Recurrent spontaneous pneumothorax in an NF1 patient with a novel causative variant: broadening genotype-phenotype correlations.

Neurofibromatosis type 1 (NF1) is an autosomal dominant disease with complete penetrance, most commonly known to affect the skin and eyes. Although lung involvement in the form of cysts and bullae occurs in up to 20% of adults, the seemingly intuitive association of NF1 and spontaneous pneumothorax is not widely recognised among clinicians. Here, we report the second case of recurring spontaneous pneumothorax in the context of NF1 with a confirmed molecular diagnosis. In both cases, the NF1 variants featured a premature stop codon in the C-terminal protein domain. Interestingly, our patient had mild skin symptoms, suggesting that spontaneous pneumothorax may not be correlated with cutaneous disease severity. More genotype-phenotype correlation studies are needed for NF1 in general and for its link to spontaneous pneumothorax in particular.

Bronchoscopic Lung Volume Reduction as the Treatment of Choice versus Robotic-Assisted Lung Volume Reduction Surgery in Similar Patients with Emphysema - An Initial Experience of the Benefits and Complications.

Objective: There is an assumption that because EBLVR requires less use of hospital resources, offsetting the higher cost of endobronchial valves, it should therefore be the treatment of choice wherever possible. We have tested this hypothesis in a retrospective analysis of the two in similar groups of patients. Methods: In a 4-year experience, we performed 177 consecutive LVR procedures: 83 patients underwent Robot Assisted Thoracoscopic (RATS) LVRS and 94 EBLVR. EBLVR was intentionally precluded by evidence of incomplete fissure integrity or intra-operative assessment of collateral ventilation. Unilateral RATS LVRS was performed in these cases together with those with unsuitable targets for EBLVR. Results: EBLVR was uncomplicated in 37 (39%) cases; complicated by post-procedure spontaneous pneumothorax (SP) in 28(30%) and required revision in 29 (31%). In the LVRS group, 7 (8%) patients were readmitted with treatment-related complications, but no revisional procedure was needed. When compared with uncomplicated EBLVR, LVRS had a significantly longer operating time: 85 (14-82) vs 40 (15-151) minutes (p<0.001) and hospital stay: 7.5 (2-80) vs 2 (1-14) days (p<0.01). However, LVRS had a similar total operating time to both EBLVR requiring revision: 78 (38-292) minutes and hospital stay to EBLVR complicated by pneumothorax of 11.5 (6.5-24.25) days. Use of critical care was significantly longer in RATS group, and it was also significantly longer in EBV with SP group than in uncomplicated EBV group. Conclusion: Endobronchial LVR does use less hospital resources than RATS LVRS in comparable groups if the recovery is uncomplicated. However, this advantage is lost if one includes the resources needed for the treatment of complications and revisional procedures. Any decision to favour EBLVR over LVRS should not be based on the assumption of a smoother, faster perioperative course.

The role of thoracic surgery in the management of complicated acute and post-acute COVID-19 pneumonia (PASC).

INTRODUCTION: COVID-19 is considered a respiratory virosis in its classic form, although it may present with heterogeneous symptoms. Thoracic complications occur in a small percentage of patients. Our objective was to evaluate existing experience with this disease and its thoracic manifestations and to determine the real-world status of care of these patients. METHODS: This study is a retrospective, single-institution analysis of a group of patients hospitalized with acute and post-acute COVID-19 pneumonia at Thomayer Hospital in Prague in the period from December 2020 to March 2022 and indicated for a thoracic surgical procedure. RESULTS: During the peak of COVID-19 pandemic, a thoracic intervention was performed in 46 admitted patients. Thoracic drainage (due to pneumothorax in 18 cases, fluidothorax in 3 cases, CT-guided lung abscess drainage in 2 cases, and CT-guided pneumatocele drainage in 2 cases) were the most common thoracic surgical procedures. Pleurectomy/decortication surgery was done in 10 cases. Additionally, 12 lung parenchyma-sparing resections were performed, while lobectomy was required in 2 cases. Resection of postintubation tracheal stenosis due to a severe course of COVID-19 pneumonia was indicated in 2 patients. CONCLUSION: Even mild COVID-19 may cause a considerable morphological a functional alteration of the respiratory system. The most common complications of COVID-19 pneumonia that require a thoracic surgical intervention include pathologies associated with an air leak and accumulation of air (pneumothorax, pneumomediastinum and subcutaneous emphysema). The development of pulmonary necrosis, symptomatic bronchiectasis, pneumatocele, and bullous-fibrotic formations may result in pneumothorax, hemothorax or thoracic empyema in sporadic cases. An early thoracic surgical intervention to treat thoracic complications of COVID-19 pneumonia can improve the survival of COVID-19 patients.

