22. Fellows’ Case Files: University of Maryland

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This week we are absolutely thrilled to be launching a new series here at Pulm PEEPs. This is the first episode in our new Fellows’ Case Files series. The purpose of this series is to highlight the incredible clinical work that is done by pulmonary and critical care fellows everywhere, share fascinating cases from across the world, and assemble a diverse network of pulmonary and critical care educators. For each episode, we will visit a different institution, and be joined by a current fellow and the Pulmonary and Critical Care Fellowship Program Director. Our aim is to learn from them, amplify some incredible teaching points, and hear about their program. We hope you enjoy it, and if you have a case you want to bring on the series reach out to us on Twitter or at our email pulmpeeps@gmail.com.

Meet Our Guests

Fahid Alghanim is a senior pulmonary and critical care fellow at the University of Maryland. He attended medical school at the Lebanese American University Gilbert and Rose-Marie Chagoury School of Medicine and completed his internal medicine residency at Johns Hopkins Bayview. He has published on topics ranging from lung transplants to patient navigators in the ICU.

Dr. Van Holden is an Associate Professor of  Medicine at the University of Maryland School of Medicine and the Pulmonary and Critical Care Fellowship Program director. Clinically, she specializes in interventional pulmonology. She is also an accomplished educator and is very active with the American Thoracic Society. She helped write the 2021 Critical Care Core Curriculum and helped coordinate the 2022 Resident Boot Camp.

Patient Presentation

A 26-year-old man presents to his primary care doctor with 1.5 months of intermittent dyspnea, cough, chest tightness, and fatigue. His dyspnea was initially exertional, and he noticed he could do less at the gym. However, in the past 3-4 weeks it has progressed to being even with mild movement. His brother was recently diagnosed and treated for acute bronchitis so he thought this could be similar. In the office, he is noted to be tachypneic with an oxygen saturation of 83% breathing ambient air. A chest X-ray is obtained and he is sent urgently to the emergency department.

Key Learning Points

**Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below

  1. Crazy Paving is a radiological term describing ground glass opacities with superimposed interlobular septal thickening. The differential diagnosis is broad and includes infectious, neoplastic, and autoimmune processes. It is not limited to just Pulmonary alveolar proteinosis (PAP) but is suggestive in an appropriate clinical setting.
  2. PAP is a disorder of surfactant production or clearance and its etiology is divided into three major subgroups. Primary or autoimmune; Secondary such as from toxic inhalations, hematological disorders, or medications; and Congenital
  3. PAP is diagnosed by positive Periodic acid-Schiff (PAS) staining of lipo-proteinaceous material in the distal bronchioles and alveoli on lung biopsy. The diagnosis can be made with PAS-positive BAL staining, but this has limited sensitivity and lung biopsy is necessary for the diagnosis in up to 30 – 35% of cases.
  4. It is important not to anchor on a diagnosis when a patient presents to you for re-evaluation even if seen by a prior expert. This was pivotal in this case!
  5. Please don’t put anything in your lung. Any toxic inhalation exposure could result in significant damage to lung parenchyma and morbidity as a result.

