Anesthetic challenges in the management of Larsen syndrome: A rare congenital anomaly

Larsen syndrome is a rare inherited disease associated with dislocations of multiple joints, typical syndromic facies, and multiple spine abnormalities. They often required multiple corrective orthopedic surgeries to regain their functional ability, thus needing repeated anesthesia. Apart from skeletal deformities, they have predicted difficult airway and need extreme care during intubation and positing of the patient. Abnormal posturing due to spinal deformity and poor pulmonary reserve due to kyphoscoliosis creates an extremely challenging situation for the anesthetist to manage the case during the perioperative period. Here we are describing the perioperative anesthetic management of a patient with Larsen syndrome. Copyright © 2022 Saudi Journal of Anesthesia Published by Wolters Kluwer - Medknow.

Case series of extra cardiac findings with increased perfusion during clinical 15O-H2O PET myocardial perfusion imaging

Aim/Introduction: Multiple extra cardiac findings with increased perfusion have been reported as coincidental findings during myocardial perfusion imaging (MPI) with various radiotracers. At our facility, 15O-H2O-PET MPI was implemented in daily clinical routine in May 2020, and we therefore anticipated similar findings using this radiotracer. The aim of this is therefore to share a case series of our initial experience regarding extra cardiac findings characterized by increased blood flow on 15O-H2O-PET. Material(s) and Method(s): Patient cases with extra cardiac findings observed during clinical 15O-H2O PET MPI scans were collected. All patients were scanned according to facility standards and international guidelines on a GE Discovery MI Digital Ready PET/CT (GE Healthcare, Waukesha, Wisconsin, USA). In contrast to the parametric myocardial 15O-H2O images, summed and gated retention images were used for assessment of extra cardiac perfusion. Biopsy histopathological diagnosis was used as reference standard in all malignant lesions and in some benign lesions. In the remaining patients, other imaging modalities were used as reference standard. Result(s): A variety of malignant lesions with increased perfusion appeared during the scans, including primary tumours of breast cancer, lung cancers, large-celled neuroendocrine tumours, lymphomas and metastases from colonic carcinomas and renal cell carcinomas. Furthermore, a number of benign conditions with increased perfusion were observed: Healing rib fractures, gynecomastia, atelectasis, acute pneumonia (disappeared on subsequent FDG-PET/CT), lung infiltrate with biopsy-verified chronic inflammation and fibrosis (FDG-positive), biopsy-verified benign lung nodulus, chronic diffuse lung infiltrates, pleural plaques and sequelae after recent COVID-19 infection (scan 12 days after positive test). In general, the benign findings were either inflammation or hyperplastic tissue, which are conditions known to be characterized by increased perfusion. Conclusion(s): Both malignant and benign extra cardiac coincidental findings with increased perfusion are readily visible and frequently encountered on 15O-H2O-PET MPI. In most cases, the coincidental findings are also visible on the low-dose attenuation correction CTscan. However, we did observe a 15O-H2O avid breast cancer tumour located outside the CT field of view, underlining the importance of evaluating the entire field of view of the retention 15O-H2O-PET and not just the reconstructed parametric myocardial images.

Clinical and Diagnostic Characteristics of Children with Sars-Cov2 Infection at a Picu in a Tertiary Hospital in Colombia: It Is Better a Two-Step Diagnostic Strategy?

BACKGROUND AND AIM: Children affected with COVID-19 infection are less likely to develop severe disease. The burden of disease and allocated resources make diagnosis and management differ among centers. Missed cases could be reduced if a second test is carried out in those with an initial negative assay. AIM: To describe the clinical characteristics, imaging finding and outcomes of COVID-19 disease in children admitted to a Pediatric Intensive Care Unit (PICU). METHOD(S): Retrospective, single-center study in patients (0-18 years) with confirmed COVID-19 infection, during one year of the SARS-CoV-2 outbreak. RESULT(S): Twenty-four patients were included, with respiratory disease (n=12, 52%), sepsis (n=5, 22%), MIS-C (n=4, 17%) and non-Covid related diagnosis (brain trauma, status epilepticus and abdominal mass). Seventeen patients (71%) had an initial positive COVID-19 test. The other seven (29%) were detected with a second RT-PCR. Supportive treatment included vasopressors (n=4), steroids (n=3), dialysis (n=2), high flow nasal cannula (n=3) and mechanical ventilation (n=9). The mean PICU length of stay was 3 days (IQR 1.5-16). Patterns in chest x-rayincluded: interstitial (n=11, 50%), alveolar (n=4, 18%) and atelectasis (n=4, 18%). Two patients died (8%), one immunocompromised with sepsis and the other because of intracranial bleeding. CONCLUSION(S): This study describes the clinical and radiological characteristics of severe COVID-19 infection in children and highlights the value of a second diagnostic test to decrease the false negative rate. Further investigation is warranted in order to evaluate the impact of this approach in terms of cost, isolation measures and inpatient infection rates. (Table Presented).

Association of Chest X- ray findings with SARS-CoV-2 severity

Background: The COVID-19 first surfaced when cluster of pneumonia patients arose in Wuhan, Hubei Province, China. Although the current gold standard for COVID-19 diagnosis is reverse transcriptase-polymerase chain reaction (RT-PCR), chest x-ray (CXR) and computed tomography (CT) play a vital role in sickness diagnosis due to their limited sensitivity and availability. Aim: To evaluate retrospectively the role of CXR, the main radiological findings in it and its diagnostic accuracy in COVID-19 pneumonia. Methods: This is a cross sectional study involving 264 PCR positive COVID-19 patients with their clinical-epidemiological findings admitted at Ziauddin Hospital from May-July 2020. CXRs were taken as digital radiographs in our emergency department's isolation wards using the same portable X-ray device, according to local norms. CXRs were taken in two directions: antero-posterior (AP) and postero-anterior (PA). The hospitals' database had all of the images. To determine the number of radiological findings, multiple radiologists on duty completed an independent and retrospective examination of each CXR. In the event of disagreement, a mutual agreement was reached. SPSS version 20 was used for statistical analysis. Results: We were able to find 264 patients who met our criteria. With a mean age of 56.4214.89, the majority of individuals were determined to be males 189(71.6%) and females 75(28.4%). (Range of 16 to 87 years). 127 patients (48.1%) had severe illness symptoms and were admitted to the ICU, while the remaining 102(38.6%) had mild to moderate disease 35(13.3%). Diffuse (29.2%) and middle and lower co-existing distribution (25.8%) whereas just lower lobe (13.3%) were the most common predominance in severity. Peripheral involvement was also seen in (8.7%) cases. Conclusion: Both lungs are equally affected with the disease having the consolidation and opacifications while the effusion is the major complication in the severe cases. Diffuse involvement of the lung lobes is seen in the study followed by the middle and lower lobe involvement.

