A three month old infant with severe respiratory distress.

This case report discusses the diagnostic challenge of congenital lobar emphysema (CLE) in a three-month old infant with severe respiratory distress. The infant was initially misdiagnosed and managed as a case of pneumothorax. This case highlights the importance of CT scans as a diagnostic tool for early diagnosis and lifesaving management of CLE. It also signifies the need for adequate funds and infrastructure in the health care system especially in rural areas of developing countries like Pakistan.

Management of Blunt Chest Trauma.

Common mechanisms of blunt thoracic injury include motor vehicle collisions and falls. Chest wall injuries include rib fractures and sternal fractures; treatment involves supportive care, multimodal analgesia, and pulmonary toilet. Pneumothorax, hemothorax, and pulmonary contusions are also common and may be managed expectantly or with tube thoracostomy as indicated. Surgical treatment may be considered in select cases. Less common injury patterns include blunt trauma to the tracheobronchial tree, esophagus, diaphragm, heart, or aorta. Operative intervention is more often required to address these injuries.

Chest tube removal at different gas flows in prolonged air leak: a randomized non-inferiority trial.

OBJECTIVES: To evaluate the safety and feasibility of removing drainage tubes at larger size of air leak in patients with prolonged air leak after pulmonary surgery. METHODS: Ninety-five patients who underwent pulmonary surgery with prolonged air leak in our centre were enrolled in this randomized controlled, single-centre, non-inferiority study. The drainage tube was clamped with a stable size of air leak observed over the last 6 h, which was quantified by gas flow rate using the digital drainage system. The control group (n = 48) and the study group (n = 46) had their drainage tube clamped at 0-20 ml/min and 60-80 ml/min, respectively. We continuously monitored clinical symptoms, conducted imaging and laboratory examinations, and decided whether to reopen the drainage tube. RESULTS: The reopening rate in the study group was not lower than that in the control group (2.08% vs 6.52%, P > 0.05). The absolute difference in reopening rate was 4.44% (95% confidence interval -0.038 to 0.126), with an upper limit of 12.6% below the non-inferiority margin (15%). There were significant differences in the length of stay [16.5 (13-24.75) vs 13.5 (12-19.25), P = 0.017] and the duration of drainage [12 (9.25-18.50) vs 10 (8-12.25), P = 0.007] between the control and study groups. No notable differences were observed in chest X-ray results 14 days after discharge or in the readmission rate. CONCLUSIONS: For patients with prolonged air leak, removing drainage tubes at larger size of air leak demonstrated similar safety compared to smaller size of air leak, and can shorten both length of stay and drainage duration. CLINICAL TRIAL REGISTRATION NUMBER: Name of registry: Gas flow threshold for safe removal of chest drainage in patients with alveolar-pleural fistula prolonged air leak after pulmonary surgery. Registration number: ChiCTR2200067120. URL: https://www.chictr.org.cn/.

[Diagnosis and management of idiopathic spontaneous pneumothorax in adolescents]. / Mise au point diagnostique et prise en charge du pneumothorax spontané idiopathique de l'adolescent.

INTRODUCTION: Due to the absence of consensual definition and agreed-upon pediatric treatment, pneumothorax (PNO) in children and adolescents often remains difficult to properly apprehend. STATE OF THE ART: While initial diagnostic suspicion is clinical, confirmation necessitates chest imaging, and lung ultrasound has become increasingly prevalent, often at the expense of chest radiography. The goal of treatment is twofold, on the one hand to a fully re-expand the lungs, and on the other hand to forestall PNO recurrence. Depending on PNO severity and clinical tolerance, it may be advisable to envision conservative management, oxygen supplementation, needle exsufflation, or chest tube drainage. PERSPECTIVES: In order to harmonize clinical practices, guidelines for the precise definition and graduated management of PNO in children and adolescents are highly advisable. CONCLUSIONS: Idiopathic spontaneous PNO frequently occurs in teenage populations, and its likewise frequent recurrence is not satisfactorily predicted by chest CT findings. It is of paramount importance that patients be fully informed of the risk of recurrence.

