Fatal pulmonary tumour thrombotic microangiopathy in patient with ovarian adenocarcinoma: review and a case report

Background Pulmonary tumour thrombotic microangiopathy (PTTM) is a fatal disease in which tumour cells embolize to the pulmonary vasculature leading to pulmonary hypertension and right heart failure. Early diagnosis is essential for timely treatment which can reduce intimal pulmonary vascular proliferation and prolong survival, improve the symptoms. Due to rare occurrences and no clear diagnostic guidelines the disorder usually is found post-mortem. We present a review of this rare disease and a case of post-mortem diagnosed pulmonary tumour thrombotic microangiopathy in a young female. Case presentation 51 years old woman presented with progressively worsening dyspnea, right ventricular failure signs and symptoms. Computerized tomography denied pulmonary embolism. 2D transthoracic echocardiography demonstrated right ventricle dilatation and dysfunction, severely increased systolic pulmonary pressure. Right heart catheterization revealed pre-capillary pulmonary hypertension with mean pulmonary artery pressure of 78 mmHg, pulmonary wedge pressure of 15 mmHg, reduced cardiac output to 1.78 L/min with a calculated pulmonary vascular resistance of 35 Wood units, and extremely low oxygen saturation (26%) in pulmonary artery. Because of worsening ascites, pelvic magnetic resonance imaging was performed, tumours in both ovaries were diagnosed. Due to the high operative risk, detailed tumour diagnosis surgically was not established. The patient developed progressive cardiorespiratory failure, unresponsive to optimal heart failure drug treatment. A postmortem morphology analyses revealed tumorous masses in pre-capillary lung vessels, right ventricle hypertrophy, ovary adenocarcinoma. Conclusions An early diagnosis of PTTM is essential. Most cases are lethal due to respiratory failure progressing rapidly. Patients with a history of malignancy, symptoms and signs implying of PH should be considered of having PTTM. If detected early enough, combination of chemotherapy with specific PH therapy is believed to be beneficial in reducing intimal proliferation and prolonging survival, along with improving the symptoms.

Fox (Vulpes vulpes) involvement identified in a series of cat carcass mutilations

This study was designed to identify the cause of mutilation and death in 32 cats, part of a larger cohort found dead in Greater London, the United Kingdom, between 2016 and 2018. At the time, discussion in the media led to concerns of a human serial cat killer (dubbed The Croydon Cat Killer) pursuing domestic cats, causing a state of disquietude. Given the link between animal abuse and domestic violence, human intervention had to be ruled out. Using a combination of DNA testing, computed tomography imaging, and postmortem examination, no evidence was found to support any human involvement. Instead, a significant association between cat carcass mutilation and the presence of fox DNA was demonstrated. Gross examination identified shared characteristics including the pattern of mutilation, level of limb or vertebral disarticulation, wet fur, wound edges with shortened fur, and smooth or irregular contours, and marks in the skin, muscle, and bone consistent with damage from carnivore teeth. Together these findings supported the theory that the cause of mutilation was postmortem scavenging by red foxes ( Vulpes vulpes). The probable cause of death was established in 26/32 (81%) carcasses: 10 were predated, 8 died from cardiorespiratory failure, 6 from blunt force trauma, one from ethylene glycol toxicity, and another from liver failure. In 6 carcasses a cause of death was not established due to autolysis and/or extensive mutilation. In summary, this study highlights the value of a multidisciplinary approach to fully investigate cases of suspected human-inflicted mutilation of animals.

