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A 54-year-old female presented with recurrent abdominal pain and new onset chest pain. Chest computed-tomography angiogram detected a thoracic aortic aneurysm with suspected Type A intramural hematoma (IMH) versus aortitis. Initially, conservative management was pursued while awaiting a definitive diagnosis. Differential workup was negative, while additional imaging modalities favored IMH, prompting expedited surgical intervention. During ascending aortic and hemiarch replacement, severe aortitis was unexpectedly discovered without evidence of IMH. Histopathological examination of the aortic specimens identified transmural aortic inflammation with lymphoplasmacytic infiltrate and irregular fibrosis. Numerous IgG4-positive plasma cells were present with IgG4/IgG ratio of 40–50% suggesting IgG4-related disease (IgG4-RD). Subsequent analysis revealed B cells positive for clonal IgH gene rearrangement, and bone marrow biopsy then revealed the same clonal B cells. She was ultimately diagnosed with CLL, the most common phenotype of monoclonal B-cell lymphocytosis, thought to account for the IgG4-predominant plasma cells causing aortitis. Although rare, this case highlights the importance of considering IgG4-related disease (IgG4-RD) as a cause of aortitis when assessing symptomatic patients with aortic pathologies, emphasizing the complexities involved in diagnosing due to a variety of imaging presentation, differentiating, and managing large-vessel vasculitides. Moreover, it underscores the importance of Multidisciplinary Aortic Team care and the use of multiple diagnostic modalities in evaluating ambiguous aortic pathologies. [ABSTRACT FROM AUTHOR]

Background: Penetrating aortic ulcers (PAU) are life-threatening conditions which derive from severely advanced atherosclerotic lesions of the aorta. The clinical course is unpredictable; thus clinical vigilance should be maintained. It is very challenging to separate PAU from co-existing AAS as predisposing factors and findings overlap. Case presentation: Case of 58-year-old gentleman, who presented for atypical chest pain in the setting of respiratory tract infection. Computed Tomographic angiography (CTA) of the chest showed a large PAU and intramural hematoma which rapidly progressed into an acute aortic dissection in the emergency department. Close follow up with cardiac point of care ultrasound one hour later detected an intimal flap which was not initially present on CTA. Patient underwent surgical aortic graft replacement and had an uneventful in-hospital stay. Discussion: This case underlines the importance of broadening differential diagnoses in atypical presentations in patients with risk factors. Prompt intervention and careful management are imperative to optimize patient outcomes and prevent complications of aortic lesions. Cardiac point of care ultrasound can help in detecting progression of dynamic atherosclerotic diseases such as acute aortic syndrome. [ABSTRACT FROM AUTHOR]

Abstract A 54-year-old female presented with recurrent abdominal pain and new onset chest pain. Chest computed-tomography angiogram detected a thoracic aortic aneurysm with suspected Type A intramural hematoma (IMH) versus aortitis. Initially, conservative management was pursued while awaiting a definitive diagnosis. Differential workup was negative, while additional imaging modalities favored IMH, prompting expedited surgical intervention. During ascending aortic and hemiarch replacement, severe aortitis was unexpectedly discovered without evidence of IMH. Histopathological examination of the aortic specimens identified transmural aortic inflammation with lymphoplasmacytic infiltrate and irregular fibrosis. Numerous IgG4-positive plasma cells were present with IgG4/IgG ratio of 40–50% suggesting IgG4-related disease (IgG4-RD). Subsequent analysis revealed B cells positive for clonal IgH gene rearrangement, and bone marrow biopsy then revealed the same clonal B cells. She was ultimately diagnosed with CLL, the most common phenotype of monoclonal B-cell lymphocytosis, thought to account for the IgG4-predominant plasma cells causing aortitis. Although rare, this case highlights the importance of considering IgG4-related disease (IgG4-RD) as a cause of aortitis when assessing symptomatic patients with aortic pathologies, emphasizing the complexities involved in diagnosing due to a variety of imaging presentation, differentiating, and managing large-vessel vasculitides. Moreover, it underscores the importance of Multidisciplinary Aortic Team care and the use of multiple diagnostic modalities in evaluating ambiguous aortic pathologies.

Abstract Background Penetrating aortic ulcers (PAU) are life-threatening conditions which derive from severely advanced atherosclerotic lesions of the aorta. The clinical course is unpredictable; thus clinical vigilance should be maintained. It is very challenging to separate PAU from co-existing AAS as predisposing factors and findings overlap. Case presentation Case of 58-year-old gentleman, who presented for atypical chest pain in the setting of respiratory tract infection. Computed Tomographic angiography (CTA) of the chest showed a large PAU and intramural hematoma which rapidly progressed into an acute aortic dissection in the emergency department. Close follow up with cardiac point of care ultrasound one hour later detected an intimal flap which was not initially present on CTA. Patient underwent surgical aortic graft replacement and had an uneventful in-hospital stay. Discussion This case underlines the importance of broadening differential diagnoses in atypical presentations in patients with risk factors. Prompt intervention and careful management are imperative to optimize patient outcomes and prevent complications of aortic lesions. Cardiac point of care ultrasound can help in detecting progression of dynamic atherosclerotic diseases such as acute aortic syndrome.

Introduction and importance: Spontaneous hemoperitoneum (SH) is a rare, life-threatening condition characterized by nontraumatic and non-iatrogenic intraperitoneal bleeding. This article explores three unique cases of SH, shedding light on unusual causes and emphasizing the critical role of diagnostic imaging and exploratory laparotomy in management. Methods: The study was a retrospective single-center non-consecutive case series. Results: We report three distinct cases of SH, each originating from uncommon sources: rupture of greater omentum arterio-venous malformation, a branch of the left gastric artery, and pathological splenic rupture. Clinical evaluation, diagnostic imaging, and surgical interventions are detailed for each case. Conclusion: These rare cases underscore the diverse etiologies of SH, including idiopathic omental bleeding, gastric intramural hematoma, and atraumatic splenic rupture. Enhanced CT imaging plays a crucial role in diagnosis, enabling the characterization of underlying pathologies. Exploratory laparotomy proves to be an essential treatment option for unstable patients with suspected or confirmed diagnoses of SH. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Blunt chest trauma caused ST‐segment elevation myocardial infarction. Diagnosis of intramural hematoma (IMH) using computed tomography was confirmed using electrocardiography, cardiac marker tests, and subsequent coronary angiography. After conservative treatment, the hematoma was completely resolved 1 year later. Differentiating IMH from other arterial injuries is critical for appropriate management. [ABSTRACT FROM AUTHOR]

