis Associated with Severe COVID-19 Infection-A Case Report.

Aortitis is a rare complication of the coronavirus disease 2019 (COVID-19) and is often treated empirically with steroids. We present a case of spontaneous resolution of aortitis without treatment. A 65-year-old man was admitted to our intensive care unit for severe COVID-19 pneumonia and underwent rehabilitation in the general ward. On day 12, he developed fever, and on day 13, he developed right cervical pain and increased inflammatory markers. On day 16, a cervical echocardiogram showed vasculitis in the right common carotid artery, and on day 17, computed tomography (CT) of the neck showed thickening of the arterial wall of the right common to the internal carotid arteries. A retrospective assessment of the CT scan on day 12 showed wall thickening from the thoracic aorta to the abdominal aorta, and a diagnosis of aortitis was made. Autoantibody analysis, culture, and magnetic resonance imaging (MRI) of the head and neck showed no abnormalities. During the investigation of the cause of aortitis, the fever and inflammatory reaction spontaneously resolved and the right cervical pain gradually improved. Therefore, the patient was diagnosed with transient COVID-19-related aortitis. To our knowledge, this is the first report describing the spontaneous resolution of COVID-19-related aortitis.

[Incidental finding aortitis: how to handle it?] / Découverte fortuite d'une aortite : quelle prise en charge ?

Aortitis may be an incidental finding at imaging. It refers to inflammation of the aortic wall and sometimes may be hard to differentiate with the periaortitis, inflammation of tissues around the vessel. Their clinical presentation is as varied as their etiologies. Appropriate early management is essential for improving patient prognosis, as the diagnostic approach remains challenging.

Une aortite, inflammation de la paroi de l'aorte, est parfois décrite à l'imagerie. Elle peut être confondue avec une périaortite, l'inflammation des tissus autour du vaisseau. La présentation clinique de ces deux atteintes est aussi diverse que leurs causes. Comme la prise en charge thérapeutique adéquate dépend de la maladie sous-jacente, un choix réfléchi d'examens paracliniques est essentiel pour améliorer le pronostic du patient.

Single-Center Analysis of Pegfilgrastim-induced Aortitis Using a Drug Prescription Database and CT Findings.

Background Pegfilgrastim-induced aortitis is a rare but serious adverse event in patients undergoing anticancer therapy with granulocyte colony-stimulating factor analogs. Despite previous case series and systemic reviews, the exact incidence, clinical presentation, and CT manifestations of pegfilgrastim-induced aortitis remain unclear. Purpose To clarify the incidence and clinicoradiologic characteristics of pegfilgrastim-induced aortitis. Materials and Methods Pegfilgrastim administration records from January 2015 to March 2021 were retrospectively collected from the drug prescription database of a single center and were matched with the relevant findings in the CT database. Corresponding CT images within 6 months were available for a total of 1462 doses of pegfilgrastim in 674 patients. Four radiologists reviewed the CT images for the presence of aortitis in two steps. Clinical information and the distribution of aortitis on CT images were examined for patients with a diagnosis of pegfilgrastim-induced aortitis. Results Pegfilgrastim-induced aortitis was observed in 18 of 674 patients (mean age, 62 years ± 13 [SD]; 424 men), resulting in incidence rates of 2.7% per patient (95% CI: 1.6, 4.2) and 1.2% per dose (95% CI: 0.7, 1.9). The most common original primary malignancies were esophageal cancer (n = 10, 9%), breast cancer (n = 3, 4%), and pancreatic cancer (n = 2, 2%). The most common anticancer drugs used at onset were 5-fluorouracil, cisplatin, and docetaxel. Seven cases were symptomatic, while the remaining 11 (61%) were asymptomatic. CT findings indicated that aortitis involved branches of the aortic arch in 13 cases (72%), aortic arch in 10 cases (56%), and abdominal aorta in two cases (11%). Conclusion Pegfilgrastim-induced aortitis may be more prevalent than previously reported and may be more common in patients with esophageal cancer and those who received 5-fluorouracil, cisplatin, and docetaxel as anticancer drugs. The findings also suggest that pegfilgrastim-induced aortitis is often characterized by aortic arch and proximal branch involvement at CT. © RSNA, 2022 Online supplemental material is available for this article. See also the editorial by Krinsky in this issue.