References and Further Reading

  1. Borie R, Danel C, Debray MP, et al. Pulmonary alveolar proteinosis. Eur Respir Rev. 2011;20(120):98-107. doi:10.1183/09059180.00001311
  2. Carey B, Trapnell BC. The molecular basis of pulmonary alveolar proteinosis. Clin Immunol. 2010;135(2):223-235. doi:10.1016/j.clim.2010.02.017
  3. Inoue Y, Trapnell BC, Tazawa R, et al. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Am J Respir Crit Care Med. 2008;177(7):752-762. doi:10.1164/rccm.200708-1271OC
  4. Kavuru MS, Malur A, Marshall I, et al. An open-label trial of rituximab therapy in pulmonary alveolar proteinosis. Eur Respir J. 2011;38(6):1361-1367. doi:10.1183/09031936.00197710
  5. Michaud G, Reddy C, Ernst A. Whole-lung lavage for pulmonary alveolar proteinosis. Chest. 2009;136(6):1678-1681. doi:10.1378/chest.09-2295
  6. Smith BB, Torres NE, Hyder JA, et al. Whole-lung Lavage and Pulmonary Alveolar Proteinosis: Review of Clinical and Patient-centered Outcomes. J Cardiothorac Vasc Anesth. 2019;33(9):2453-2461. doi:10.1053/j.jvca.2019.03.047
  7. Tazawa R, Ueda T, Abe M, et al. Inhaled GM-CSF for Pulmonary Alveolar Proteinosis. New England Journal of Medicine. 2019;381(10):923-932. doi:10.1056/NEJMoa1816216
  8. Tung AH, Grace J, O’Kane GM, Kumar K. Transbronchial lung biopsy (TBLB) in diagnosing pulmonary alveolar proteinosis (PAP): forgotten role in Australia? Respirology Case Reports. 2015;3(4):145-147. doi:10.1002/rcr2.129
  9. Werner AK, Koumans EH, Chatham-Stephens K, et al. Hospitalizations and Deaths Associated with EVALI. New England Journal of Medicine. 2020;382(17):1589-1598. doi:10.1056/NEJMoa1915314

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21. Post Intensive Care Syndrome (PICS)

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Today on Pulm PEEPs, we are joined by two pioneers in the field of post-intensive care outcomes and delirium research. Drs. Dale Needham and Wes Ely talk to us all about the Post Intensive Care Syndrome (PICS) and cover everything from how it was first recognized, to the impact it has, and, most importantly, what we can do to prevent it. This is a huge topic in the field of critical care and we’re thrilled to be delving into it with such knowledgeable guides.

Meet Our Guests

Wes Ely is the Grant W. Liddle Chair in Medicine and a Professor of Medicine at Vanderbilt University Medical Center. He is also the Associate Director of Aging Research at the VA Tennessee Valley Geriatric Research and Education Clinical Center and the co-director of the Critical, Illness, Brain Dysfunction and Survivorship Center. He has published 100s of manuscripts on critical illness survivorship and delirium. He also published a book called “Every Deep-Drawn Breath” about his and his patients’ experiences in the ICU and about the ramifications of critical illness. All net proceeds for the book are going to the CIBS Center Endowment for Survivorship

Dale Needham is a Professor of Medicine at Johns Hopkins, where he is also the Medical Director of the Critical Care Physical Medicine and Rehabilitation Program and the Director of the Outcomes After Critical Illness and Surgery Group. He is the author of 100s of publications focusing on post-ICU outcomes and has received numerous research grants from the NIH and other organizations.

Key Learning Points

Visit our website www.pulmpeeps.com to see the key learning points from this episode summarized in two infographics.