Abstract Background Aortic intramural hematoma (IMH) is one of the typical entities of acute aortic syndrome and probably accounts for 5–25% of all cases. The ulcer-like projections (ULP), which are described as a focal, blood-filled pouch protruding into the hematoma of the aortic wall, are regarded as one of the high-risk imaging features of IMH and may cause initial medical treatment failure and death. Case presentation We present a case report of an acute type B IMH patient with impaired renal function and newly developed ULP in the acute phase. The 18F-fluorodeoxyglucose positron emission tomography/magnetic resonance imaging (18F-FDG PET/MR) was performed to evaluate the condition of aortic hematoma. The 18F-FDG focal uptake along the aortic wall of the hematoma was normal compared to the background (SUVmax 2.17; SUVSVC 1.6; TBR 1.35). We considered the IMH stable in such cases and opted for medical treatment and watchful observation. Six months after discharge, the patient’s recovery was satisfactory, and aortic remodeling was ideal. Conclusions The 18F-FDG PET/MR is a novel tool to evaluate the risk of IMH patients and thus provides information for therapy selection.

Introduction: This study aimed to emphasize the importance of cranial nerve (CN) palsies in spontaneous cervical artery dissection (sCeAD). Methods: A search term-based literature review was conducted on "cervical artery dissection" and "cranial nerve palsy." English and German articles published until October 2023 were considered. Results: Cranial nerve (CN) palsy in sCeAD is evident in approximately 10% of cases. In the literature, isolated palsies of CN II, III, VII, IX, X, and XII have been reported, while CN XI palsy only occurs in combination with other lower cranial nerve palsies. Dissection type and mural hematoma localization are specific to affected CN as CN palsies of II or III are solely evident in those with stenoocclusive vessel pathologies located at more proximal segments of ICA, while those with CN palsies of IX, X, XI, and XII occur in expansive sCeAD at more distal segments. This dichotomization emphasizes the hypothesis of a different pathomechanism in CN palsy associated with sCeAD, one being hypoperfusion or microembolism (CN II, III, and VII) and the other being a local mass effect on surrounding tissue (CN IX, X, XI, and XII). Clinically, the distinction between peripheral palsies and those caused by brainstem infarction is difficult. This differentiation is key, as, according to the reviewed cases, peripheral cranial nerve palsies in sCeAD patients mostly resolve completely over time, while those due to brainstem stroke do not, making cerebrovascular imaging appraisal essential. Discussion: It is important to consider dissections as a potential cause of peripheral CN palsies and to be aware of the appropriate diagnostic pathways. This awareness can help clinicians make an early diagnosis, offering the opportunity for primary stroke prevention. [ABSTRACT FROM AUTHOR]

Objective: This single-center retrospective study evaluated early and midterm outcomes of 100 consecutive patients with type A intramural hematoma. Methods: Initial watch-and-wait strategy was indicated if the maximum aortic diameter was < 50 mm, pain score was < 3/10 on the numerical rating scale, and no ulcer-like projection was observed in the ascending aorta. The primary endpoints of this study were all-cause and aorta-related deaths, and the secondary endpoint was aortic events. Results: Initial watch-and-wait strategy was indicated in 52 patients. Emergency aortic repair was indicated in the remaining 48 patients; 2, 31, and 15 patients died before surgery, underwent emergency surgery, and declined emergency surgery, respectively. Among the watch-and-wait group, 11 (21%) patients underwent aortic repair during hospitalization. In-hospital mortality rates, 5-year survival rates, and 5-year freedom from aorta-related death were not significantly different between the initial watch-and-wait strategy and emergency surgery (2% vs. 6%, 92% vs. 82%, and 100% vs. 94%, respectively). In the initial watch-and-wait strategy group, 5-year freedom from aortic events and freedom from aortic events involving the ascending aorta were 60% and 66%, respectively. Conclusions: The early and midterm outcomes with the initial watch-and-wait strategy in patients with type A intramural hematoma with a maximum aortic diameter of ≤ 50 mm, pain score of ≤ 3/10, and no ulcer-like projection in the ascending aorta were favorable with no aorta-related death. [ABSTRACT FROM AUTHOR]

Spontaneous coronary artery dissection (SCAD) is a rare but well-recognized cause of acute coronary syndrome, especially important in women. Invasive coronary angiography (ICA) is the fundamental diagnostic technique for the confirmation of SCAD. Knowing the angiographic patterns suggestive of SCAD is essential for the correct identification of patients with this entity. In this narrative review, the main angiographic characteristics of SCAD lesions as detected by ICA are presented and discussed. In addition to the specific angiographic classification of SCAD, several authors have described complementary angiographic patterns suggestive of SCAD. Knowledge and correct identification of these angiographic patterns is essential for the correct diagnosis of patients with clinical suspicion of SCAD. [ABSTRACT FROM AUTHOR]

Purpose: This study reports the outcomes from a Multicenter Registry on unibody stent-graft system for the treatment of spontaneous infrarenal acute aortic syndrome (MURUSSIAS registry). Materials and methods: The retrospective MURUSSIAS registry included spontaneous infrarenal acute aortic dissection (IAAS) managed with the unibody stent-graft system (AFX endovascular AAA system; Endologix Inc., Irvine, California) outside the current instruction for use. IAAS considered aortic dissection (AD), intramural hematoma (IMH), and penetrating aortic ulcer (PAU). Indications to IAAS treatment were symptoms, associated dilated abdominal aorta (>3 cm), rapidly-growing (>0.5 cm/6 months) aorta, IAAS disease progression. Measured results were technical success, early (within 30 days) and midterm outcomes (after 30 days), including mortality, complications, symptoms recurrence, type I/III endoleak occurrence, stent-graft patency, survival, and freedom from reintervention. The mean follow-up was 22.12 ± 17 months. Results: The MURUSSIAS registry included 83 patients from 7 participating centers. IAAS indication to treatment were symptoms in 42 (51%). In 14 (17%) patients, the infrarenal aortic length was <80 mm, and in 28 (34%), the aortic bifurcation diameter was <16 mm. Technical success was 100%. Mortality occurred early in 1 (1%) and at the midterm in 3 (4%) patients. Complications occurred early in 10 (12%) patients (1 severe, 3 moderates, and 6 mild) and at midterm in 2 (2%) (2 moderate). No symptoms' recurrence or type I/III endoleaks were registered. The 36-month estimated survival and freedom from reinterventions were 89% and 92%, respectively. Conclusions: The MURUSSIAS registry is the largest collection of spontaneous IAAS managed endovascularly using the AFX endovascular AAA system. The IAAS peculiar anatomic features were fitted with the used technique with excellent results. This treatment strategy might be considered in IAAS unless specifically-designed endovascular solutions will be available also in the emergent setting. Further studies are required to assess the longer-term performances and the stability of the reported technique. Clinical Impact: The lack of specifically designed devices for infrarenal acute aortic syndrome (IAAS) disease remains an issue principally for its specific anatomic features. The MURUSSIAS registry retrospectively examined the outcomes of spontaneous IAAS treated using the unibody stent-graft system in a spontaneous national study; and reports the largest available data on this topic. The use of the unibody stent-graft system showed to fit the anatomic peculiarities of IAAS with excellent outcomes. This IAAS treatment strategy should be considered unless specifically designed endovascular solutions will be available. [ABSTRACT FROM AUTHOR]