Neumotórax catamenial con fuga aérea persistente / Catamenial pneumothorax with persistent air leak

El neumotórax catamenial (NC) es aquel neumotórax espontáneo y recurrente que se presenta en mujeres en edad reproductiva y en relación temporal con la menstruación. Se han descrito múltiples variaciones en cuanto a la relación temporal, aunque suele producirse 24 horas antes del inicio de la menstruación o 72 horas después. Su consideración de patología poco frecuente podría deberse a que sea infradiagnosticada debido a la falta de conocimiento. El diagnóstico de NC no suele ser fácil; depende principalmente de la historia clínica pero también puede ser un diagnóstico quirúrgico o histopatológico. Las estrategias de manejo del NC pueden incluir cualquier combinación de terapia hormonal, pleurodesis, resección de parénquima pulmonar y resección/reparación del diafragma. Se presenta este caso de neumotórax catamenial de manifestación atípica para resaltar la importancia de tener un adecuado conocimiento de esta enfermedad que, por su aparente baja incidencia, puede pasar desapercibida. (AU)

Catamenial pneumothorax is a spontaneous recurrent pneumothorax that occurs in women of reproductive age and in temporal relationship with the menses. It usually occurs within 24 hours before or 72 hours after the start of the menstrual cycle; however, multiple variations have been described in terms of the temporal relationship. To date, it is considered a rare condition; this may be justified by the limited available knowledge on this disease leading to mis-diagnosis. Often, making a diagnosis of catamenial pneumo-thorax is complicated; it mainly depends on the medical history; also, it may be a surgical or histopathological diagnosis. Management strategies for catamenial pneumothorax may include combinations of hormonal therapy, pleurodesis, lung parenchyma resection, and diaphragm resection/repair.The aim of reporting this atypical case of catamenial pneumothorax is to highlight the relevance of having the adequate knowledge on this entity so it does not go unnoticed despite its apparent low incidence. (AU)

[Right upper lobe pulmonary sequestration as a rare cause of recurrent spontaneous pneumothorax]. / Sekvestratsiya verkhnei doli pravogo legkogo ­ redkaya prichina pravostoronnego retsidiviruyushchego spontannogo pnevmotoraksa.

A 19-year-old patient after previous wedge resection of the right upper pulmonary lobe a year ago urgently admitted with recurrent right-sided spontaneous pneumothorax. According to standard management of spontaneous pneumothorax, we performed diagnostic thoracoscopy and drainage of the right pleural cavity with regular X-ray examinations. However, these measures were ineffective. The patient was scheduled for surgery, and we intraoperatively observed an unusual cause of pneumothorax. Thus, we present spontaneous pneumothorax following right upper lobe pulmonary sequestration. The uniqueness of this case is associated with unusual manifestation and non-standard localization of rare lesion. A few cases of pneumothorax in similar patients are described in the world literature. The key limiting factor in diagnosis of such defects (identification of aberrant vessel supplying abnormal lung parenchyma) is the lack of routine CT angiography in patients diagnosed with pneumothorax. That is why CT changes were interpreted as postoperative ones, and the true cause was established only during redo surgery. A thorough inspection of the pleural cavity and alertness regarding unusual appearance of the right upper pulmonary lobe made it possible to suggest a non-standard diagnosis, avoid complications (bleeding from afferent vessel) and perform adequate lung resection.

Endometriosis first presenting in pleural fluid cytology.

Most patients with thoracic endometriosis present with catamenial pneumothorax, a rare condition in which recurrent episodes occur within 72 h before or after the start of menstruation. We report a case of thoracic endometriosis presenting with recurrent bloody pleural effusions without pneumothorax diagnosed on pleural fluid cytology. We describe the cytomorphology and immunoprofile of thoracic endometriosis and discuss the differential diagnoses, including neoplastic processes. We also highlight the importance of communication with clinicians for timeliness of diagnosis and treatment, especially when thoracic endometriosis is not suspected.

