This review article summarizes the main treatments for chronic obstructive pulmonary disease, their mechanisms, and the key evidence from trials supporting their use. Drug classes covered were short acting beta agonists (SABA), short acting muscarinic antagonists (SAMA), long acting beta agonists (LABA), long acting antimuscarinics (LAMA), inhaled corticosteroids (ICS), LABA/ICS combinations, specific phosphodiesterase (PDE4) inhibitors, non-specific PDE inhibitors, mucolytics, and oxygen. Non-specific therapies, such as opiates for relief of dyspnoea and therapies for smoking cessation, are also covered briefly. For each class of drug, mechanisms of action are described, key clinical trial results are reported, and available agents compared. Finally, the place of each drug in therapy is compared between current worldwide guidelines.

History: A 32-year-old woman was admitted to the emergency department because of acute dyspnea and syncope. A few minutes before the onset of symptoms, she had self-administered an intravenous injection of one gram of heroin combined with grinded flunitrazepam tablets.Investigations: Signs of acute cor pulmonale were detected on transthoracic echocardiography despite lack of pulmonary embolism in computed tomography. It was assumed that microembolisms were the cause of acute pulmonary hypertension after intravenous injection of heroin and flunitrazepam.Treatment and course: Because of lack of thrombus in CT scan therapeutic anticoagulation with unfractionated heparin and oxygen insufflation was initiated resulting in rapid improvement of oxygen saturation and blood pressure. On the following day pulmonary pressure in transthoracic echocardiography was already decreased significantly. Without signs of deep venous thrombosis in duplex scan and only a marginal sub segmental perfusion deficit in ventilation-perfusion-scintigraphy therapeutic anticoagulation was recommended for three months.

Conclusion:

The most likely cause of micro embolisms in this case are particles of talc, which are often used to cut heroin, or the microcrystalline cellulose used in tablets. There have been reports of tissue necrosis due to arterial embolism/vasospasm by crystalloid or oily substances (embolia cutis medicamentosa) in the extremities after intraarterial injection of grinded flunitrazepam tablets. Therefore it seems plausible that intravenous application may cause a serve but transient deficit of perfusion in pulmonary circulation.

SESSION TYPE: Pleural Global Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Hemoptysis complicating paracentesis had been described with dry tap, we were able to find one case report of thoracentesis leading to fatal massive hemoptysis in the literature. The hemoptysis is usually caused by puncture of the lungs and puncture sites had been found on postmortem exam, it is unusual to get this complication with successful removal of pleural fluid when the lung is atelectatic.

CASE PRESENTATION:

79 YEAR OLD FEMALE WITH STAGE 4 LUNG CANCER NSCLCA WITH MALIGNANT PLEURAL EFFUSION, PRESENTED WITH DYSPNEA AND HYPOXEMIA AND ACCUMILATION OF RIGHT SIDED MASSIVE PLEURAL EFFUSION LEADING TO ATELECTASIS OF THE LEFT LUNG is 79-year-old female with stage IV non-small cell lung CA bronchogenic carcinoma with malignant pleural effusion, presented with dyspnea and hypoxemia, chest x-ray showed massive right-sided pleural effusion, with complete right lung atelectasis, the patient had mild episodes of hemoptysis which was attributed to the bronchogenic carcinoma, the patient was hemodynamically stable, nevertheless, her breathing was labored, and needed 60% oxygen supplement by Venturi mask, on a lateral decubitus chest x-ray film, the pleural fluid was freely floating in the pleural space, the patient underwent right thoracentesis with removal of 1000 cc of serous sanguinous pleural fluid, there was no blood through the needle upon entering the pleural space, the patient's dyspnea improved towards the end of the thoracentesis procedure, and the patient lied in bed comfortably, 5 minutes following the procedure the patient started coughing with large amounts of blood rushing through the airways, attempts to intubate the patient failed secondary to obscuring of the airways by the massive hemoptysis, the patient desaturated quickly, became bradycardic, and developed asystolic cardiac arrest, the family elected not to pursue CPR, patient expired.

