Episode Of Spontaneous Pneumothorax Research Articles

The prognostic effect of concomitant COVID-19 with spontaneous pneumothorax.

The aim of this study was to investigate the prevalence of novel coronavirus disease 2019 (COVID-19) in patients hospitalized with primary spontaneous pneumothorax and to evaluate its possible effects on the clinical course, treatment, and the prognosis. Between April 2020 and January 2021, a total of 86 patients (78 males, 8 females; mean age: 27±5 years; range, 16 to 40 years) who had no underlying lung disease and were diagnosed with the first episode of spontaneous pneumothorax were retrospectively analyzed. At the same time of diagnosis, all patients were screened for COVID-19 via polymerase chain reaction test of nasopharyngeal swabs. According to the test results, the patients were divided into two groups as COVID-19(+) and COVID-19(-). The duration of air leak, hospital stay, recurrence rates and treatment modalities, and mortality rates of the two groups were compared. Following a pneumothorax diagnosis, 18 (21%) patients were diagnosed with COVID-19. In COVID-19(+) patients, the mean air leak and lung expansion duration were significantly longer (p<0.0001 for both). In these patients, the mean length of hospital stay was also significantly longer (p<0.0001). During the median follow-up of six months, no mortality was observed and the recurrence rate was similar between the two groups (p=0.998). Our study results suggest that COVID-19 negatively affects the recovery time in patients with spontaneous pneumothorax.

Video-assisted thoracoscopic (VATS) era in surgical treatment of spontaneous pneumothorax

Background: VATS is now regarded by many surgeons to be the gold standard in the surgical management of a variety of thoracic conditions, such as spontaneous pneumothorax (SP), pleural diseases, and indeterminate lung nodules. Aim and objectives: to describe different VATS techniques in patients with spontaneous pneumothorax.Summary: Several clinical management issues remain unanswered. Firstly, the duration of prolonged air leak that warrants VATS remain controversial. Current recommendations range from five days by BTS to three days advocated by our institute with more than a decade of experience with VATS for SP. Furthermore, whether VATS should be offered to all patients with low general anesthetic risk presenting with first episode of SP is unclear. Furthermore, the evolution of surgical techniques to allow ipsilateral thoracoscopic approach to treat contralateral bullous lesion may redefine the indications for CT scan in SP. Technically, the debate will continue among the surgeons on the type of VATS procedure that will minimize pneumothorax recurrence. Most surgeons will agree that some form of bullectomy (endoscopic staple or suturing) is mandatory. However, the nature of pleurodesis (mechanical, chemical, or both) that follow is often at the discretion of the individual clinician.

Dysphagia Aortica: A Case Report and Review of Treatment Options

A 64-year-old, cachectic man with body mass index (BMI) &lt;19 visited in clinic with the chief complaint of dysphagia for 6 months. He reported a 2-year history of reflux and heartburn, for which he has been taking pantoprazole but reported only 40% relief of reflux symptoms. He reported progressive solid food mid-chest dysphagia. Additional comorbidities included severe pulmonary bronchiectasis and bullous emphysema and a history of treated pulmonary tuberculosis 40 years prior and two previous episodes of spontaneous pneumothorax in the right-sided lung treated with a chest tube. A chest X-ray showed a calcified aorta crossing the midline (Figure 1). A CT scan done for assessment of pneumothorax showed a torturous descending thoracic aorta and a dilated mid-/proximal esophagus. The diameter of the descending aorta was 41 mm, and it crossed the midline. For evaluation of dysphagia, a barium swallow was performed, which showed a narrowing in the mid-esophagus with proximal dilation and lack of peristalsis (Figure 2). Upper gastrointestinal endoscopy showed a dilated esophagus with eccentric extraluminal compression. High-resolution manometry (HRM) showed an absence of peristalsis and a vascular pressure artifact around the mid-esophagus correlating with the external aortic compression (Figure 3). Due to alarming weight loss and a BMI &lt;19, we recommended a feeding jejunostomy to maintain nutrition before scheduling definitive treatment.