A Rare Presentation of Minimally Invasive Mesothelioma as a Large Tension Pneumothorax.

Development of mesothelioma is associated with asbestos exposure. Common presentations are with pleural-based plaques invading the chest wall and/or pleural effusion on chest imaging. The intent of this case report is to describe a rare presentation of mesothelioma, which presented atypically as a large tension pneumothorax. A 93-year-old male presented with a history of dyspnea that started after a coughing episode. On physical examination he was hemodynamically stable, but was hypoxic requiring 2L of supplemental oxygen. Computed tomography of the chest revealed a large right tension pneumothorax. A chest tube was placed and connected to suction (-20cmH20), but he continued to have an unresolving air leak over the following 2-week period. Upon video-assisted thoracotomy there were no blebs or adhesions seen. Right apical wedge resection and talc pleurodesis were performed. Pathologic examination revealed an atypical mesothelial cell proliferation with minimal, focal invasion into the pulmonary parenchyma. Tumor spread along the visceral pleura was thought to be the underlying cause of the pneumothorax. The surgical margins were uninvolved by the tumor, and the patient was later discharged home in stable condition. This was a rare presentation of what could best be described as minimally invasive mesothelioma arising in a background of probable mesothelioma in situ, which presented atypically as a large tension pneumothorax. This case highlighted the importance of establishing a pathologic diagnosis from pleural effusion cytology and/or pleural biopsy in persons presenting with spontaneous pneumothorax, and the difficulty in confirming a pathologic diagnosis of early mesothelial neoplasia.

Bilateral Pneumothorax after a Transbronchial Lung Cryobiopsy for Interstitial Lung Disease.

We herein report a case of bilateral pneumothorax after a unilateral transbronchial lung cryobiopsy (TBLC). A 73-year-old man with no history of cardiothoracic surgery underwent a TBLC for the reevaluation of interstitial lung disease. Five hours later, he developed bilateral pneumothorax, pneumomediastinum, and subcutaneous emphysema. He underwent bilateral chest drainage and was discharged 18 days later. The lung biopsy specimens obtained from the TBLC contained visceral pleura and bronchial cartilage, suggesting bronchial injury as the cause of the bilateral pneumothorax.

[Spontaneous pneumothorax: new concepts and current management]. / Pneumothorax spontané: nouveaux concepts et prise en charge actuelle.

Spontaneous pneumothorax is a classical medical condition encountered in emergency centers, and by primary care and respiratory physicians. The traditional distinction between primary and secondary pneumothorax, although old and increasingly blurred, still allows to guide initial management and to determine whether pleurodesis is indicated. In case of spontaneous pneumothorax, a targeted family history is essential because it can suggest the presence of a genetic syndrome as the underlying cause of the pneumothorax, a condition often associated with a high risk of pneumothorax recurrence, and the occurrence of extrathoracic manifestations which may be serious if recognized late. This review addresses the classification of spontaneous pneumothorax, its pathogenesis, the risk factors of occurrence including genetic syndromes, and its management.

Le pneumothorax spontané constitue une situation médicale classique rencontrée dans les centres d'urgence, chez le médecin de premier recours et le pneumologue. La traditionnelle distinction entre pneumothorax primaire et secondaire, bien qu'ancienne et de plus en plus incertaine, permet encore de diriger la prise en charge initiale et de décider si une pleurodèse est indiquée. En cas de pneumothorax spontané, une anamnèse familiale ciblée est primordiale car elle peut suggérer la présence d'un syndrome génétique à l'origine du pneumothorax, souvent associé à un risque élevé de récidive et à la survenue de manifestations extrathoraciques qui peuvent être graves si diagnostiquées tardivement. Cet article aborde la classification du pneumo thorax spontané, sa patho genèse, ses facteurs de risque y compris génétiques, et sa prise en charge.

Massive Subcutaneous Emphysema and Pneumothorax in a Case of Attacked by a Domesticated Bull - An Autopsy Based Diagnosis.