References and links for further reading

  1. Devlin JW, Skrobik Y, Gélinas C, et al. Executive Summary: Clinical Practice Guidelines for the Prevention and Management of Pain, Agitation/Sedation, Delirium, Immobility, and Sleep Disruption in Adult Patients in the ICU. Critical Care Medicine. 2018;46(9):1532-1548. doi:10.1097/CCM.0000000000003259
  2. Ely EW. The ABCDEF Bundle: Science and Philosophy of How ICU Liberation Serves Patients and Families. Crit Care Med. 2017;45(2):321-330. doi:10.1097/CCM.0000000000002175
  3. Mikkelsen ME, Still M, Anderson BJ, et al. Society of Critical Care Medicine’s International Consensus Conference on Prediction and Identification of Long-Term Impairments After Critical Illness. Crit Care Med. 2020;48(11):1670-1679. doi:10.1097/CCM.0000000000004586
  4. Needham DM, Sepulveda KA, Dinglas VD, et al. Core Outcome Measures for Clinical Research in Acute Respiratory Failure Survivors. An International Modified Delphi Consensus Study. Am J Respir Crit Care Med. 2017;196(9):1122-1130. doi:10.1164/rccm.201702-0372OC
  5. Needham DM, Wozniak AW, Hough CL, et al. Risk Factors for Physical Impairment after Acute Lung Injury in a National, Multicenter Study. Am J Respir Crit Care Med. 2014;189(10):1214-1224. doi:10.1164/rccm.201401-0158OC
  6. Semler MW, Bernard GR, Aaron SD, et al. Identifying Clinical Research Priorities in Adult Pulmonary and Critical Care. NHLBI Working Group Report. Am J Respir Crit Care Med. 2020;202(4):511-523. doi:10.1164/rccm.201908-1595WS
  7. Spruit MA, Holland AE, Singh SJ, Tonia T, Wilson KC, Troosters T. COVID-19: Interim Guidance on Rehabilitation in the Hospital and Post-Hospital Phase from a European Respiratory Society and American Thoracic Society-coordinated International Task Force. Eur Respir J. Published online August 13, 2020:2002197. doi:10.1183/13993003.02197-2020
  8. Turnbull AE, Sepulveda KA, Dinglas VD, Chessare CM, Bingham CO, Needham DM. Core Domains for Clinical Research in Acute Respiratory Failure Survivors: An International Modified Delphi Consensus Study. Crit Care Med. 2017;45(6):1001-1010. doi:10.1097/CCM.0000000000002435
  9. Ward DS, Absalom AR, Aitken LM, et al. Design of Clinical Trials Evaluating Sedation in Critically Ill Adults Undergoing Mechanical Ventilation: Recommendations From Sedation Consortium on Endpoints and Procedures for Treatment, Education, and Research (SCEPTER) Recommendation III. Crit Care Med. 2021;49(10):1684-1693. doi:10.1097/CCM.0000000000005049
  10. Ozga D, Krupa S, Witt P, Mędrzycka-Dąbrowska W. Nursing Interventions to Prevent Delirium in Critically Ill Patients in the Intensive Care Unit during the COVID19 Pandemic—Narrative Overview. Healthcare. 2020;8:578. doi:10.3390/healthcare8040578

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Radiology Rounds – 7/12/22

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Time for another #RadiologyRounds!! This week we’re looking at the coronal CT scan views, which can be extremely helpful and are often under-utilized. Follow us on Twitter to work through Radiology Rounds cases as they come out.

Our patient had an elevated VEGF-D level, a renal angiomyolipoma identified on CT abdomen, and imaging with diffuse cystic lung disease confirming her diagnosis of LAM. Make sure to check out the ICUOnePager made by Dr. Nick Mark.

20. Top Consults: Pulmonary Hypertension Diagnosis

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This week on Pulm PEEPs, we are continuing our Top Consults series with a discussion on the work-up and diagnosis of Pulmonary Hypertension. See our prior Radiology Rounds on signs of PAH on CT scan, and listen to our follow-up episode on right heart catheterizations for some background before this episode… or dive right in! We’ll cover everything from history and physical, to recent guideline changes in the definition of PH, and much, much more!

Meet Our Guests

Erika Berman Rosenzweig is a Professor of Pediatrics and the Director of the Pulmonary Hypertension Center and CTEPH Program at Columbia University Medical Center / New-York Presbyterian Hospital. She is an active member of the Pulmonary Hypertension Association, was the Editor-in-Chief of Advances in Pulmonary Hypertension and is on the Scientific Board of the World Symposium on PH.

Catherine Simpson is an Assistant Professor of Medicine at Johns Hopkins Hospital and is one of the faculty members in our Pulmonary Hypertension group. Her clinical and research areas of expertise are in pulmonary vascular disease and right heart function. Her research is focused on novel biomarker discovery and metabolomics in pulmonary vascular disease.

Cyrus Kholdani is an Instructor in Medicine at Beth Israel Deaconess Medical Center and Harvard Medical School. He is also the director of the Pulmonary Hypertension Program at BIDMC, and is actively involved in clinical care and clinical research in a variety of pulmonary vascular disease domains.

Consult Patient

Ms. Pamela Harris (PH) is a 47-year-old woman with PMH of migraines, obesity s/p gastric sleeve (BMI now 33), and a history of remote DVT in her 20s while on OCP s/p 6 months of AC who is referred to pulmonary hypertension clinic for evaluation of dyspnea on exertion. She has actually had dyspnea for some time and previously it has been attributed to her weight. Based on this, she pursued a gastric sleeve and has lost 55 pounds, but continues to have shortness of breath. She has no cough, and does not get dyspnea at rest, but notes that after 1 flight of stairs, or 2-3 blocks on flat ground she has shortness of breath. She saw her PCP and had basic labs, basic spirometry, and an echocardiogram. He did not note anything significant on examination in the notes.