SECONDARY SPONTENEOUS PNEUMOTHORAX AS A PRESENTATION OF BREAST CANCER RECURRENCE: CASE REPORT

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Spontaneous pneumothorax is a common pathology described as gas trapped in the pleural space, not caused by an external event. When a lung associated pathology is found as the cause of the spontaneous pneumothorax then a subclassification arises and the events are characterized as Secondary spontaneous pneumothorax. Secondary spontaneous pneumothorax (SSP) are most frequently caused by chronic obstructive lung disease, necrotizing lung infections, genetically associated disease like cystic fibrosis or primary lung malignancy. There are lesser-known causes of SSP and, this is one such example where we showcase an unusual presentation revealing a unique etiology. CASE PRESENTATION: A 60-year-old Hispanic female with hypothyroidism who underwent left radical mastectomy 10 years ago for breast CA presented with sudden onset left-sided chest pain. She denied shortness of breath, recent trauma or smoking history. Heart rate, peripheral oxygen saturation, and laboratory tests were unremarkable, including 12 lead electrocardiogram. Physical examination was remarkable for decreased breath sounds on the left side of the chest. Chest CT scan revealed large left pneumothorax with paraaortic, right paratracheal and subcarinal lymph nodes with central necrosis. Also, a tissue density within the left main bronchus and hilum was identified, resulting in partial obstruction of the left lower lobe bronchus. General surgery service was consulted for emergent chest tube placement. A bronchoscopy was performed, and the endobronchial mass was biopsied. Pathology results showed metastatic recurrence of breast cancer. The patient was observed for three days after chest tube placement and chest radiography demonstrated resolution of pneumothorax. At that moment, the chest tube was removed, and the patient was discharged home the next day. DISCUSSION: Presently, the patient continues stable since being discharged from our institution. She was started on Letrozole and Palbociclib by the Oncology Service and has had no new episode of spontaneous pneumothorax. The patient will continue to receive conservative management by Oncology at our institution. CONCLUSIONS: This vignette illustrates a unique presentation of a metastatic breast cancer recurrence debuting as spontaneous pneumothorax. The most common type of malignancies associated with secondary spontaneous pneumothorax are primary lung cancer and sarcomas. To our knowledge, there are no cases that report an SSP as the presentation of breast cancer recurrence. Reference #1: Srinivas, S., & Varadhachary, G. (2000). Spontaneous pneumothorax in malignancy: A case report and review of the literature. Annals of Oncology, 11(7), 887–889. doi: 10.1023/a:1008323632078 Reference #2: Bini, Alessandro; Zompatori, Maurizio; Ansaloni, Luca; Grazia, Manuele; Stella, Franco; Bazzocchi, Ruggero. (2000). Bilateral recurrent pneumothorax complicating chemotherapy for pulmonary metastatic breast ductal carcinoma: Report of a case. Surgery today. 30. 469-72. 10.1007/s005950050628. DISCLOSURES: No relevant relationships by Juan Feliciano-FIgueroa, source=Web Response No relevant relationships by Angel Laureano, source=Web Response No relevant relationships by Giovanni Veloz Irizarry, source=Web Response

Conservative treatment for recurrent secondary spontaneous pneumothorax in patients with a long recurrence-free interval.

BackgroundAlthough recurrence is included in surgical indications, there is no definitive guideline for managing recurrent spontaneous pneumothorax (SP) according to characteristics of recurrence, such as the type [primary SP (PSP) or secondary SP (SSP)] and recurrence-free interval (RFI). Actually, some patients with early cessation of air leak after closed thoracostomy tubes, a long RFI, or medical comorbidities are often managed conservatively without surgery. However, the validity of this management is unclear. The aims of the present study are to analyze treatment outcomes for recurrent SP according to the type of SP and RFI, and to check the validity of conservative treatment for patients according to type of SP and RFI.MethodsWe included 1,250 consecutive cases (624 right sided and 626 left side cases for the first episode) who were hospitalized and treated from January 2012 to June 2018. To investigate recurrence according to treatment modality (surgical or conservative treatment), we estimated RFI in each group during the observation period. RFI was measured from the completion of treatment to recurrence or last follow-up. We divided patients into two groups [the early (EG) and the late (LG) recurrence group] according to 1-year. Recurrence was defined as a subsequent episode of an ipsilateral SP, while a contralateral SP was regarded as an independent case in the present study. RFI between subgroups was compared using the Kaplan-Meier method with the log-rank test. A P value less than 0.05 (two-sided) was regarded as statistically significant.ResultsRecurrence occurred in 47 cases after surgical intervention for the first episode (585 cases). Recurrence occurred in 265 cases after conservative treatment for the first episode of SP (665 cases). For the first episode, the surgical group (SG) had a significantly longer RFI than the conservative group (CG), regardless of the type (both, P<0.001). Conservative treatment and surgical intervention for the second episode after conservative treatment for the first episode were performed in 98 and 167 cases, respectively. For the second episode after conservative treatment for the first episode, SG also had a significantly longer RFI than CG, regardless of the types (PSP P<0.001, SSP P=0.031). To check the validity of conservative treatment for patients with a long RFI, we analyzed recurrence by dividing patients into two groups according to one-year RFI. For PSP, the early recurrence group (EG, RFI ≤1 year) had 99 cases and the late recurrent group (LG, RFI >1 year) had 67 cases. SG had a significantly longer RFI than CG in both EG and LG (EG, P<0.001 and LG, P=0.001). In addition, for SSP, there were 67 cases of EG and 32 cases of LG, SG had significantly longer RFIs than CG in EG (P=0.007). However, there was no significant difference in RFI between SG and CG in LG (P=0.748).ConclusionsThe present study revealed diversity of management outcomes according to characteristics of recurrence and provides some evidences of the validity of conservative treatment in recurrent SSP with a long RFI. Further large-scale prospective randomized trials are required to validate these findings.