ACCELERATED GRANULOMATOUS LYMPHOCYTIC INTERSTITIAL LUNG DISEASE DUE TO COVID-19 IN A PATIENT WITH COMMON VARIABLE IMMUNODEFICIENCY PRESENTING WITH RECURRENT PNEUMOTHORACES

SESSION TITLE: Post-COVID-19 Infection Complications SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Patients with COVID-19 usually recover completely in the acute setting but it has been demonstrated post-infectious complications include continued dyspnea, myalgias, and other long-term complications which are not fully known yet. A case published in the British Medical Journal by Nunna demonstrated the development of a large spontaneous pneumothorax in a middle-aged patient after his Covid-19 infection [1]. Here, we present a 60-year-old female with a history of common variable immunodeficiency (CVID) and hypothyroidism presenting with recurrent pneumothoraxes as a late complication of COVID-19. CASE PRESENTATION: A 60-year-old female with a history of CVID and COVID-19 pneumonia complicated by chronic hypoxic respiratory failure and a right sided loculated hydropneumothorax diagnosed in December of 2020, on 3 to 5 liters of home oxygen, presents to the emergency department due to dyspnea and left-sided pleuritic chest pain in July of 2021. On presentation, the patient was tachypneic, had labored breathing and was requiring 7 liters of oxygen to saturate adequately. Repeat imaging of the chest showed a large tension pneumothorax on the left side with near complete collapse of the lung and tracheal deviation to the right [figure 1]. At that time, a chest tube was placed to re-expand the lung. After 5 days of treatment, repeat imaging showed marked improvement, with the pneumothorax decreasing significantly [figure 2]. Pulmonary function testing in the outpatient setting showed a moderate restrictive lung defect with sever decrease in diffusion capacity. The patient continued to have dyspnea so, the decision was made for the patient to undergo an open lung biopsy. The pathology report showed noncaseating granulomas with focal interstitial fibrosis and lymphocytic infiltrates consistent with granulomatous lymphocytic interstitial lung disease (GLILD), which is a complication of CVID [figure 3]. Roughly 1 in 5 patients with CVID develop histopathological findings consistent with GLILD[2]. We believe these changes were accelerated due to her COVID-19 infection. DISCUSSION: This case habits the importance of continued consideration for long-term complications of COVID-19, especially in patients who are immunocompromised. Reports of diffuse alveolar injury caused by the virus can result in emphysematous changes ultimately leading to alveolar rupture such as in this patient [3]. Although pneumothorax is an uncommon late complication, it should be on the differential diagnosis for COVID-19 patients with sudden respiratory decompensation. As a life-threatening event, it requires prompt recognition and treatment. CONCLUSIONS: Patients who have CVID complicated by GLILD accelerated by COVID-19, are more prone to life-threatening tension pneumothoraxes and they should be encouraged to seek lung transplantation as this could be the only way to stop the formation of these pneumothoraxes. Reference #1: Nunna, K., & Braun, A. B. (2021). Development of a large spontaneous pneumothorax after recovery from mild COVID-19 infection. BMJ Case Reports, 14(1), e238863. Reference #2: Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD): A Spectrum of Pulmonary Histopathological Lesions in Common Variable Immunodeficiency (CVID) - Histological and Immunohistochemical Analysis of 16 cases. (n.d.). Reference #3: Gradica, F. (2020). Spontaneous Pneumothorax in Covid-19 Pneumonia. Case report. Clinical Orthopaedics and Trauma Care, 2(1), 01–03. https://doi.org/10.31579/2694-0248/010 DISCLOSURES: No relevant relationships by Elizabeth Bankstahl No relevant relationships by Talal Bazzi No relevant relationships by Mujtaba Cherri No relevant relationships by Khairya Fatouh

MUCORMYCOSIS-RELATED CENTRAL AIRWAY OBSTRUCTION MANAGED BY CUSTOM 3-D PRINTED AIRWAY STENT

SESSION TITLE: Great Procedural Cases: Fire, Ice, Struts, Valves, and Glue SESSION TYPE: Case Reports PRESENTED ON: 10/19/2022 11:15 am - 12:15 pm INTRODUCTION: While rarely reported, there has been an increasing incidence of tracheobronchial mucormycosis in patients infected with COVID-19, especially when associated with uncontrolled diabetes mellitus. We describe a complex case of central airway obstruction due to mucormycosis that was managed with a custom 3D printed silicone Y-stent. CASE PRESENTATION: A 54-year-old woman with diabetes, prior COVID-19 infection, presented with dyspnea and cough. She underwent a CT chest which showed left lower lobe atelectasis and left main stem bronchus (LMSB) obstruction. Bronchoscopy showed a large exophytic mass extruding from the LMSB. There was necrosis leading to a fistula between the left and right mainstem bronchi distal to the carina. Pathology of the mass showed necrotic bronchial mucosa and cartilage with invasive mucormycosis. She underwent placement of a 10X40mm covered stent in LMSB. However, due to granulation tissue and recurrent mucus plugging, she needed a bronchoscopy every 3-4 weeks and multiple stent revisions. Despite these interventions, her respiratory symptoms did not change significantly. Ultimately, her airway was also complicated by tracheobronchomalacia (TBM) of the right main stem bronchus (RMSB). Subsequently a custom printed 3D silicone Y-stent from VisionAir was placed that allowed successful recanalization of LMSB and management of the TBM of RMSB simultaneously. The patient reported significant improvement in respiratory symptoms. She was maintained on Isavuconazole for mucormycosis suppression therapy DISCUSSION: Mucormycosis infections commonly occur in the pulmonary or rhino-cerebral region with high morbidity and mortality. Mucor can involve the major airways as well as rarely invade the tracheal cartilage leading to TBM. There is often granulation tissue, gray-white mucoid material, with edematous and necrotic airway. This can be associated with complex central airway obstruction. While the covered tracheobronchial stent (Bonastent) allowed us to recanalize LMSB, it was complicated by obstructive granulation tissue formation and mucous plugging requiring frequent stent revision to maintain stent patency. At the same time, the TBM in the right airway was contributing significantly to dyspnea and cough. While a standard silicon Y stent was considered for the management of bilateral mainstem bronchi disease, due to the significant distortion in airway anatomy this was not an ideal option. By using the VisionAir stent, we placed a custom stent that would best fit her airway anatomy. The patient had sustained improvement in her symptoms for several months following the procedure. CONCLUSIONS: This is the first case report of a custom designed and 3D printed stent for the treatment of benign central airway obstruction caused by tracheobronchial mucormycosis. Custom stents are a promising tool to individualize and tailor intervention for patients with complex airway anatomy. Reference #1: Tracheal Mucormycosis Pneumonia: A Rare Clinical Presentation. Satyawati Mohindra, Bhumika Gupta, Karan Gupta and Amanjit Bal. Respiratory Care November 2014, 59 (11) e178-e181 Reference #2: Keshishyan S, DeLorenzo L, Hammoud K, Avagyan A, Assallum H, Harris K. Infections causing central airway obstruction: role of bronchoscopy in diagnosis and management. J Thorac Dis. 2017;9(6):1707-1724. doi:10.21037/jtd.2017.06.31 Reference #3: Leon CA, Inaty H, Urbas A, Grafmeyer K, Machuzak M, Sethi S, Gildea T. Early outcomes with 3D printing and airway stents. CHEST 2019 annual meeting s. DISCLOSURES: No relevant relationships by Sisir Akkineni No relevant relationships by Kelly Daymude No relevant relationships by Wissam Jaber No relevant relationships by Abesh Niroula

