ALK-rearranged histiocytosis: Report of two cases with involvement of the central nervous system.

AIMS: Histiocytoses are a heterogeneous group of localized or disseminated diseases. Clinical presentation and patients' outcome vary greatly, ranging from mild to life-threatening disorders. Rare cases of systemic or localized histiocytosis harboring ALK rearrangement have been reported. METHODS: Two cases of CNS histiocytosis were thoroughly investigated by implementing multiple molecular tests, i.e. FISH, RT-qPCR, NGS analysis. RESULTS: In a 10-month old girl (patient #1), MRI showed two left hemispheric lesions and a right fronto-mesial lesion histologically consisting of a moderately cellular infiltrative proliferation, composed by CD68(PGM1)+/CD163+ spindle cells. ALK 5'/3'-imbalance and a KIF5B(exon 24)-ALK(exon 20) fusion were documented by RT-qPCR and NGS analysis, respectively. A subsequent CT scan showed multiple hepatic and pulmonary lesions. The patient was started on chemotherapy (vinblastine) associated to an ALK-inhibitor (Alectinib) with remarkable response. In a 11-year-old girl (patient #2), MRI showed a right frontal 1.5 cm lesion. Neuropathological examination revealed a histiocytic proliferation composed by medium sized CD68(PGM1)+/HLA-DR+ cells, showing moderate ALK1 positivity. ALK rearrangement and a KIF5B(exon 24)-ALK(exon 20) fusion were demonstrated also in this case. Subsequent CT, 18F-FDG-PET and MRI scans showed the presence of a single right femoral lesion, proved to be a fibrous cortical defect. CONCLUSIONS: In ALK-histiocytoses, CNS involvement may occur as part of a systemic disease or, rarely, as its only primary disease localization, which could remain otherwise asymptomatic. The diagnosis often relies on neuropathological examination of brain biopsy, which may pose a diagnostic challenge due to the variable histopathological features. An integrated histological and molecular approach in such cases is recommended.

Comparison Between Three Commonly Used Large-Bore Aspiration Catheters in Terms of Successful Recanalization and First-Passage Effect.

OBJECTIVES: To compare three commonly used large-bore aspiration catheters in terms of final successful recanalization rate and first-passage successful and complete recanalization rates (the so called "first-passage effect"). MATERIALS AND METHODS: it is an observational retrospective multicenter study conducted in three Italian high-volume tertiary stroke centers between January 2017 and May 2019. The study population included all consecutive patients with an ischemic stroke due to middle cerebral artery occlusion (M1 segment only) that underwent intra-arterial mechanical thrombectomy with contact aspiration as first-line strategy within 24 hours from symptoms onset. RESULTS: Three hundred twenty-one patients were included in the study. Multivariable logistic regression analysis comparing the three catheters revealed no differences in terms of successful recanalization. Sofia 6 Plus catheter was associated with better first-passage successful recanalization [OR, 9.09; 95% CI, 2.66-31.03] (p<0.001) and first-passage complete recanalization [OR: 3.73; 95% CI: 1.43-9.72] (p=0.007) whereas rt-PA was associated with worse first-passage recanalization [OR: 0.52; 95% CI: 0.29-0.93] (p=0.028). CONCLUSIONS: No differences between the three catheters were reported in terms of successful recanalization. Sofia 6 Plus has proven to be superior in achieving both successful and complete first-passage recanalization. Conversely, rt-PA was found to be a negative predicting factor of first-passage effect.

An evaluation of the objectivity and reproducibility of shear wave elastography in estimating the post-mortem interval: a tissue biomechanical perspective.

Cadaveric rigidity-also referred to as rigor mortis-is a valuable source of information for estimating the time of death, which is a fundamental and challenging task in forensic sciences. Despite its relevance, assessing the level of cadaveric rigidity still relies on qualitative and often subjective observations, and the development of a more quantitative approach is highly demanded. In this context, ultrasound shear wave elastography (US SWE) appears to be a particularly well-suited technique for grading cadaveric rigidity, as it allows non-invasive quantification of muscle stiffness in terms of Young's modulus (E), which is a widely used parameter in tissue biomechanics. In this pilot study, we measured, for the first time in the literature, changes in the mechanical response of muscular tissues from 0 to 60 h post-mortem (hpm) using SWE, with the aim of investigating its applicability to forensic practice. For this purpose, 26 corpses were included in the study, and the muscle mechanical response was measured at random times in the 0-60 hpm range. Despite the preliminary nature of this study, our data indicate a promising role of SWE in the quantitative determination of cadaveric rigidity, which is still currently based on qualitative and semiquantitative methods. A more in-depth study is required to confirm SWE applicability in this field in order to overcome some of the inherent limitations of the present work, such as the rather low number of cases and the non-systematic approach of the measurements.