Pneumothorax spontané récidivant révélant un syndrome de Marfan

Because of its rarity and lack of specific symptoms, the diagnosis of Marfan's syndrome can be delayed. Though cardiovascular, skeletal and ophthalmological manifestations are the most frequent features, the respiratory system can also be involved. We report the case of a 35-year-old patient who presented with a large, right sided, encysted pyopneumothorax. The history revealed two episodes of spontaneous pneumothorax, cataract surgery ten years before his admission, and a similar case in the family. Clinical examination showed skeletal abnormalities of the wrist and thumb as well as deformity of the hindfoot with a flat foot. He had no characteristic thoracic deformity. Ophthalmologically, the right eye had a subluxed lens and the left eye had a totally subluxed cataract. The development of the third episode of pneumothorax led to the diagnosis of Marfan's syndrome thanks to the patient's combination of symptoms. To avoid diagnostic delay, criteria were established in 1996and revised in 2010to ensure an early diagnosis of this condition.

UNANTICIPATED CONSEQUENCES OF MARIJUANA LEGALIZATION

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: A nineteen year old male, never a tobacco smoker, with no significant history, presented to clinic after three episodes of spontaneous pneumothorax in two months. CASE PRESENTATION: After presenting to clinic, he denied smoking, substance use, and environmental exposures. Physical exam was unremarkable. Between his first and second events, CT scans revealed apical bullae. His three episodes required thorascopy, left apical bullectomy, and pleurectomy. Pathology showed respiratory bronchiolitis and inflammation with abundant eosinophils, raising suspicion for undisclosed smoking habits. The patient retroactively admitted to smoking marijuana and inhaling heated oils daily for “medical purposes,” due to chronic back pain, and had obtained a “medical card” to purchase marijuana. DISCUSSION: This case of respiratory bronchiolitis and bullae due to marijuana and heated oil inhalation exemplifies unintended consequences of marijuana legalization, and delineates a perception that marijuana use is largely harmless. It suggests that there is confusion regarding the distinction between recreational and medical marijuana, among users and medical providers. Moreover, it outlines a perceived probability that obtaining a license for marijuana use permits recreational procurement, by way of claiming to be affected by a condition which may not have entirely objective diagnostic criteria. CONCLUSIONS: Marijuana is one of the most commonly used substances, and has been used extensively in medicine. Chronic pain is the most common reason for use of medical marijuana, which is legalized and approved for specific uses. Despite known complications, marijuana use remains prevalent and may become the first Schedule I substance to traverse the gap between substance use and medical therapy. Amid constituent compounds, cannabidiol is believed to correlate with analgesia, and whether it can be administered without exposing lung parenchyma to inflammatory states is worth exploring. Describing the risks and benefits of marijuana can bolster public knowledge as its legal status continues to broaden. Reference #1: Peacock A, Leung J, Larney S, et al. Global statistics on alcohol, tobacco and illicit drug use: 2017 status report. Addiction. 2018:113(10):1905-1926. Reference #2: Aggarwal SK, Carter GT, Sullivan MD, et al. Medicinal use of cannabis in the United States: historical perspectives, current trends, and future directions. J Opioid Manag. 2009;5:153–168. Reference #3: Wilkinson S, Yarnell S, Radhakrishnan R, Ball S, D’Souza D. Marijuana Legalization: Impact on Physicians and Public Health. Annu Rev Med. 2016;67:253-466. DISCLOSURES: No relevant relationships by Humberto Choi, source=Web Response No relevant relationships by Charlie Lin, source=Web Response

Management and recurrence of spontaneous pneumothorax in children.