Bronchial Valves for Persistent Air Leak: A Systematic Review and Meta-analysis.

BACKGROUND: Patients with persistent air leak (PAL) pose a therapeutic challenge to physicians, with prolonged hospital stays and high morbidity. There is little evidence on the efficacy and safety of bronchial valves (BV) for PAL. METHODS: We systematically searched the PubMed and Embase databases to identify studies evaluating the efficacy and safety of BV for PAL. We calculated the success rate (complete resolution of air leak or removal of intercostal chest drain after bronchial valve placement and requiring no further procedures) of BV for PAL in individual studies. We pooled the data using a random-effects model and examined the factors influencing the success rate using multivariable meta-regression. RESULTS: We analyzed 28 observational studies (2472 participants). The pooled success rate of bronchial valves in PAL was 82% (95% confidence intervals, 75 to 88; 95% prediction intervals, 64 to 92). We found a higher success rate in studies using intrabronchial valves versus endobronchial valves (84% vs. 72%) and in studies with more than 50 subjects (93% vs. 77%). However, none of the factors influenced the success rate of multivariable meta-regression. The overall complication rate was 9.1% (48/527). Granulation tissue was the most common complication reported followed by valve migration or expectoration and hypoxemia. CONCLUSION: Bronchial valves are an effective and safe option for treating PAL. However, the analysis is limited by the availability of only observational data.

Successful Needle Aspiration of a Traumatic Pneumothorax: A Case Report and Literature Review.

Traumatic pneumothorax (PTX) occurs in up to 50% of patients with severe polytrauma and chest injuries. Patients with a traumatic PTX with clinical signs of tension physiology and hemodynamic instability are typically treated with an urgent decompressive thoracostomy, tube thoracostomy, or needle decompression. There is recent evidence that non-breathless patients with a hemodynamically stable traumatic PTX can be managed conservatively through observation or a percutaneous pigtail catheter. We present here a 52-year-old woman who presented to the emergency department with a 55 mm traumatic PTX. Following aspiration of 1500 mL of air, a clinical improvement was immediately observed, allowing the patient to be discharged shortly thereafter. In hemodynamically stable patients with a post-traumatic PTX, without specific risk factors or oxygen desaturation, observation or simple needle aspiration can be a reasonable approach. Although the recent medical literature supports conservative management of small traumatic PTXs, guidelines are lacking for hemodynamically stable patients with a significantly large PTX. This case report documents our successful experience with needle aspiration in such a setting of large traumatic PTX. We aimed in this article to review the available literature on needle aspiration and conservative treatment of traumatic pneumothorax. A total of 12 studies were selected out of 190 articles on traumatic PTX where conservative treatment and chest tube decompression were compared. Our case report offers a novel contribution by illustrating the successful resolution of a sizable pneumothorax through needle aspiration, suggesting that even a large PTX in a hemodynamically stable patient, without other risk conditions, can be successfully treated conservatively with simple needle aspiration in order to avoid tube thoracostomy complications.

Prospective Observation Study for Primary Spontaneous Pneumothorax: Incidence of and Risk Factors for Postoperative Neogenesis of Bullae.

PURPOSE: Details of the neogenesis of bullae (NOB), which causes recurrent primary spontaneous pneumothorax (PSP) following bullectomy, have not been reported and risk factors for NOB remain unclear. We aimed to clarify the details of NOB. METHODS: We conducted a prospective study using three computed tomography (CT) examinations performed 6, 12, and 24 months after bullectomy to identify the incidence of and risk factors for NOB. We enrolled 50 patients who underwent bullectomy for PSP. RESULTS: After excluding 11 patients who canceled the postoperative CT examination at 6 months after bullectomy, only 39 patients were analyzed. The incidence of NOB at 6, 12, and 24 months after bullectomy was 38.5%, 55.2%, and 71.2%, respectively. The rate of NOB in the operated lung was almost 2 times higher than that in the contralateral nonoperative lung. Male sex, multiple bullae on preoperative CT, long stapling line (≥7 cm), deep stapling depth (≥1.5 cm), and heavier resected sample (≥5 g) were suggested to be risk factors for NOB. CONCLUSIONS: We recognized a high incidence of postoperative NOB in PSP patients. Bullectomy itself seems to promote NOB. Postoperative NOB occurs frequently, especially in patients who require a large-volume lung resection with a long staple line.