CHLORINE GAS EXPOSURE AND OBSTRUCTIVE AIRWAY DISEASE: A CASE REPORT AND CASE-BASED DISCUSSION

SESSION TITLE: Occupational and Environmental Lung Disease Cases SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 12:25 pm - 01:25 pm INTRODUCTION: Chlorine gas is a pulmonary irritant with pungent odor that damages the respiratory tract. Chlorine gas exposure occurs in industrial or household exposures,Chlorine gas has two forms either a liquid or gas, toxicity of chlorine gas depends on the dose and duration of exposure. Chlorine gas used in manufacturing products like paper, insecticides, Chlorine is used to treat bottled and swiming pool water. CASE PRESENTATION: A 37 Y.O Male, no PMH presents with progressive dyspnea for three days worse with activity,decreases with rest, denied cough fever or chest pain he is vaccinated for COVID,no smoking history. The patient worked at a chlorine gas factory in the Dominican Republic for 15 years. Exam: Vitals: BP 124/72 mmHg. HR 100 BPM. RR 21 BPM. SpO2 84%. General: acute distress. Heart: normal S1, S2. RRR. Lung: wheeze bilaterally. Abdomen: Soft. Musculoskeletal: no pitting edema. he was placed on 6 LPM NC saturation improved to 90%. CBC and Chemistry were unremarkable, he was started on steroid, breathing treatment with antibiotics. ABG showed hypoxemia. he was placed on Venturi mask and his saturation improved to 95%.CTA was negative for PE. EKG, troponin were unremarkable. A proBNP normal. The antibiotics were discontinued because of a negative workup. A TTE study was normal. HRCT scan of the chest, showed atelectasis and infiltrates of lower lobes. No interstitial fibrosis.A PFT showed obstructive airway disease. He was discharged on oral and inhaled steroids.Hi new onset obstructive airway could be due to chlorine gas exposure. DISCUSSION: Chlorine gas causes cellular injury through oxidative damage but further damage results from activation and recruitment of inflammatory cells with subsequent release of oxidants and proteolytic enzymes. Humans can detect chlorine gas odor at a concentration between 0.1-0.3 ppm. At 1-3 ppm,it causes irritation of oral,eye mucosal membranes. At 30-40 ppm causes cough, chest pain, and SOB. At 40-60 ppm, toxic pneumonitis and pulmonary edema and can be fatal at 430 ppm concentration or higher within thirty minutes. Chronic exposure to chlorine gas lead to chest pain, cough, sore throat, hemoptysis, recurrent asthma. Physical exam findings include tachypnea cyanosis, wheezing, intercostal retractions, decreased breath sounds. Pulmonary function tests may reveal obstructive lung function disease. Chronic exposure to a low level was found to be associated with an increased risk of asthma in swimmers. CONCLUSIONS: Chlorine exposure results in direct chemical toxicity to the airways with acute airways obstruction or airways hyperreactivity, presentation varies from acute overwhelming intoxication with acute lung injury and or death, occupational exposure increase the likelihood of chronic bronchitis or isolated wheezing attacks. Treatment for chlorine exposure is largely supportive. Reference #1: 1- Center of disease control and prevention website/emergency preparedness and response/ https://emergency.cdc.gov/agent/chlorine/basics/facts.asp Reference #2: 2- C- Morim A, Guldner GT. Chlorine Gas Toxicity. [Updated 2021 Jul 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing;2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537213/. Reference #3: A- Gummin DD, Mowry JB, Beuhler MC, et al. 2020 Annual Report of the American Association of Poison Control Centers’ National Poison Data System (NPDS): 38th Annual Report. Clin Toxicol (Phila). 2021;59(12):1282-1501. doi:10.1080/15563650.2021.1989785 DISCLOSURES: No relevant relationships by Abdallah Khashan No relevant relationships by Samer Talib no disclosure on file for Matthew Yotsuya;