The labs had no anemia, and normal renal and liver function. Her serum bicarbonate was 25 and there was no blood gas. Spirometry showed an FVC 82% predicted, FEV1 83% predicted, and FEV1/FVC was 99% predicted. The echocardiogram had normal LVEF, mild LVH, normal RV size and function qualitatively. There was mild TR with tricuspid valve peak regurgitant velocity of 3.4 m/sec. The estimated PASP + RA pressure (based on normal IVC diameter 2.1 cm) was 46 mmHg.

RHC: Systemic BPs 140s/90s, with O2 saturations 97-98% on RA throughout. RA mean pressure was 9, RV was 48 with an RVEDP of 17, PA was 48/27 with mean of 34, and PCWP mean was 11. CO/CI by Fick was 5.56 / 2.42, and by thermo was similar, 5.8 / 2.52. Her PA sat was 62%, and PVR was 3.97 WU.

Key Learning Points

History

  • Understand the constellation of symptoms and the functional limitation
    • The goal is to assign a WHO functional class by the end of the visit
  • Evaluate the time course and evolution of the symptoms
  • Concerning symptoms that need to be addressed
    • Palpitations
    • Pre-syncope
    • Syncope
    • Chest pain
    • LE edema
  • Evaluate for risk factors to explain or contribute to pulmonary hypertension
    • Signs or symptoms of OSA
    • Signs or symptoms of auto-immune disease
      • Raynauds
      • Skin changes
    • Family history
      • Heritable lung disease
      • Clotting disorders
      • Auto-immune disease
    • Social history
      • Exposure history
      • Smoking

Physical Exam

  • Look for signs that confirm PH
    • Loud P2
      • Accentuated with elevated PVR
      • Can hear pretty early on. Could be one of the earliest findings
    • TR murmur – pansystolic murmur at RUSB
    • Diastolic murmur if severe pulmonary insufficiency
  • Look for signs of right heart failure
    • JVD
    • S4 gallop – later in course
    • RV heave – later in course
    • Peripheral edema
    • Pulsatile liver or hepatosplenomegaly
  • Look for signs of other secondary causes of PH
    • Mitral regurgitation or aortic stenosis murmur
    • Asymmetric lower extremity edema
    • Pulmonary edema
    • Skin findings concerning for auto-immune disease or liver disease
    • Arthritis

Work up for etiology of PH

  • CBC with diff – myeloproliferative and hemolytic anemia
  • CMP – renal function, liver function
  • Serologies – lupus, scleroderma, vasculitis – broad evaluation
  • HIV, hepatitis
  • Liver duplex if concerned
  • ECHO with bubble
  • Consider cardiac MRI
  • History of toxin and anorexigenic use
  • CT scan of the chest
  • PFTs including lung volumes and DLCO to evaluate for lung disease
  • Pulse oximetry at rest and with exercise
  • A sleep study or nocturnal oximetry
  • V/Q scan for all patients

References and links for further reading

  1. Bonno EL, Viray MC, Jackson GR, Houston BA, Tedford RJ. Modern Right Heart Catheterization: Beyond Simple Hemodynamics. Advances in Pulmonary Hypertension. 2020;19(1):6-15. doi:10.21693/1933-088X-19.1.6
  2. Augustine DX, Coates-Bradshaw LD, Willis J, et al. Echocardiographic assessment of pulmonary hypertension: a guideline protocol from the British Society of Echocardiography. Echo Res Pract. 2018;5(3):G11-G24. doi:10.1530/ERP-17-0071
  3. Callan P, Clark AL. Right heart catheterisation: indications and interpretation. Heart. 2016;102(2):147-157. doi:10.1136/heartjnl-2015-307786
  4. Chokkalingam Mani B, Chaudhari SS. Right Heart Cardiac Catheterization. In: StatPearls. StatPearls Publishing; 2022. Accessed April 18, 2022. http://www.ncbi.nlm.nih.gov/books/NBK557404/
  5. D’Alto M, Dimopoulos K, Coghlan JG, Kovacs G, Rosenkranz S, Naeije R. Right Heart Catheterization for the Diagnosis of Pulmonary Hypertension: Controversies and Practical Issues. Heart Failure Clinics. 2018;14(3):467-477. doi:10.1016/j.hfc.2018.03.011
  6. Galiè N, McLaughlin VV, Rubin LJ, Simonneau G. An overview of the 6th World Symposium on Pulmonary Hypertension. European Respiratory Journal. 2019;53(1). doi:10.1183/13993003.02148-2018
  7. Rosenkranz S, Preston IR. Right heart catheterisation: best practice and pitfalls in pulmonary hypertension. European Respiratory Review. 2015;24(138):642-652. doi:10.1183/16000617.0062-2015