Systematic literature review and meta-analysis of Clostridium septicum aortitis.

BACKGROUND: Clostridium septicum bacteremia is often associated with occult malignancies (approximately 80%), especially of the right colon. Furthermore, inflammation of the aortic wall can rapidly lead to aneurysm induction through bacterial seeding into atheromatous lesions with consecutive life-threatening rupture. We summarize all published data on this rare and lethal disease to evaluate therapeutic approaches and give valid treatment recommendations because there are no guidelines. METHODS: A systematic review of the literature was conducted screening EMBASE and MEDLINE databases following the PRISMA guidelines with search period from first description to August 25, 2021. RESULTS: There were 72 cases of C septicum aortitis reported in 64 publications. Endovascular aortic repair (EVAR) was performed in a minority of patients (n = 6) unfit for surgery but lacked long-term survivors. Antibiotic treatment was beneficial in a bridge to surgery concept, but up to now harbored a 6-month mortality rate of 100% (median overall survival, 0.5 months) when no additional aortic repair was performed. Open aortic repair was the only potential curative approach but was accompanied with a 90-day-mortality of 26.7% (4/15). CONCLUSIONS: Open aortic repair combined with perioperative antibiotic treatment should be offered to all patients as the only potentially curative approach. If applicable, resection of a coexisting colonic tumor should be performed after successful aortic repair. Alternatively, long-term antibiotic treatment can be offered to patients unfit for surgery in a palliative setting. Endovascular aortic repair has been performed on a minority of patients with a high risk for stent graft infection and should remain a salvage strategy when therapeutic pressure demands acute intervention in patients unfit for surgery.

A methodological framework for AI-assisted diagnosis of active aortitis using radiomic analysis of FDG PET-CT images: Initial analysis.

BACKGROUND: The aim of this study was to explore the feasibility of assisted diagnosis of active (peri-)aortitis using radiomic imaging biomarkers derived from [18F]-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography (FDG PET-CT) images. METHODS: The aorta was manually segmented on FDG PET-CT in 50 patients with aortitis and 25 controls. Radiomic features (RF) (n = 107), including SUV (Standardized Uptake Value) metrics, were extracted from the segmented data and harmonized using the ComBat technique. Individual RFs and groups of RFs (i.e., signatures) were used as input in Machine Learning classifiers. The diagnostic utility of these classifiers was evaluated with area under the receiver operating characteristic curve (AUC) and accuracy using the clinical diagnosis as the ground truth. RESULTS: Several RFs had high accuracy, 84% to 86%, and AUC scores 0.83 to 0.97 when used individually. Radiomic signatures performed similarly, AUC 0.80 to 1.00. CONCLUSION: A methodological framework for a radiomic-based approach to support diagnosis of aortitis was outlined. Selected RFs, individually or in combination, showed similar performance to the current standard of qualitative assessment in terms of AUC for identifying active aortitis. This framework could support development of a clinical decision-making tool for a more objective and standardized assessment of aortitis.

The Femoral Vein as a Long-Term Aorto-Iliac Graft for Aortic Infection and Aortitis.