Abstract 17180: Complications Of A Severe Case Of Multisystem Inflammatory Syndrome In Children

Case Presentation: A 10 year old male with prior COVID-19 exposure presented with 7 days of fever, rash, cough, vomiting, and hypotension. Laboratory evaluation was notable for SARS-CoV2 antibodies, elevated cardiac enzymes, BNP, and inflammatory markers. Initial echocardiogram showed normal cardiac function and a small LAD coronary aneurysm. He was diagnosed with Multisystemic Inflammatory Syndrome in Children (MIS-C) and given methylprednisolone and IVIG. Within 24 hours, he developed severe LV dysfunction and progressive cardiorespiratory failure requiring VA-ECMO cannulation and anticoagulation with bivalirudin. Cardiac biopsy demonstrated lymphocytic infiltration consistent with myocarditis. On VA-ECMO, he had transient periods of complete AV block. With immunomodulator treatment (anakinra, infliximab) and 5 days of plasmapheresis, inflammatory symptoms and cardiac function improved. He weaned off ECMO, and anticoagulation was transitioned to enoxaparin. He had left sided weakness 5 days later, and brain MRI revealed an MCA infarct. Ten days later, he had focal right sided weakness and repeat MRI showed multiple hemorrhagic cortical lesions, thought to be thromboembolic with hemorrhagic conversion secondary to an exaggerated inflammatory response to an MSSA bacteremia in the setting of MIS-C. Enoxaparin was discontinued. After continued recovery and a slow anakinra and steroid wean, he has normal coronary arteries, cardiac function, and baseline ECG but requires ongoing neurorehabilitation. Discussion: COVID-19 infection in children is often mild, but MIS-C is an evolving entity that can present with a wide range of features and severity. This case highlights two concepts. While first degree AV block is often reported in MIS-C, there is potential for progression to advanced AV block. Close telemetry monitoring is critical, especially if there is evidence of myocarditis. MIS-C shares features with Kawasaki disease, with a notable difference being a higher likelihood of shock and cardiac dysfunction in MIS-C. In MIS-C patients with cardiovascular collapse requiring ECMO, there is a risk for stroke. There should be a low threshold for neuroimaging and multidisciplinary effort to guide anticoagulation in these complex cases.

Treating Congenital Diaphragmatic Hernia with ECMO: First Successful Case in Romania

We present the first successful case of extracorporeal membrane oxygenation (ECMO) at „M.S. Curie” Emergency Clinical Hospital for Children, Newborn Intensive Care Unit: a term neonate with cardiorespiratory failure secondary to left sided congenital diaphragmatic hernia. The patient was placed on veno-venous ECMO at three days of life; however, on the fourth day, due to unstable right ventricular function, conversion to veno-arterial ECMO was mandatory. At one week of life, the patient was operated on-site for diaphragmatic hernia without ECMO support and then reintroduced on ECMO immediately after the surgical procedure, being on ECMO support for a total of 8 days. Antithrombotic treatment was administered for significant occlusion of the right common carotid artery and right internal jugular vein (complication of the cannulation for ECMO) and also long term treatment for Persistent Pulmonary Hypertension was needed. The patient was discharged at the age of four months with moderate neurodevelopmental delay. The literature review indicates that neonatal ECMO procedure in Romania is still in its early stages. Despite this state, our current case proves that ECMO can be successfully performed with increased chances of survival for neonates with severe prognosis after failure of conventional therapy.

Optimizing Outcomes in Extracorporeal Membrane Oxygenation Postcardiotomy in Pediatric Population

Extracorporeal membrane oxygenation (ECMO) is a rapidly emerging advanced life support technique used in cardiorespiratory failure refractory to other treatments. There has been an influx in the number of studies relating to ECMO in recent years, as the technique becomes more popular. However, there are still significant gaps in the literature including complications and their impacts and methods to predict their development. This review evaluates the available literature on the complications of ECMO postcardiotomy in the pediatric population. Areas explored include renal, cardiovascular, hematological, infection, neurological, and hepatic complications. Incidence, risk factors and potential predictors, and scoring systems for the development of these complications have been evaluated.