Utility of artificial intelligence for detection of pneumothorax on chest radiopgraphs done after transthoracic percutaneous transthoracic biopsy guided by computed tomography.

OBJETIVE: To assess the ability of an artificial intelligence software to detect pneumothorax in chest radiographs done after percutaneous transthoracic biopsy. MATERIAL AND METHODS: We included retrospectively in our study adult patients who underwent CT-guided percutaneous transthoracic biopsies from lung, pleural or mediastinal lesions from June 2019 to June 2020, and who had a follow-up chest radiograph after the procedure. These chest radiographs were read to search the presence of pneumothorax independently by an expert thoracic radiologist and a radiodiagnosis resident, whose unified lecture was defined as the gold standard, and the result of each radiograph after interpretation by the artificial intelligence software was documented for posterior comparison with the gold standard. RESULTS: A total of 284 chest radiographs were included in the study and the incidence of pneumothorax was 14.4%. There were no discrepancies between the two readers' interpretation of any of the postbiopsy chest radiographs. The artificial intelligence software was able to detect 41/41 of the present pneumothorax, implying a sensitivity of 100% and a negative predictive value of 100%, with a specificity of 79.4% and a positive predictive value of 45%. The accuracy was 82.4%, indicating that there is a high probability that an individual will be adequately classified by the software. It has also been documented that the presence of Port-a-cath is the cause of 8 of the 50 of false positives by the software. CONCLUSIONS: The software has detected 100% of cases of pneumothorax in the postbiopsy chest radiographs. A potential use of this software could be as a prioritisation tool, allowing radiologists not to read immediately (or even not to read) chest radiographs classified as non-pathological by the software, with the confidence that there are no pathological cases.

Intravenous Opioid Medication with Piritramide Reduces the Risk of Pneumothorax During CT-Guided Percutaneous Core Biopsy of the Lung.

PURPOSE: CT-guided percutaneous core biopsy of the lung is usually performed under local anesthesia, but can also be conducted under additional systemic opioid medication. The purpose of this retrospective study was to assess the effect of intravenous piritramide application on the pneumothorax rate and to identify risk factors for post-biopsy pneumothorax. MATERIALS AND METHODS: One hundred and seventy-one core biopsies of the lung were included in this retrospective single center study. The incidence of pneumothorax and chest tube placement was evaluated. Patient-, procedure- and target-related variables were analyzed by univariate and multivariable logistic regression analysis. RESULTS: The overall incidence of pneumothorax was 39.2% (67/171). The pneumothorax rate was 31.5% (29/92) in patients who received intravenous piritramide and 48.1% (38/79) in patients who did not receive piritramide. In multivariable logistic regression analysis periinterventional piritramide application proved to be the only independent factor to reduce the risk of pneumothorax (odds ratio 0.46, 95%-confidence interval 0.24, 0.88; p = 0.018). Two or more pleura passages (odds ratio 3.38, 95%-confidence interval: 1.15, 9.87; p = 0.026) and prone position of the patient (odds ratio 2.27, 95%-confidence interval: 1.04, 4.94; p = 0.039) were independent risk factors for a higher pneumothorax rate. CONCLUSION: Procedural opioid medication with piritramide proved to be a previously undisclosed factor decreasing the risk of pneumothorax associated with CT-guided percutaneous core biopsy of the lung. LEVEL OF EVIDENCE 4: small study cohort.