No guideline clearly prescribes an approach to management of spontaneous pneumothorax in children. The objectives of this study were to evaluate practice variation in the management of spontaneous pneumothorax in children and its probability of recurrence. This study was a retrospective chart review followed by a phone follow-up that included all children who had visited a tertiary care paediatric hospital for a first episode of spontaneous pneumothorax between 2008 and 2017. The primary outcomes were the management of pneumothorax (observation, oxygen, needle aspiration, intercostal chest tube, surgery) and the probability of recurrence. All charts were evaluated by a rater using a standardized report form and 10% of the charts were evaluated in duplicate. All children/families were contacted by phone to assess recurrence. The primary analyses were the proportions of each treatment modalities and recurrence, respectively. During the study period, 76 children were deemed eligible for the study. Among them, 59 had a primary spontaneous pneumothorax while 17 were secondary. The most common first therapeutic approaches were chest tube insertion (31), oxygen alone (27), and observation (14). A total of 54 patients were available for follow-up among whom a recurrence was observed in 28 (37% of the total cohort or 52% of available children). Chest tube insertion was the first line of treatment in about 40% of children with a first spontaneous pneumothorax. In this population, the recurrence probability is established between 37 and 52% and the majority occurs in the following months.

The first episode of spontaneous pneumothorax (errors, hazards, complications)

To analyze the errors and complications of surgical care in patients with the first episode of spontaneous pneumothorax at different hospitals. From 2005 to 2015 three hundreds and seventeen patients with the first episode of spontaneous pneumothorax have been treated at the thoracic department of Volgograd State Medical University. Patients were divided into 2 groups: 79 of them underwent thoracotomy while in 238 cases minimally invasive technologies were applied. Faster preoperative management and earlier active surgical tactics were suggested. There were 6 the most typical violations of surgical care in 42% of patients with the first episode of spontaneous pneumothorax. Medical and diagnostic violations at regional centers and central district hospitals were the same, but there were differences in certain types of errors and their incidence. Minimally invasive technologies do not significantly affect the number of violations of thoracic surgery principles. Early procedures against the recurrence by using of thoracoscopic interventions reduce postoperative morbidity from 1.2 to 0.3% and mortality by 8.25 times.

Relapse Bullous Emphysema Complicated by Spontaneous Pneumothorax, after Radical Bullectomy

Actuality The social significance of spontaneous pneumothorax due to the fact that in recent years a growing number of patients with pulmonary bullous emphysema and, accordingly, with spontaneous pneumothorax, as well as the fact that the structure is dominated by patients of working age persons. To date, some difficulties persist in choosing the adequate diagnostic and treatment tactics in this disease.&#x0D; Objective In a clinical example to show the importance of an integrated approach to of surgical treatment for spontaneous pneumothorax, caused bullous emphysema.&#x0D; Materials and methods A clinical case of recurrent bullous emphysema complicated by recurrent spontaneous pneumothorax in a patient 32 years old, six years earlier underwent radical surgery - bullectomy on the first episode of spontaneous pneumothorax.

Catamenial pneumothorax since introduction of video-assisted thoracoscopic surgery : A systematic review.

Catamenial pneumothorax is an uncommon form of spontaneous pneumothorax in women. The exact epidemiology and pathogenesis remain elusive. Video-assisted thoracoscopic surgery is used for diagnostic and therapeutic purposes. The aim of this review was to analyze the demographic features, intraoperative findings, treatment methods and outcome in catamenial pneumothorax patients. In addition, we assessed the relationship between catamenial pneumothorax and pelvic endometriosis. A PubMed search of medical literature, published from January 1993 (video-assisted thoracoscopic surgery first described in literature) to January 2015, using the keywords "catamenial pneumothorax" was performed. Our study complied with the preferred reporting of items for systematic reviews and meta-analysis principles. Atotal of 182 patients were included in the analysis, including 4patients treated at our institution. The inclusion criteria of were recurrent (at least two) episodes of spontaneous pneumothorax in relation to onset of menses. Age at time of diagnosis, side affected, diagnosis of pulmonary endometriosis, intraoperative findings, histological confirmation of thoracic endometriosis, methods of treatment and outcome were recorded. In 2.9% of the patients no pathological lesions were found; however, 59.3% had endometrial implants and 57.0% diaphragmatic perforations. Pelvic endometriosis was reported in 39.5% patients. Patients with diagnosed pelvic endometriosis showed asignificantly higher rate of endometrial implants and histologically confirmed endometriosis lesions than patients without pelvic endometriosis. In 26.9% of patients, recurrence was observed after treatment. Video-assisted thoracoscopic surgery provides good diagnostic and therapeutic results; however, 25% of patients experienced recurrence despite adequate treatment. Astrong association exists between thoracic and pelvic endometriosis in catamenial pneumothorax patients.