Background: Aortic intramural hematoma (IMH) is one of the typical entities of acute aortic syndrome and probably accounts for 5–25% of all cases. The ulcer-like projections (ULP), which are described as a focal, blood-filled pouch protruding into the hematoma of the aortic wall, are regarded as one of the high-risk imaging features of IMH and may cause initial medical treatment failure and death. Case presentation: We present a case report of an acute type B IMH patient with impaired renal function and newly developed ULP in the acute phase. The 18F-fluorodeoxyglucose positron emission tomography/magnetic resonance imaging (18F-FDG PET/MR) was performed to evaluate the condition of aortic hematoma. The 18F-FDG focal uptake along the aortic wall of the hematoma was normal compared to the background (SUVmax 2.17; SUVSVC 1.6; TBR 1.35). We considered the IMH stable in such cases and opted for medical treatment and watchful observation. Six months after discharge, the patient's recovery was satisfactory, and aortic remodeling was ideal. Conclusions: The 18F-FDG PET/MR is a novel tool to evaluate the risk of IMH patients and thus provides information for therapy selection. [ABSTRACT FROM AUTHOR]

This work aims to provide a comprehensive description of the characteristics of a group of acute aortic diseases that are all potentially life-threatening and are collectively referred to as acute aortic syndromes (AASs). There have been recent developments in the care and diagnostic plan for AAS. A substantial clinical index of suspicion is required to identify AASs before irreversible fatal consequences arise because of their indefinite symptoms and physical indicators. A methodical approach to the diagnosis of AAS is addressed. Timely and suitable therapy should be started immediately after diagnosis. Improving clinical outcomes requires centralising patients with AAS in high-volume centres with high-volume surgeons. Consequently, the management of these patients benefits from the increased use of aortic centres, multidisciplinary teams and an "aorta code". Each acute aortic entity requires a different patient treatment strategy; these are outlined below. Finally, numerous preventive strategies for AAS are discussed. The keys to good results are early diagnosis, understanding the natural history of these disorders and, where necessary, prompt surgical intervention. It is important to keep in mind that chest pain does not necessarily correspond with coronary heart disease and to be alert to the possible existence of aortic diseases because once antiplatelet drugs are administered, a blocked coagulation system can complicate aortic surgery and affect prognosis. The management of AAS in "aortic centres" improves long-term outcomes and decreases mortality rates. [ABSTRACT FROM AUTHOR]

We aimed to investigate predictors of intervention of acute type B aortic penetrating ulcer (PAU) and intramural hematoma (IMH). We conducted a retrospective chart review of all patients admitted for acute type B PAU or IMH in a tertiary referral hospital. Indications to intervention were "complicated" (rupture, impending rupture, malperfusion) or "high risk for unfavorable outcome" (refractory hypertension and/or pain despite best medical treatment, morphologic aortic evolution, transition to a new aortic syndrome, or increase in IMH/PAU depth >5 mm) during the acute/subacute phase. The primary outcomes were overall mortality, aortic-related mortality, and freedom from intervention. Time-dependent outcomes were estimated with Kaplan-Meier curves. Cox proportional hazards models were used to identify predictors of intervention and mortality. There were 54 acute aortic syndromes, 37 PAUs and 17 IMHs. Mean age was 69 ± 14 years and 33 patients (62.2%) were male. Six (11.5%) patients had complicated aortic syndromes and underwent urgent repair. Two (3.7%) additional patients developed an impending rupture during the acute phase. Eleven (21.1%) patients were classified as at "high risk" during the initial hospitalization. Overall, 22 (40.7%) patients required an aortic intervention during the initial admission (n = 16, 72.7% during the acute phase; n = 6, 27.3% during the subacute phase). In-hospital mortality was 5.5% (1 PAU and 2 IMH), and was aorta-related in all cases. For IMH, disease extension in >3 aortic zones (HR 1.94, 95%CI 1.17–32.6; p = 0.038) and presence of ulcer-like projections (ULPs) (HR 1.23, 95%CI 1.02–9.41; p = 0.042) were associated with the need for intervention. There were no aortic-related deaths or intervention during the chronic phase. PAU width >20 mm (HR 1.68, 95%CI 1.07–16.08; p = 0.014), PAU depth >15 mm (HR 6.74, 95%CI 1.31–34.18; p = 021), PAU depth/total aortic diameter >0.3 (HR 4.31, 95%CI 1.17–20.32; p = 0.043), and location at the level of the paravisceral aorta (HR 2.24, 95%CI 1.23–4.70; p = 0.035) were significantly associated with need for intervention. Six additional (16.2%) PAUs required intervention during the chronic phase owing to PAU growth. Maximum aortic diameter >35 mm was significantly associated with intervention (HR 1.45, 95%CI 1.00–2.32; p = 0.037). Acute symptomatic type B IMHs and PAUs are characterized by a high risk of complications during the first month from presentation. Morphologic features associated with intervention were IMH with ULPs or extension in more than 3 aortic zones, as well as PAUs with depth>15 mm, width >20 mm, or depth/aortic diameter ratio>0.3. A strict follow-up protocol or consideration for early intervention within 30 days from presentation should be taken into account for these high-risk patients. During the chronic phase imaging follow-up is particularly important for PAUs in order to identify progression to saccular aneurysms. [Display omitted] [ABSTRACT FROM AUTHOR]

Key Clinical Message Blunt chest trauma caused ST‐segment elevation myocardial infarction. Diagnosis of intramural hematoma (IMH) using computed tomography was confirmed using electrocardiography, cardiac marker tests, and subsequent coronary angiography. After conservative treatment, the hematoma was completely resolved 1 year later. Differentiating IMH from other arterial injuries is critical for appropriate management.