Clinical manifestations and outcome of SARS-CoV-2 infections in children and adolescents with rheumatic musculoskeletal diseases: data from the National Paediatric Rheumatology Database in Germany

ObjectivesThis study aimed to investigate the clinical manifestations, course and outcome of SARS-CoV-2 infection among children and adolescents with rheumatic and musculoskeletal diseases (RMD). Due to their underlying disease as well due to therapeutic immunosuppression, these patients may be at risk for a severe course of COVID-19 or for a flare of the underlying disease triggered by SARS-CoV-2 infection.MethodsDemographic, clinical and treatment data from juvenile patients with RMD as well as data about SARS-CoV-2 infection like test date and method, clinical characteristics, disease course, outcome and impact on the disease activity of the RMD were documented on a specific SARS-CoV-2 questionnaire implemented in the National Paediatric Rheumatology Database (NPRD) in Germany. The survey data were analysed descriptively.ResultsFrom 17 April 2020 to 16 February 2021, data were collected from 76 patients (52% female) with RMD and laboratory-proven SARS-CoV-2 infection with median age of 14 years, diagnosed with juvenile idiopathic arthritis (58%), autoinflammatory (24%) and connective tissue disease (8%). Fifty-eight patients (76%) received disease-modifying antirheumatic drugs (DMARDs), 41% biological DMARDs and 11% systemic glucocorticoids. Fifty-eight (76%) had symptoms of COVID-19. Disease course of SARS-CoV-2 infection (classified as asymptomatic, mild, moderate, severe, life-threatening) was mild and outcome of COVID-19 (classified as recovered, not yet recovered, permanent damage or deceased) was good (recovered) in the majority of patients. Two patients were hospitalised, one of whom required intensive care and died of cardiorespiratory failure. In 84% of SARS-CoV-2-positive patients, no relevant increase in disease activity of the RMD was observed.ConclusionsIn our cohort, SARS-CoV-2 infection in juvenile patients with RMD under various medications was mild with good outcome in the majority of cases and does not appear to have a relevant impact on disease activity of the underlying condition.

POS1199 CLINICAL MANIFESTATIONS OF SARS-CoV2 INFECTIONS IN CHILDREN AND ADOLESCENTS WITH RHEUMATIC AND MUSCULUSKELETAL DISEASES – SURVEY DATA FROM GERMANY

Background:Although children and adolescents are less likely to develop COVID-19 and generally show milder disease courses, it is unclear what impact the SARS-CoV2 infection has on children and adolescents with rheumatic and musculoskeletal disease (RMD). Due to their underlying disease as well as therapeutic immunosuppression these patients may be at higher risk of being more severely affected by SARS-CoV2. Furthermore, SARS-CoV2 infection might trigger a flare of the underlying disease.Objectives:To evaluate clinical characteristics and disease course of COVID-19 in children and adolescents with RMD and to analyze possible effects of SARS-CoV2 infection on the underlying disease under different therapeutic regimens.Methods:Data from juvenile patients with RMD recorded via the SARS-CoV2 questionnaire within the National Pediatric Rheumatology Database and the registry for hospitalized children and adolescents with COVID-19 of the German Society for Pediatric Infectious Diseases were analyzed. In addition to age, sex and diagnosis, information was collected about the date and method of a positive SARS-CoV2 testing, reason for testing, on clinical manifestations, disease course, treatment and outcome of COVID-19, on drug therapy at the time of virus detection, on disease activity (NRS 0 – 10, 0 = best) of the underlying disease at the last visit before and after the SARS-CoV2 infection.Results:From April 17th 2020 until January 25th 2021, data of 67 patients with RMD and confirmed SARS-CoV2 infection were collected. Mean age was 13.5 ± 3.9 years with equal sex distribution. The majority of patients were diagnosed with juvenile idiopathic arthritis (JIA, 64%), 12 (18%) patients had an autoinflammatory disease (FMF, CAPS, PFAPA, TRAPS) and 5 (7%) a connective tissue disease. Fifty-two patients (78%) were treated with a disease modifying antirheumatic drug (DMARD), 39% with a biological DMARD and 9% systemic glucocorticoids at the time of SARS-CoV-2 infection. Nineteen patients (28%) were tested for SARS-CoV-2 because of typical symptoms, the majority (67%) because of contact to an infected person. PCR was used most often (in 60 %).52 patients (78%) developed symptoms of COVID-19, 15 patients remained asymptomatic. The most common symptom of COVID-19 was rhinitis (42%) and fever (38%), followed by fatigue (34%), taste/smell disorder (33%), sore throat (27%) and cough (23%).Disease severity was graded as mild in 44 of 52 (85%) symptomatic patients, only two patients were hospitalized, one of whom required intensive care and died of cardiorespiratory failure 3 days after symptom onset. In 22 of 26 (85%) SARS-CoV2-positive patients, no relevant increase in disease activity (difference in NRS ≤ 1 before/after infection) of the underlying disease was observed 31 days after symptom onset (median, IQR 17-52 days). One patient, who had paused tocilizumab for 2 doses, experienced a flare of his seronegative polyarthritis 2 months after asymptomatic SARS-CoV-2 infection.Conclusion:In our cohort, the clinical picture of COVID-19 in children and adolescents with RMD was similar to that of healthy peers. The majority of patients showed mild disease course with good outcome under various medications, however, one patient with a severe course of COVID-19 died. In addition, SARS-CoV2 infection does not appear to have a relevant impact on the underlying disease activity, whereas discontinuation of therapy might pose a risk of flare.Disclosure of Interests:None declared.