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19. Severe COPD and Lung Volume Reduction

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We are extremely excited for the third and final installment in our Pulm PEEPs and ATS Clinical Problems Assembly collaborative series on COPD. Today, we are joined by Drs. Jessica Bon, Michael Lester, and Niru Putcha to discuss severe COPD management and the role of lung volume reduction procedures. If you missed the first two parts of our series, make sure to check out episode 1 on COPD diagnosis and initial management, and episode 2 on COPD exacerbations.

Meet our Guests

Jessica Bon is an Associate Professor of Medicine at the University of Pittsburgh School of Medicine where she is also the Program Director for the Pulmonary and Critical Care Medicine Fellowship. Her research and clinical interests focus on lung disease progression in COPD and she manages patients with difficult-to-treat and severe COPD and evaluates patients for lung volume reduction surgery. Jessica was the chair of the ATS Clinical Problems Assembly Programming Committee from 2021 – 2022.

Michael Lester is an Assistant Professor of Medicine at Vanderbilt University Medical Center. Michael’s interests span both pulmonary and critical care medicine. He specializes in patients with advanced COPD and evaluation for bronchoscopic lung volume reduction surgery.

Niru Putcha is an Associate Professor of Medicine at Johns Hopkins School of Medicine and is an integral member and mentor in the Obstructive Lung Disease Group. Her research and clinical interests focus on the role of comorbidities on clinical outcomes in individuals with COPD. She also manages patients with difficult-to-treat and severe COPD and evaluates patients for lung volume reduction surgery. Niru is also the new chair of the ATS  Clinical Problems Assembly Programming Committee.

Key Learning Points

Patients with advanced COPD should also be considered for lung transplantation. We will have an episode on lung transplant coming up soon!

References

  1. Criner GJ, Sternberg AL. A Clinician’s Guide to the Use of Lung Volume Reduction Surgery. Proc Am Thorac Soc. 2008;5(4):461-467. doi:10.1513/pats.200709-151ET
  2. A Randomized Trial Comparing Lung-Volume–Reduction Surgery with Medical Therapy for Severe Emphysema. New England Journal of Medicine. 2003;348(21):2059-2073. doi:10.1056/NEJMoa030287
  3. Valipour A, Slebos DJ, Herth F, et al. Endobronchial Valve Therapy in Patients with Homogeneous Emphysema. Results from the IMPACT Study. Am J Respir Crit Care Med. 2016;194(9):1073-1082. doi:10.1164/rccm.201607-1383OC
  4. Sciurba FC, Ernst A, Herth FJF, et al. A Randomized Study of Endobronchial Valves for Advanced Emphysema. New England Journal of Medicine. 2010;363(13):1233-1244. doi:10.1056/NEJMoa0900928
  5. Klooster K, Slebos DJ. Endobronchial Valves for the Treatment of Advanced Emphysema. Chest. 2021;159(5):1833-1842. doi:10.1016/j.chest.2020.12.007
  6. Choi M, Lee WS, Lee M, et al. Effectiveness of bronchoscopic lung volume reduction using unilateral endobronchial valve: a systematic review and meta-analysis. Int J Chron Obstruct Pulmon Dis. 2015;10:703-710. doi:10.2147/COPD.S75314

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Radiology Rounds – 6/14/22

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This week on #RadiologyRounds we are extremely excited to share a case brought to you by one of our new Associate Editors, Leon Mirson! Enjoy, and follow us on Twitter and Instagram for content delivered to you weekly!