Abstract Coronary heavy calcification (HC) poses a sturdy challenge to percutaneous coronary intervention (PCI). Scores considering calcification length, thickness, or circumferential extent, are widely accepted to dictate upfront calcium modification to improve PCI outcomes. Although often marginalized, calcification shape (morphology) may require consideration during procedure planning in selected cases. This case demonstrates how a focal but spur-shaped calcification led to a massive proximal left anterior descending (LAD) dissecting intramural hematoma.

Amyloid light-chain (AL) amyloidosis rarely causes colorectal submucosal hematoma. A 76-year-old man presented with a complaint of bloody stool. An initial colonoscopy revealed ulcerative lesions in the descending colon, leading to a diagnosis of ischemic colitis. One month later, he presented with cardiac failure, suspected cardiac amyloidosis, and underwent a second colonoscopy. Although it revealed multiple ulcerative lesions from the ascending to transverse colon, biopsy samples did not confirm amyloid deposition. He underwent a third colonoscopy 3 weeks later due to recurrent bloody stool. It showed multiple submucosal hematomas from the ascending to descending colon concomitant with ulcerative lesions in the descending colon and multiple elevated lesions in the sigmoid colon. Biopsy samples confirmed amyloid deposition. Using a systemic search, multiple myeloma with AL amyloidosis was diagnosed. Colorectal submucosal or intramural hematomas are conditions usually encountered in trauma, antithrombotic use, or coagulation disorders. Based on our review of the literatures, we identified several differences between colorectal intramural hematoma caused by amyloidosis and those caused by other etiologies. We believe that amyloidosis should be considered when relatively small and multiple colorectal hematomas, not restricted to the sigmoid colon, and with concomitant findings of erosions and ulcers, are observed. [ABSTRACT FROM AUTHOR]

Coronary heavy calcification (HC) poses a sturdy challenge to percutaneous coronary intervention (PCI). Scores considering calcification length, thickness, or circumferential extent, are widely accepted to dictate upfront calcium modification to improve PCI outcomes. Although often marginalized, calcification shape (morphology) may require consideration during procedure planning in selected cases. This case demonstrates how a focal but spur-shaped calcification led to a massive proximal left anterior descending (LAD) dissecting intramural hematoma. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES This study aimed to investigate changes in haematoma thickness during the early period and their association with disease progression in patients who received initial medical treatment for type A intramural haematoma (IMH). METHODS Medical records and serial computed tomography angiography (CTA) images of patients who did not undergo emergency aortic repair for type A IMH upon presentation were retrospectively reviewed. The haematoma remodelling rate was determined using the following equation: thickness   of   the   haematoma   on   the   first   CTA   (mm)   -   thickness   of   the   haematoma   on   the   second   CTA   (mm) time   between   the   first   and   second   CTAs   (h). RESULTS Among the 40 patients included in this study, 38 were indicated for initial watch-and-wait strategy, whereas 2 were indicated for emergency aortic repair but declined it. During hospitalization, 10 patients developed disease progression, with 2 in-hospital mortality cases. Analysis of the haematoma remodelling rate in 39 patients revealed that such a rate was significantly associated with the reciprocal of the time from onset. Analysis of all 70 CTA examinations performed within 24 h after the onset of IMH showed that haematoma thickness was significantly associated with the logarithm of the time from onset. Initial regression of the haematoma was not necessarily associated with avoidance of disease progression. CONCLUSIONS In type A IMH, the thickness of the haematoma in the ascending aorta tended to decrease in the very early period; however, prompt regression of the haematoma was not necessarily associated with avoidance of disease progression. [ABSTRACT FROM AUTHOR]

Acute aortic syndrome; includes aortic dissection, intramural hematoma, symptomatic penetrating aortic ulcer. However, these three pathologies form a broad terminology group, including thoracic aortic aneurysm and traumatic aortic rupture, depending on their progression. Aortic dissection is the most common type. Type A aortic dissection requires emergency surgery, while Type B requires endovascular therapy. Intramural hematoma was defined as aortic wall hematoma without intimal flap. The ruptured aorta may progress to aortic dissection, aneurysm or pseudoaneurysm, or it may show complete resolution. It can be divided into proximal (type A) and distal (type B) Acute Aortic Syndrome according to the aortic segment involved surgically and prognostically. Surgical treatment can be recommended in most cases of type A intramural hematomas. In intramural hematomas localized in the descending aorta, there was generally no difference in survival with surgical and medical treatment. Penetrating aortic ulcer; It describes ulceration of an atherosclerotic lesion that progresses to the media layer by perforating the internal elastic lamina. These patients may not progress clinically. Traumatic aortic rupture or transection are also aortic lesions that require early intervention. Treatment methods, surgical or endovascular interventions can be adjusted according to the clinical conditions of the patients. [ABSTRACT FROM AUTHOR]

(1) Background: Spontaneous isolated intramural hematoma of the superior mesenteric artery (SIHSMA) is a rare entity often considered as a subset of spontaneous isolated dissection of the superior mesenteric artery (SIDSMA). It is characterized by a completely thrombosed false lumen with or without an ulcer-like projection with computed tomography (CT) imaging. The recent literature describes few reports with a relatively short-term follow-up. The natural course, prognosis, and treatment options for SIHSMA still lack consensus. We present two cases of acute abdominal pain in a young man due to IMH of the superior mesenteric artery with an extensive literature review. (2) Case report: A 46-year-old male patient was submitted to an urgent CTA for acute abdominal pain, showing the presence of an isolated dissection of the superior mesenteric artery, determining significant stenosis of the vessel with collateral vessel patency. The patient referred to a recent COVID-19 infection, whose course was paucisymptomatic. He was conservatively treated with antiplatelet therapy and corticosteroid treatments, and, after a few days, the symptomatology completely regressed; also, the 2-month-control CTA showed complete IMH regression and the absence of any signs of residual stenosis. The second patient was a 61-year-old male patient who was submitted to an urgent CTA for acute abdominal pain, showing the presence of an isolated dissection of the superior mesenteric artery, not determining significant vessel stenosis. He was conservatively treated with antiplatelet therapy and corticosteroid treatment, and after a few days, the symptomatology completely regressed and the radiological control showed complete dissection regression. (3) Conclusion: SISHSMA is a rare entity of vascular pathology, and conservative management represents the best medical strategy. We propose corticosteroid treatment as one of the most appropriate tools in the conservative treatment of SISHSMA. [ABSTRACT FROM AUTHOR]