DISCUSSION:

THIS UNEXPECTED COMPLICATION LEAD TO THE THINKING THAT LARGE VOLUME THORACENTESIS FOR MASSIVE PLEURAL EFFUSION MAY LEAD TO COMPLICATIONS RELATED TO THE REEXPANSION OF THE ATELECTATIC LUNG, WHICH IS NOT LIMITED TO REEXPANSION PULMONARY EDEMA . The mechanism of hemoptysis in this patient was thought not to be secondary to needle puncture of the lungs, rather, relieving a tympanotomy and pressure exerted by the malignant massive pleural effusion on the bronchogenic carcinoma, once the tympanotomy and pressure was relieved by thoracentesis, hemoptysis occurred. There had been a description of one case in the literature where the patient developed massive fatal hemoptysis. 3 minutes following a dry pleural tap. The cause of hemoptysis was described as direct puncture of the lungs, and puncture sites were found on the posterior surface of the lungs on the postmortem examination, in our case, there was massive pleural effusion, which provided a good distance between the needle and the lungs making the lung. Posterior much less likely, and our tap was not dry, on the contrary, large volume of fluid was removed with improvement of the patient's sense of dyspnea, since the patient did not get the postmortem exam is very difficult to determine whether the mechanism postulated of the sudden release of tympanotomy and pressure exerted by me pleural effusion had led to the decompression of the Brooklyn genic carcinoma, leading to the massive hemoptysis

CONCLUSIONS:

ALTHOUGH THIS IS AN UNUSUAL PRESENTATION AND COMPLICATION, ONME CAN LEARN TO BE CAREFUL WITH LARGE VOLUME THORACENTESIS IN A PATIENT WITH MALIGNANTPLEURAL EFFUSION AND HISTORY OF HEMOPTYSIS although this is an unusual presentation and complication of large-volume thoracentesis and malignant pleural effusions, one can learn to be more careful with the removal of pleural fluid and a patient with endobronchial extension of malignant neoplasms who had history of hemoptysis, insertion of the pigtail tube, and gradual removal of fluid may be preferred to large-volume thoracentesis in such patients, more research is needed to confirm this hypothesis.1) Seneff MG, Corwin RW, Gold LH, Irwin RS: Complications associated with thoracocentesis. Chest 1986; 90:97-1002) Virshup B, Coombs RH: Physicians' adjustment to retirement. West J Med 1993; 158: 142-1443) Collins TR, Sahn SA: Thoracocentesis: Clinical value, complications, technical problems, and patient experience. Chest 1987; 91:817-822DISCLOSURE: The following authors have nothing to disclose: Islam IbrahimNo Product/Research Disclosure InformationIMC, Jeddah, Saudi Arabia.

SESSION TYPE: Infectious Disease Student/Resident Case Report Posters IPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Disseminated histoplasmosis can rarely be found in patients without known underlying immunodeficiency.

CASE PRESENTATION:

58 year-old woman presented for evaluation of progressive dyspnea with non-productive cough and generalized weakness for eight months. She also reported intermittent fever, pleuritic chest pain, dysphagia, and easy bruisability. Her past medical history was unremarkable. She was not taking immunosuppressive medications. She did not have sick contacts nor did she have outdoor activities such as camping or travelled recently. At presentation, she was febrile, tachycardic, tachypneic, and hypotensive. She had diffuse crackles on lung auscultation. Initial laboratory tests showed hemoglobin of 9.1 g/dL, platelet count of 82 x 10^9/L, and WBC count of 3.9 x10^9 /L with 4% lymphocytes and CD4 count of 78. High resolution chest CT revealed bilateral nodular and ground-glass peribronchiolar infiltrates, cavitary lesion in left upper lobe, hilar and mediastinal adenopathy. She was admitted and empiric antibacterial therapy was initiated. Viral serology for HIV, EBV, and Influenza, RSV and Parvovirus PCR, urine Legionella antigens, Mycobacterium TB QuantiFERON were negative. Fungal serology was positive for histoplasma. The patient's hypoxic respiratory failure worsened and required intubation. Bronchoscopy with bronchoalveolar lavage was negative for malignancy, but positive for both Histoplasma and Pneumocystis by PCR. Blood cultures grew Histoplasma capsulatum. Urine histoplasma antigen was detected at high levels. Bone marrow biopsy was performed and extensive involvement by histoplasma was determined to be the cause of her pancytopenia. Antimicrobial therapy was changed to amphotericin B and sulfamethoxazole/trimethoprim along with methylprednisolone for treatment of disseminated histoplasmosis and Pneumocystis pneumonia. She improved significantly shortly after initiation of appropriate treatment. She tolerated extubation and ultimately was discharged from the hospital in good condition.