Subcutaneous emphysema is a condition when air or gas gets trapped within the subcutaneous layer. It is characterised by crackling feeling on palpation of the skin known as subcutaneous crepitation which is described as touching rice krispies. A 70 years male from hilly region of Nepal with agricultural background suffered multiple injuries sustained due to an attack by domesticated bull in his house. Upon the incident the injured male was taken to hospital, where he was declared "Brought Dead" by the Emergency Department of Dhulikhel Hospital, Kathmandu University Hospital. His body was brought for autopsy in Department of Forensic Medicine and Toxicology of Kathmandu University School of Medical Sciences. On complete autopsy, massive subcutaneous emphysema and pneumothorax was demonstrated. The details of finding are discussed in detail as follows.

A unique case of AH-dominant type nodular pulmonary amyloidosis presenting as a spontaneous pneumothorax: a case report and review of the literature.

Amyloidosis is a rare metabolic disorder primarily brought on by misfolding of an autologous protein, which causes its local or systemic deposition in an aberrant fibrillar form. It is quite rare for pulmonary tissue to be impacted by amyloidosis; of the three forms it can take when involving pulmonary tissue, nodular pulmonary amyloidosis is the most uncommon. Nodular pulmonary amyloidosis rarely induces clinical symptoms, and most often, it is discovered accidentally during an autopsy or via imaging techniques. Only one case of nodular pulmonary amyloidosis, which manifested as a spontaneous pneumothorax, was found in the literature. In terms of more precise subtyping, nodular amyloidosis is typically AL or mixed AL/AH type. No publications on AH-dominant type of nodular amyloidosis were found in the literature. We present a case of an 81 years-old male with nodular pulmonary AH-dominant type amyloidosis who presented with spontaneous pneumothorax. For a deeper understanding of the subject, this study also provides a review of the literature on cases with nodular pulmonary amyloidosis in relation to precise amyloid fibril subtyping. Since it is often a difficult process, accurate amyloid type identification is rarely accomplished. However, this information is very helpful for identifying the underlying disease process (if any) and outlining the subsequent diagnostic and treatment steps. Even so, it is crucial to be aware of this unit and make sure it is taken into consideration when making a differential diagnosis of pulmonary lesions.

Pulmonary interstitial glycogenosis in Birt-Hogg-Dubé syndrome-associated lung cysts: A new insight into the pathogenesis?

Multiple lung cysts are one of the major features of Birt-Hogg-Dubé syndrome (BHD), but little is known about their nature and pathogenesis. We report a case of a woman diagnosed with BHD lung cysts who exhibited pulmonary interstitial glycogenosis (PIG), a mesenchymal abnormality hitherto undescribed in this disease, in specimens resected at 14 and 29 years of age. Histopathologically, oval to spindle clear cells were seen in the subepithelial interstitial tissue of septal structures and the walls of the cysts. They had abundant periodic acid-Schiff-positive cytoplasmic glycogen. Immunohistochemically, these cells were positive for a few markers of mesenchymal stem cell-like lineage, including vimentin, CD44, and CD10, and negative for markers of epithelial or specific mesenchymal differentiation; these results were consistent with the reported immunophenotype of PIG cells. These PIG cells were more abundant in her specimen at age 14 years than in the second specimen from adulthood. The present case suggests that BHD lung cysts belong to a group of pulmonary developmental disorders characterized by combined PIG and alveolar simplification/cystic change. Disorders with PIG may persist until adulthood and may be of clinical and pathological significance.

[Soft tissue emphysema in spontaneous pneumothorax. Features of medical approaches]. / Massivnaya emfizema myagkikh tkanei pri spontannom pnevmotorakse. Osobennosti lechebnoi taktiki.

