Lobe Hematoma Research Articles

Clinical and non-hemorrhagic neuroimaging indicators of probable cerebral amyloid angiopathy as a cause of non-traumatic lobar hematomas

Objective. To identify clinical and non-hemorrhagic neuroimaging indicators of probable CAA in patients with acute non-traumatic lobar hematomas. Cerebral amyloid angiopathy (CAA) is a microangiopathy affecting the leptomeningial and cortical vessels of the brain due to the deposition of pathological b-amyloid in them. The most common clinical manifestation of CAA is lobar hematomas (LH) – spontaneous intracerebral hemorrhages localized between the cerebral cortex and basal ganglia. LH can also occur in hypertensive cerebral microangiopathy (hCMA) in patients with arterial hypertension. Since the tactics of managing patients with CAA and hCMA differ, it is important to determine the genesis of LH correctly. Materials and methods. A comparative analysis of clinical and neuroimaging characteristics of acute non-traumatic hypertension in 32 patients with probable CAA and hCMA was carried out. Along with neurological examination and neuroimaging, all patients underwent a study using the Montreal Cognitive Assessment Scale and the Benson Complex Figure Test to reveal visuospatial impairments. The diagnosis of probable CAA was carried out in accordance with the updated Boston criteria of 2010, the diagnosis of hCMA was based on clinical data, anamnesis and results of neuroimaging of the brain. Results. Probable CAA was diagnosed in 16 patients, and in all these cases it was combined with hCMA (1st subgroup). Isolated hCMA as a cause of LH was also observed in 16 patients (2nd subgroup). Patients of subgroup 1 were statistically significantly more likely to have clinically pronounced visual impairments, performed the MoCA subtest and the Benson Complex Figure Test worse, and the overall assessment of their cognitive functions according to Mo SA was lower than in patients of subgroup 2. According to neuroimaging data, in the 1st subgroup of patients, an expansion of perivascular spaces in the semi-oval center and a zero or negative value of the front-occipital gradient were more often detected. The application of the logistic regression method made it possible to integrate potential CAA indicators and create a prognostic model for revealing this pathology in patients with hypertension. Conclusions. Clinically pronounced disorders of primary and higher visual functions, a negative front-occipital gradient and expansion of perivascular spaces in the semi-oval centers can serve as indicators of probable CAA in patients with acute lobar hematoma. On admission of such patients to the vascular center, it is advisable to include iron-sensitive pulse sequences in the neuroimaging screening protocol to verify the diagnosis of CAA.

Input of exome sequencing in early-onset cerebral amyloid angiopathy.

Genetics of cerebral amyloid angiopathy (CAA) remains understudied. We assessed variants in Alzheimer's disease (AD) risk factor genes and differential diagnosis genes by performing exome sequencing among 78 patients with early-onset definite or probable CAA, after negative screening for APP mutation or duplication. Among 14 genes involved in non-Aβ CAA, or vascular leukoencephalopathies, we detected pathogenic NOTCH3 variants in two patients, who exhibited lobar hematomas at the ages of 58 and 65, leading to a diagnosis redirection toward CADASIL. Of the remaining 76 patients, 23.1% carried at least one apolipoprotein E (APOE) ε2 allele and 43.6% carried at least one APOE ε4 allele, known asCAA risk factors. A total of 15 out of 76 (19.7%) carried either a loss-of-function or a rare predicted damaging missense or known AD risk variant in SORL1,TREM2,ABCA7,ABCA1,andATP8B4. Exome sequencing allowed the redirection toward CADASIL in two patients and suggested shared genetic factors between AD and CAA, beyond the APOE gene. The genetic component of cerebral amyloid angiopathy (CAA) remains understudied.Rare differential diagnoses such as CADASIL should be considered, even in cases of cerebral hemorrhage.Our study suggests shared genetic factors between AD and CAA, beyond the APOE gene.Rare variants in SORL1, TREM2, ABCA7, ABCA1 and ATP8B4 might be susceptibility factors in early-onset CAA.

