Impact of SARS-CoV-2 infection in patients with hereditary hemorrhagic telangiectasia: epidemiological and clinical data from the comprehensive Italian retrospective multicenter study.

Rare Disease patients manifested high concern regarding the possible increased risk of severe outcomes and worsening of disease-specific clinical manifestation due to the impact of COVID-19. Our aim was to assess the prevalence, outcomes, and impact of COVID-19 in patients with a rare disease such as Hereditary Hemorrhagic Telangiectasia (HHT) in Italian population. A nationwide, multicentric, cross-sectional observational study was conducted on patients with HHT from five Italian HHT centers by online survey. The association between COVID-19-related signs and symptoms and nosebleeds worsening, the impact of personal protective equipment on nosebleeds pattern, and the relationship between the presence of visceral AVMs and severe outcomes were analyzed. Out of 605 total survey responses and eligible for analysis, 107 cases of COVID-19 were reported. A mild-course COVID-19 disease, not requiring hospitalization, was observed in 90.7% of patients, while the remaining eight cases needed hospitalization, two of them requiring intensive-care access. No fatal outcome was recorded and 79.3% of patients reported a complete recovery. No difference in infection risk and outcome between HHT patients and general population was evidenced. No significative interference of COVID-19 on HHT-related bleeding was found. The majority of patients received COVID-19 vaccination, with relevant impact on symptoms and need for hospitalization in case of infection. COVID-19 in HHT patients had an infection profile similar to the general population. COVID-19 course and outcome were independent from any specific HHT-related clinical features. Moreover, COVID-19 and anti-SARS-CoV-2 measures did not seem to affect significantly HHT-related bleeding profile.

A Case Series of Non-Traumatic Pediatric Intracerebral Hemorrhage Hospitalized in Picu within a 10-Year Period

BACKGROUND AND AIM: Intracerebral hemorrhage (ICH) accounts for about half of all childhood strokes and is a notable contributor to childhood morbidity and mortality. The aim of this study is to describe the characteristics of cases of ICH that were hospitalized in a PICU during a 10-year period. METHOD(S): Patients' files between 2011-2021 were studied retrospectively. Cases with non-traumatic intracerebral hemorrhage were included in the study. RESULT(S): 18 patients were examined (38.8% male) with a median age of 7 years (3 months-14 years). 83% were of Greek origin and mean duration was 6.3 days. Half of our patients were hospitalized between 2020-2021 (see image 1.). 33.3% had relevant comorbidities such as arteriovenous malformation (AVM), hemangiomas and neurofibromatosis type. Presenting symptoms were headache (55.5%), nausea and emesis (66.6%), seizures (27.7%), altered level of consciousness (65.5%), paresis (22%). Glasgow Coma Scale at admission was 13-15 in 66.6%, 9-12 in 22.2% and < 9 in 5%. Diagnosis was made using CT (88,9%) and MRI scans (11.1%). 61% needed surgery while 72% underwent digital subtraction angiography (DSA) and 38.4% of them were embolized. Vascular malformations were the main causes: AVM (38.8%), aneurysm (5%), pseudoaneurysm (5%), hemangioma (5%) but 46,2% remained unidentified. No patient died but 33.3% showed neurological deficits after discharge CONCLUSION(S): Intracerebral Hemorrhage in children is an urgent situation that needs collaboration between intensivists and neurosurgeons. The increase in frequency during COVID-19 pandemic is remarkable and needs further studying to determine a possible causative relation.

Awake Craniotomy for Cerebral Abscess with Pulmonary Arteriovenous Malformation

A cerebral abscess can be a life-threatening complication of pulmonary arteriovenous malformations (PAVM), thus posing significant morbidity if left untreated. We report a case of an incidental finding of a PAVM in a patient diagnosed with cerebral abscess. A 22-year-old male presented to the emergency department with acute onset right-sided weakness in both upper and lower limbs for 1 week. Magnetic resonance imaging showed a ring-enhancing lesion within the left parasagittal frontoparietal region s/o intracerebral abscess. High-resolution computed tomography was done as a protocol in patients posted for surgery due to coronavirus disease 2019 and coincidentally, it showed a single well-defined parenchymal nodule, 4 x 3.4 cm in the lateral basal segment of the left lower lobe. The knowledge of the pathophysiology of PAVM and expected complications during general anesthesia (GA) and positive pressure mechanical ventilation is essential. In such conditions, awake craniotomy under conscious sedation and scalp block may be considered as an alternative to GA.