IntroductionThis study aimed to emphasize the importance of cranial nerve (CN) palsies in spontaneous cervical artery dissection (sCeAD).MethodsA search term-based literature review was conducted on “cervical artery dissection” and “cranial nerve palsy.” English and German articles published until October 2023 were considered.ResultsCranial nerve (CN) palsy in sCeAD is evident in approximately 10% of cases. In the literature, isolated palsies of CN II, III, VII, IX, X, and XII have been reported, while CN XI palsy only occurs in combination with other lower cranial nerve palsies. Dissection type and mural hematoma localization are specific to affected CN as CN palsies of II or III are solely evident in those with steno-occlusive vessel pathologies located at more proximal segments of ICA, while those with CN palsies of IX, X, XI, and XII occur in expansive sCeAD at more distal segments. This dichotomization emphasizes the hypothesis of a different pathomechanism in CN palsy associated with sCeAD, one being hypoperfusion or microembolism (CN II, III, and VII) and the other being a local mass effect on surrounding tissue (CN IX, X, XI, and XII). Clinically, the distinction between peripheral palsies and those caused by brainstem infarction is difficult. This differentiation is key, as, according to the reviewed cases, peripheral cranial nerve palsies in sCeAD patients mostly resolve completely over time, while those due to brainstem stroke do not, making cerebrovascular imaging appraisal essential.DiscussionIt is important to consider dissections as a potential cause of peripheral CN palsies and to be aware of the appropriate diagnostic pathways. This awareness can help clinicians make an early diagnosis, offering the opportunity for primary stroke prevention.

BackgroundIntracranial artery dissection (IAD) often causes headache and cerebral vascular ischemic events. The imaging characteristics of IAD remain unclear. This study aims to characterize the appearance of culprit and non-culprit IAD using high-resolution cardiovascular magnetic resonance imaging (hrCMR) and quantify the incremental value of hrCMR in identifying higher risk lesions.MethodsImaging data from patients who underwent intervention examination or treatment using digital subtraction angiography (DSA) and hrCMR using a 3 T CMR system within 30 days after the onset of neurological symptoms were collected. The CMR protocol included diffusion-weighted imaging (DWI), black blood T1-, T2- and contrast-enhanced T1-weighted sequences. Lesions were classified as culprit and non-culprit according to imaging findings and patient clinical presentations. Univariate and multivariate analyses were performed to assess the difference between culprit and non-culprit lesions and complementary value of hrCMR in identifying higher risk lesions.ResultsIn total, 75 patients were included in this study. According to the morphology, lesions could be classified into five types: Type I, classical dissection (n = 50); Type II, fusiform aneurysm (n = 1); Type III, long dissected aneurysm (n = 3); Type IV, dolichoectatic dissecting aneurysm (n = 9) and Type V, saccular aneurysm (n = 12). Regression analyses showed that age and hypertension were both associated with culprit lesions (age: OR, 0.83; 95% CI 0.75-0.92; p

Introduction: In India, Cerebrovascular Disease (CVD) remains to be one of the leading causes of mortality and morbidity. In recent times, the imaging of cerebrovascular disorders has undergone various advancements with the advent of digital technologies. Two novel imaging modalities include Magnetic Resonance Angiography (MRA) and Computed Tomographic Angiography (CTA). Aim: To assess and compare Computed Tomography Angiogram (CTA) brain vs non contrast MR angiogram brain in evaluation of CVD. Materials and Methods: A cross-sectional observational study was conducted in the Department of Radiology, Arupadai Veedu Medical College and Hospital, Puducherry, India, from November 2020 to July 2022. All suspected cases of cerebrovascular accidents who were above 18 years of age were screened for the study. Finally, a total of 60 patients were recruited for the study. Demographic parameters like age, gender and occupation were collected. Past history of chronic illness, vitals, serum urea and creatinine were also assessed. MRA findings including stenosis, occlusion, and irregularity of vessels and features of stroke were assessed and compared to the findings on CTA images. Data was compiled and analysed using Statistical Package for the Social Sciences (SPSS) software version 19.0. Results: A maximum of 26 (43.3%) participants were in the age group of 61-70 years of age and, 60% of the cases were males and 40% of the cases were females. Out of the 60 patients, 40 (80%) had characteristics of CVD based on CTA, and 47 (78.3%) of the individuals indicated of CVD based on MRA results. The differences between MRA and CTA for CVD alterations were significant (p-value

Abstract Background Intramural hematoma (IMH) and Aortic dissection (AD) have overlapping features. The aim of this study was to explore the differences between them by comparing the clinical manifestations and imaging features of patients with acute Stanford type B IMH-like lesions and acute Stanford type B AD (ATBAD). Methods This study retrospectively analysed the clinical and computed tomography angiography (CTA) imaging data of 42 IMH-like lesions patients with ulcer-like projection (ULP) and 38 ATBAD patients, and compared their clinical and imaging features. Results (1) The IMH-like lesions patients were older than the ATBAD patients (64.2 ± 11.5 vs. 50.9 ± 12.2 years, P 0.05). (3) The maximum diameter of the ULPs in IMH-like lesions was smaller than that of the intimal tears in ATBAD (7.4 ± 3.4 vs. 10.8 ± 6.8 mm, P = 0.005). The lumen compression ratio in the ULPs plane and the maximum compression ratio of the aortic lumen in the IMH-like lesions group were smaller than that in the ADBAD group (P

Objective: Antithrombotic therapy has the potential to interfere with false lumen thrombosis. In type B acute aortic syndrome, the degree of false lumen thrombosis affects clinical outcomes. We aimed to explore the association of antithrombotic therapy with the prognosis of patients with type B acute aortic syndrome. Methods: We reviewed 406 patients with type B acute aortic syndrome who were discharged alive with and without antithrombotic therapy. The primary outcome was aorta-related adverse events, defined as a composite of aorta-related death, aortic rupture, aortic repair, and progressive aortic dilation. Results: Of the 406 patients, 64 (16%) were discharged with antithrombotic therapy and 342 (84%) were discharged without antithrombotic therapy. A total of 249 patients (61%) presented with intramural hematoma with complete thrombosis of the false lumen, and 157 patients (39%) presented with aortic dissection. During a median follow-up of 4.6 years, 32 patients (50%) in the antithrombotic group and 93 patients (27%) in the nonantithrombotic group had a primary outcome event. Cumulative incidence of aorta-related events at 1 and 3 years with death as the competing risk was higher in the antithrombotic group than in the nonantithrombotic group (19% ± 5% vs 9% ± 2% at 1 year and 40% ± 7% vs 17% ± 2% at 3 years, P