Time for “Code ICH”? Workflow Metrics of Hyperacute Treatments and Outcome in Patients with Intracerebral Haemorrhage

Introduction: Knowledge about uptake and workflow metrics of hyperacute treatments in patients with non-traumatic intracerebral haemorrhage (ICH) in the emergency department is scarce. Methods: Single-centre retrospective study of consecutive patients with ICH between 01/2018–08/2020. We assessed uptake and workflow metrics of acute therapies overall and according to referral mode (stroke code, transfer from other hospital, or other). Results: We enrolled 332 patients (age 73 years, IQR: 63–81 and GCS 14 points, IQR: 11–15, onset-to-admission time 284 min, IQR: 111–708 min), of whom 101 patients (35%) had lobar haematoma. Mode of referral was stroke code in 129 patients (38%), transfer from other hospital in 143 patients (43%), and arrival by other means in 60 patients (18%). Overall, 143 of 216 (66%) patients with systolic blood pressure >150 mm Hg received IV-antihypertensive treatment, and 67 of 76 (88%) on therapeutic oral anticoagulation received prothrombin complex concentrate treatment (PCC). Forty-six patients (14%) received any neurosurgical intervention within 3 h of admission. Median treatment times from admission to first IV-antihypertensive treatment were 38 min (IQR: 18–72 min) and 59 min (IQR: 37–111 min) for PCC, with significant differences according to mode of referral (p p = 0.92). The median time in the emergency department was 139 min (IQR: 85–220 min), and among patients with elevated blood pressure, only 44% achieved a successful control (p = 0.008) and non-significant trends towards better functional outcome measured using the modified Rankin scale score at 3 months (aOR for ordinal shift 0.54, 95% CI: 0.26–1.12, p = 0.097). Conclusion: Uptake of hyperacute therapies for ICH treatment in the ED is heterogeneous. Treatment delays are short, but not all patients achieve treatment targets during ED stay. Code ICH-concordant treatment may improve clinical outcomes. Further improvements seem achievable by advocating for a “code ICH” to streamline acute treatments.

CADMUS: A Novel MRI-Based Classification of Spontaneous Intracerebral Hemorrhage Associated With Cerebral Small Vessel Disease

Cerebral small vessel disease (SVD) is the major cause of intracerebral hemorrhage (ICH). There is no comprehensive, easily applicable classification of ICH subtypes according to the presumed underlying SVD using MRI. We developed an MRI-based classification for SVD-related ICH. We performed a retrospective study in the prospectively collected Swiss Stroke Registry (SSR, 2013-2019) and the Stroke InvestiGation in North And central London (SIGNAL) cohort. Patients with nontraumatic, SVD-related ICH and available MRI within 3 months were classified as Cerebral Amyloid angiopathy (CAA), Deep perforator arteriopathy (DPA), Mixed CAA-DPA, or Undetermined SVD using hemorrhagic and nonhemorrhagic MRI markers (CADMUS classification). The primary outcome was inter-rater reliability using Gwet's AC1. Secondary outcomes were recurrent ICH/ischemic stroke at 3 months according to the CADMUS phenotype. We performed Firth penalized logistic regressions and competing risk analyses. The SSR cohort included 1,180 patients (median age [interquartile range] 73 [62-80] years, baseline NIH Stroke Scale 6 [2-12], 45.6% lobar hematoma, systolic blood pressure on admission 166 [145-185] mm Hg). The CADMUS phenotypes were as follows: mixed CAA-DPA (n = 751 patients, 63.6%), undetermined SVD (n = 203, 17.2%), CAA (n = 154, 13.1%), and DPA (n = 72, 6.3%), with a similar distribution in the SIGNAL cohort (n = 313). Inter-rater reliability was good (Gwet's AC1 for SSR/SIGNAL 0.69/0.74). During follow-up, 56 patients had 57 events (28 ICH, 29 ischemic strokes). Three-month event rates were comparable between the CADMUS phenotypes. CADMUS, a novel MRI-based classification for SVD-associated ICH, is feasible and reproducible and may improve the classification of ICH subtypes in clinical practice and research.

Hypertensive supratentorial intracerebral hemorrhage: clinical picture, diagnosis, treatment