DISCUSSION:

The presented patient did not have history of immunosuppression. She underwent extensive negative evaluation by immunologists for underlying immunosuppressive disorders such as cellular or humoral immunodeficiencies. Nevertheless, she developed severe disseminated histoplasmosis with bone-marrow involvement. This caused pancytopenia with a low CD4 count which ultimately predisposed her to pneumocystis infection. This case is an example of disseminated histoplasmosis with subsequent co-infections in apparently immunocompetent host.

CONCLUSIONS:

Histoplasmosis should be in the differential diagnosis when a patient presents with pulmonary infiltrates, adenopathy, cytopenia, even when he/she is not from the Mississippi and Ohio River valleys.1) Disseminated histoplasmosis: clinical and pathologic correlations. Goodwin RA Jr, Shapiro JL, Thurman GH, Thurman SS, Des Prez RM. Medicine (Baltimore). 1980;59(1):1DISCLOSURE: The following authors have nothing to disclose: Benyam Addissie, Joseph SkalskiNo Product/Research Disclosure InformationMayo Clinic, Rochester, MN.

SESSION TYPE: ILD Student/Resident Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Hypersensitivity pneumonitis (HP) is a known cause of non-caseating granuloma. Without a thorough history, diagnosis can be missed. Since exposure elimination can be curative, diagnosis is crucial. We present three initially misdiagnosed patients with different lung disorders, but based on environmental history and elevated antigen-specific antibodies, were found to have HP.

CASE PRESENTATION:

A 58-year-old female presented with cough but subsequently developed progressive dyspnea with pulmonary infiltrates, not resolved after multiple antibiotics. Evaluation revealed carpal tunnel, Raynaud's, positive rheumatoid factor and high sedimentation rate. Initially diagnosed as scleroderma lung disease, although autoimmune work up was negative. Lung biopsy showed scattered, peribronchial, poorly formed granulomas, lymphocytic fibrosis, and eosinophils. Further questioning revealed that, dyspnea began after the patient moved to a new workplace. HP antibody panel was significantly positive for A.fumigatus precipitins, supporting HP. 44-year-old woman presented with cough for two months. Cough and rhinoconjunctivitis began shortly after cleaning her backyard. Imaging revealed bilateral hilar lymphadenopathy and lymph node biopsy revealed non-necrotizing granulomas with eosinophils. She was treated for sarcoidosis with steroids and improved without further treatment. However, autoimmune markers were never elevated. Further evaluation revealed positive hypersensitivity screen for Thermoactinomyces vulgaris IgG. Avoiding her backyard, she has remained asymptomatic, and her infiltrates/lymphadenopathy have resolved. 50-year-old male living in a damp basement presented with asthma, positive skin test to mold allergens, pulmonary infiltrates, and negative hypersensitivity screen and was originally diagnosed and treated as allergic bronchopulmonary aspergillosis. Chest CT revealed nodules bilaterally. Nodules, septal perforation and positive P-ANCA led to diagnosis of vasculitis. Nasal biopsy however revealed only chronic inflammation without evidence of vasculitis or granulomas. Lung biopsy revealed non-necrotizing granulomas, which the pathologist felt to be consistent with HP. The patient moved out of the moldy basement and not only clinically improved, but the p-ANCA became negative.

DISCUSSION:

These initially misdiagnosed cases demonstrate unusual presentations of HP. Two patients had abundant eosinophils in their granulomas not commonly seen with HP. Negative hypersensitivity screen in the third patient does not exclude HP in the setting of strong history of exposure, radiologic and histologic findings, and improvement after removal of the offending agent.

CONCLUSIONS:

High level of suspicion and a thorough environmental history are crucial in diagnosing HP.1) Hypersensitivity Pneumonitis: A Historical, Clinical, and Radiologic Review-Jan V. Hirschmann, MD, Sudhakar N. J. Pipavath, MBBS and J. David Godwin, MDDISCLOSURE: The following authors have nothing to disclose: Lahari Rampur, Shravan Kooragayalu, Purvi Parikh, Sunit Jariwala, Golda Hudes, D. RoseinstreichNo Product/Research Disclosure InformationAlbert Einstein College of Medicine/Montefiore Medical Center, Bronx, NY.

SESSION TYPE: Miscellaneous Case Report Posters IIPRESENTED ON: Tuesday, October 23, 201 at 01:30 PM - 02:30 PM

INTRODUCTION:

Patients with sarcoidosis are susceptible to the development of various manifestations of infiltrative disease including cardiac involvement. Cardiac manifestations range from conduction system aberrancies to myocardial infiltration. Pericardial manifestations are rarely described.