A Case of COVID-19 ARDS with Unusual Refractory Hypoxia

Aim and background: Since the beginning of COVID-19 pandemic, we have come across a large number of ARDS patients with different presentations and clinical manifestations. The usual management is using a lung-protective ventilatory strategy followed by proning to improve oxygenation. Here, we present a case where the usual management failed to improve oxygenation which led us to think of co-existing alternative diagnosis. Case description: A 59-year-old male with a history of cardiovascular disease, presented with cough and breathing difficulty for 10 days and COVID RT PCR was positive. He was started on remdesivir, steroids, anti-coagulants, and other supportive measures but worsened and had to be intubated and mechanically ventilated. Lung-protective ventilation was initiated but the patient remained hypoxic even at 100% fiO2. Chest X-ray and HRCT did not show much severity and the measured lung compliance was also good. A transesophageal ECHO showed good LV function and no significant diastolic dysfunction. 2 sessions of proning were done and yet the oxygenation did not improve. Repeat HRCT + CTPA was done to look for pulmonary embolism but it instead revealed a pulmonary AV malformation. Coiling of the AV malformation was done. Oxygenation then substantially improved. Further sessions of proning were done and patient was gradually weaned off. Conclusion: There may be several co-existing causes of ventilation-perfusion mismatch which needs to be looked for. Pulmonary AV malformation, though rare, can cause shunting and hence persistent hypoxia.

Anaesthetic management of a parturient with Klippel-Trenaunay syndrome

Introduction: Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular disease which is characterised by capillary, venous and lymphatic malformations. We describe the anaesthetic management of a 36 year old parturient with COVID-19 and KTS, who underwent an elective caesarean section. Very few such cases have been described and the multi-system nature of condition poses various challenges to both the obstetrician and the anaesthetist. Case Report: We had a 36-year-old woman who had experienced three previous normal vaginal deliveries and an elective caesarean section (CS) four years previously under general anaesthetic(GA) at 36 weeks gestation. She was told by a vascular surgeon that she was not suitable for regional anaesthesia. There was no recent imaging of her back to rule out arteriovenous(AV) malformations. Her past history included gastric bypass surgery under GA two years ago. She also had depression, varicose veins and three previous deep venous thrombosis andwas on prophylactic lowmolecularweight heparin. She had tested positive for COVID-19 4 days previously, and had mild symptoms of cough and sore throat. After a multi-disciplinary discussion involving an obstetrician, vascular surgeon, haematologist and anaesthetist, a decision was made to proceed with GA despite recent COVID-19, because of the possibility of AV malformations, in agreement with the patient. After securing two wide bore cannulae and adequate preoxygenation, a modified Rapid Sequence Induction was performed, and a tracheal tube was secured. Anaesthesia was maintained with oxygen, nitrous oxide and sevoflurane. After delivery of the baby, oxytocin 5U, followed by an infusion, midazolam, morphine, ondansetron and dexamethasone were administered. Extubation was performed when the patient was fully awake. In recovery, further opioids were given for pain. There were no concerns for the newborn. Estimated blood loss was 200 mL. Discussion: Gestation and its physiology may further exacerbate the manifestations of KTS, with increased obstetric risk. The success in the management of these patients requires the participation of a multidisciplinary team, consisting of obstetrician, anaesthetist, urologist, haematologist and vascular surgeon, with appropriate collaboration among the professionals involved. Periodic imaging and clotting tests are recommended to evaluate the evolution of vascular malformations in the pelvis, uterus and vagina, and identify neuraxial changes, to guide the safest way of delivery and anaesthesia.

Cerebral Venous Sinus Thrombosisin the Setting of an AVMWhile on Anticoagulation: A Case Report