Background. The paper analyzed the severity and prevalence of hypertensive supratentorial intracerebral hemorrhages (ICH). This pathology is an urgent problem in the global health context, as it leads to severe consequences, including disability and death of patients. Recent discoveries and clinical guidelines provide physicians and researchers with new opportunities to improve clinical practice and improve the effectiveness of treatment for these complications. An increase in the number of cases of hypertensive supratentorial intracerebral hemorrhages and their impact on public health makes this problem extremely relevant in the field of medicine. Materials and methods. Two hundred and thirty-two patients with supratentorial intracerebral hemorrhages caused by hypertension were examined and treated in 2019–2020 at the Kyiv City Clinical Emergency Hospital. The average age of patients was 60 years: for men, it was 56 years, for women 69 years. They were observed during treatment in the hospital for up to 21 days. On the 21st day, the degree of disability was assessed. All patients underwent a neurological examination with an assessment of consciousness according to the Glasgow Coma Scale, Intracerebral Hemorrhage Score, Barthel index, modified Rankin scale, National Institutes of Health Stroke Scale (NIHSS). We have used neuroimaging methods (computed tomography, magnetic resonance angiography, if necessary). Clinical and laboratory studies were conducted. Results. It was found that 127 (57 %) patients had putaminal supratentorial hemorrhage, 24 (10 %) — thalamic, 34 (15 %) — subcortical (in the white matter of the cerebral hemispheres), 47 (20 %) — mixed. Seventy-seven (33 %) patients were hospitalized in a coma (Glasgow Coma Scale ≤ 8 points), 36 (16 %) in a sopor, 81 (35 %) in a stunned state, and 31 (13 %) patients were conscious. Patients over 60 years of age had ICH of deep localization, while young patients had cortical ones. Ninety-four surgical interventions were performed in 84 patients with lobar and lateral hematomas, as well as with breakthrough into the ventricular system (liquid drainage surgery). General mortality was 39.5 %, surgical — 46.4 %. Contraindications for surgical interventions were as follows: an existing severe coma, unstable hemodynamics, the need for constant administration of vasopressors, as well as severe comorbid conditions. Conclusions. It was found that the clinical picture of acute supratentorial intracerebral hemorrhages against the background of hypertension is characterized by polymorphic neurological symptoms according to the localization of the hematoma, its volume, cerebral edema, and intracranial hypertension. The results of the treatment of patients with hypertensive ICH indicate the need for early hospitalization, early computed tomography scan, and a differentiated approach to surgical removal of the hematoma. The favorable outcome of supratentorial ICH is more typical for patients with initial mild and moderate neurological symptoms in case of a hemorrhage volume of less than 30 cm3. The presence of blood breakthrough into the ventricular system increases the risk of death by 35 times.

Useful treatment paradigms: Decompressive hemicraniectomy with hematoma evacuation in larger intracranial hemorrhage. Insights from a leading vietnamese hospital

Background and PurposeSpontaneous intracerebral hemorrhage (SICH) bears a poor prognosis, with a 30-day mortality rate of 35%–52%. The mass effects of hematoma and surrounding edema in larger supratentorial SICH lead to an increased intracranial pressure (ICP) and subsequent brain herniation. Multiple trials have failed to prove the superiority of surgical evacuation over conservative methods. However, surgery in a carefully selected set of patients is beneficial in reducing mortality and limiting disability. The aim of this study was to investigate the effectiveness of decompressive hemicraniectomy (DHC) with hematoma evacuation for treating patients with larger supratentorial SICH. MethodsThis was a retrospective study conducted at our hospital between December 2016 and April 2020. A total of 55 patients with large supratentorial SICH treated with DHC and hematoma evacuation during this period. Patient characteristics and clinical information were analyzed, including age, sex, Glasgow Coma Scale (GCS), stroke severity (e.g. National Institutes of Health Stroke Scale), computed tomography (CT) scan data (signs of herniation, side and size of hematoma, midline shift), the time interval between ictus and surgery, surgical characteristics, and time of ventilation. Outcome was assessed by modified Rankin Scale (mRS) at six months after surgery. ResultsFifty-five patients (82% males) were treated with DHC. The mean age was 55 years. The median NIHSS (National Institutes of Health Stroke Scale) score at admission was 26 (range 9–32). Approximately two-thirds of the patients (69.1%) had hematoma located within the basal ganglia, while 30.9% had lobar hematomas. The median midline shift was 10.9 mm (range 4–20 mm). Intraventricular extensions were observed in 23 patients on the pre-operative CT scan. At discharge, the median mRS score was 5 (range 4–6), and 5 patients (9.1%) died. Six months after surgery, 17 patients (31%) had favorable outcomes (mRS scores 0–3), while the remaining patients had poor outcomes (mRS scores 4–6). The mortality rate was 25.5% (n = 14 patients). In comparison to the poor outcome group, those with favorable outcomes had a significantly higher GCS score (mean, 9.5 [SD = 2.5] vs 8.0 [SD = 1.9]; p = 0.013). Conversely, those who had poor outcomes were more likely to be older (mean 57.2 [SD = 10.4] vs 49.7 [SD = 10.7]; p = 0.017) and had a larger hematoma volume (mean 95.8 [SD = 32] vs 71.6 [SD = 33.0]; p = 0.018). ConclusionsDecompressive hemicraniectomy with hematoma evacuation could be beneficial for a selected group of young patients with large SICH.