CASE PRESENTATION:

A 68 yo man with multi-organ sarcoidosis and diabetes mellitus presented with complaints of 4 days of worsening dyspnea and subjective fevers. The patient was on outpatient corticosteroid therapy and infliximab with recently negative fungal and tubercular studies. He was in his usual state of health 2 weeks before admission when his outpatient pulmonologist initiated a corticosteroid taper. Admission radiograph demonstrated increase in chronic interstitial changes. EKG was consistent with baseline studies including normal intervals and ST segments. Exam revealed mild hypoxemia, relative hypotension, coarse pulmonary crackles, distant heart sounds and 1+ bilateral lower extremity edema. VQ scan showed low probability of pulmonary embolism. Transthoracic echocardiogram showed normal systolic function, PA systolic pressure of 61mmHg, a moderate pericardial effusion composed primarily of peri-right ventricular fibrinous organized material and a small focal mass attached to the LV surface without evidence of collapse in any chamber. Right heart catheterization demonstrated CVP of 15 mmHg, PCWP of 8 mmHg, PA pressure of 40/20 mmHg and systemic BP of 185/101 mmHg. The patient was given corticosteroids, antibiotics and diuresis. On day 4, he became hypercapnic and altered, prompting initiation of mechanical ventilation. Bronchoalveolar lavage cultures grew Enterobacter cloacae. Therapy continued with significant clinical improvement. Cardiac MRI demonstrated an abnormal focus of enhancement within the inferior LV wall consistent with infiltrative disease. The previously noted effusion was not visualized. The patient continued on corticosteroids and was discharged at baseline level of cardiopulmonary functionality.

DISCUSSION:

The finding of a fibrinous pericardial mass may be an atypical cardiac manifestation of sarcoidosis but did not appear to have clinical significance in this patient's presentation.

CONCLUSIONS:

This case illustrates the importance of using the preponderance of data in clinical decision making. Although the patient had a fibrinous mass on echocardiogram, his symptomatology primarily stemmed from pneumonia and sepsis in the setting of immunosuppresion.1) Uma S. Ayyala, Ajith P Nair, Maria L Padilla. Cardiac Sarcoidosis. Clin Chest Med 29 (2008) 493-508.DISCLOSURE: The following authors have nothing to disclose: Patrick Aguilar, Karla Diaz, Diego Maselli, Adriel Malave, Marcos RestrepoNo Product/Research Disclosure InformationUniversity of Texas Health Science Center San Antonio, San Antonio, TX.

SESSION TYPE: Bronchology Student/Resident Case Report PostersPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Thoracic tumors are a common cause of SVC syndrome. The mass can cause complete or partial obstruction of the vessel lumen. The most common malignancies known to cause this syndrome are of bronchogenic origin. Because of the distension and back pressure caused by the tumor occluding the superior vena cava, bleeding is a feared and known complication. There has been no publication that we are aware of looking at EBUS for diagnosis of this condition.

CASE PRESENTATION:

72 year old woman with a past medical history of hypertension presented with cough, facial edema, and dyspnea to our emergency department. Patient had an extensive smoking history, more than 100pkyrs, and quit 6months ago. She also reported anorexia and weight loss. Chest radiography revealed tracheal deviation to the left and mediastinal fullness. Computerized Tomography of the chest was positive for a mediastinal mass invading the proximal part of the superior vena cava ( Fig 1), and the clinical diagnosis of SVC syndrome was made. Patient was treated with steroids. An EBUS guided needle aspiration was performed along the right paratracheal and subcarinal lymph nodes region ( Fig 2). Rapid on site evaluation by a pathologist was diagnostic for non-small cell malignancy, later confirmed as adenocarcinoma of primary pulmonary origin. Radiation therapy was instituted and the patient was discharged to home with scheduled follow-up visits. No complications were noted with the procedure.

DISCUSSION:

We illustrate the safety of EBUS in a case of SVC syndrome. Prior studies were conducted on safety of blind transbronchial needle biopsies in the diagnosis of SVC syndrome with rapid on site evaluation of pathological specimen (Brundyn et al). The use of EBUS while performing the transbronchial biopsy has a dual benefit. The vessels can be identified and avoided during the biopsy procedure increasing safety, and a suitable area of the mass identified increasing yield.