Preoperative Direct Puncture Embolization of Castleman Disease of the Parotid Gland: A Case Report.

BACKGROUND: Castleman disease (CD) is an uncommon benign lymphoproliferative disease characterized by hypervascular lymphoid hyperplasia. We present a unique case of unicentric CD of the parotid gland treated by preoperative direct puncture embolization. CASE PRESENTATION: A 27-year-old female patient was admitted for a right neck mass. Ultrasound examination and MRI scan documented a hypervascular mass within the right parotid gland. Preoperative embolization was performed by direct puncture technique: a needle was inserted into the core of the mass under both ultrasound and fluoroscopic guidance and SQUID 12 was injected into the mass under fluoroscopic control, achieving a total devascularization. CONCLUSION: Preoperative direct puncture embolization was safe and effective and provides excellent hemostatic control during the surgical operation, limiting the amount of intraoperative bleeding.

Flow Diverter Devices in the Treatment of Anterior Communicating Artery Region Aneurysms: Would the Regional Anatomy and the Aneurysm Location Affect the Outcomes?

BACKGROUND: In this study, the authors evaluated the efficacy and the safety of flow diverter devices (FDD) in anterior communicating artery (ACoA) region aneurysms, focusing on anatomical factors that could affect the outcome, such as the location of the aneurysm along the ACoA (centered on ACoA or decentered on the A1-A2 junction) and the anatomy of the ACoA region. METHODS: Clinical, procedural and follow-up data were analyzed. Aneurysms were classified according to the location along the ACoA (centered or decentered on the A1-A2 junction) and on the basis of the anatomical configuration of the ACoA region. Safety was assessed by recording intraprocedural, periprocedural and delayed complications to determine the morbidity and mortality rates. The functional outcome was evaluated with the modified Rankin scale (mRS) prior to and after the endovascular procedure. To assess the efficacy, midterm and long-term clinical, angiographic and cross-sectional imaging follow-ups were recorded. Subgroup analysis according to the different ACoA regional anatomical configurations and the ACoA aneurysm locations were performed. RESULTS: 33 patients (17 males; 16 females) with ACoA region aneurysms were treated with FDDs. 27 aneurysms were located at the A1-A2 junction (82%) while the remaining six aneurysms were centered on the ACoA. No mortality was recorded. The overall morbidity rate was 6% (2/33 procedures). Major complications occurred in 33% (2/6) of ACoA aneurysms and in the 0% of A1-A2 junction aneurysms. Mid-term and long-term neuroimaging follow-ups showed the occlusion of the aneurysm in 28/33 cases (85%). Complete occlusion rates were 93% in the A1-A2 junction aneurysms and 50% in ACoA aneurysms. CONCLUSIONS: The FDD is a safe and effective tool that can be used in the treatment of selected cases of ACoA region aneurysms. The location of the aneurysm along the ACoA and the regional anatomy of the ACoA complex could affect the efficacy and safety.

Radiological Reporting of Urgencies Related to Medical Devices: Commentary on a Possible Systematic Approach.

Most medical devices are routinely recognized on radiological images and described as normal findings in the radiological report, but sometimes they can cause patient access to the emergency department. Multiple possible complications have been described and most of them require prompt recognition by radiologists for proper clinical management. This commentary proposes a systematic approach to radiological reporting of the most common emergent complications related to medical devices with the intent to avoid the omission of important findings in the final radiological report.

Posterior Circulation Endovascular Thrombectomy for Large Vessels Occlusion in Patients Presenting with NIHSS Score ≤ 10.

Mechanical thrombectomy (MT) is currently the gold standard treatment for ischemic stroke due to large vessel occlusion (LVO). However, the evidence of clinical usefulness of MT in posterior circulation LVO (pc-LVO) is still doubtful compared to the anterior circulation, especially in patients with mild neurological symptoms. The database of 10 high-volume stroke centers in Europe, including a period of three year and a half, was screened for patients with an acute basilar artery occlusion or a single dominant vertebral artery occlusion ("functional" BAO) presenting with a NIHSS ≤10, and with at least 3 months follow-up. A total of 63 patients were included. Multivariate analysis demonstrated that female gender (adjusted OR 0.04; 95% CI 0-0.84; p = 0.04) and combined technique (adj OR 0.001; 95% CI 0-0.81; p = 0.04) were predictors of worse outcome. Higher pc-ASPECTS (adj OR 4.75; 95% CI 1.33-16.94; p = 0.02) and higher Delta NIHSS (adj OR 2.06; 95% CI 1.16-3.65; p = 0.01) were predictors of better outcome. Delta NIHSS was the main predictor of good outcome at 90 days in patients with posterior circulation LVO presenting with NIHSS score ≤ 10.