Effects of blood pressure and tranexamic acid in spontaneous intracerebral haemorrhage: a secondary analysis of a large randomised controlled trial

BackgroundTranexamic acid reduced haematoma expansion and early death, but did not improve functional outcome in the tranexamic acid for hyperacute spontaneous intracerebral haemorrhage-2 (TICH-2) trial. In a predefined subgroup, there...

Frequency of deep-seated cerebral microbleeds in patients with lobar hemorrhages and histopathological evidence for cerebral amyloid angiopathy.

Cerebral amyloid angiopathy (CAA) is a common disease and the most common cause of lobar hemorrhages in the elderly. Usually, deep-seated microhemorrhages preclude the diagnosis of CAA. In this study, we sought to estimate the frequency of deep-seated microbleeds on MRI in patients with lobar hemorrhages and histopathological evidence for cerebral amyloid angiopathy. In addition, we describe a cohort of patients with cortical and deep-seated microbleeds on MRI and a histopathological specimen available from lobar hematoma evacuation. Retrospective database search for histopathological specimens from lobar hematoma evacuation and review of imaging findings (CT and MRI) and patient charts was performed. Between 1 January 2012 and 31 December 2020, 88 specimens from 88 patients were available. A total of 56 specimens were excluded (no brain tissue in the specimen n = 4, other diagnosis n = 8, no MRI n = 43, and no BOLD-based sequence n = 1). Of the remaining 32 patients, 25 patients (78%) did not harbor deep-seated lesions on MRI, of which 17 patients had histopathological features of CAA. A total of seven patients harbored deep-seated CMB. Of these seven patients, three (3/20, 15%) had histopathological features of CAA. Approximately 15% of patients with histopathologically diagnosed CAA harbor deep-seated microbleeds. This finding may add to the discussion on how to identify patients with CAA and deep-seated CMB.

Potential of Satellite Sign for Prediction of Hematoma Expansion in Small Spontaneous Hematoma within 7 Days' Follow-Up.

Background Hematoma expansion (HE) is the most important modifiable predictor that can change the clinical outcome of intracerebral hemorrhage (ICH) patients. The study aimed to investigate the potential of satellite sign for prediction of HE in spontaneous ICH patients who had follow-up non-contrast computed tomography (NCCT) within 7 days after the initial CT scan. Methods We retrospectively reviewed data and NCCT from 142 ICH patients who were treated at our hospital at Bangkok, Thailand. All included patients were treated conservatively, had baseline NCCT within 12 hours after symptom onset, and had follow-up NCCT within 168 hours after baseline NCCT. HE was initially estimated by two radiologists, and then by image analysis software. Association between satellite sign and HE was evaluated. Results HE occurred in 45 patients (31.7%). Patients with HE had significantly higher activated partial thromboplastin time ( p = 0.001) and baseline hematoma volume ( p = 0.001). The prevalence of satellite sign was 43.7%, and it was significantly independently associated with HE ( p = 0.021). The sensitivity, specificity, and accuracy of satellite sign for predicting HE was 57.8, 62.9, and 61.3%, respectively. From image analysis software, the cutoff of greater than 9% relative growth in hematoma volume on follow-up NCCT had the highest association with satellite sign ( p = 0.024), with a sensitivity of 55%, specificity of 64.6%, and accuracy of 60.5%. Conclusion Satellite sign, a new NCCT predictor, was found to be significantly associated with HE in Thai population. With different context of Thai population, HE was found in smaller baseline hematoma volume. Satellite sign was found more common in lobar hematoma. Further studies to validate satellite sign for predicting HE and to identify an optimal cutoff in Thai population that is correlated with clinical outcomes are warranted.

Can novel CT-and MR-based neuroimaging biomarkers further improve the etiological diagnosis of lobar intra-cerebral hemorrhage?