BACKGROUND: Reconstruction of the aorto-iliac segment with femoral vein (FV) as substitute for infected synthetic grafts or mycotic aneurysms constitutes the most sustainably convenient alternative. The aim of this study was to evaluate the long-term outcome of up to 16 years of follow-up, analysing the morphologic adaption of the FV with special emphasis on the distal and proximal anastomoses. METHODS: We conducted a retrospective study of 22 patients with 109 computed tomography angiograms (CTAs) treated between August 2001 and January 2020 in case of aortic infection/aortitis. Morphologic changes like anastomotic dilatation/stenosis as well as changes of FV wall thickness were retrospectively analysed in pre- and postoperative CTAs. RESULTS: Elective procedure was done in 17/22 (77%) cases, and 5/22 (23%) patients required emergent surgery. The median follow-up was 91.5 months (P25;P75 = 21;117). Cross-sectional diameter of proximal (20.38 ± 3.77 vs 22.04 ± 3.97 mm, p = 0.007) and distal anastomoses (13.05 ± 4.23 vs 14.61 ± 5.19 mm, p = 0.05) increased significantly, as well as the proximal and distal anastomotic areas (3.36 ± 1.29 vs 4.32 ± 1.63 mm2, p = 0.04 and 0.99 ± 0.48 vs 1.25 ± 0.72 mm2, p = 0.023, respectively). Venous wall thickness was significantly reduced at the anastomotic site (1.74 ± 0.46 vs 1.24 ± 0.31 mm, p = 0.001). The upper thigh diameter did not differ before and after harvesting of the FV (161.6 ± 29.1 vs. 178.2 ± 23.3 mm, p = 0.326, respectively). CONCLUSION: This long-term CTA follow-up study showed that the FV wall becomes thinner at the anastomotic site, and the anastomoses dilate with time without rupture. The FV is a durable conductor after replacement of the aorto-iliac segment due to aortic infection. Further CTA studies from more centres are warranted to evaluate the risk of vein rupture.

How to mimic a type A intramural hematoma: A case of IgG4-related aortitis associated with cerebral hemorrhage.

IgG4-related aortitis is an inflammatory condition of the aorta, characterized by aortic wall thickening and periaortic soft-tissue involvement. Therefore, this condition can mimic an aortic intramural hematoma (IMH), due to similar radiological findings. We hereby report the case of an IgG4-related aortitis misdiagnosed as an IMH, associated with cerebral hemorrhage, possibly due to cerebral vascular system involvement.

Semi-Quantitative [18F]FDG-PET/CT ROC-Analysis-Based Cut-Offs for Aortitis Definition in Giant Cell Arteritis.

[18F]fluorodeoxyglucose-positron emission tomography/computed tomography ([18F]FDG-PET/CT) is used to diagnose large vessel vasculitis in giant cell arteritis (GCA). We aimed to define a semi-quantitative threshold for identifying GCA aortitis from aortic atheroma or the control. Contrast enhanced computed tomography (CECT) was used as the reference imaging for aortic evaluation and to define aortitis, aortic atheroma and control aortas. [18F]FDG-PET/CT was performed on 35 GCA patients and in two different control groups (aortic atheroma (n = 70) and normal control (n = 35)). Aortic semi-quantitative features were compared between the three groups. GCA patients without aortitis on CECT were excluded. Of the GCA patients, 19 (54.3%) were not on glucocorticoids (GC) prior to [18F]FDG-PET/CT. The SUVmax, TBRblood and TBRliver aortic values were significantly higher in the GCA aortitis group than in the aortic atheroma and control groups (p < 0.001). Receiver operating characteristic curve analyses brought to light quantitative cut-off values allowing GCA aortitis diagnosis with optimal sensitivity and specificity versus control or aortic atheroma patients for each PET-based feature analyzed. Considering the overall aorta, a SUVmax threshold of 3.25 and a TBRblood threshold of 1.75 had a specificity of 83% and 75%, respectively, a sensitivity of 81% and 81%, respectively, and the area under the ROC curve (AUC) was 0.86 and 0.83, respectively, for aortitis detection compared to control groups in GCA cases with GC. A SUVmax threshold of 3.45 and a TBRblood threshold of 1.97 had a specificity of 90% and 93%, respectively, a sensitivity of 89% and 89%, respectively, with an AUC of 0.89 and 0.96, respectively, for aortitis detection compared to the control in GC-free GCA cases. Discriminative thresholds of SUVmax and TBRblood for the diagnosis of GCA aortitis were established using CECT as the reference imaging.

Ascending aortic aneurysm associated with lymphoplasmacytic thoracic aortitis mimics acute type A intramural hematoma - case report.

INTRODUCTION: Lymphoplasmacytic thoracic aortitis is still underrecognized cause of thoracic aortic aneurysm. CASE REPORT: We herein report a case of symptomatic inflammatory ascending aortic aneurysm and pericarditis diagnosed on CT scan as acute type A intramural hematoma. Patient underwent emergency supracoronary ascending aorta replacement with a prosthetic graft. Marked inflammatory thickening of aneurysm wall was the intraoperative finding. Histopathologic examination of the excised aortic tissue showed inflammation with lymphoplasmacytic pattern of aortitis. CONCLUSION: This case demonstrates that lymphoplasmacytic aortitis may mimic symptoms and radiological findings of intramural hematoma.