Endovascular treatment of cerebral dural arteriovenous fistulas with SQUID 12.

BACKGROUND: Endovascular therapy with liquid embolic agents (LEAs) is the gold standard for the treatment of cerebral dural arteriovenous fistulas (cDAVFs). The aim of the study is to retrospectively evaluate effectiveness, safety, and midterm follow-up results of endovascular treatment of cDAVFs using SQUID 12. METHODS: Between June 2017 and January 2020 the authors retrospectively reviewed clinical, demographic and embolization data of 19 consecutive patients with cDAVF who underwent embolization using SQUID 12. The number of arteries catheterized for each procedure, the total amount of embolic agent, the occlusion rate, the injection time, any technical and/or clinical complications were recorded. Mid-term follow-up with DSA was reviewed. RESULTS: 20 procedures were performed in 19 patients. A transarterial approach was accomplished in 19 procedure; a combined transvenous-transarterial approach was realized in 1 treatment. The average time of injection was 33 minutes (2-82 minutes), and the average amount of SQUID 12 was 2.8 mL (0.5-6 mL). Complete angiographic cure at the end of the procedure was achieved in 17 patients. No major periprocedural adverse events were recorded. Mid-term follow-up was achieved in 15 out of 19 patients and confirmed complete occlusion of the cDAVFs in 13/15 patients (87%); in 2 of the initially cured patients a small relapse was detected. CONCLUSIONS: The treatment of the cDAVFs using SQUID 12 was effective and safe. The lower viscosity seems to allow an easier penetration of the agent with a high rate of complete occlusion of the cDAVFs.

A Rare Case of Postinfectious Moyamoya Syndrome: Case Report and Review of the Literature.

BACKGROUND: Postinfectious Moyamoya syndrome (MMS) is a rare vasculopathy that can follow meningitis. Only 9 cases of MMS after meningitis have been reported in the literature. We present a unique case of MMS after meningitis caused by Aspergillus fumigatus and Escherichia coli and review all cases of MMS postmeningitis in the literature. CASE DESCRIPTION: A 41-year-old man was admitted to our emergency department for sudden hypoesthesia in the left arm and an intense headache not responsive to drugs. Computed tomography scan and magnetic resonance imaging showed acute ischemic lesions in the right centrum semiovale associated with bilateral chronic watershed cerebral ischemic lesions. The cerebral digital subtraction angiography documented a typical Moyamoya pattern. In anamnesis, the patient suffered from meningitis caused by A. fumigatus and E. coli infection after neurosurgery for subependymoma of the fourth ventricle 2 years before. Laboratory tests, clinical investigation, and imaging ruled out any other cause of vasculopathy and led to the final diagnosis of postinfectious MMS. The patient started medical therapy with oral acetyl salicylic acid, verapamil, and prednisone while surgical approach was excluded in the first instance. CONCLUSIONS: Physicians should be aware of an uncommon but severe complication of meningitis such as MMS, even several years after the infection. Neuroimaging is essential for the diagnosis and to exclude other causes of neurologic symptoms in these patients.

Radiological features and management of retained needles.

The identification of retained needles is essential because of their sharp structure with possible life-threatening complications. However, radiological evaluation could be challenging, especially in case of needles' relatively poor conspicuity and small dimension. This pictorial essay focuses on clinical issues (needle features, retention mechanisms and associated complications) and technical aspects (choice of the best diagnostic modality and technique) that can lead the radiologist to an earlier and proper diagnosis of needle retention in order to provide the best treatment for the patient.

Acute hemobilia from a pseudoaneurysm of the cystic artery arising from the left hepatic artery: Case report and literature review.

INTRODUCTION: Hemobilia represents only 6% of all causes of upper gastrointestinal bleeding. PRESENTATION OF CASE: We report a rare case of a bleeding pseudoaneurysm of the cystic artery, due to a re-activation of a chronic cholecystitis, which arose with a mixed symptomatology: jaundices and hematemesis. DISCUSSION: The rarity of our patient is increased for some vascular anatomic variations detected by Computed Tomography that influenced the management of the disease. Our patient was treated by endovascular embolization of the pseudoaneurysm and subsequent cholecystectomy. CONCLUSION: About pseudoaneurysm of the cystic artery only few cases have been already reported and to date there are no guidelines for its management. We describe both diagnostic features and therapeutic strategies in comparison to the most recent literature.