Lobar hematomas represent around half of all supratentorial hemorrhages and have high mortality and morbidity. Their management depends on the underlying cause. Apart from local causes such as vascular malformation, which are rare and can usually be easily excluded thanks to imaging, the vast majority of lobar hematomas equally frequently result from either hypertensive arteriolopathy (HA) or cerebral amyloid angiopathy (CAA). Distinguishing between CAA and HA is important for prognostication (risk of recurrence nearly sevenfold higher in the former), for decision-making regarding, e.g., antithrombotic therapies (for other indications) and for clinical trials of new therapies. Currently, a non-invasive diagnosis of probable CAA can be made using the MR-based modified Boston criteria, which have excellent specificity but moderate sensitivity against histopathological reference, leading to the clinically largely irrelevant diagnosis of "possible CAA". Furthermore, the Boston criteria cannot be applied when both lobar and deep MRI hemorrhagic markers are present, a not uncommon situation. Here we propose to test whether new CT and MR-based imaging biomarkers, namely finger-like projections of the hematoma and adjacent subarachnoid hemorrhage on acute-stage CT or MRI, and remote punctate diffusion-weighted imaging ischemic lesions on acute or subacute-stage MRI, have the potential to improve the performance of the Boston criteria. Furthermore, we also propose to test whether clinical-radiological biomarkers may also allow a positive diagnosis of HA to be made in lobar hematomas, which, if feasible, would not only further reduce the prevalence of "possible CAA" but also permit a diagnosis of HA and/or CAA to be made in the presence of mixed deep and lobar MRI hemorrhagic markers.

The course of intracerebral hypertensive hemorrhages depending on age and gender

Objective ‒ to analyze the clinical course of patients with intracerebral hypertensive hemorrhages depending on age and gender.Materials and methods. An analysis of the treatment of 238 patients with hypertensive intracerebral hemorrhages, who were treated at the Kyiv City Clinical Hospital of Emergency Medical Care from 2018‒2019, was conducted. The majority of patients were men (163).Results. The average age of patients was 60 years. Patients are divided into age groups according to the WHO classification (2016). Young age (18‒44 years) ‒ 25 (23 men and 2 women), average age (45‒59 years) ‒ 91 (74 men and 17 women), old age (60‒74 years) ‒ 90 (52 men and 38 women), old age (75‒90 years) ‒ 32 (14 men and 18 women). There are no long-lived people (>90 years). 84 patients were operated on, and 94 surgical interventions were performed. Patients with lobar and lateral hematomas, as well as with significant hemorrhages with a breakthrough in the ventricular system, who underwent fluid-draining surgical interventions, were operated on. The total mortality was 39.5 %. Surgical mortality rate was 46.4 %.Conclusions. According to the conducted analysis and the data of the Center for Medical Statistics of the Ministry of Health of Ukraine, the incidence of hemorrhagic stroke is many times more frequent in men, which is most likely due to a more aggressive lifestyle, lack of control over arterial hypertension, bad habits (alcohol consumption and smoking). The highest mortality rate was observed in elderly patients (47 %) and young patients (48 %), regardless of the patient’s condition at the time of admission.

Endovascular Management of Intracranial Pial Arteriovenous Fistula with Giant Venous Varix: Case Report and Review of Literature.

Abstract: Background: Intracranial pial arteriovenous fistulas (AVFs) are rare intracranial vascular malformation and their treatment is challenging because of high-flow pial supply of the lesion. We report a case of intracranial pial AVF with associated giant venous varices managed successfully by embolization. Case Description: A 25-year-old male presented with sudden onset headache and altered sensorium. Computed tomographic scan was suggestive of frontal lobar hematoma that had dissected into the third ventricle with associated intraventricular hemorrhage. Magnetic resonance imaging revealed presence of large vascular channels with the presence of multiple flow voids in bilateral frontal location. Digital subtraction angiography revealed pial fistula with arterial supply from hypertrophied left anterior cerebral artery. Treatment was done using combined coil and onyx embolization after careful analysis of angioarchitecture of the lesion using three-dimensional rotational angiogram cross-sectional reconstructions. Postprocedure angiogram revealed closure of fistula and giant varices. Conclusion: Pial AVFs with giant venous varix in adults are rare vascular malformation and understanding the angioarchitecture with exact identification of fistulous point is important to plan for surgical/endovascular management strategies. Combined strategy of embolization using coils and onyx is a safe option in such cases with giant varices.