Soft tissue emphysema (including subcutaneous emphysema) is common in pneumothorax. In most cases, this condition is of little clinical significance and regresses under standard medical procedures. However, progressive soft tissue emphysema poses a threat to the patient's life in case of compression of the upper respiratory tract in some cases. The world literature describes various approaches to the treatment of these patients. Standard medical care for progressive soft tissue emphysema following pneumothorax is pleural drainage with active aspiration. Despite unequivocal treatment tactics, this may not be enough in case of massive air release. Surgical treatment may be accompanied by surgical and anesthetic difficulties including difficult intubation. The authors present persistent tension pneumothorax and soft tissue emphysema, features of surgical and anesthetic management, as well as current treatment options.

Clinical and genetic features of 334 Asian patients with Birt-Hogg-Dubé syndrome (BHDS) who presented with pulmonary cysts with or without a history of pneumothorax, with special reference to BHDS-associated pneumothorax.

BACKGROUND: The clinical pulmonary manifestations and genetic features of Birt-Hogg-Dubé syndrome (BHDS) in Asian patients remained unclear. We aimed to clarify the clinical features of BHDS-associated pneumothorax (PTX) and retrospectively investigate potential contributing factors in the largest Asian cohort to date. METHODS: We reviewed the clinical and genetic data collected in 2006-2017, from the BHDS patients who were Asian and presented with pulmonary cysts with or without a history of PTX. RESULTS: Data from 334 (41.3% males; 58.7% females) patients from 297 unrelated families were reviewed. Among them, 314 (94.0%) patients developed PTX. The median age at the first occurrence of PTX was 32 years, which was significantly lower in males (P = 0.003) and patients without notable skin manifestations (P < 0.001). Seventy-six (24.2%) patients experienced their first PTX episode before the age of 25 years. PTX simultaneously occurred in the bilateral lungs of 37 (11.8%) patients. Among 149 patients who had their first PTX episode at least 10 years before BHDS diagnosis, PTX occurred more frequently in males (P = 0.030) and light smokers than in nonsmokers (P = 0.014). The occurrence of PTX peaked in the early 30s and gradually decreased with age but remained high in females (P = 0.001). We identified 70 unique FLCN germline variants, including duplications (46.4%), substitutions (7.1%), insertions/deletions (30.0%), and variants affecting splicing (12.5%). Approximately 80% of Asian patients suspected of having BHDS could be genetically diagnosed by examining FLCN exons 7, 9, 11, 12, and 13. No apparent genotype-phenotype correlation regarding pulmonary manifestations was identified. CONCLUSIONS: Our findings indicate that sex, smoking history, and skin manifestations at BHDS diagnosis significantly influence the clinical features of BHDS-associated PTX. These findings may contribute to the appropriate management and treatment of BHDS-associated PTX.

Pulmonary Point-of-Care Ultrasonography in the Intensive Care Unit.

Pulmonary point-of-care ultrasonography (POCUS) is a quick and essential tool in the diagnosis of various pulmonary pathologies. Pulmonary POCUS can aid in the detection of pneumothorax, pleural effusion, pulmonary edema, and pneumonia, with sensitivity and specificity comparable, if not superior, to those of chest radiograph and chest computed tomography. Knowledge of anatomy and scanning of both lungs in multiple positions is essential for effective pulmonary POCUS. In addition to identifying pertinent anatomic structures such as the diaphragm, liver, spleen, and pleura and identifying specific ultrasonography findings such as A-lines, B-lines, lung sliding, and dynamic air bronchograms, POCUS helps detect pleural and parenchymal abnormalities. Proficiency in pulmonary POCUS is an attainable and essential skill in the care and management of the critically ill patient.

Expert Review on Spontaneous Pneumothorax: Advances, Controversies, and New Directions.

For decades, there has been scanty evidence, most of which is of poor quality, to guide clinicians in the assessment and management of pneumothorax. A recent surge in pneumothorax research has begun to address controversies surrounding the topic and change the face of pneumothorax management. In this article, we review controversies concerning the etiology, pathogenesis, and classification of pneumothorax, and discuss recent advances in its management, including conservative and ambulatory management. We review the evidence base for the challenges of managing pneumothorax, including persistent air leak, and suggest new directions for future research that can help provide patient-centered, evidence-based management for this challenging cohort of patients.