The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes). A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate. Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease. [ABSTRACT FROM AUTHOR]

Although endovascular technology has resulted in a paradigm shift in treatment, medical management remains the standard of care for penetrating aortic ulcer (PAU) and intramural hematoma (IMH). This study aimed to detail the short- and long-term outcomes of symptomatic PAU/IMH. Institutional data on symptomatic PAU/IMH were gathered (2005-2020). The primary outcome was the composite of recurrent symptoms, radiographic progression, intervention, rupture, and death from related or unknown cause. Factors associated with the primary outcome were determined using a Fine-Gray model with death from an unrelated cause as a competing risk. A total of 83 symptomatic patients treated with medical management aside from ruptures and type A dissections: 21 isolated PAU, 30 isolated IMH, and 32 IMH and PAU. Adverse outcomes included symptom recurrence in 14 (16.9%), radiographic progression to dissection or saccular aneurysm in 17 (20.5%), surgery in 20 (24.1%) (17 thoracic endovascular aortic repair, 1 endovascular aortic repair, 1 frozen elephant trunk, and 1 open repair), and rupture in 4 (4.8%). Twenty-seven patients (32.5%) died during follow-up: 6 from IMH treatment complications, 8 from an unknown cause, and 13 from other causes. The 30-day, 1-year, and 5-year cumulative incidences of the primary outcome was 26.5% (95% confidence interval [CI], 16.9%-37.0%), 44.9% (95% CI, 32.8%-56.2%), and 57.5% (95% CI, 42.4%-69.9%), respectively. IMH with PAU was associated with a significantly higher risk of the primary outcome compared with isolated IMH (subdistribution hazard ratio, 2.21; 95% CI, 1.09-4.50; P =.027) and isolated PAU (subdistribution hazard ratio, 3.58; 95% CI, 1.44-8.88; P =.006). Complications from symptomatic PAU and IMH are frequent, with intervention, recurrent symptoms, radiographic progression, rupture, or death affecting 25% of patients at 30 days after diagnosis and almost one-half of patients 1 year after diagnosis. Given the high rate of adverse events in this population, investigation into a more aggressive interventional strategy may warranted, especially in patients with a combined IMH and PAU. Although endovascular technology has resulted in a paradigm shift in treatment, medical management often remains the standard of care for symptomatic penetrating aortic ulcer (PAU) and intramural hematoma (IMH). Complications of intervention, recurrent symptoms, radiographic progression, rupture, or death affected 25% of patients at 30 days after diagnosis and almost one-half of patients 1 year after diagnosis. Medical management alone may be inappropriate for symptomatic PAU, IMH, and IMH with PAU. Thoracic endovascular aortic repair could be a more effective aggressive interventional strategy for IMH with PAU. [ABSTRACT FROM AUTHOR]

Intracranial artery dissection accounts for a small percentage (1%-2%) of all ischemic strokes. Vertebral artery dissection sometimes extends to the basilar artery but very rarely to the posterior cerebral artery. We report a case of bilateral vertebral artery dissection extending to the left posterior cerebral artery with the characteristic distribution of intramural hematoma. A 51-year-old woman presented with right hemiparesis and dysarthria 3 days after sudden neck pain. Magnetic resonance imaging on admission revealed infarcts in the left thalamus and temporo-occipital lobe and findings suggestive of bilateral vertebral artery dissection. No infarct was detected in the brainstem. The patient was treated conservatively. Initially, we suspected that infarction in the left posterior cerebral artery territory had been caused by artery-to-artery embolism from the dissected vertebral arteries. However, T1-weighted imaging on day 15 of admission revealed intramural hematoma extending from the left vertebral artery to the left posterior cerebral artery. Therefore, we diagnosed bilateral vertebral artery dissection extending to the basilar artery and the left posterior cerebral artery. The patient's symptoms subsequently improved with conservative treatment, and she was discharged with a modified Rankin Scale score of 1 on day 62 of admission. In this case, intramural hematoma of the basilar artery was found in the anterior vessel wall. Brainstem infarction is less likely when intramural hematoma is located in the anterior vessel wall of the basilar artery in vertebrobasilar artery dissection. T1-weighted imaging is useful for the diagnosis of this rare condition and can predict potentially impaired branches and possible symptoms.

Abstract Background The current treatment for retrograde ascending aortic intramural hematoma (RAIMH) remains challenging. This study aims to summarize the short-term results of endovascular repair in the treatment of retrograde ascending aortic intramural hematoma. Methods Between June 2019 and June 2021, 21 patients (16 males and 5 females) with a retrograde ascending aortic intramural hematoma, aged 53 ± 14years, received an endovascular repair in our hospital. All cases involved an ascending aortic or aortic arch intramural hematoma. 15 patients had an ulcer on the descending aorta combined with an intramural hematoma in the ascending aorta and 6 patients had typical dissection changes on the descending aorta combined with an intramural hematoma in the ascending aorta. All patients had a successful endovascular stent-graft repair, with 10 cases operated on in the acute phase (

Abstract We reported the case of a 33‐year‐old male who presented a dengue infection complicated by spontaneous coronary artery intramural hematoma associated with acute myocarditis. The initial presentation was a typical acute coronary syndrome with ST‐segment elevation. Coronary angiography and endocoronary optical coherence tomography confirmed the diagnosis of left anterior descending artery intramural hematoma. Cardiac magnetic resonance imaging revealed not only typical ischaemic injury but also lesions of acute myocarditis confirmed by native T1‐ and T2‐mapping, sub‐epicardial late gadolinium enhancement and pericardial effusion. This case highlights the multiple cardiac damages caused by dengue virus, their possible association (coincidental or linked?), and the impact of multimodal imaging on diagnosis and management.