CONCLUSIONS:

EBUS can be performed safely in a patient with SVC syndrome, and has the potential to increase safety and yield compared to other diagnostic procedures.1) Brundyn K, Koegelenberg CF, Diacon AH, et al.Transbronchial fine needle aspiration biopsy and rapid on-site evaluation in the setting of superior vena cava syndrome Diagn Cytopathol. 2011 Nov 18.DISCLOSURE: The following authors have nothing to disclose: Bala Prakash, Shweta Upadhyay, Veronica Brito, Shalinee Chawla, Jonathan IlowiteNo Product/Research Disclosure InformationWinthrop University Hospital, Mineola, NY.

SESSION TYPE: Pleural Cases IIPRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM

INTRODUCTION:

Pleuroscopy is a minimally invasive procedure done under conscious sedation to visualize and biopsy the pleura. We present the case of a patient diagnosed by semi-rigid pleuroscopy with malignant mesothelioma in which the biopsy was performed through the novel use of a basket retrieval device typically used to remove foreign bodies within the gastrointestinal or respiratory tracts.

CASE PRESENTATION:

Our patient was a 79 year old man who presented with gradually progressive dyspnea. He was a former smoker and had a history of asbestos exposure. Chest x-ray revealed a massive unilateral pleural effusion. Ultrasound showed free flowing fluid and multiple pleural based polypoid masses. Large volume thoracentesis demonstrated a lymphocytic exudate. No malignant cells were found. As the clinicoradiographic appearance was concerning for maliganancy, semi-rigid pleuroscopy was performed. The parietal pleura and diaphragm were noted to be studded with polypoid tumors that were soft, fragile, and difficult to grasp. Using an endoscopic basket retrieval device we were able to ensnare and remove a 1.5 cm tumor en bloc for histopathologic evaluation. The patient was ultimately diagnosed with malignant mesothelioma.

DISCUSSION:

When we inspected the pleura, we noted very soft and fragile polypoid masses. Due to the consistency, the standard flexible biopsy forceps could only remove small fragments of tissue. Smaller fragments of tissue in pleural tumors are often non-diagnostic. A major criticism of the single port semi-rigid pleuroscopy is that biopsy samples are smaller than in rigid thoracoscopic biopsies. The basket retrieval device allowed us to obtain very large biopsy specimens that were comparable to rigid techniques yet still used the semi-rigid pleuroscopy technique.

CONCLUSIONS:

This case illustrates the novel use of the endoscopic basket retrieval device within the pleural space to overcome some of the inherent limitations of semirigid pleuroscopic biopsies and increase the yield for diagnosis with this minimally invasive technique. Understanding the technical limitations of our newer minimally invasive procedures allows us to apply novel approaches to overcome these limitations and ultimately improves patient care.1) Asge Technology Committee, Diehl DL, Adler DG, Conway JD, Farraye FA, Kantsevoy SV, Kaul V, Kethu SR,Kwon RS, Mamula P, Rodriguez SA, Tierney WM. Endoscopic retrieval devices. Gastrointest Endosc. 2009 May;69(6):997-1003.2) Michaud G, Berkowitz DM, Ernst A. Pleuroscopy for diagnosis and therapy for pleural effusions. Chest. 2010 Nov;138(5):1242-6.3) Tassi GF, Davies RJ, Noppen M. Advanced techniques in medical thoracoscopy. Eur Respir J. 2006 Nov;28(5):1051-9.DISCLOSURE: The following authors have nothing to disclose: Andrew Philip, Scott Parrish, Robert BrowningNo Product/Research Disclosure InformationWalter Reed National Military Medical Center, Bethesda, MD.

SESSION TYPE: Diagnostic BronchoscopyPRESENTED ON: Tuesday, October 23, 2012 at 04:30 PM - 05:45 PM

PURPOSE:

Tracheobronchopathia Osteochondroplastica (TBPOCP), has been depicted as a rare disease with an elusive pathology. Given the paucity of published data on the natural history of the disease, there was a compelling need for further elucidation of its underlying demographic trends, clinical features, diagnostic and histological findings, and treatment approaches. In response, an extensive search of the literature was performed.

METHODS:

98 case reports involving 331 cases of TBPOCP, from 1947 through 2012, were identified via MEDLINE and EMBASE. Variables including patient data, disease presentation, diagnostic modalities and treatment approaches were extracted. Multivariate regression was employed to explore relationships of interest.