PLEOMORPHIC CARCINOMA IN THE MIDST OF COVID

SESSION TITLE: Pathology Identifying Chest Infections Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Pleomorphic carcinoma is a subtype of sarcomatoid carcinomas that represents <1 % of all primary lung neoplasms. This case highlights a recent diagnosis of a patient with pleomorphic carcinoma in the midst of COVID-19 pneumonia. CASE PRESENTATION: A 75 year old female with a 180-pack year smoking history presented to the emergency department with dyspnea and chest discomfort. Vital signs significant for oxygen saturation at 93% on room air. The patient had been admitted to the hospital 7 months prior for acute hypoxemic respiratory failure due to COVID-19 pneumonia. At that point, computed tomography (CT) of the chest showed a right lower lobe 5.5 cm juxtapleural lesion measuring fluid attenuation by Hounsfield units without intralesional enhancement. The lesion was initially thought to be secondary to the patient's COVID-19 pneumonia and was not investigated further. The patient was subsequently lost to follow up. Seven months later the patient presented with worsening shortness of breath. Chest CT revealed large right complex pleural effusion with near complete lung collapse. The patient underwent pigtail catheter placement with partial re-expansion of the lung. Pleural fluid analysis showed an exudative effusion with no malignant cells on cytology. Follow-up CT imaging showed a large mass-like area in the right mid and lower hemithorax. Video assisted thorascopic surgery (VATS) decortication and thoracotomy revealed a right lower lobe abscess and empyema. Pathology samples collected during procedure showed malignant cells of sarcamatoid features found in right lung and intraparenchymal lymph nodes. Histology and immunostaining showed a tumor composed of a component of poorly differentiated adenocarcinoma and more than 10% spindle/pleomorphic cells. Immunostaining showed the poorly differentiated adenocarcinoma component was positive for moc 31, Ber-EP4, cytokeratin AE1/AE3, CAM 5.2, lack TTF-1 and p40. The spindle/pleomorphic component was negative for cytokeratins. DISCUSSION: Pulmonary pleomorphic carcinoma (PC) is a rare, poorly differentiated non-small cell lung cancer (NSCLC) that contains at least 10% spindle and/or giant cells or a carcinoma consisting only of spindle and giant cells. PC has poor response to conventional treatments for NSCLC and subsequently poor 5 year survival. It more common in men and smokers. COVID-19 causes a variety of pulmonary radiographic manifestations, including nodules and mass-like consolidations. Superimposed bacterial infections are also common. Our case, however, highlights the importance of serial radiographic monitoring and, when indicated, tissue sampling to rule out alternative explanations for abnormal CT findings. CONCLUSIONS: Appropriate screening and careful follow up of suspicious lung lesions is vital to ensure prompt diagnosis and treatment of lung malignancy. Reference #1: WHO Classification of Tumours Editorial Board. Thoracic Tumours. In: WHO Classification of Tumours,Earke 5th ed, IARC Publications, 2021. Vol 5. Reference #2: Ito K, Oizumi S, Fukumoto S, Harada M, Ishida T, Fujita Y, Harada T, Kojima T, Yokouchi H, Nishimura M;Hokkaido Lung Cancer Clinical Study Group. Clinical characteristics of pleomorphic carcinoma of the lung. Lung Cancer. 2010 May;68(2):204-10. doi: 10.1016/j.lungcan.2009.06.002. Epub 2009 Jul 3. PMID: 19577320. Reference #3: Maneenil K, Xue Z, Liu M, Boland J, Wu F, Stoddard SM, Molina J, Yang P. Sarcomatoid Carcinoma of the Lung: The Mayo Clinic Experience in 127 Patients. Clin Lung Cancer. 2018 May;19(3):e323-e333. doi: 10.1016/j.cllc.2017.12.008. Epub 2017 Dec 21. PMID: 29454534. DISCLOSURES: No relevant relationships by Rachel Earle No relevant relationships by Samantha Gillenwater No relevant relationships by Miquel Gonzalez No relevant relationships by Sikandar Khan No relevant relationships by Christopher Lau no disclosure submitted for Jinesh Mehta;

BNT162B2 MRNA BOOSTER VACCINE-RELATED PERICARDITIS: A CASE COMPLICATED BY PERICARDIAL AND PLEURAL EFFUSIONS

SESSION TITLE: Issues After COVID-19 Vaccination Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Immune mediated vaccine related pericarditis reports have been well documented;albeit rare and generally well tolerated, it remains a real complication with possible devastating impacts. The incidence has increased even more with the covid vaccine.3 Here we describe a 72 year old female that received her 4th BNT162b2 dose, 5 months after the 3rd dose, and subsequently developed pericarditis. CASE PRESENTATION: 72 year old female, previously well, who presented with a 3 day history of central, sticking chest pain associated with exertional dyspnea, cough, palpitations and malaise. She denied any coryzal symptoms. On presentation she was hemodynamically stable but hypoxic and tachycardic. Laboratory investigations revealed leucocytosis and troponinemia of 0.13ng/ml. ECG showed diffuse ST elevations. A transthoracic echo showed a small pericardial effusion with normal LV and RV function, EF 60-65% and a CT Pulmonary Angiogram revealed a small sub-segmental pulmonary embolism with atelectasis and trace effusions. On further questioning she revealed that the symptoms started 3 days after she received her 2nd booster dose of the Pfizer covid vaccine. She was started on colchicine and apixaban and was discharged home with follow-up. Two days after discharge the patient represented to the hospital with worsening chest pain. Investigations revealed worsening leucocytosis and increased inflammatory markers (CRP 303mg/L, ESR 62mm/h). A new finding of a small pericardial effusion and bilateral pleural effusions with consolidations were noted on a repeat CT scan. Decision was made to continue colchicine and commence prednisone. Other infectious and inflammatory causes of pericarditis were ruled out. The COVID spike IgG was negative and the NAAT Cov 2 IgG showed titres >250 (<50). DISCUSSION: The exact pathogenesis of the COVID-19 vaccine-induced pericarditis remains unknown. It is thought that mRNA vaccines produce a large number of antibodies which elicit a multi-system inflammatory response1;despite this, steroid therapy remains controversial given the risk of recurrent pericarditis.2 A shorter vaccine interval has been associated with adverse outcomes. CDC extended the dosing interval in young persons to reduce the risk of severe myocarditis;however the interval for persons 65 years or more and immunocompromised remained unchanged. Our case and the identical case described by Singh et al1 reinforces the need to determine the best time interval for administration of the covid booster vaccines;especially in patients more than 65 years. CONCLUSIONS: More research needs to be done as to the most appropriate interval between booster doses to reduce the inflammatory complications related to the vaccine. A consideration should also be made to determine if the measurement of SARS COV-2 IgG spike titres have any role in determining the timing of subsequent booster doses. Reference #1: Singh A, Nguyen L, Everest S, et al. (February 12, 2022) Acute Pericarditis Post mRNA-1273 COVID Vaccine Booster. Cureus 14(2): e22148. DOI 10.7759/cureus.22148 Reference #2: Hajjo R., Sabbah D.A., Bardaweel S.K., Tropsha A. Shedding the Light on Post-Vaccine Myocarditis and Pericarditis in COVID-19 and Non-COVID-19 Vaccine Recipients. Vaccines. 2021;9:1186. doi: 10.3390/vaccines9101186. Reference #3: Diaz GA, Parsons GT, Gering SK, Meier AR, Hutchinson IV, Robicsek A. Myocarditis and Pericarditis After Vaccination for COVID-19. JAMA. 2021 Sep 28;326(12):1210-1212. doi: 10.1001/jama.2021.13443. PMID: 34347001;PMCID: PMC8340007. DISCLOSURES: No relevant relationships by Zachary Banbury No relevant relationships by Michael Basir No relevant relationships by Alexandra Gottdiener No relevant relationships by Janeen Grant-Sittol No relevant relationships by Srikant Kondapaneni No relevant relationships by Ross Lavine No relevant relationships by Anesha White