Objective: To increase the awareness of neurological complications of arteriovenous malformation (AVM) due to obstruction of the venous drainage despite being on anticoagulants. Background: Cerebral AVMs are high-flow intracranial vascular malformation comprised of feeding arteries, a nidus of vessels with intervening brain parenchyma through which arteriovenous shunting occurs and dilated draining veins allowing significant hemodynamic gradient without an interposed resistance. Venous drainage stenosis or occlusion will increase the hemodynamic pressure gradient within the AVM compartments and potentially lead to redistribution of flow resulting in cerebral venous sinus thrombosis or hemorrhagic stroke from nidus rupture. This effect might be worsened in the presence of a generalized hypercoagulable state causing microvascular injury and thrombosis;despite adequate anticoagulation therapy. Design/Methods: N/A Results: 66-year-old obese woman with history of atrial fibrillation, coronary artery disease, diabetes, hypertension, hyperlipidemia, prior stroke, small left frontal AVM diagnosed on conventional angiogram and recent COVID-19 infection presented to our comprehensive stroke center with seizures and right hemiparesis. MRI brain showed T2/FLAIR hyperintense lesion in the left frontal/parasagittal region with an extensive vasogenic edema, heterogeneous diffusion restriction, and gyriform contrast enhancement. Conventional angiogram showed AVM without nidus opacification but with an associated mass effect correlating with parenchyma edema and early venous shunting. Patient was initially misdiagnosed as low-grade neoplasm although accurate diagnosis of left parasagittal frontal venous infarct in the setting of spontaneous venous thrombosis of left frontal AVM was made with conventional angiogram. Conclusions: Venous infarct due to CVST is a devastating complication of AVM. The hemodynamic pressure gradient within the AVM might play a larger role in contributing to hypercoagulable state within the venous system leading to cerebral venous sinus thrombosis despite patient being on therapeutic anticoagulation.

INDICATIONS AND DIAGNOSTIC YIELD OF SINGLE-BALLOON ASSISTED ENTEROSCOPY: AN EXPERIENCE IN A TERTIARY CARE CENTER OF A LOW INCOME COUNTRY

AIM: Enteroscopy is an endoscopic procedure to approach the small intestine in order to diagnose and treat various pathologies. The aim of this study is to share the experience of our center with this modality and highlight the main indications and diagnostic yield pf the procedure. METHODS: This is a single center retrospective study at Aga Khan University Hospital Karachi, Pakistan in which we included all the enteroscopies done in last five years. RESULTS: A total of 37 enteroscopies were done in past five years, the numbers has reduced significantly in the Covid-19 era. The mean age was 50.4 ± 16.5 years with 19 (51.4%) females and 18 (48.6%) males. The most common indications were jejunal thickening on CT scan 17 (45.9%) followed by obscure gastrointestinal bleeding 7 (18.9%) and chronic diarrhea 5 (13.5%). Most of the enteroscopies showed no gross pathology 17 (45.9%). However, jejunal ulcers 6 (16.2%), arteriovenous malformations 4 (10.8%), jejunal polyps 3 (8.1%), erythematous mucosa 2 (5.4%), pale mucosa 2 (5.4%), jejunal mass 2 (5.4%) and gastric polyp 1 (2.7%) are the findings noticed in rest of the enteroscopies performed. In 18 (48.6%) no biopsies were taken due to normal findings. 12 (32.4%) had nonspecific chronic inflammation in the jejunum. The remaining seven patient's histopathology showed giardiasis, MALTOMA, high grade B cell lymphoma, moderately differentiated adenocarcinoma, inflammatory polyp, hamartomatous polyp and adenomatous polyp. CONCLUSIONS: We shared the five year experience of enteroscopies done in our center which belongs to a low income country. The number of enteroscopies has greatly affected by the cost, low socioeconomic status of patients. Covid-19 has further reduced the number of diagnostic procedures in general. Most of the enteroscopies didn't reveal significant findings however there are cases where lymphoma and adenocarcinoma has been diagnosed.

Pulmonary arteriovenous malformation and inherent complications with solitary lung nodule biopsy-literature overview and case report.

Pulmonary arteriovenous malformation, also known as an arteriovenous fistula, is typically a congenital disease caused by structural deficiencies, particularly the lack of capillary wall development, leading to the abnormal dilation of the pulmonary capillaries. The majority of pulmonary arteriovenous malformation cases are associated with Rendu-Osler-Weber syndrome, also known as hereditary hemorrhagic telangiectasia. Pulmonary arteriovenous malformation rarely occurs due to chest trauma. Pulmonary arteriovenous malformations are long-lasting and often first diagnosed in adults. More than two-thirds of pulmonary arteriovenous malformation lesions are found in the lower lung lobe and the subpleural area, and the vast majority of cases present with the monofocal form. The initial diagnosis is often based on the identification of a solitary pulmonary nodule. However, a solitary nodule detected on chest computed tomography that is not correctly diagnosed as pulmonary arteriovenous malformation, even after intravenous contrast injection, can lead to the performance of a transthoracic biopsy. Biopsy of pulmonary arteriovenous malformations can lead to stroke occurrence, during which the patient often presents with severe pleural bleeding, which can have lifelong consequences if not immediately treated. We report a case of pulmonary arteriovenous malformation that was discovered incidentally in an adult patient who underwent non-contrast computed tomography. Misdiagnosis occurred, and transthoracic lung biopsy was performed. Complications were discovered late, and the patient underwent surgical pulmonary arteriovenous malformation removal and was treated for hemothorax.

