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      Vanishing Lung Syndrome, or Idiopathic Giant Bullous Emphysema, with Pneumothorax, and Subcutaneous Emphysema in a 58-Year-Old Female Smoker with Chronic Obstructive Pulmonary Disease

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          Abstract

          Patient: Female, 58-year-old

          Final Diagnosis: Giant bullae • spontaneous pneumothorax • subcutaneous emphysema

          Symptoms: Crepitus • dysphagia • dysphonia • facial swelling

          Medication: —

          Clinical Procedure: —

          Specialty: Critical Care Medicine • Pulmonology

          Objective:

          Unusual clinical course

          Background:

          Vanishing lung syndrome (VLS), also known as idiopathic giant bullous emphysema, is a rare manifestation of chronic obstructive pulmonary disease (COPD) and usually occurs in middle-aged smokers. This report presents a 58-year-old female smoker with COPD and VLS who presented with spontaneous pneumothorax. The pneumothorax was managed with a chest tube and was later complicated by massive subcutaneous emphysema.

          Case Report:

          A 58-year-old woman with a past medical history of long-term smoking and COPD presented with worsening shortness of breath. Upon initial evaluation, she had tachypnea and hypoxia (SpO 2 93%). Chest radiography revealed a new right-sided pneumothorax on top of extensive bullous disease, which the patient already had. The drainage of the pneumothorax was successful with a pigtail catheter. However, during the following night, after insertion of the pigtail catheter, the patient developed massive subcutaneous emphysema, which was confirmed with imaging. The patient remained hemodynamically stable, and diffuse subcutaneous crepitus was present on examination. The pigtail catheter was repositioned, resulting in complete resolution of the subcutaneous emphysema in the following 2 weeks.

          Conclusions:

          This case highlights the importance of a timely diagnosis and management of the possible presentations and complications of VLS. Complications such as pneumothorax are life-threatening and require urgent management, taking precedence over the curative treatment for VLS, surgical bullectomy.

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          Most cited references16

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          Elective surgery for giant bullous emphysema: a 5-year clinical and functional follow-up.

          So far, very few studies in the literature have reported data on the long-term follow-up of patients who have undergone surgery for giant bullous emphysema (GBE), and much still needs to be known on the late fate of these patients. To evaluate patients who have undergone elective surgery due to GBE, early and late mortality following surgery, the early and late reappearance of bullae, and the early and late modifications of clinical and functional data. Forty-one consecutive patients (36 men; mean [+/- SD] age, 48.4 +/- 14.8 years) who underwent elective surgery for GBE were enrolled in a prospective study, and were studied both before and after undergoing bullectomy for a 5-year-follow-up period. Analyses were performed on the whole population and on two subgroups of patients who were divided on the basis of the absence of underlying diffuse emphysema (group A; n = 23) or the presence of underlying diffuse emphysema (group B; n = 18). The early mortality rate was 7.3% (within the first year), and the late mortality rate was 4.9% (overall mortality rate at 5 years, 12.2%; mortality rate in group B, 27.8%). Bullae did not reappear and residual bullae did not become enlarged in any patients at the site of the bullectomy. During the follow-up, the dyspnea score was reduced significantly soon after bullectomy and up to the fourth year of follow-up; intrathoracic gas volume also was reduced significantly (average, 0.7 L). The same was true for the FEV1 percent predicted and the FEV1/vital capacity ratio, which kept increasing until the second year; then, from the third year of follow-up these values were reduced, yet remained above the prebullectomy values until the fifth year of follow-up. When considered separately, the patients in group B appeared to be the most impaired, clinically and functionally (eg, FEV1 showed a similar significant increase up to the second year in both groups after surgery, while a different mean annual decrease was appreciable from the second to the fifth year of follow-up: group A, 25 mL/year; group B, 83 mL/year. Furthermore, patients in group B were the only ones who contributed to the mortality rate, on the whole showing a behavior similar to that of patients who had undergone lung volume reduction surgery. In patients with GBE who were enrolled in the study prospectively and were investigated yearly during a 5-year-follow-up period, elective surgery appears to have been fairly safe, and allowed clinical and functional improvement for at least 5 years. Better results may be expected in patients without underlying diffuse emphysema.
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            Emphysema and secondary pneumothorax in young adults smoking cannabis.

