Lymphangioleiomyomatosis (LAM) is an abnormal proliferation of smooth muscle-like cells and may occur sporadically or in association with tuberous sclerosis complex. Patients are typically female, nonsmoking and may have cystic lung disease with pneumothorax. Diagnosis can be made by compatible imaging findings with a history of tuberous sclerosis complex, or in conjunction with vascular endothelial growth factor-D 800 pg/ml or greater, a highly specific finding. Sirolimus is first line treatment for LAM.

Primary spontaneous pneumothorax occurs in patients without apparent clinical lung disease, with a higher incidence in tall, thin males between the ages of 10 and 30. Tension pneumothorax is a life-threatening condition that can develop within minutes due to progressive air accumulation in the pleural space; mechanical pressure can lead to significant cardiorespiratory compromise. Tobacco association with a higher incidence of spontaneous pneumothorax has been well documented, but marijuana and spontaneous pneumothorax connection has not been well studied. However, it has been observed that patients who use marijuana and tobacco simultaneously have a higher incidence of spontaneous tension and larger pneumothoraces, as well as longer postoperative stay and higher recurrence than cigarette-only users. We present a case of a 26-year-old young male with a history only significant for excessive tobacco and marijuana smoking who developed multiple recurrent spontaneous pneumothorax and had to undergo right-sided video-assisted thoracoscopic surgery (VATS) with minimally invasive thoracotomy and had a prolonged hospital stay. With our case report, we hope to add to the evidence the effects of combined marijuana and tobacco smoking on bullous lung disease and pneumothorax while emphasizing the importance of conducting a detailed substance use history in patients with spontaneous pneumothorax.

Lymphangioleiomyomatosis (LAM) is a rare, slow-growing metastasizing neoplasm in which smooth muscle-like cells infiltrate the lung parenchyma and cause cystic lung damage. The common early symptoms include shortness of breath, pneumothorax, and chest pain. Lymphangioleiomyomatosis mainly involves the lungs, kidneys, and lymph nodes. This study reviews the characteristics of lymphangioleiomyomatosis to identify any possible changes in the prevalence of symptoms of the disease. We conducted a literature review of case reports on lymphangioleiomyomatosis from PubMed and Google Scholar. Variables of interest were age, gender, symptoms, vitals, immunostaining, and radiological findings. Data were transferred to an Excel spreadsheet (Microsoft Corporation, Redmond, WA), and mean, median, standard deviation, frequencies, and proportions were calculated using R version 1.1.456 (RStudio: Integrated Development for R. RStudio, PBC, Boston, MA). Lymphangioleiomyomatosis is a rare case and so not much of the literature could be found online. Thirty-three case reports were included in this study, and females accounted for 78.78% of the presentations. The average age was 38 years, SD 14.41 years. Shortness of breath was the most frequent symptom (60.6%), followed by pneumothorax (57.57%), chest pain (42.42%), cough (24.24%), and pleural effusion (1.25%).

Sjogren\'s syndrome (SS) is a chronic exocrinopathy caused by lymphocytic infiltration and is associated with numerous manifestations and morbidities. We discuss a case of a 60-year-old female who presented to the Acute Medical Assessment Unit complaining of progressive shortness of breath for one month, not associated with chest pain or lower limb swelling. She also reported joint pain involving both wrists and proximal interphalangeal (PIP) joints, oral dryness, hair loss, and numerous tongue ulcerations. Blood workup revealed triple-negative SS, negative rheumatoid factor, anti-SSA and anti-SSB, a high erythrocyte sedimentation rate (ESR), and antinuclear antibody (ANA) titer of 640. A diagnosis of SS was made. Nevertheless, her CT chest showed massive left-sided pneumothorax; subsequently, a chest tube was urgently inserted. The chest tube was removed two days later with complete resolution on chest X-ray (CXR). However, one week later, she presented with a recurrent pneumothorax that persisted and required surgical intervention that led to complete recovery afterward. Pneumothorax is an extremely rare but potentially unfavorable complication related to SS, with only two cases reported in the literature so far and usually associated with underlying lung pathology.

Granulomatosis with polyangiitis is a small vessel vasculitis that manifests as multisystemic inflammation predominantly affecting the lungs, upper respiratory tract, and the kidneys. Granulomatosis with polyangiitis commonly presents with elevated inflammatory markers and has a strong association with cytoplasmic antinuclear antibodies. Pulmonary manifestations of the disease include nodules, alveolar hemorrhage, and respiratory failure. The prevalence of pleural involvement is low, but can present as pleural effusion, wall thickening, and rarely pneumothorax. We describe the first report of recurrent pneumothorax secondary to presumed granulomatosis with polyangiitis.