We describe herein an autopsy case involving fatal hemorrhagic shock due to idiopathic sigmoid colonic intramural hematoma rupturing into the abdominal cavity. Antemortem computed tomography revealed a mass lesion in the sigmoid colon and a large amount of hemoperitoneum. On forensic autopsy, intramural hematoma of the sigmoid colon with ruptured serosa was identified, while the mucosa remained intact. Microscopically, hematomas were apparent, mainly in the muscularis propria. We diagnosed the cause of death as hemorrhagic shock due to idiopathic intramural hematoma of the sigmoid colon rupturing into the abdominal cavity. Although the patient had been receiving peritoneal dialysis, no relationship was identified between dialysis catheters and sigmoid colon intramural hematoma rupture. On computed tomography, the mass lesion was initially considered a submucosal neoplastic lesion or endometriotic lesion. Intramural hematoma should be considered as a differential diagnosis.

IntroductionSCAD involves a sudden tear or separation within the layers of the coronary artery wall, resulting in blood flow obstruction and subsequent myocardial ischemia.Materials and methodsA comprehensive literature search was conducted to identify relevant published cases of cutting balloon use in patients diagnosed with spontaneous coronary artery dissection. Electronic databases including PubMed, MEDLINE, Embase, Cochrane Library and Google Scholar were systematically searched from inception until the present using terms “cutting balloon,” “SCAD,” “acute coronary syndrome,” “intramural hematoma,” and “angioplasty.”ResultsA total of 32 published cases of cutting balloon use in spontaneous coronary artery dissection were analyzed in this study. The majority of the patients included in the analysis were female without prior history of cardiovascular disease. The median age of the SCAD population was approximately 46 years. The most frequently affected artery in SCAD cases was the Left Anterior Descending artery. Intravascular ultrasound was utilized more frequently than other modalities of adjunctive imaging techniques. The most frequent complication was the distal propagation of hematoma. Despite the successful dilation achieved with the cutting balloon, in some cases stenting was required to provide additional support.ConclusionThe results of this analysis demonstrate that cutting balloon use in SCAD cases yields favorable outcomes.

Thoracic endovascular aortic repair (TEVAR) is the preferred treatment for complicated type B aortic dissection (TBAD) or intramural hematoma (IMH). This study aimed to investigate the association of the proximal landing zone and its morphology with long-term outcomes in patients with TBAD or IMH. A total of 94 patients who underwent TEVAR for TBAD or IMH between 10/2003 and 01/2020 were included. The cohort was divided according to the proximal landing in Ishimaru zone 2 or 3 and the presence of a healthy landing zone (HLZ; non-dissected or aneurysmatic, ≥2 cm length). Primary outcome was freedom from aortic reintervention. Secondary endpoints were freedom from aortic growth, stroke, spinal cord ischemia, retrograde dissection, proximal stent-graft induced new entry (pSINE), debranching failure, and mortality. Outcomes were assessed using Cox proportional hazard models with mortality as a competing risk. A proximal TEVAR landing in zone 2 was associated with higher rates of reinterventions compared to zone 3 (33% vs. 15%, p = 0.031), spinal cord ischemia (8% vs. 0%, p = 0.037), and pSINE (13% vs. 2%, p = 0.032). No difference was found for the other outcomes, including mortality. Landing in dissected segments was not associated with impaired results. Proximal TEVAR landing in zone 3 may be preferable with regard to long-term aortic reintervention in patients with TBAD or IMH. [ABSTRACT FROM AUTHOR]

Background: Intramural hematoma (IMH) and Aortic dissection (AD) have overlapping features. The aim of this study was to explore the differences between them by comparing the clinical manifestations and imaging features of patients with acute Stanford type B IMH-like lesions and acute Stanford type B AD (ATBAD). Methods: This study retrospectively analysed the clinical and computed tomography angiography (CTA) imaging data of 42 IMH-like lesions patients with ulcer-like projection (ULP) and 38 ATBAD patients, and compared their clinical and imaging features. Results: (1) The IMH-like lesions patients were older than the ATBAD patients (64.2 ± 11.5 vs. 50.9 ± 12.2 years, P < 0.001). The D-dimer level in the IMH-like lesions group was significantly higher than that in the ATBAD group (11.2 ± 3.6 vs. 9.2 ± 4.5 mg/L, P < 0.05). The incidence rate of back pain was significantly higher in the ATBAD group than in the IMH-like lesions group (71.1% vs. 26.2%, P < 0.05). (2) The ULPs of IMH-like lesions and the intimal tears of ATBAD were concentrated in zone 4 of the descending thoracic aorta. The ULPs of IMH-like lesions and the intimal tears of ATBAD were mainly in the upper quadrant outside the lumen (64.3% vs. 65.8%, P > 0.05). (3) The maximum diameter of the ULPs in IMH-like lesions was smaller than that of the intimal tears in ATBAD (7.4 ± 3.4 vs. 10.8 ± 6.8 mm, P = 0.005). The lumen compression ratio in the ULPs plane and the maximum compression ratio of the aortic lumen in the IMH-like lesions group were smaller than that in the ADBAD group (P < 0.05). Fewer aortic segments were involved in IMH-like lesions patients than in ATBAD patients (5.6 ± 2.2 vs. 7.1 ± 1.9 segments, P < 0.005). The IMH-like lesions group had less branch involvement than that of the ATBAD group (P < 0.001). Conclusion: The degree of intimal tears, lumen compression ratio, extent of lesion involvement, and impact on branch arteries in ATBAD are more severe than that of IMH-like lesions. But for the ULPs of IMH-like lesions and intimal tears of ATBAD, they have astonishing similarities in the location of the partition and the lumen quadrant, we have reason to believe that intimal tear is the initial factor in the pathogenesis of this kind of disease, and their clinical and imaging manifestations overlap, but the severity is different. Concerning similarities between these two conditions, these two may be a spectrum of one disease. [ABSTRACT FROM AUTHOR]