Spontaneous pneumothorax in diffuse cystic lung diseases.

Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and pulmonary Langerhans cell histiocytosis. DCLDs are responsible for approximately 10% of apparent primary spontaneous pneumothoraces. Computed tomography screening for DCLDs (Birt-Hogg-Dubé syndrome, lymphangioleiomyomatosis, and pulmonary Langerhans cell histiocytosis) following the first spontaneous pneumothorax has recently been shown to be cost-effective and can help facilitate early diagnosis of the underlying disorders. Patients with DCLD-associated spontaneous pneumothorax have a very high rate of recurrence, and thus pleurodesis should be considered following the first episode of spontaneous pneumothorax in these patients, rather than waiting for a recurrent episode. Prior pleurodesis is not a contraindication to future lung transplant. Although DCLDs are uncommon, spontaneous pneumothorax is often the sentinel event that provides an opportunity for diagnosis. By understanding the burden and implications of pneumothoraces in DCLDs, clinicians can facilitate early diagnosis and appropriate management of the underlying disorders.

Tuberous sclerosis associated with autosomal dominant polycystic kidney disease: Case report about of the TSC2/PKD1 contiguous gene syndrome

We present a case of a young woman with chronic kidney disease, with a history of seizures, episodes of spontaneous pneumothorax and left nephrectomy. The retrospective study of the image exams showed liver, kidney and lung cysts; in addition to cortical tubers and subependymal nodules in the brain. The pathologic evaluation of kidney surgically removed characterised adult renal polycystic disease. The clinical review also with these tests revealed the diagnosis of tuberous sclerosis, and autosomal dominant polycystic kidney disease, suggesting contiguous gene syndrome TSC2/PKD1

Recurrent Afebrile Episodes of Spontaneous Pneumothorax in a Child with Well Controlled Systemic Lupus Erythematosus Disease

Systemic Lupus Erythematosus is an autoimmune disorder with multiple system involvement. Despite its pleuro-pulmonary involvement, recurrent spontaneous pneumothoraces are rare complication of childhood SLE. We report a 12 year old girl having SLE with nephritis, who developed recurrent episodes of spontaneous pneumothoraces. Surgical management with prolonged evacuation and aggressive immunosuppressive therapy improves the outcome for these patients. J Nepal Paediatr Soc 2016;36(1):94-96.

Prise en charge du premier épisode de pneumothorax spontané

The management of a first episode of spontaneous pneumothorax is controversial and the best technique to be used as an initial intervention, aspiration or intercostal drainage, is still debated. We present a retrospective case series during two and a half consecutive years describing the immediate management of spontaneous pneumothoraces, comparing aspiration versus thoracic drainage. One hundred and thirty-three clinical files from patients with spontaneous pneumothoraces were analyzed (17 primary and 116 secondary). The pneumothoraces were of varying size and different etiologies. Patients were initially treated with simple aspiration in 68 cases, with an immediate success rate of 37.5%, intercostal drainage in 49 cases, and by rest alone in 16 cases. In case of secondary pneumothorax, aspiration appeared to offer advantages as an initial strategy over intercostal drainage in terms of hospital stay (11 versus 22 days), and with significant effectiveness (37.5%).