Arteriovenous Malformation of Upper Lip - a short cure for gross deformity

Aim: To evaluate the safety and success of facial artery ligation alone in arteriovenous malformation of upper lip in non-affording patients. Methodology: This prospective study was conducted at Department of Plastic surgery, on 11 consecutive patients with recurrent upper lip arteriovenous malformation who couldn’t afford angioembolization and frequent hospital visits for staged procedures. We did debulking of lesion after facial artery ligation and cosmetic lip correction. Results: Total 11 patients (9 males and 2 females) were included in this study. Majority of patients showed satisfactory results with facial artery ligation alone. One patient lost the follow up. No significant complication was noted in any patient. No recurrence was noted at 6 months and 1 year follow-up. Patient satisfaction rate remained 8.6±0.96 as per VAC. Conclusion: facial artery ligation alone provides an easy and approachable option for arteriovenous malformation where cost affordability for angioemboization and repeated hospital visits are main limitations.

Intracranial Hemorrhage Due to Potential Rupture of an Arteriovenous Malformation after BNT162b2 COVID-19 mRNA Vaccination in a Young Korean Woman: Case Report.

Inoculation with the Pfizer-BioNTech coronavirus infection-19 (COVID-19) vaccine (BNT162b2) has been approved in Korea. Although it is generally safe, several possible side effects have been reported. The present report describes a 28-year-old woman who developed an intracerebral hemorrhage in her right temporal lobe after the first dose of the Pfizer-BioNTech COVID-19 vaccine. The patient complained of a persistent headache for four days after the first dose, along with right third nerve palsy and drowsiness. Non-enhanced brain computed tomography confirmed a 5.0 × 3.7 × 5.0 cm3-sized intracranial hemorrhage in the right temporal lobe due to the rupture of an arteriovenous malformation (AVM). Transfemoral cerebral angiography revealed that blood was supplied to the AVM by the right middle cerebral artery branch and drained into the right transverse sinus. The patient underwent surgical treatment for AVM nidus removal with hematoma evacuation on the day of admission. Her condition stabilized 10 days postoperatively. These findings indicate that clinicians should be aware that cerebral hemorrhage caused by AVM rupture may be a side effect of inoculation with the BNT162b2 mRNA COVID-19 vaccine.

Acute Endovascular Management of a Ruptured Choroidal Artery Arteriovenous Malformation in a Peripartum COVID-19-Positive Patient

Coronavirus disease 2019 (COVID-19) pandemic has led to significant reduction in specialty care causing delayed presentations and decreased resources for elective procedures. We present here a case of a 29-year-old female, 34 weeks primigravida who presented with a twice ruptured right choroidal artery arteriovenous malformation (AVM). She underwent an immediate external ventricular drain placement followed by an emergency cesarean. She underwent a diagnostic angiogram showing a right choroidal AVM with a feeder artery aneurysm along with small supply to its cortical component by the M4 segment of right middle cerebral artery, draining into the basal vein of Rosenthal and vein of Labbe. The patient underwent embolization of the aneurysm and the choroidal feeders. She improved symptomatically following the procedure with external cerebrospinal fluid diversion for 5 days. At follow-up magnetic resonance imaging, the residual AVM remained stable and is planned for Gamma knife radiosurgery. The newborn was taken care at a neonatal intensive care unit and was started on breastfeed on 16th day of birth once the mother had a negative COVID-19 reverse transcription polymerase chain reaction. In this study, we highlight our experience with the above-mentioned patient profile, the multidisciplinary effort during the pandemic, and the measures taken (isolation ward, COVID-19 dedicated Cath-lab and personal precautions) and advised for tackling COVID-19 patients for endovascular procedures.

Urgent Neurosurgical Interventions in the COVID-19-Positive Pediatric Population.