GRANULOMATOUS-LYMPHOCYTIC INTERSTITIAL LUNG DISEASE AS THE NONINFECTIOUS COMPLICATION OF COMMON VARIABLE IMMUNODEFICIENCY

SESSION TITLE: Difficult Diffuse Lung Disease SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Common Variable Immunodeficiency (CVID) is one of the most common humoral immunodeficiency disorders and usually manifests as infectious complications. However, noninfectious complications such Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) can convey a much poorer prognosis in patients with CVID. In this case report, we present a patient with GLILD who presented with cough and shortness of breath. CASE PRESENTATION: A 66 year old female with past medical history of provoked DVT (off anticoagulation), cervical cancer (s/p resection) presented to pulmonology clinic with complaints of chronic cough and shortness of breath on exertion. She had a negative smoking history and no occupational exposures. She was up to date on vaccinations and COVID was negative. Vitals were stable and physical exam was normal including clear breath sounds. CXR revealed emphysema and hazy opacities in the lung bases. PFTs demonstrated moderate obstructive pattern with no response to bronchodilator, normal lung volumes, and DLCO of 76%. Due to lack of improvement in her cough, CT Chest was done which revealed diffuse pulmonary nodules, bronchiectasis with possible atelectasis or scarring in the RML and lingula, and a prominent subcarinal lymph node. EBUS TBNA of station 7 returned negative for malignancy. Culture showed polymicrobial growth with negative AFB and fungi. Patient was treated without antibiotics, but due to family history of immunodeficiency, immunoglobulin panel was sent which returned low IgG subclasses. She then received IVIG. However, given the centrilobular nodules and lack of response to IVIG, repeat bronchoscopy with TBBx and BAL was performed. BAL revealed lymphocytic predominance and tissue biopsy showed non-caseating granulomas and negative cultures. Eventually patient was diagnosed with GLILD and started on 6 weeks of prednisone 40 mg daily along with PJP prophylaxis. However, her symptoms remained same and rituximab was prescribed with improvement in the symptoms. DISCUSSION: Although recurrent sinopulmonary infections are common in CVID patients, if clinical response to IVIG is minimal to none, GLILD should be considered on the differential. Centrilobular nodules and ground glass opacities should raise suspicion of GLILD and tissue sample should be obtained in these patients to confirm the diagnosis. Appropriate treatment with prednisone or rituximab along with IVIG improves GLILD patient symptoms and yields better outcomes in terms of morbidity and quality of life. CONCLUSIONS: Appropriate treatment with prednisone or rituximab along with IVIG improves GLILD patient symptoms and yields better outcomes in terms of morbidity and quality of life. Reference #1: Hurst JR, Verma N, Lowe D, Baxendale HE, Jolles S, Kelleher P, et al. British lung foundation/United Kingdom primary immunodeficiency network consensus statement on the definition, diagnosis, and management of granulomatous-lymphocytic interstitial lung disease in common variable immunodeficiency disorders. J Allergy Clin Immunol Pract. (2017) 5:938– 45. doi: 10.1016/j.jaip.2017.01.021 DISCLOSURES: No relevant relationships by Benjamin Butler No relevant relationships by Abdulmetin Dursun No relevant relationships by Badri Giri No relevant relationships by Emily Smallwood

MANAGEMENT OF PNEUMOTHORAX AND PNEUMOMEDIASTINUM IN COVID-19 PATIENTS WITH ARDS

SESSION TITLE: What Lessons Will We Take From the Pandemic? SESSION TYPE: Rapid Fire Original Inv PRESENTED ON: 10/19/2022 11:15 am - 12:15 pm PURPOSE: Since the emergence of COVID-19, many serious complications have arisen especially in the setting of Acute Respiratory Distress Syndrome (ARDS) in the intensive care unit. Historically, ARDS and mechanical ventilation is associated with higher rates of pneumothorax. It has been well described that ARDS is the result of inflammatory lung injury, with increased activation of circulating neutrophils, complement and proinflammatory mediators leading to loss of surfactant, alveolar atelectasis, and fibrin deposition. This leads to a less compliant lung parenchyma, and higher airway pressures, which has been attributed as a risk factor for pneumothorax. The management of pneumothorax varies depending on the etiology of the pneumothorax. In the case of iatrogenic pneumothorax (i.e. mechanical ventilation), tube thoracostomy is generally recommended, however the size of the chest tube inserted remains less clear. METHODS: This is a multi-center retrospective cohort study of 88 hospitalized patients with a diagnosis of COVID-19 and pneumothorax or pneumomediastinum between the dates of 3/11/2020 to 01/26/2021. Patient demographics, comorbidities, laboratory and hemodynamic data were collected as well as ventilator settings, lung mechanics, and clinical patient outcome data including type of intervention and rate of resolution of pneumothorax. Final statistical analysis is pending. RESULTS: Our preliminary results reveal that there was a higher rate of resolution of pneumothorax with a large bore chest tube (LBCT) compared to a small bore chest tube (SBCT). There was no significant difference in lung compliance or tidal volume in cc/kg between the patients with a resolving pneumothorax compared to the patients with a nonresolving pneumothorax. Overall mortality rate among all patients was 70.4%. CONCLUSIONS: Patients with a large bore chest tube placed are more likely to have resolution of pneumothorax compared to small bore chest tube or serial X-ray. Lung compliance and tidal volume were not significantly different between patients that had a resolving pneumothorax compared to nonresolving pneumothorax. It is important to manage a pneumothorax early on to reduce associated morbidity. CLINICAL IMPLICATIONS: The development of pneumothorax in COVID patients with ARDS has significant associated morbidity and mortality. Utilization of a large bore chest tube may result in improved rates of resolution of pneumothorax. DISCLOSURES: No relevant relationships by Nathalie Antonios No relevant relationships by Colby Baker No relevant relationships by Jessica Johnson No relevant relationships by Karen Sayad