Video-assisted and thoracoscopic surgeries in the first episode of spontaneous pneumothorax

Video-assisted and thoracoscopic surgeries in the first episode of spontaneous pneumothorax

178 Prognostic impact of recurrent pneumothoraces in adults with cystic fibrosis

Objectives: Spontaneous pneumothorax (PTx) is a well recognised complication in CF and is associated with recurrence and increased morbidity and mortality. To inform future management, we looked at the treatment and outcome of PTx in CF patients attending our large adult centre. Method: We reviewed 64 episodes of spontaneous PTx in 28 CF patients (mean FEV1 32% predicted [range 16−68%], 36% male), focussing on modalities of treatment and ultimate outcomes. Results: Immediate treatments are shown in the table. Nine patients (32%) sustained bilateral PTx and 50% had ipsilateral recurrence (mean interval 4 months). Twenty four have died (63% at 1 year, 80% at 2 years, median survival 7 months [0−68]) after first PTx, with the remaining 4 alive at 6−34 months post PTx. Conclusions: We have confirmed that spontaneous PTx is a poor prognostic factor in CF with poor survival rates and a recurrence or a contralateral PTx increases mortality. A multidisciplinary approach towards the treatment modalities for the management of recurrent or persistent PTx is essential when dealing with this complication.

177 Management and outcomes following pneumothorax in cystic fibrosis patients

Objectives: Spontaneous pneumothorax (PTx) is a well recognised complication in CF and is associated with recurrence and increased morbidity and mortality. To inform future management, we looked at the treatment and outcome of PTx in CF patients attending our large adult centre. Method: We reviewed 64 episodes of spontaneous PTx in 28 CF patients (mean FEV1 32% predicted [range 16−68%], 36% male), focussing on modalities of treatment and ultimate outcomes. Results: Immediate treatments are shown in the table. Nine patients (32%) sustained bilateral PTx and 50% had ipsilateral recurrence (mean interval 4 months). Twenty four have died (63% at 1 year, 80% at 2 years, median survival 7 months [0−68]) after first PTx, with the remaining 4 alive at 6−34 months post PTx. Conclusions: We have confirmed that spontaneous PTx is a poor prognostic factor in CF with poor survival rates and a recurrence or a contralateral PTx increases mortality. A multidisciplinary approach towards the treatment modalities for the management of recurrent or persistent PTx is essential when dealing with this complication.

Thoracic endometriosis as cause of recurrent pneumothorax

We present a 44-year-old woman with no personal history of relevance but for a heterozygous mutation G1691A of the gene of Leiden factor V. Her history starts 2 years ago with two consecutive, 1 month apart, right pneumothoraces that were diagnosed as primary spontaneous pneumothorax (PSP). In both cases she was treated with endopleural aspiration and respiratory physical therapy, with full recovery. The patient remained asymptomatic for a year, but she suffered another right spontaneous pneumothorax (Figure 1). She was treated with endopleural aspiration. Videothoracoscopy was performed and revealed a bullae in the inferior lobe, a fibroid plaque on the posterior thoracic wall and a hypervascularized lesion with multiple fenestrations localized at the diaphragmatic pleura. A biopsy was taken from the hypervascularized lesion and showed pleural with fibrosis with a hyperplasic mesothelial reaction. Resection of the bullae and pleural abrasion were performed. Figure 1. Chest radiograph showing a right-sided pneumothorax. The next year the patient suffered her fourth episode of spontaneous pneumothorax and was treated again with endopleural aspiration and respiratory physical therapy. After 1 …

Pneumothorax and Bullae in Marfan Syndrome

Background: Increased risk of spontaneous pneumothorax has been described in patients with Marfan syndrome and has been attributed, in part, to the presence of apical blebs and bullae. Objectives: We assess the risk of pneumothorax and its relationship to the presence of apical blebs and bullae in patients with Marfan syndrome in the era of CT imaging. Methods: A retrospective cohort study was performed of all patients 13 years or older with Marfan syndrome evaluated at the Mayo Clinic, Rochester, Minn., USA, from 1998 through 2008. One hundred and sixty-six patients met the current diagnostic criteria for Marfan syndrome and had chest imaging studies available for review. Results: The median age was 40 years (range 14–71); 37% had a smoking history. Eight of 166 patients (4.8%) had experienced 1 or more episodes of spontaneous pneumothorax, and 2 of these 8 patients had 2 or more episodes. Apical blebs or bullae were identified on radiologic imaging in 16 patients (9.6%). Four of 16 (25%) patients with apical blebs or bullae had a history of spontaneous pneumothorax compared to 4 of 150 patients (2.7%) without blebs or bullae (p = 0.003). Conclusions: The frequency of blebs is relatively low in patients with Marfan syndrome but the risk of pneumothorax is significantly higher in those with radiologically detectable blebs or bullae. Chest CT scanning to identify blebs and bullae may allow risk stratification for pneumothorax in patients with Marfan syndrome.