RESULTS:

TBPOCP affects predominantly middle-aged males. The most common disease manifestation involves recurrent cough, generally lasting for several months. Of note, those presenting with hemoptysis were less likely to see disease progression than those presenting with dyspnea or stridor. The majority of afflicted patients are non-smokers and there doesn't appear to be any hereditary component. The most common predisposing risk factor is asthma or chronic obstructive pulmonary disease (COPD) - no association with infectious process, prior malignancy or autoimmune disease was evident. Of note, individuals presenting with TBPOCP as well as COPD/asthma are more likely to see disease stabilization with surgical treatment of TBCOP than those TBPOCP patients without COPD/asthma (suggesting TBPOCP may not be caused by COPD/asthma - rather, it may actually be causing a form of obstructive pulmonary disease). Multivariate linear regression indicates nodular size is inversely correlated with %FEV. Multivariate logistic regression indicates disease resolution is positively correlated with age and whether the patient has underlying risk factors for TBPOCP that are modifiable or preventable.

CONCLUSIONS:

The above findings serve to further augment our understanding of this complex disease and help improve our diagnostic and treatment capabilities.

CLINICAL IMPLICATIONS:

This is the first study to rigorously define the underlying patient population of TBPOCP as well as identify the potential risk factors associated with TBPOCP presentation and progression.DISCLOSURE: The following authors have nothing to disclose: Abubakr Chaudhry, Elie Donath, Atul MehtaNo Product/Research Disclosure InformationUniversity of Miami Miller School of Medicine, Palm Beach Regional Campus, West Palm Beach, FL.

SESSION TYPE: Miscellaneous Case Report Posters IIPRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION:

Complete unilateral pulmonary agenesis is an extremely rare congenital condition, characterized by complete absence of bronchi, parenchyma, vessels and pleural cavity in the affected lung. Fifty percent of infants born with pulmonary aplasia are stillborn or die within the first five years of life. We present a patient with right pulmonary agenesis complicated by ASD that has survived to adulthood.

CASE PRESENTATION:

A 21 year-old female presents with new onset of hypoxic respiratory failure and dyspnea after diagnosis of community-acquired pneumonia. She has a right pulmonary agenesis, dextrocardia and ASD. Initial diagnosis in the first week of life and ASD surgical repair at 7 months of age, complicated with chronic hypoxic respiratory failure and tracheostomy till age of 5. She also has severe pulmonary hypertension. She was lost to follow up until the current presentation. She was not on supplemental oxygen until current hospitalization. Community-acquired pneumonia treated with Moxifloxacin 10 days course, discharged on 6 L/min of oxygen at rest. Physical exam: BMI 45, vesicular breath sounds over left chest and transmitted breath sounds over right chest, normal S1 and loud S2 over right hemithorax. Echocardiogram: dextrocardia, estimated RV pressure of 89 mmHg with moderate mitral regurgitation. PFT shows obstruction with FEV1/FVC 68%, FEV1 0.81 L (28.7%) and FVC 1.2 L (37.9 %). Chest roenterogram: homogenously opaque right hemithorax with mediastinal shift and hyperlucency of left lung field. CT chest: left lung emphysema, right mediastinal shift, right lung agenesis and heart in right hemithorax.

DISCUSSION:

There is no specific therapy for pulmonary agenesis. We took a symptomatic approach to therapy, treating her obstructive lung disease with Albuterol, Tiotropium, and Mometasone, pulmonary rehabilitation with improved functional status. Planned left and right cardiac catheterization to evaluate for any remediable anatomic cardiovascular problems that may not be evident by ECHO but may contribute to pulmonary hypertension and assess responsiveness to pulmonary vasodilators.

CONCLUSIONS:

Lung aplasia is often associated with acute respiratory distress and a high mortality rate early in life. This case highlights the fact that children with complicated congenital pulmonary diseases are increasingly living into adulthood. As adult pulmonologists, we must be more aware of these diseases and their pathophysiological changes to be able to adequately diagnose and treat our patients.1) Skandalakis JE, Gray SW, Symbas P: The trachea and the lungs. In: Skandalakis JE, Gray SW, editors. Embryology for Surgeons. 2nd ed. Baltimore, MD: Williams and Wilkins; 1994. p 429-32.2) Krivchenya DU, Rudenko EO, Lysak SV, Dubrovin AG, Khursin VN, Krivchenya TD. Lung aplasia: Anatomy, history, diagnosis and surgical management. Eur J Pediatr Surg 2007;17:244-50.3) Kumar B, Kandpal DK, Sharma C, Sinha DD. Right lung agenesis. Afr J Paediatr Surg 2008;5:102-4DISCLOSURE: The following authors have nothing to disclose: Olena Lineberry, P.Jim Murphy, Craig Piquette, Kristina BaileyNo Product/Research Disclosure InformationUNMC, Omaha, NE.