What abnormalities do you see on this CXR to help explain the patient’s presentation?

The CT scan has reticular changes consistent with interstitial lung disease and there are multiple features that help us define the pattern of the ILD. His CT notably has very few ground-glass opacities, there is traction bronchiectasis, and honeycombing with a basilar and peripheral / sub-pleural predominance.

Taking all these features together, the patient’s radiographic presentation is consistent with Usual Interstitial Pneumonia (UIP)

This patient had a thorough history taken and he had no prior smoking and no occupational or environmental exposures of significance. He had no family history of interstitial lung disease. A broad history was taken regarding symptoms of connective tissue disease and a broad serologic workup was sent, all of which were unremarkable. What would you want to do next diagnostically?

If you want to learn more about diagnosing interstitial lung disease, listen to our prior Top Consults episode on diagnosing ILD with experts in the field and see these prior #RadiologyRounds on Fibrotic NSIP and Sarcoidosis.

18. A Case of Severe Weakness in the ICU

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We are thrilled here @PulmPEEPS to have our first episode with one of our new Associate Editors Luke Hedrick, and our first nephrology consultant Jeff William. Luke will walk us through an interesting case presentation, and we will discuss an approach to severe weakness in our patient in the ICU.

Meet Our Guests

Jeff William is an Assistant Professor of Medicine at Harvard Medical School and Beth Israel Deaconess Medical Center, where he is also the Associate Director of the Nephrology Fellowship Program. He completed a Medical Education Research Fellowship at Harvard Medical School, and is very involved in residency, fellowship and medical student education.

Patient Presentation

We have a man in his 40s with a past medical history of asthma, hypertension, and acid reflux who was brought in by EMS with back pain and profound proximal lower extremity weakness. He reports mild weakness in his legs which started 2 days ago, but this morning his weakness acutely worsened to the point that he can’t lift his legs out of the bed. He also has some cramping pain in his thighs. He additionally has had mild shortness of breath and yesterday went to an urgent care where he was given steroids and swabbed for COVID (which was negative).

Key Learning Points

**Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below

Although our patient’s etiology of severe hypokalemia was thought to be secondary to thiazide diuretic use, it is important to be familiar with hypokalemic periodic paralysis.

References

  1. Knochel JP, Schlein EM. On the mechanism of rhabdomyolysis in potassium depletion. J Clin Invest. 1972 Jul;51(7):1750-8. doi: 10.1172/JCI106976.
  2. Wang X, Han D, Li G. Electrocardiographic manifestations in severe hypokalemia. J Int Med Res. 2020 Jan;48(1):300060518811058. doi: 10.1177/0300060518811058.
  3. Venance SL, Cannon SC, Fialho D, Fontaine B, Hanna MG, Ptacek LJ, Tristani-Firouzi M, Tawil R, Griggs RC; CINCH investigators. The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain. 2006 Jan;129(Pt 1):8-17. doi: 10.1093/brain/awh639.
  4. Lin SH, Lin YF, Halperin ML. Hypokalaemia and paralysis. QJM. 2001 Mar;94(3):133-9. doi: 10.1093/qjmed/94.3.133. 
  5. Lin SH, Lin YF, Chen DT, Chu P, Hsu CW, Halperin ML. Laboratory tests to determine the cause of hypokalemia and paralysis. Arch Intern Med. 2004 Jul 26;164(14):1561-6. doi: 10.1001/archinte.164.14.1561.

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Radiology Rounds – 5/31/22

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We are excited to bring you another #RadiologyRounds which applies some of the knowledge from our most recent episode on pneumothorax.

She is presenting with a 1.5 cm left pneumothorax. You can see lucency representing air in the pleural space. There are a lack of blood vessels or lung markings extending to the periphery and you can see the visceral pleura.

She is presenting with her first pneumothorax which is a small, spontaneous pneumothorax secondary to her underlying cystic lung disease. She was managed conservatively and followed closely outpatient with ultimate resolution of her pneumothorax.