            We observed a remarkable increase in the number of young patients who presented with lung emphysema and secondary spontaneous pneumothorax (SSP) at our institution for over a period of 30 months; most of them have a common history of marijuana abuse. Retrospective case series. Seventeen young patients presented with spontaneous pneumothorax with bullous lung emphysema were systematically evaluated over a period of 30 months. All were regular marijuana smokers. Clinical history, chest X-ray, CT-scan, lung function test, and laboratory and histological examinations were assessed. We compared the findings of this group (group I) with the findings of non-marijuana smoking patients (group II) in the same period. The findings of this series were also compared with the findings of 75 patients presented with pneumothorax in a previous period from January 2000 till March 2002 (group III). In group I, there were 17 patients: the median age of the patients was 27 years (range 19-43 years), 16 males and 1 female. All were living in Switzerland. All but one smoked marijuana daily for a mean of 8.8 years and tobacco for 11.8 years. CT-scan showed multiple bullae at the apex or significant bullous emphysema with predominance in the upper lobes only in two patients. Only two patients had reduced forced first second expiratory volume (FEV1) and one reduced vital capacity (VC) below the predicted 50%. This correlated with the subjectively asymptomatic condition of the patients. All but two patients were treated by video-assisted thoracoscopic surgery (VATS) for prevention of relapsing pneumothorax. Histology showed severe lung emphysema, inflammation, and heavily pigmented macrophages. In group II, there were 85 patients: there were 78 males, the median age was 24 years (range 17-40 years), 74 patients smoked tobacco for 13.4 years but no marijuana. CT-scan in 72 patients showed only small bullae at the apex but no significant emphysema; other clinical, laboratory, and histopathological findings showed no significant difference in group I. In group III, there were 75 patients: there were 71 males and 4 females. Mean age was 25 years (range 16-46 years). Six smoked marijuana daily for a mean of 3.2 years, and 62 smoked tobacco for 14 years. CT-scan done in 59 patients showed few small bullae at the apex but no significant lung emphysema. The presence of lung emphysema on CT-scan in group I was significantly different than in groups II and III (p=0.14). No significant difference was found among all groups in the form of clinical, laboratory, and histopathological findings. In case of emphysema in young individuals, marijuana abuse has to be considered in the differential diagnosis. The period of marijuana smoking seems to play an important role in the development of lung emphysema. This obviously quite frequent condition in young and so far asymptomatic patients will have medical, financial, and ethical impact, as some of these patients may be severely handicapped or even become lung transplant candidates in the future.
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              Bullous lung disease due to marijuana.

              In contrast to the well-described effects of tobacco smoking upon pulmonary emphysema, with approximately 15% of smokers being affected at the age of 65 years, the effects of marijuana smoking are rarely reported and poorly understood. We report a series of 10 patients (mean age 41 +/- 9 years, eight male, two female), who presented over a period of 12 months to our respiratory unit with new respiratory symptoms, and who admitted to regular chronic marijuana smoking (>1 year continuously). Symptoms on presentation were dyspnoea (n = 4), pneumothorax (n = 4) and chest infection (n = 2). High-resolution CT revealed asymmetrical, variably sized, emphysematous bullae in the upper and mid zones. However, the CXR was normal in four patients and lung function was normal in five. Marijuana smoking leads to asymmetrical bullous disease, often in the setting of normal CXR and lung function. In subjects who smoke marijuana, these pathological changes occur at a younger age (approximately 20 years earlier) than in tobacco smokers.
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                Author and article information

                Journal
                Am J Case Rep
                Am J Case Rep
                amjcaserep
                The American Journal of Case Reports
                International Scientific Literature, Inc.
                1941-5923
                2022
                02 December 2022
                : 23
                : e938063-1-e938063-6
                Affiliations
                [1 ]Department of Internal Medicine, Lincoln Medical Center, Bronx, NY, USA
                [2 ]Department of Pulmonary and Critical Care, Spectrum Health – Michigan State University, Grand Rapids, MI, USA
                [3 ]Department of Internal Medicine, Springfield Memorial Hospital, Springfield, IL, USA
                Author notes
                Corresponding Author: Haris Sohail, e-mail: sohailh@ 123456nychhc.org

                Authors’ Contribution:

                [A]

                Study Design

                [B]

                Data Collection

                [C]

                Statistical Analysis

                [D]

                Data Interpretation

                [E]

                Manuscript Preparation

                [F]

                Literature Search

                [G]

                Funds Collection

                Financial support: None declared

                Conflict of interest: None declared

                Author information
                https://orcid.org/0000-0001-6720-7178
                https://orcid.org/0000-0002-0863-8944
                Article
                938063
                10.12659/AJCR.938063
                9724188
                36457206
                a3f3ba4d-5e08-4a43-9978-bcde48da6d76
                © Am J Case Rep, 2022

                This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International ( CC BY-NC-ND 4.0)

                History
                : 09 August 2022
                : 06 October 2022
                : 14 November 2022
                Categories
                Articles

                emphysema,pulmonary bullae causing pneumothorax,subcutaneous emphysema

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