Neumomediastino espontáneo: una manifestación extramuscular de la dermatomiositis anti-MDA5. Reporte de 2 casos / Spontaneous pneumomediastinum: An extra muscular manifestation of anti-MDA5 dermatomyositis. Report of 2 cases

El uso de anticuerpos específicos en miopatías inflamatorias ha mejorado la caracterización de esta enfermedad identificando distintos fenotipos clínicos. Los pacientes con dermatomiositis y anticuerpos anti-MDA5 muestran síntomas típicos en la piel, un menor compromiso muscular y una prevalencia de enfermedad pulmonar intersticial de hasta el 91%. Además de la enfermedad pulmonar intersticial, se ha identificado el neumomediastino espontáneo como una manifestación pulmonar rara pero potencialmente mortal. Se reportan 2 casos de esta manifestación en pacientes con dermatomiositis anti-MDA5.(AU)

The use of specific antibodies in inflammatory myopathies has improved the characterization of this disease, identifying different clinical phenotypes. Patients with dermatomyositis and anti-MDA5 antibodies display typical skin symptoms, lesser muscular involvement, and a prevalence of interstitial lung disease of up to 91%. Beyond interstitial lung disease, spontaneous pneumomediastinum has been identified as a rare but potentially fatal pulmonary manifestation. Two cases of this complication in patients with anti-MDA5 dermatomyositis are reported.(AU)

The prevalence of pneumothorax in human immunodeficiency virus patients. A single center study.

OBJECTIVES: To assess the prevalence, risk factors, and associated complications of pneumothorax that are present in patients with human immunodeficiency virus (HIV) at our institution and to provide an updated local study addressing the association between pneumothorax and HIV. METHODS: This retrospective cohort study examined 161 patients who were admitted with a diagnosis of HIV from June 2017 to May 2022. They were divided into 2 groups depending on the presence of pneumothorax during their stay. Multiple variables were studied, including age, gender, tuberculosis infection, pneumocystis jiroveci pneumonia (PJP)infection, bacterial pneumonia, and pneumothorax type and treatment course. RESULTS: There were 11 patients diagnosed with pneumothorax (prevalence rate: 6.8%). Bacterial lung infection was found in 9 (81.8%) of these patients, while fungal infection was found in 6 (54.5%) (p<0.001, 0.010). The MTB was found in 3 (27.3%) patients (p=0.728), while none were infected with PJP. Intercostal tube insertion was attempted in 9 (81.8%) patients, the mean duration of tube stay was 39.3±30.7 days, and the mortality rate was 72.7% (p=0.007). CONCLUSION: Pneumothorax in patients with HIV is a manifestation of the progression of the disease and its poor outcome. It has a complicated treatment course and a high mortality rate.

Medical and surgical management of pneumothorax in diving and hyperbaric chambers.

The presence of a pneumothorax within a pressurized chamber represents unique diagnostic and management challenges. This is particularly the case in the medical and geographic remoteness of many chamber locations. Upon commencing chamber decompression, unvented intrapleural air expands. If its initial volume and/or degree of chamber pressure reduction is significant enough, a tension pneumothorax will result. Numerous reports chronicle failure to diagnose and manage in-chamber pneumothorax with resultant morbidity and one fatal outcome. Such cases have occurred in both medically remote and clinically based settings. This paper reviews pneumothorax and tension pneumothorax risk factors and clinical characteristics. It suggests primary medical management using the principle of oxygen-induced inherent unsaturation in concert with titrated chamber decompression designed to prevent intrapleural air expanding faster than it contracts. Should this conservative approach prove unsuccessful, and surgical venting becomes necessary or otherwise immediately indicated, interventional options are reviewed.

Pneumothorax during manned chamber operations: A summary of reported cases.

In-chamber pneumothorax has complicated medically remote professional diving operations, submarine escape training, management of decompression illness, and hospital-based provision of hyperbaric oxygen therapy. Attempts to avoid thoracotomy by combination of high oxygen partial pressure breathing (the concept of inherent unsaturation) and greatly slowed rates of chamber decompression proved successful on several occasions. When this delicate balance designed to prevent the intrapleural gas volume from expanding faster than it contracts proved futile, chest drains were inserted. The presence of pneumothorax was misdiagnosed or missed altogether with disturbing frequency, resulting in wide-ranging clinical consequences. One patient succumbed before the chamber had been fully decompressed. Another was able to ambulate unaided from the chamber before being diagnosed and managed conventionally. In between these two extremes, patients experienced varying degrees of clinical compromise, from respiratory distress to cardiopulmonary arrest, with successful resuscitation. Pneumothorax associated with manned chamber operations is commonly considered to develop while the patient is under pressure and manifests during ascent. However, published reports suggest that many were pre-existing prior to chamber entry. Risk factors included pulmonary barotrauma-induced cerebral arterial gas embolism, cardiopulmonary resuscitation, and medical or surgical procedures usually involving the lung. This latter category is of heightened importance to hyperbaric operations as an iatrogenically induced pneumothorax may take as long as 24 hours to be detected, perhaps long after a patient has been cleared for chamber exposure.