Transcatheter aortic valve implantation for severe pure aortic regurgitation due to active aortitis.

Aortitis is an uncommon systemic inflammatory disease affecting the aorta and its main branches. Severe aortic regurgitation (AR) represents a fearsome complication of aortitis and is associated with an increased mortality rate. Surgical aortic valve replacement represents the only treatment choice for these patients. However, it is associated with a higher risk of medium to long-term complications such as prosthetic valve detachment. This is the first reported case where severe AR secondary to aortitis was managed with transcatheter aortic valve implantation (TAVI). TAVI was safe and effective in this clinical setting and may be considered a viable alternative to high-risk surgery in these complex patients.

Gas Where It Shouldn't Be! Imaging Spectrum of Emphysematous Infections in the Abdomen and Pelvis.

OBJECTIVE. The purpose of this article is to review the spectrum, etiopathogenesis, clinical presentation, imaging features, differential diagnoses, and management of emphysematous infections of the abdomen and pelvis. CONCLUSION. Emphysematous infections are associated with high morbidity and mortality and thus need urgent medical and surgical interventions. CT is the most sensitive modality to detect gas; CT provides definitive diagnosis in most cases and can depict the extent of involvement.

Aortic involvement in relapsing polychondritis: case-based review.

Relapsing polychondritis is a systemic inflammatory disease that mainly affects ears, nose, eyes, joints, and large airway. Relapsing polychondritis may also affect cardiac valves and large vessels with the aorta being most frequently involved. We conducted a systematic literature review to delineate the clinical characteristics, treatment, and outcome of relapsing polychondritis patients with aortic involvement including thoracic and abdominal aorta, aortic valve, and coronary arteries. 113 patients reported in 85 manuscripts were retrieved through the systematic literature search and references of the selected manuscripts. With the addition of a patient from our center, a total of 114 patients were included in the analyses. Aortic vessel involvement was the predominant type of involvement that was identified in 93 (82%) patients, while aortic valve involvement was identified in 41 patients (36%). The median age at aortic involvement was 37 years [IQR: 30-53] with a delay of 5 years [IQR: 1-8] between first relapsing polychondritis symptom and aortic involvement. Nineteen percent of the patients were asymptomatic at the time of aortic involvement diagnosis. The initial treatment was immunosuppressives in 41 patients (56%) and surgery in 28 patients (38%). The mortality ratio was 27% in a 24 month follow-up [IQR: 7.5-54 months]. Aortic dissection or rupture was the most frequent causes of mortality. Concomitant coronary artery involvement suggested a worse outcome. Aortic involvement in relapsing polychondritis is a mortal complication despite medical and surgical treatments. It may be asymptomatic in 19% of the patients which warrants the importance of screening.

Brucellosis aortitis: An interesting case.

A 24-year-old woman referred to our radiology department with the diagnosis of new positive blood culture brucellosis and severe aortic regurgitation for evaluation of periaortic thickening. Cardiac magnetic resonance imaging elegantly revealed the presence of active aortitis, most probably caused by brucellosis.

Aortitis masquerading as intramural hematoma: When to observe, when to operate? A case report.

Radiologic evidence of aortic disease is not always consistent with the diagnosis. With a lack of accompanying symptoms or with an atypical presentation, diagnosis, and management of aortic pathology rely greatly on imaging techniques. We report the case of a 58-year-old female who presented with incidental radiographic findings consistent with a type A aortic intramural hematoma and a vague left-sided chest discomfort. After follow-up, imaging was consistent with disease progression and hematoma expansion; the affected segment was resected and pathology reported lymphoplasmacytic aortitis as the underlying etiology of the imaging findings rather than an intramural hematoma. The patient lacked symptoms or serology consistent with the rheumatologic disease, and the postoperative course was uneventful. The management of a suspected ascending intramural hematoma is controversial, especially when the patient presents with atypical signs and symptoms. Features of disease progression may warrant urgent surgical intervention.