Deeplearningapproachfor detection of multiple respiratory diseasesfrom chest X-rayanalysis:Asurvey

Deep learning approach for detecting various respiratory diseases hasbeen challenging and mostdemanding research area. Withrapidly increase in number of patients suffering from respiratory diseases quick method hasbecome necessary for classification and detection of respiratory diseases. This survey paper offers a comparative study of various deep learning techniques that can use chest X-raysfordetection of various thoracic diseases.There is possibility of severe respiratory failure in some thoracic diseases if they are not treated in initial stages. Many digital image processing techniques,machine learning and deep learning models have been developed for this purpose[17]. Different forms of existing deep learning techniques including convolutional neural network (CNN), visual geometry group based neural network (VGG-16 and VGG-19) have been developed for respiratory disease prediction. But these all models have some limitations that they do not cover all respiratory diseases including Covid-19, Viral pneumonia and Tuberoculosis on single platform. Therefore, we propose our customized new deep learning model Clx-Net by using data augmentation technique to enlarge the area of available dataset[1][2] to make model more efficient with less time consumption per epoch and provide localization to identify infected region by examining chest X-ray images. Our focus is to develop a new unique deep learning based model Clx-Net which will be able to detect almost all major respiratorydiseases including Covid-19. It will simplify the detection of respiratory diseases and also find the location of infected chest area to make task easy for radiologists.

TRACHEOBRONCHIAL ASPIRATION IN THE ELDERLY

CASE: A 68-year-old male with a past medical history of hypertension and null smoking history presented with insidious onset dyspnea for the past three days. On physical exam, he had inspiratory rhonchi and was hypoxic, saturating to 88% in room air, requiring 6L oxygen. Laboratory studies were unremarkable, including a negative COVID PCR test. Chest X-ray demonstrated right-sided hilar prominence, and CT of the chest revealed an 8 mm endobronchial. On the day of his bronchoscopy evaluation, the patient expectorated a brownish undercooked pea while receiving nebulizer treatment and repeat chest CT revealed the resolution of the previous endobronchial lesion. IMPACT/DISCUSSION: Foreign body aspiration (FBA) has a bimodal presentation with a second peak in adults above 50 years. Although FBA most commonly presents with abrupt onset cough and dyspnea, the immediate presentation may not be evident in the geriatric population given the lack of cough reflex and cognitive decline. A retrospective study performed with data from 140 patients with FBA noted that 44.3% of patients did not present to the emergency in the first 24 hours of aspiration. Physical exam findings depend on the location of foreign body(FB) dislodgement, but around half the time, the exam could be unremarkable. A radiograph could reveal the object if the aspirated FB is radiopaque;hence a negative radiograph does not rule out the diagnosis of FBA. However, when present, the most common radiographic findings are inspiratory-expiratory abnormalities. High clinical suspicion is required to diagnose FBA to prevent chronic respiratory manifestations. An undiagnosed FB could travel distally and present as pneumonia, bronchiectasis, atelectasis, asthma/COPD-like illness. However, our patient presented with an endobronchial mass that was suspicious for malignancy. We found a similar presentation described by Bader et al. in a case about a 41-year-old woman who underwent chest CT for chronic cough, revealing a mass lesion in the right main bronchus. Bronchoscopic examination showed no growth;instead, the team found a plastic foreign body. The patient admitted aspirating this plastic object in her early 20s. If FBA is suspected, bronchoscopy is the study of choice to evaluate the airway, and extraction of FB can be performed with flexible or rigid bronchoscopy. Although flexible bronchoscopy requires only local anesthesia and a rigid bronchoscopy requires general anesthesia, the latter is safer in preventing damage to the airway. Given that each case of FBA can present unique challenges and might occasionally need endotracheal intubation or tracheostomy, only experts should perform bronchoscopic extraction of FB. CONCLUSION: In this COVID era, it is very reasonable to be anchored to a diagnosis of COVID for every patient who presents with dyspnea. FBA should be one of the differential diagnoses for geriatric patients presenting with newonset respiratory symptoms even when no physical or radiographic signs are evident.

False positive lung perfusion/ventilation scan due to rapidly resolved pulmonary abnormalities: Importance of SPECT/CT imaging

An eighty-year-old lady a with history of treated tuberculosis decades ago and polymerase chain reaction (PCR) proven COVID-19 pneumonia about four months ago was referred to our department for ventilation/perfusion scan due to deteriorating dyspnea to rule out P.E. Planar perfusion scan showed bilateral accentuated apical perfusion gradient and multiple segmental and non-segmental perfusion defects in both lungs, which were mismatched with relatively normal planar ventilation images. Perfusion SPECT images also showed multiple segmental, sub-segmental and semi-segmental perfusion defects, which proved to be concordant with consolidations, bilateral pleural effusion, right lung pneumothorax, and atelectasis which were consultant with CT findings. The ventilation SPECT/CT images also demonstrated uniform tracer activity throughout both lungs, with almost complete improvement in consolidations, lung pneumothorax, pleural effusion, and atelectasis. Our case highlights the importance of SPECT/CT imaging in avoiding false-positive interpretation of pulmonary embolism as well as the possibility for rapid resolution of the lung parenchymal abnormalities

An IoMT-Based Federated and Deep Transfer Learning Approach to the Detection of Diverse Chest Diseases Using Chest X-Rays

Since chest illnesses are so frequent these days, it is critical to identify and diagnose them effectively. As such, this study proposes a model designed to accurately predict chest disorders by analyzing multiple chest x-ray pictures obtained from a dataset, consisting of 112,120 chest X-ray images, obtained the National Institute of Health (NIH) X-ray. The study used photos from 30,805 individuals with a total of 14 different types of chest disorder, including atelectasis, consolidation, infiltration, and pneumothorax, as well as a class called "No findings" for cases in which the ailment was undiagnosed. Six distinct transfer-learning approaches, namely, VGG-16, MobileNet V2, ResNet-50, DenseNet-161, Inception V3, and VGG-19, were used in the deep learning and federated learning environment to predict the accuracy rate of detecting chest disorders. The VGG-16 model showed the best accuracy at 0.81, with a recall rate of 0.90. As a result, the Fl score of VGG-16 is 0.85, which was higher than the Fl scores computed by other transfer learning approaches. VGG-19 obtained a maximum rate of accuracy of 97.71% via federated transfer learning. According to the classification report, the VGG-16 model is the best transfer-learning model for correctly detecting chest illness.