Intracerebral haemorrhage in bacterial meningitis

ObjectiveTo determine the incidence, clinical course, radiological patterns, and clinical outcome of intracerebral haemorrhage (ICH) complicating community-acquired bacterial meningitis. MethodsThe clinical characteristics and outcome of patients with ICH complicating bacterial meningitis were studied in a prospectively nationwide cohort in the Netherlands performed from 2006 to 2018. ResultsICH was identified in 44 of 2306 episodes of bacterial meningitis (1.9%). Nine of these patients (20%) were diagnosed with ICH on admission and 35 (80%) during clinical course after a median of 5 days (1–9). ICH occurred in 4 patients with endocarditis (9%), 9 patients on anticoagulation (vitamin K antagonists and heparin; 20%), and 10 patients with cerebral infarctions (23%). In 31 patients (70%) ICH was a lobar haematoma. ICH in bacterial meningitis was associated with high rates of death (24 of 44 [55%] vs. 346 of 2200 [16%]; P < 0.001) and unfavourable outcome compared to non-ICH patients (39 of 44 [89%] vs. 798 of 2200 [36%]; P < 0.001). Neurological sequelae on discharge occurred frequently in ICH survivors compared to non-ICH patients (15 of 20 [75%] vs. 203 of 1669 [12%]; P < 0.001). ConclusionsICH is a rare but severe complication in patients with bacterial meningitis occurring in those with endocarditis, cerebral infarction, and anticoagulant use. ICH complicating bacterial meningitis is associated with high rates of death and morbidity.

Improving the Percutaneous Approach for the Management of Trigeminal Neuralgia

Trigeminal neuralgia, a debilitating pain disorder affecting the trigeminal nerve distribution, is often treated through percutaneous procedures using the route of Härtel. Miscannulation and excessive attempts at traversing the foramen ovale can negatively affect surgical outcomes. Complications attributed to miscannulation include blindness, brainstem hematoma, temporal lobe hematoma, and carotid artery hemorrhage. Currently, there is disagreement regarding the best skin entry point for the percutaneous approach. However, identifying an optimum entry point for cannulation can decrease the number of attempts necessary for a successful cannulation and also decrease the amount of fluoroscopic radiation exposure for both the patient and the surgeon. Therefore, this cadaveric study assessed a total of 56 cadavers to identify the optimum skin entry point. Calvariae, brains, and trigeminal nerves were removed, and the foramina ovalae were exposed. A lumbar needle was inserted through each porous trigeminus and foramen ovale at varied angles. The cannulation procedure was “reversed” by inserting the needle from the interior of the cranium through the porous trigeminus, foramen ovale, and skin. The exit location of the needle relative to facial structures was assessed. The exit points from the facial skin, which would otherwise represent the optimal entry points for the percutaneous procedure, were recorded. The results of this study represent an improvement in the percutaneous approach for the management of trigeminal neuralgia.

Intracranial primary synovial sarcoma mimicking a spontaneous cerebral hematoma–a case report and review of the literature

BackgroundSynovial sarcoma is a soft tissue sarcoma, of uncertain histological origin, usually located near large joints and concerning mainly young adults. Intracranial presentation in the form of metastasis from a primitive body sarcoma has been rarely reported. However, intracranial primitive synovial sarcoma (IPSS) is extremely rare and only a few cases have been reported in the literature. Case descriptionWe present the case of a 48-year-old man, with no particular medical history, that was referred to our hospital for severe headache with a normal neurological exam and a CT cerebral scan showing a left frontal lobe hematoma. The initial cerebral CT scan didn’t show any vascular malformation and the body CT scan was negative for a primitive lesion. A close follow-up with a cerebral MRI three months later, demonstrated a T1 enhanced lesion with an important volume progression. The patient underwent a complete surgical removal of this lesion and the first pathology diagnosis was compatible with a meningioma. After further proofreading by an expert and molecular analysis, the diagnosis of monophasic synovial sarcoma was confirmed. Nine months after the first surgery, the follow-up MRI showed the progressive recurrence of the lesion and in this context the patient underwent a second surgery with total resection of the tumor and frontal thin margin excision. Afterwards, the patient was treated with adjuvant radiotherapy, with a good clinical evolution, and till now the follow-up shows no recurrence. ConclusionIPSS is an extremely rare sarcoma, with challenging diagnosis and difficult management. Specific molecular analysis is necessary. Complete resection followed by radiotherapy seem to be the most appropriate therapeutic approach. However, the prognosis is still poor. Our case is even rarer because of the initial presentation as a cerebral hematoma.