Endobronchial valves for emphysema and persistent air-leak: 10-year experience in an Asian country.

BACKGROUND: Endobronchial valve (EBV) therapy, a validated method for bronchoscopic lung volume reduction (BLVR) in severe emphysema, has been explored for persistent air-leak (PAL) management. However, its effectiveness and safety in the Asian population require further real-world evaluation. In this study, we assessed the outcomes of treatment with EBV within this demographic. METHODS: We conducted a retrospective analysis of medical records from 11 Korean centers. For the emphysema cohort, inclusion criteria were patients diagnosed with emphysema who underwent bronchoscopy intended for BLVR. We assessed these patients for clinical outcomes of chronic obstructive pulmonary disease. All patients with PAL who underwent treatment with EBV were included. We identified the underlying causes of PAL and evaluated clinical outcomes after the procedure. RESULTS: The severe emphysema cohort comprised 192 patients with an average age of 70.3 years, and 95.8% of them were men. Ultimately, 137 underwent treatment with EBV. Three months after the procedure, the BLVR group demonstrated a significant improvement in forced expiratory volume in 1 s (+160 mL vs. +30 mL; P = 0.009). Radiographic evidence of lung volume reduction 6 months after BLVR was significantly associated with improved survival (adjusted hazard ratio 0.020; 95% confidence interval 0.038-0.650; P = 0.010). Although pneumothorax was more common in the BLVR group (18.9% vs. 3.8%; P = 0.018), death was higher in the no-BLVR group (38.5% vs. 54.5%, P = 0.001), whereas other adverse events were comparable between the groups. Within the subset of 18 patients with PAL, the predominant causes of air-leak included spontaneous secondary pneumothorax (44.0%), parapneumonic effusion/empyema (22.2%), and post-lung resection surgery (16.7%). Following the treatment, the majority (77.8%) successfully had their chest tubes removed. Post-procedural complications were minimal, with two incidences of hemoptysis and one of empyema, all of which were effectively managed. CONCLUSIONS: Treatment with EBV provides substantial clinical benefits in the management of emphysema and PAL in the Asian population, suggesting a favorable outcome for this therapeutic approach.

The sensitivity of a neck CT scan in detecting pneumothoraces in nonpenetrating trauma.

BACKGROUND: The trauma-related pneumothorax is a common intrathoracic injury and can go undetected with detrimental outcomes. Chest computed tomography (CT) investigation in low- to middle-income countries (LMIC) is not always readily available during emergency situations and increased workloads. However, alternative investigations all have limitations in including pneumothoraces. Patients may have trauma indications for CT of the head and neck. The neck CT scan may hold an extra potential advantage in pneumothorax detection. This study aimed to assess its sensitivity in pneumothorax detection in nonpenetrating trauma. METHODS: A retrospective study was conducted from 1 January 2016 to 31 December 2021. All adult patients sustaining nonpenetrating injuries, and investigated with chest and neck CT scans were included. The chest CT scan was the gold standard against,which the neck CT scan was compared to determine the accuracy of pneumothorax detection. Stata version 16 was used for descriptive statistical analysis, and a p-value of 0.05 was considered statistically significant. RESULTS: One thousand ninety three were eligible for evaluation; 204 (18.66%) pneumothoraces were detected on chest CT scans, 200 (98.0%) of which were also detected on the neck CT scan, producing a sensitivity of 98.0% (95% CI 95.1-99.5%) and a specificity of 100% (95% CI 99.6-100%). Most pneumothoraces were detected at T1 level (45.09%), followed by T2 (43.62%). CONCLUSION: The neck CT scan has demonstrated high sensitivity and specificity in pneumothorax detection. Thus, it may be used as an additional tool for those who could not receive or do not need a formal chest CT but have an indication for neck CT scans.

Pneumothorax during shoulder surgery. / Pneumothorax under skulderkirurgi.