Catamenial pneumothorax is a unique condition associated with thoracic endometriosis. It often presents in females of reproductive age as a recurrent pneumothorax aligned with the menstrual cycle. We present a case of a young female diagnosed with catamenial pneumothorax within one year of experiencing a stroke. The clinical presentation related to the stroke allowed for a unique diagnostic process and management considerations. The patient was successfully treated with progesterone-based contraception in the setting of an estrogen contraindication.

In this report, we describe a 35-year-old female with neurogenic thoracic outlet syndrome (NTOS) who presented with a post-pull pneumothorax following first rib resection and anterior scalenectomy (FRRAS). During the FRRAS, a large amount of scar tissue was observed surrounding the first rib, resulting in a tear in the pleura as the rib was resected. This created a pneumothorax which required chest tube placement and observation overnight. The patient was discharged 1 day post-operatively, feeling well but was then re-admitted 2 days later due to the presence of a recurrent pneumothorax. A chest tube was placed again, and the patient was monitored until symptom resolution. The occurrence of a post-pull pneumothorax has never been reported following FRRAS in TOS patients. The patient\'s symptoms have improved 4 months post operation.

OBJECTIVE: Primary spontaneous pneumothorax (PSP) is an urgent/emergency condition. Treatment with intercostal chest drainage (ICD) is necessary, particularly in symptomatic patients or those with tension. A previous study found that systematic breathing exercise significantly reduced ICD duration when compared with controls. This study aimed to evaluate if pulmonary rehabilitation can reduce the duration of ICD treatment in patients with PSP.
METHODS: This was a retrospective study of patients diagnosed with PSP treated with ICD. Duration of ICD treatment was recorded from patients\' medical charts. Factors associated with ICD duration were calculated using linear regression analysis.
RESULTS: There were 66 patients who met the study criteria, with average (SD) age and body mass index of 31.68 (13.53) years and 20.94 (2.72) kg/m2. The majority of the patients were male (72.73%), and average (SD) duration of ICD treatment was 9.90 (7.83) days. Three factors remained in the final model: body mass index, systolic blood pressure, and recurrent PSP. Two factors were independently associated with longer ICD duration: systolic blood pressure and recurrent PSP, with adjusted coefficients of 0.21 (p value 0.041) and 7.69 (p value 0.039), respectively. Pulmonary rehabilitation was not included in the final model.
CONCLUSIONS: Patients with a history of recurrent PSP or high systolic blood pressure at presentation may require longer ICD duration. Pulmonary rehabilitation was not associated with the duration of ICD treatment.

BACKGROUND: The typical non-muscle complications of long-surviving X-linked myotubular myopathy (XLMTM) include scoliosis, head deformity, macrocephaly, gastroesophageal reflux disease and peliosis hepatis. Recently, pulmonary blebs and recurrent pneumothorax have also been reported as uncommon complications, whereas no reports on autopsy cases have focused on lung lesions.
METHODS: An 8-year-old boy with XLMTM presented recurrent pneumothorax requiring bleb resection and pleurodesis. He subsequently developed multiple pulmonary mass lesions. He died of hemorrhagic shock due to peliosis hepatis. Autopsy showed multiple peliosis-like hematomas in the blebs of the lung. The histopathological examination of the hematomas revealed pooled blood without a pathway to bronchus. No apparent increase in desmin- or α-smooth muscle actin (α-SMA)-positive cells, namely myofibroblasts, was observed around hematomas, suggesting that the mutation in the myotubularin gene was involved in the defective repair process in the liver and lung tissues.
CONCLUSIONS: Recurrent pneumothorax should be considered as a non-muscle complication of XLMTM. Peliosis-like intrapulmonary hematoma may also be a critical complication caused by poor proliferation of myofibroblasts in the tissue repair process.

Pneumothorax is defined as the condition in which air is collected between the visceral and parietal pleura. Pneumothorax as a complication of coronavirus disease 2019 (COVID-19) infection has been reported in relatively few cases and recurrent pneumothorax is even rarer. We present a case of a 50-year-old critically ill patient who required mechanical ventilation for 55 days and developed recurrent bilateral pneumothorax. The patient initially presented with shortness of breath and cough. He was found to be COVID-19 positive on the polymerase chain reaction (PCR) test. Subsequently, his oxygen demand increased, and he ultimately needed mechanical ventilation. He developed four episodes of pneumothorax. The patient was managed in all four episodes with intercostal tube insertion. To prevent subsequent episodes, pleurodesis was performed after the fourth episode of pneumothorax.