Factor VII Deficiency Presenting with Recurrent Intracranial Bleeding and rFVIIa Prophylaxis.

Factor VII deficiency is an autosomal recessively inherited bleeding disease with a frequency of 1/500000. Intracranial bleeding may be seen in the newborn and infancy period. In this report, a patient having reccurrent intracranial bleedings with the diagnosis of Factor VII deficiency that is treated and given the prophylaxis of rFVIIa is discussed. The patient was a 44 months old boy who was given fresh frozen plasma at the 2 and 4 months of age. He had subdural hematoma, intracranial bleeding and hydrocephalus when he was 5 months of age and ventriculoperitoneal shunt operation was done. Replacement therapy with rFVIIa was given. When he was 11 months of age he had 2 attacks of intracranial bleeding and right cerebellar hematoma was diagnosed at that time. He was treated with 14 days of rFVIIa replacement therapy, without the need of any surgical intervention. In the 15th month he had a 3rd attack of intracranial bleeding and after that bleeding he had left occipital lobe hematoma. The valve of his shunt was replaced. He was treated with 14 days of rFVIIa replacement therapy. During 15–26 months of age the patient was given fresh frozen plasma twice a week for 6 months and then continued with 10 ml/kg of fresh frozen plasma once a week. He had 4 minor mucosal bleeding attack. At the 26th month he had the 4th intracranial bleeding attack and subarachnoid hemorrhage that is treated with 14 days of rFVIIa replacement therapy. No surgical intervention was done.After the 4th attack, once a week rFVIIa prophylaxis was started. He had 4 minor mucosal bleeding during the prophylaxis. At the 34th month due to his social security problems the patient could not get the drug and he had the 5th bleeding and had subarachnoid bleeding in the 35th month. Both replacement therapy and prophylaxis were restarted. The patient is being followed for 7 months with rFVIIa prophylaxis and he had no new bleeding episode.

Current treatment options and prognostic factors for ruptured distal anterior cerebral artery aneurysms.

Background: Distal anterior cerebral artery (ACA) aneurysms are rare, representing 1–9% of all intracranial aneurysms. The best treatment strategy for these aneurysms continues to be debated. We clarified the clinical features and treatment outcomes of patients with ruptured distal ACA aneurysms according to the treatment options at our institute.Methods: Thirty-seven consecutive patients (26 women; mean age, 65.2 years) with ruptured distal ACA aneurysms who underwent surgical clipping or coil embolization between 2012 and 2018 were included in the study. Clinical presentations, radiographic findings, and outcomes were retrospectively reviewed and compared between patients who underwent either surgical clipping or coil embolization. Risk factors associated with poor outcomes (modified Rankin Scale 4–6) were analyzed using multiple regression analysis.Results: Nineteen patients (51.4%) had World Federation Neurological Surgeons (WFNS) Grade IV-V, 18 (48.7%) had frontal lobe hematomas, and 13 (35.1%) had multiple aneurysms. Surgical clipping and endovascular coiling were performed in 28 (75.7%) and nine (24.3%) patients, respectively. Aneurysms located at the A4-5 portions were mainly treated by surgical clipping (P = 0.04). There were no significant between-group differences in procedure-related morbidity and mortality; however, the complete occlusion rate was higher in the surgical group (P < 0.01). Overall, a favorable neurological outcome at discharge (mRS 0–3) was obtained in 23 patients (62.5%). Multiple regression analysis revealed that WFNS Grade IV-V and frontal lobe hematomas were risk factors for poor outcomes (mRS 4–6).Conclusion: Acceptable outcomes were obtained in 62.5% of cases, and there were no significant between-group differences in treatment results between clipping and coiling. A poor WFNS grade and intracerebral hematomas were risk factors for a poor prognosis.