Human metapneumovirus infection in the cardiac paediatric ICU before and during COVID-19 pandemic: a retrospective cohort analysis.

INTRODUCTION: This study investigates the hygiene standards in the context of the COVID-19 pandemic and their impact on the perioperative incidence of human metapneumovirus as well as the typical symptom burden of human metapneumovirus-infected children with CHDs. MATERIALS AND METHODS: Between March 2018 and July 2021, all patients of a cardiac paediatric ICU of a German university hospital were included in this retrospective cohort analysis. RESULTS: A total of 589 patients with CHD were included in the analysis. Three hundred and fifty-two patients (148 females and 204 males) were admitted before the introduction of social distancing and face masks between March 2018 and 15 April 2020 (cohort A). Two hundred and thirty-seven patients (118 females and 119 males) were admitted after the introduction between April 16 and July 2021 (cohort B). In cohort A, human metapneumovirus was detected in 11 out of 352 patients (3.1%) during their stay at cardiac paediatric ICU. In cohort B, one patient out of 237 (0.4%) tested positive for human metapneumovirus. Patients who tested positive for human metapneumovirus stayed in cardiac paediatric ICU for a median of 17.5 days (range, 2-45 days). Patients without a detected human metapneumovirus infection stayed in the cardiac paediatric ICU for a median of 4 days (range, 0.5-114 days). Nine out of 12 (75%) human metapneumovirus-positive patients showed atelectasis. CONCLUSION: Perioperative human metapneumovirus infections prolong cardiac paediatric ICU stay in children with CHD. In affected patients, pulmonary impairment with typical symptoms appears. Under certain circumstances, a complication-rich perioperative infection with human metapneumovirus could be prevented in paediatric cardiac high-risk patients by prophylactic hygiene intervention.

A Case of Pulmonary Cryptococcosis After Severe COVID-19 Pneumonia T. Roesch

Introduction / Case Presentation:46yo female with a history of CKD, atrial flutter, bioprosthetic valve with mitral ring, and recent COVID-19 pneumonia who presented to the emergency department (ED) with shortness of breath, fevers, and fatigue. Three months prior, she had been diagnosed with severe COVID-19 pneumonia, for which she received dexamethasone, remdesivir, tocilizumab, anakinra, and IVIG. She was discharged to a nursing facility with a prolonged steroid taper, ending 1 month prior to admission.In the ED, the patient had a chest x-ray that demonstrated bibasilar atelectasis and opacification, and a CT chest revealed right lower lobe consolidation and surrounding ground glass opacities. A respiratory pathogen PCR swab was negative. Sputum culture was negative for bacterial and fungal growth. Blood cultures did not grow any organisms. Given recent immunosuppression and imaging findings, a serum Cryptococcal antigen was drawn, which was positive with a titer of 1:128. A transthoracic needle biopsy of the patient's right lower lung was then performed. The specimen did not grow any bacteria or fungi and AFB stain on the tissue was negative. Pathology demonstrated a collection of histiocytes, neutrophils, and necrotic debris. PAS, GMS, and mucicarmine stains were positive for fungal organisms consistent with Cryptococcus species. Discussion: Cryptococcosis is a fungal infection due predominately to one of two encapsulated yeasts, Cryptococcus neoformans or Cryptococcus gattii. C. neoformans is found in soil worldwide, and infection typically begins with spore inhalation. Clinically significant disease is seen mostly in immunocompromised patients.Corticosteroids and interleukin inhibitors, such as anakinra (IL-1) and tocilizumab (IL-6), are used in the treatment of COVID-19. These medications have been associated with increased risk for opportunistic infections, including invasive fungal infections. The diagnosis of pulmonary cryptococcosis may be challenging, as symptoms are often nonspecific and may radiographically resemble bacterial pneumonia, malignancy, or other infections. Serum cryptococcal antigen detection tests may be helpful in establishing the diagnosis, as well as histopathology showing narrow-based budding yeast. Conclusion: Patients with prior COVID-19 infection commonly return to healthcare settings with sequelae of their previous coronavirus infection. In our case, it was the prior treatment of COVID-19, which included immunomodulating therapy, that lead to a secondary pulmonary cryptococcal infection. When evaluating pulmonary processes that evolve after an acute infection with COVID-19, it is important to keep a broad differential, including uncommon and/or opportunistic infectious etiologies, particularly when a patient has received prolonged courses of steroids and tocilizumab.

A Case of Symptomatic Endobronchial Hamartoma Causing Right Upper Lobe (RUL) Atelectasis

Introduction: Pulmonary Hamartomas are the most common benign tumors of lungs. Hamartomas are usually asymptomatic and are incidentally diagnosed on chest imaging. Symptomatic cases usually involve endobronchial mucosa. We describe a case of a symptomatic patient who was noted to have a right upper lobe collapse by an endobronchial lesion. This lesion was confirmed to be a cartilaginous hamartoma and was definitively treated by electrosurgical by Argon plasma (APC). Description: A 37 year old obese male active smoker presented to the ER for worsening Shortness of air (SOA) for two weeks. He had dry cough for similar duration without any fevers. On physical examination he was afebrile with normal vital signs but without hypoxia and otherwise normal physical examination. Labs revealed normal white cell count. A COVID test was negative. CT chest showed an endobronchial lesion obstructing the right upper lobe (RUL) with some internal fat density measuring at least 1.5cm causing complete collapse of RUL. No mediastinal or hilar adenopathy was noted. A flexible bronchoscopy showed a pedunculated smooth lesion arising from RUL bronchus anteriorly occluding the RUL bronchus. FNA of the lesion did not show any malignant cells. Using electrosurgery by APC the lesion was removed en bloc. A histopathology showed findings of cartilaginous hamartoma. A follow up Chest X-ray showed improvement in RUL atelectasis. Discussion: Pulmonary hamartomas are the most common benign pulmonary tumors and comprise 6% of solitary pulmonary nodules. Mostly detected on chest imaging as an incidental finding however CT chest has characteristic finding of pop corn shaped calcifications, fat density. Symptomatic cases are usually endobronchial and can be definitively treated with endoscopic techniques.