A characteristic feature of communicating aortic dissections (CD) is the dissection flap between the true and false lumen. However, in intramural hematomas (IMH) a flap is not visible. We aimed to determine if cross-sectional HU variability allow reliable identification of aortic dissections including IMH. We included 362 patients presenting with acute chest pain (CP) or respiratory distress (RD) and underwent contrast-enhanced CTA with or without ECG-gating. In the derivation group we included 72 CP patients with and 74 without AAS. In the validation group we included 108 CP or RD patients with and 108 without AAS. The adventitial border of the aorta was visually identified and measurements were performed at 6 locations along the ascending and descending aorta. At each cross-section 5 circular ROI measurements of HU were made and the maximum HU difference calculated. In the derivation and validation group the maximum difference in HUs at any one location was significantly higher for AAS subjects than controls (validation group: median = 128.5 vs. 34.0, p -value Wilcoxon two-sample test <0.001). In the validation group, the estimated AUC was 0.939 with 95% CIs of [0.906, 0.972], indicating that the maximum difference in HUs is a strong predictor of AAS (p < 0.001). Our data provide evidence that cross-sectional variability of Hounsfield Unit reliably identifies aortic dissection including IMH in dedicated ECG-gated aorta scans but also non-gated chest CTs with limited aortic contrast enhancement. These results suggest that this approach could be feasible for an automated algorithm for identification of AAS. • We examined if cross-sectional Hounsfield Units (HU) variability allow reliable identification of aortic dissections including IMH. • 362 patients presenting with acute chest pain or respiratory distress were included. • the aorta was segmented and HU measurements were made at cross-sections in the ascending and descending aorta. • maximum difference in HUs at any one location was significantly higher for Acute Aortic Syndromes (AAS) subjects than controls. • cross-sectional variability of HU reliably identifies aortic dissection including IMH and could be feasible for an automated identification algorithm. [ABSTRACT FROM AUTHOR]

Introduction: In India, Cerebrovascular Disease (CVD) remains to be one of the leading causes of mortality and morbidity. In recent times, the imaging of cerebrovascular disorders has undergone various advancements with the advent of digital technologies. Two novel imaging modalities include Magnetic Resonance Angiography (MRA) and Computed Tomographic Angiography (CTA). Aim: To assess and compare Computed Tomography Angiogram (CTA) brain vs non contrast MR angiogram brain in evaluation of CVD. Materials and Methods: A cross-sectional observational study was conducted in the Department of Radiology, Arupadai Veedu Medical College and Hospital, Puducherry, India, from November 2020 to July 2022. All suspected cases of cerebrovascular accidents who were above 18 years of age were screened for the study. Finally, a total of 60 patients were recruited for the study. Demographic parameters like age, gender and occupation were collected. Past history of chronic illness, vitals, serum urea and creatinine were also assessed. MRA findings including stenosis, occlusion, and irregularity of vessels and features of stroke were assessed and compared to the findings on CTA images. Data was compiled and analysed using Statistical Package for the Social Sciences (SPSS) software version 19.0. Results: A maximum of 26 (43.3%) participants were in the age group of 61-70 years of age and, 60% of the cases were males and 40% of the cases were females. Out of the 60 patients, 40 (80%) had characteristics of CVD based on CTA, and 47 (78.3%) of the individuals indicated of CVD based on MRA results. The differences between MRA and CTA for CVD alterations were significant (p-value<0.0001). MRA had a diagnostic efficacy of 95% and had a sensitivity and specificity of 95.8% and 91.7% respectively, with PPV of 97.9%, and NPV of 84.6% as compared to CTA. Conclusion: The results of the present study demonstrate that MRA is equally effective as CTA in diagnosing cases with CVD. [ABSTRACT FROM AUTHOR]

Background and Purpose: Some cervical artery dissection (CAD) can't be easily confirmed by commonly used angiography techniques in clinical practice. We aimed to compare the abilities of the vessel wall magnetic resonance imaging (MRI) techniques including simultaneous noncontrast angiography and intraplaque hemorrhage (SNAP) sequence and T1-weighted volumetric isotropic turbo spin echo acquisition (T1-w VISTA) sequence alone for evaluating CAD. Materials and Methods: From July 2017 to October 2020, 59 patients underwent MRI examinations including SNAP and T1-w VISTA sequences for cervical artery pathologies. SNAP and T1-w VISTA images were retrospectively and independently reviewed to evaluate their diagnostic performances of CAD by using the final diagnosis as the reference standard which was established by clinical history, physical examination, and all available images. The agreement between T1-w VISTA and SNAP in the identification of the imaging features of CAD, including intramural hematoma (IMH), intimal flap, and double lumen, were compared. The IMH-wall contrasts by T1-w VISTA and SNAP were also compared. Results: CAD was confirmed in 43 of the 59 patients. T1-w VISTA and SNAP showed the same diagnostic performance, and their consistencies with the final diagnosis were good (κ = 0.776, p < 0.001). The sensitivity and specificity in CAD diagnosis were 0.978 and 0.750 for T1-w VISTA and SNAP. The IMH, intimal flap, and double lumen observed on SNAP were also determined by T1-w VISTA (κ = 1.000, p < 0.001 for all). The SNAP sequence showed higher IMH-wall contrast than T1-w VISTA (7.34 ± 4.56 vs. 3.12 ± 1.17, p < 0.001). Conclusions: SNAP and T1-w VISTA sequences had the same performance in CAD diagnosis, thus they were both recommended. In addition, SNAP showed better IMH-wall contrast than T1-w VISTA. [ABSTRACT FROM AUTHOR]

People with hemophilia (PWH), especially severe hemophilia, often experience bleeding episodes, which occur mostly at major joints. Intramural hematoma of the gastrointestinal (GI) tract is a rare, potentially life-threatening clinical bleeding manifestation in PWH. Prompt identification and timely administration of clotting factor concentrates are of utmost importance for effective management and optimal patient outcomes. In this report, we present the case of a 48-year-old male with severe hemophilia A. The patient developed a spontaneous intramural hematoma of the jejunum, leading to signs of acute abdomen, bloody stool, and paralytic ileus. Conservative management with factor VIII (FVIII) infusion was successfully administered. However, within a span of three months, the patient suffered from a recurrent episode of intramural hematoma, which was again effectively treated with conservative therapy. Subsequently, prophylactic FVIII therapy was administered to the patient, resulting in the absence of recurrence for over three years. Inspired by this case, we conducted a comprehensive review of the relevant literature and gathered data from 79 reported cases of intramural hematoma that were documented between the years 1956 and 2022. We classified these cases based on the site affected within the gastrointestinal (GI) tract (spread across five different locations) and proceeded to conduct a simple pooling analysis on the data collected, which subsequently revealed that the overall mortality rate of intramural hematoma in people with hemophilia (PWH) was found to be 12.2%, while children have a higher mortality rate (23.3%) than adults (4.9%). We hope this case report and literature review increase awareness of this rare bleeding manifestation in PWH, the effectiveness of conservative treatment, and the possibility of prophylaxis against recurrence. [ABSTRACT FROM AUTHOR]