BACKGROUND: Urgent neurosurgical interventions for pediatric patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are rare. These cases pose additional stress on a potentially vulnerable dysregulated inflammatory response that can place the child at risk of further clinical deterioration. Our aim was to describe the perioperative course of SARS-CoV-2-positive pediatric patients who had required an urgent neurosurgical intervention. METHODS: We retrospectively analyzed pediatric patients aged ≤18 years who had been admitted to a quaternary children's hospital with a positive polymerase chain reaction test result for SARS-CoV-2 virus from March 2020 to October 2021. The clinical characteristics, anesthetic and neurosurgical operative details, surgical outcomes, and non-neurological symptoms were collected and analyzed. RESULTS: We identified 8 SARS-CoV-2-positive patients with a mean age of 8.83 years (median, 8.5 years; range, 0.58-18 years). Of the 8 patients, 6 were male. All children had had mild or asymptomatic coronavirus disease 2109. The anesthetic and surgical courses for these patients were, overall, uncomplicated. All the patients had been admitted to a specialized isolation unit in the pediatric intensive care unit for cardiopulmonary and neurological monitoring. The use of increased protective personal equipment during anesthesia and surgery did not impede a successful neurosurgical operation. CONCLUSIONS: SARS-CoV-2-positive pediatric patients with minimal coronavirus disease 2019-related symptoms who require urgent neurosurgical interventions face unique challenges regarding their anesthetic status, operative delays due to SARS-CoV-2 polymerase chain reaction testing, and requirements for additional protective personal equipment. Despite these clinical challenges, the patients in our study had not experienced adverse postoperative consequences, and no healthcare professional involved in their care had contracted the virus.

Screening in cryptogenic brain abscess: Do not forget pulmonary arteriovenous malformations.

BACKGROUND: Brain abscess usually occurs secondary to trauma, through contiguous spread (e.g.; dental infections, [paranasal] sinusitis, otitis, and mastoiditis), after intracranial neurosurgical procedures, or through hematogenous spread in case of an arteriovenous (AV) shunt, for example; atrial septum defect. Although uncommon, another possible cause of AV shunt which can facilitate brain abscess is a pulmonary arteriovenous malformation (PAVM). We report a case of brain abscess secondary to a solitary PAVM and review the literature. CASE DESCRIPTION: A 74-year-old male patient presented with headaches, fatigue, low-grade fever, and homonymous hemianopsia. He was diagnosed with a brain abscess in the left occipital lobe. A chest computed tomography (CT) with intravenous (IV) contrast was performed because of fever and respiratory insufficiency in a period where screening for COVID-19 in suspected patients was important. A solitary PAVM of the left lung was diagnosed. Initial stereotactic burr hole drainage of the abscess was insufficient and resection of the abscess was deemed necessary. Routine workup did not reveal any additional pathology apart from the PAVM. After treatment of the cerebral abscess, the PAVM was treated with embolization using an endovascular plug. CONCLUSION: It is recommended to screen for PAVM by chest CT with IV contrast in patients with brain abscess when no obvious source of infection can be identified.

Incidence of hemorrhagic cerebrovascular disease due to vascular malformations during the COVID-19 national quarantine in Italy.

BACKGROUND: hemorrhagic cerebrovascular disease due to vascular malformations represents an emergency for neurosurgery and neuro-interventional departments. During the COVID-19 pandemic, a dramatic reduction in the number of hospitalizations for acute myocardial infarction or stroke and a larger time interval from symptom onset to first medical contact have been reported. This study aims to verify the hypothesis that there would also have been a reduction of admissions for hemorrhagic cerebrovascular disease during the Italian lockdown. MATERIAL AND METHOD: s A multicenter, observational survey was conducted to collect data on hospital admissions for hemorrhagic cerebrovascular disease due to vascular malformations throughout two-months (March 15th to May 15th); the years 2020 (COVID-19 Italian lockdown), 2019 and 2018 were compared. Cases were identified by ICD-9 codes 430, 431, 432.1, 432.9, 747.81 of each hospital database. The statistical significance of the difference between the event rate of one year versus the others was evaluated using Poisson Means test, assuming a constant population. RESULTS: During the 2020 lockdown, the total number of admissions for hemorrhagic cerebrovascular disease was 92 compared with 116 in 2019 and 95 in 2018. This difference was not significant. GCS upon admission was 3-8 in 44 % of cases in 2020 (41 patients), 39.7 % in 2019 (46 patients) and 28 % in 2018 (27 patients). CONCLUSION: Reduction of admissions for hemorrhagic cerebrovascular disease due to vascular malformations during the COVID-19 lockdown was not confirmed. Nevertheless, some patients reached the emergency rooms only several days after symptoms onset, resulting in a worse clinical condition at admission.