Isolated pauci-immune pulmonary capillaritis associated with an extensive bilateral hemothorax and pleuro-pericarditis

Introduction: Isolated pauci-immune pulmonary capillaritis (IPIPC) is a rare disorder characterized by small vessel vasculitis limited to alveolar capillaries in the absence of systemic manifestations. There are very few case reports of this disorder in the medical literature. Case Report: A 37-yo male with no known history of autoimmune pathology who was admitted to the hospital for evaluation and treatment of dyspnea and thoracalgia. Peripheral blood cultures, serum studies to detect Legionella and Pneumococcus antigens, and a nasopharyngeal swab test for covid-19 were all negative. Chest imaging revealed bilateral pleural effusions from the base to the apices with concomitant atelectasis of the adjacent lung parenchyma. Although the results of an 18F-PET-CT scan revealed no pathological uptake, video-assisted thoracoscopy revealed diffusely edematous pleura with crater-like patches with new onset of venous vessel varicosities, intra-alveolar hemorrhages associated with disordered vascularization, suggesting small vessel vasculitis. Histologic findings included widespread intra-alveolar hemorrhage with organizing injury, hemosiderin-laden macrophages, scattered intra-arterial thrombi, and diffuse perivascular neutrophilic infiltrates consistent with a diagnosis of capillaritis. Conclusions: Given the negative immune studies (save for a weakly-positive lupus anticoagulant and no evidence for extra-pulmonary vasculitis, the diagnosis was Isolated pauci-immune pulmonary capillaritis. The patient recovered in response to immunosuppressive/anti-inflammatory therapy.

Safe and effective administration of multiple recombinant factors on VA-ECMO via post-oxygenator pigtail during acute massive pulmonary hemorrhage

Introduction: Massive bleeding on extracorporeal membrane oxygenation (ECMO) is associated with multiple coagulation defects, including depletion of coagulation factors and development of acquired von Willebrand syndrome (AVWS). The use of recombinant factors, in particular recombinant activated factor VII (rFVIIa, Novoseven), to treat severe refractory hemorrhage in ECMO has been described. However, the use of multiple recombinant factors has been avoided in large part due to concern for circuit complications and thrombosis. Here, we describe the safe and effective administration of rFVIIa and recombinant von Willebrand factor complex (vWF/ FVIII, Humate-P) via post-oxygenator pigtail catheter on VA-ECMO for the treatment of massive pulmonary hemorrhage. Case Description: A 21-month-old (13.4 kg) girl with a recent history of COVID-19 infection presented to an outside hospital with parainfluenza bronchiolitis resulting in acute refractory hypoxemic respiratory failure (oxygenation index 58), refractory septic shock, and myocardial dysfunction. She was cannulated to VA-ECMO and subsequently diagnosed with necrotizing pneumonia from Pseudomonas and herpes simplex infections. Her course was complicated by a large left-sided pneumatocele and bronchopleural fistula requiring multiple chest tubes. She also had right mainstem bronchus obstruction from necrotic airway debris and complete right lung atelectasis. She was noted to have prolonged episodes of mucosal and cutaneous bleeding (oropharynx, chest tube insertion sites, peripheral IV insertion sites) associated with absent high molecular weight von Willebrand multimers consistent with AVWS. Tranexamic acid infusion was initiated and bivalirudin anticoagulation was discontinued. VA-ECMO flows were escalated to 140-160 ml/kg/min to maintain circuit integrity and meet high patient metabolic demand in the absence of anticoagulation. On ECMO day 26, she underwent bronchoscopy to clear necrotic debris from her airway to assist with lung recruitment. The procedure was notable for mucosal bleeding requiring topical epinephrine and rFVIIa. Post-procedure, she developed acute hemorrhage from her right mainstem bronchus, resulting in significant hemothorax (estimated 950 ml) with mediastinal shift, increased venous pressures, desaturation and decreased ECMO blood flow rate, necessitating massive transfusion of 2,050 ml (150 ml/kg) of packed red blood cells, platelets, plasma and cryoprecipitate. An airway blocker was placed in the mid-trachea to control bleeding. In addition to transfusion of appropriate blood products and continuation of tranexamic acid infusion, she was given both rFVIIa (100mcg/kg) and vWF-FVIII (70 units vWF/kg loading dose on the day of hemorrhage, followed by 40 units vWF/kg every 12 hours for 3 additional doses). Both products were administered over 10 minutes through a post-oxygenator pigtail to allow the product to circulate throughout the patient prior to entering the ECMO circuit. The circuit was closely monitored during administration and no changes to circuit integrity were noted in the subsequent hours while hemostasis was achieved. The ECMO circuit remained without thrombosis for 9 days after the bleeding event. Discussion: Balancing anticoagulation and hemostasis is a central challenge in maintaining ECMO support, especially given the prevalence of acquired coagulopathies such as AVWS. For our patient, AVWS contributed to mucosal bleeding necessitating cessation of anticoagulation and utilization of a high ECMO blood flow strategy to minimize circuit clot burden. This was further complicated by absent native lung function and minimal myocardial function, resulting in complete dependence on ECMO. An acute massive pulmonary hemorrhage was treated with multiple recombinant factors (rFVIIa and vWF/FVIII), that are often avoided on ECMO. To minimize clotting risk to the circuit and to maximize transit of these factors to our patient, we added a post-oxygenator pigtail for administration. While this approach was the result of extreme circumstances, th use of a post-oxygenator pigtail for administration of recombinant factors may represent a viable strategy for refractory hemorrhage while on ECMO.