Intraoperative Computed Tomography-Based Navigation with Augmented Reality for Lateral Approaches to the Spine

Background. Lateral approaches to the spine have gained increased popularity due to enabling minimally invasive access to the spine, less blood loss, decreased operative time, and less postoperative pain. The objective of the study was to analyze the use of intraoperative computed tomography with navigation and the implementation of augmented reality in facilitating a lateral approach to the spine. Methods. We prospectively analyzed all patients who underwent surgery with a lateral approach to the spine from September 2016 to January 2021 using intraoperative CT applying a 32-slice movable CT scanner, which was used for automatic navigation registration. Sixteen patients, with a median age of 64.3 years, were operated on using a lateral approach to the thoracic and lumbar spine and using intraoperative CT with navigation. Indications included a herniated disc (six patients), tumors (seven), instability following the fracture of the thoracic or lumbar vertebra (two), and spondylodiscitis (one). Results. Automatic registration, applying intraoperative CT, resulted in high accuracy (target registration error: 0.84 ± 0.10 mm). The effective radiation dose of the registration CT scans was 6.16 ± 3.91 mSv. In seven patients, a control iCT scan was performed for resection and implant control, with an ED of 4.51 ± 2.48 mSv. Augmented reality (AR) was used to support surgery in 11 cases, by visualizing the tumor outline, pedicle screws, herniated discs, and surrounding structures. Of the 16 patients, corpectomy was performed in six patients with the implantation of an expandable cage, and one patient underwent discectomy using the XLIF technique. One patient experienced perioperative complications. One patient died in the early postoperative course due to severe cardiorespiratory failure. Ten patients had improved and five had unchanged neurological status at the 3-month follow up. Conclusions. Intraoperative computed tomography with navigation facilitates the application of lateral approaches to the spine for a variety of indications, including fusion procedures, tumor resection, and herniated disc surgery.

Role of COVID-19 in Hepatic Damage and Its Cellular Entrance and Mechanism of Action: A Mini-Review

Context: COVID-19 induces damage to the liver in addition to the pulmonary system. This event can alter hepatic biochemical and histological variables. Therefore, the present mini-review study investigated the effect of COVID-19 on hepatic damages and its cellular mechanism of action. Evidence Acquisition: In this mini-review study, the effects of COVID-19 on liver cells were sought in PubMed without restriction. The related components were searched in the Me SH term system and also used in two groups of COVID-19 and hepatocytes in combination with each other. Then, the titles and abstracts of the selected articles were assessed, and unrelated articles were removed. Results and Conclusion: COVID-19 leads to liver tissue damage and hepatocyte apoptosis by inducing increased cytokines, ischemia, and hepatic hypoxia following cardiorespiratory failure, coagulation disorders, and intracellular calcium. The SARS-CoV-2 virus exerts this effect through the ACE2 receptor and increasing its expression in cholangiocytes.