A Rare Case of Severe Hemophilia B Combined with Hematomesenchymal Dysplasia

BACKGROUND: The prevalence of hemophilia B in the global population is approximately 1:60,000. Undifferentiated systemic mesenchymal dysplasia (uSMD) is diagnosed rather frequently, about 1:5, according to Russian authors; no data on uSMD prevalence in other countries are available. The combination of hemophilia and uSMD has grouped under the term hematomesenchymal dysplasia (HMD). This combination significantly worsens the clinical picture, prognosis, and quality of life of the patient. AIM: In this article, we present a rare clinical case of a child with a combination of hemophilia B and HMD. CASE REPORT: A clinical case of a male patient, 5 years old with hemophilia B, severe form combined with HMD, complicated by hemothorax, abscessed pulmonary lobe hematoma has presented. The presence of HMD and the above complications had an unfavorable effect on the severity of the clinical picture and the abnormal response to treatment. The main diagnostic procedures were the assessment of the phenotypic signs of HMD in combination with laboratory and instrumental examination methods such as ultrasound, computed tomography (CT) scan, and echocardiography. Vital treatment is factor IX replacement therapy and supportive one. CONCLUSION: This clinical example highlights the importance of clinical alertness to hereditary coagulopathies, which often lead to life threatening, sometimes disabling complications that significantly reduce the quality of life of children with hemophilia. Consequently, full-scale epidemiological studies of the prevalence of HMD in the population are an urgent task for the near future.

Apolipoprotein E genotype predicts subarachnoid extension in spontaneous intracerebral haemorrhage

Spontaneous intracerebral haemorrhage (ICH) with subarachnoid extension (SAHE) predicts poor outcomes and haematoma expansion in spontaneous ICH and is also a potential predictor of the severity of vascular amyloid deposition. The biological underpinnings of SAHE remain elusive. A study was conducted to identify risk factors associated with SAHE. A retrospective analysis was performed of an ongoing prospective cohort of primary spontaneous supratentorial ICH patients admitted to Tongji Hospital. SAHE was rated on baseline noncontrast computed tomography images by investigators blinded to the clinical data. A total of 189 patients were enrolled. Apolipoprotein E (APOE) ε2 copies (p=0.020), but not APOE ε4 copies (p>0.2), were more common in patients with SAHE in univariate analysis. After controlling for confounding factors in multiple logistic regression, lobar haematoma (odds ratio [OR] 14.21, 95% confidence interval [CI] 5.89-34.33; p<0.001), large haematoma volume (OR 1.04, 95% CI 1.02-1.06; p<0.001) and APOE ε2 copies (OR 3.07, 95% CI 1.05-8.97; p=0.041) were three independent predictors of SAHE. For subgroup analysis stratified by location, APOE ε2 showed a possible association with SAHE in lobar ICH (p=0.026) but not in deep ICH (p>0.2). No significant association was found between APOE ε4 copies and either lobar (p>0.2) or deep ICH (p>0.2). The APOE ε2 allele predicts SAHE in spontaneous supratentorial ICH. The association may predominantly apply to lobar ICH. Given the established relationship between the APOE ε2 allele and pathological cerebrovascular changes, our findings suggest that SAHE involves genetically driven vessel pathology.

Bypassing the Ordinary: A Patient's Unique Journey to Reach His Destination

<h3>Introduction</h3> Advanced heart failure therapies include an armamentarium of mechanical circulatory support (MCS). We present a case for which an array of mechanical support systems were utilized to save a patient. <h3>Case Report</h3> A 71-year old male with HTN, HLD, and smoking history presented with expressive aphasia due to an embolic CVA. Initial workup revealed an ejection fraction <20% with severe global hypokinesis on TEE, and an occluded distal left main and multi-vessel disease by left heart catheterization. Patient underwent a noncomplicated quadruple bypass surgery. Post-operative day #3, he suffered a ventricular fibrillation cardiac arrest and was promptly taken to the OR for emergency IABP insertion followed by central cannulation for VA-ECMO. Post-operative day #4, his pressor requirements increased and a TEE revealed an EF of 5% despite IABP and VA-ECMO support. He was taken back again to the OR for axillary Impella 5.0 implantation and decannulation of VA-ECMO. Over the next couple weeks, he was weaned from pressors, but developed fevers of unknown origin. A CT head revealed a new frontal lobe hematoma in addition to a subacute CVA. He remained neurologically intact, though unable to wean from ventilation. A tracheostomy and PEG tube were inserted. The patient was highly functioning prior to his presentation and wanted everything done including LVAD implantation. A midline sternotomy approach was not suitable due to recent CABG and emergent central cannulation of ECMO; therefore, he underwent Heartmate 3 LVAD implantation via left thoracotomy approach with descending aorta outflow graft cannulation (see Figure 1) as destination therapy. He was discharged to acute rehab post-operative day# 55 and is now home, with tracheostomy and PEG discontinued, and able to walk with limited assistance. <h3>Summary</h3> In cases necessitating various MCS modalities, a left thoracotomy approach with descending aorta outflow graft cannulation for LVAD may be a viable alternative to midline sternotomy.