Background: Pneumothorax following shoulder arthroscopy, although rare, is documented in over 30 PubMed case reports as occurring during or within 10 hours post-procedure. Case Presentation: A fit septuagenarian underwent a two-hour arthroscopic rotator cuff repair with IV anaesthesia and laryngeal mask airway, without a nerve block. With one hour remaining of the operation, the patient had desaturation and hypotension. Lung sliding was absent on ultrasound and x-ray confirmed left-sided tension pneumothorax. Successful thoracic drain insertion and lung re-expansion facilitated his recovery, allowing discharge after 24 hours and symptom-free status at 6 months. Interpretation: This case highlights pneumothorax as an uncommon yet possible post-arthroscopic event. The speculated aetiology is the surgical procedure, where pump-induced pressure fluctuations may displace air into surrounding tissue. Instances of pneumomediastinum and subcutaneous emphysema without pneumothorax suggest arthroscopic origin of air. Prompt perioperative ultrasound can aid in detecting such critical complications.

Pneumothorax, pneumomediastinum and subcutaneous emphysema as respiratory complications of COVID-19.

BACKGROUND: Pneumothorax, pneumomediastinum and subcutaneous emphysema are respiratory complications of Coronavirus disease 2019 occurring with noteworthy frequency in patients especially with severe disease. They can be life-threatening and often complicate patient managment. METHODS: This was a retrospective, observational study of patients admitted in Nepal Armed Police Force hospital from 13/05/2020 to 28/12/2021 diagnosed with pneumothorax, pneumomediastinum or subcutaneous emphysema singly or in combination. Data were collected from clinical charts, imaging records and electronic medical records of Severe Acute Respiratory Syndrome Coronavirus-type 2 positive patients 18 years and older. The frequency and type of the defined complications, the inflammatory markers and ventilatory parameters just prior to their diagnosis, the duration of hospitalization and ICU admission and in-hospital mortality rate were studied. RESULTS: Out of 4013 COVID-19 patients admitted in the hospital during the period, a total of 28 patients were observed to develop the complications, the overall incidence being 0.7% among hospitalized patients and 5.6% among ICU patients. The proportion of subcutaneous emphysema (64.3%) was highest followed by pneumomediastinum (46.4%) and then pneumothorax (39.3%) existing singly or in combination among the 28 patients, where four patients developed the complications spontaneously. Mean Positive End Expiratory Pressure of 12.1±2.6 cmH2O and Peak Inspiratory Pressure or Pressure Support of 30.9±10.3 cmH2O were observed for patients under positive pressure ventilation. Most of the patients who developed the complications (78.6%) died during treatment. CONCLUSIONS: Pulmonary air leak complications occur frequently in COVID-19 patients treated with or without positive pressure ventilation signifying increased disease severity, risk of ICU admission and high mortality rate. Hence, clinicians should be vigilant of these complications in all patients affected with COVID-19 and institute timely management.

Native-lung complications following single-lung transplantation for interstitial lung disease: an in-depth analysis.

BACKGROUND: Interstitial lung disease (ILD) represents a heterogeneous group of lung disorders characterized by fibrotic lung tissue changes. In regions with severe donor shortages, single-lung transplantation (SLTx) is often preferred over bilateral lung transplantation for advanced ILD. However, temporal changes and complications in the retained native lung remain poorly understood. METHODS: A retrospective analysis of 149 recipients who had undergone SLTx was conducted, including 34 ILD SLTx recipients. Native-lung volume, radiological alterations, and perfusion were assessed at distinct post-SLTx time points. Statistical analyses compared ILD and non-ILD SLTx groups. RESULTS: Our study revealed a progressive reduction in native-lung volume over time, accompanied by radiographic deterioration and declining perfusion. Complications in the retained native lung were observed, such as pneumothorax (29.4%), pulmonary aspergillosis (11.8%), and acute exacerbation (8.9%). Long-term survival rates were similar between ILD and non-ILD SLTx recipients. CONCLUSIONS: This study illuminates the unique challenges and complications with respect to the native lung following SLTx for ILD. Ongoing monitoring and tailored management are essential. Despite limitations, this research contributes to our understanding of the temporal progression of native-lung complications post-SLTx for ILD, underscoring the need for further investigation.