A 24-year-Old woman with recurrent pneumothoraces.

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.

Recurrent Spontaneous Pneumothorax Secondary to Marijuana and Tobacco Abuse.

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.

The Epidemiology and Clinical Features of Lymphangioleiomyomatosis (LAM): A Descriptive Study of 33 Case Reports.

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%).

Catamenial pneumothorax, a commonly misdiagnosed thoracic condition: case report.

A 43-year-old woman presented with right-sided chest discomfort associated with dyspnea. The symptoms were related to the menstrual cycle and the patient has already presented several similar episodes. The Chest computed tomography (chest-CT) showed a partial right pneumothorax. A thoracoscopy was performed and demonstrated some diaphragmatic fenestrations. The diagnosis of a catamenial pneumothorax was established. The catamenial pneumothorax is a rare condition affecting the women in the reproductive period and is located most of the time in the right-side. The treatment is mainly surgical with the realization of a thoracoscopy. However, a medical treatment may be sometimes necessary.

Triple-Negative Sjogren's Syndrome and Recurrent Pneumothorax: An Uncommon Presentation of Autoimmune Disease.

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.

Catamenial Pneumothorax in the Setting of a Recent Stroke.

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.

Unusual Case of Recurrent Pneumothorax in Granulomatosis With Polyangiitis.

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.

Recurrent Pneumothorax Following First Rib Resection and Anterior Scalenectomy.

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.

An autopsy case of recurrent pneumothorax and peliosis-like intrapulmonary hematoma with X-linked myotubular myopathy.

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. CASE PRESENTATION: 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. CONCLUSION: 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.

Factors Associated with Duration of Intercostal Chest Drainage in Patients with Primary Spontaneous Pneumothorax and the Role of Pulmonary Rehabilitation.

PURPOSE: 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. PATIENTS AND 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. CONCLUSION: 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.

Recurrent Pneumothorax in an Adult Male With Bilateral COVID-19 Pneumonia.

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.

Endometriosis torácica como causa de neumotórax espontáneo recurrente en mujer de 53 años / Thoracic endometriosis presenting as recurrent spontaneous pneumothorax in a 53-year-old female

Una mujer de 53 años asintomática es diagnosticada de tercer episodio de neumotórax en hemitórax derecho. En episodios previos no se había objetivado causa del neumotórax salvo bullas subpleurales. Una videotoracoscopia muestra lesiones típicas de endometriosis torácica junto con múltiples perforaciones diafragmáticas. Tras la reparación quirúrgica la paciente recibió tratamiento anti-estrogénico con buena evolución. Se trata de un caso excepcional por su forma de presentación, por la edad de la paciente y por la afectación diafragmática. (AU)

A 53-year-old asymptomatic female presented with a third episode of spontaneous pneumothorax in right hemithorax. Previous studies did not demonstrate the etiology of this disease but only sub-pleural bullae. We found typical thoracic endometriosis lesions through video thoracoscopy. After surgical correction and anti-estrogenic treatment, the patient remains asymptomatic. This case is relevant due its presentation, diaphragmatic involvement and the age of the patient. (AU)

Ehlers-Danlos syndrome presenting as cystic lung disease with recurrent pneumothorax: a case report.

A 19-year-old male patient was referred to our hospital for recurrent pneumothorax. He previously experienced seven episodes of pneumothorax refractory to conventional treatment including pleurodesis and wedge resection. On admission, chest computed tomography scan showed multiple cystic lesions with surrounding ground-glass opacities and several nodules in both lungs. Detailed history revealed that the patient experienced haemoptysis whenever pneumothorax developed and had a family history of sudden death. Physical examination showed large eyes with conjunctival injection, hypermobile joints, and hyper-extensive and easily bruised skin. All these findings led to the suspicion of vascular Ehlers-Danlos syndrome (EDS). Genetic testing for the diagnosis of vascular EDS was performed and a heterozygous mutation in COL3A1 gene, c.1662+1G>A (IVS23(+1) G>A), was confirmed. Clinicians should consider vascular EDS as the differential diagnosis of cystic lung disease with recurrent pneumothorax.

Wedge resection on recurrent pneumothorax, failed lung expansion after needle aspiration: A case report.

BACKGROUND: Pneumothorax has several classifications, including based on etiology, location, extent, and degree of collapse as well as by mechanism and type. CASE PRESENTATION: A 61-years-old man with the main complaint of sudden shortness of breath after lifting a birdcage. The complaint worsened, and it was accompanied by nausea, sweating, and decreased vital signs. The patient was in a life-threatening condition with a tension pneumothorax and treated with needle aspiration (NA). On the second day of treatment, a clinical evaluation showed recurrent dyspnea. Lung physical examination and chest X-ray evaluation showed recurrent pneumothorax with subcutaneous emphysema. Installation of chest tube drainages (CTD) with active continuous suction of -20 cmH2O. High-resolution CT (HRCT) showed right pneumothorax with multiple blebs, bullae, and bronchopleural fistula. Video-assisted thoracic surgery (VATS) was carried out to repair bronchopleural fistula (BPF). However, pre-surgery found multiple bullae and multiple fistulas accompanied by adhesion to the chest wall, thus the procedure could not be conducted. As an alternative, thoracotomy was performed, followed by wedge resection and fistula reparation. DISCUSSION: Diagnosis of pneumothorax is based on clinical manifestations. Conservative management by providing oxygen or NA/CTD insertion. Needle aspiration is a simple and alternative treatment and performed for an outpatient indication, whereas CTD requiring hospitalization and is performed by experts. Management aims to restore clinical symptoms, restore lung expansion and prevent a recurrence. CONCLUSION: The choice of thoracoscopy/VATS or thoracotomy needs to be considered according to the indications so that complications do not occur and have a good prognosis.

Delayed recurrent spontaneous pneumothorax post-recovery from COVID-19 infection.

Pneumothorax, as a consequence of coronavirus disease 2019 (COVID-19) infection, has become an established entity but the delayed occurrence of pneumothorax, after recovery from the illness, is less commonly reported. We present a case of delayed recurrent spontaneous pneumothorax, presenting 4 weeks after recovery from COVID-19 in a previously healthy middle-aged gentleman, for which uniportal video-assisted thoracoscopic surgery (VATS) pleurectomy was performed, but the cause of pneumothorax could not be ascertained. This report brings to light, the importance of continued surveillance of COVID-19 survivors, the unpredictability of the disease process, and the challenges of thoracic surgery in this unique subset of patients.

Pleural aspergillosis in a patient with recurrent spontaneous pneumothorax: The challenge of an optimal therapeutic approach.

Pleural aspergillosis (PA) is a rare but potentially fatal disease. Most cases are secondary to bronchopleural fistulae or pleural intervention and can occur in the absence of immunosuppression. We report a case of PA in a young patient after pleurodesis for recurrent pneumothorax. Clinical resolution was achieved with systemic and local antifungal therapy combined with surgical debridement. Hepatotoxicity led to a switch from voriconazole to isavuconazole, with a successful outcome.

A delayed and recurrent pneumothorax complicating a fractured clavicle - A novel presentation.

There are very few reports in the literature about clavicular fractures being associated with a pneumothorax. With this combination of injuries, there are also minimal reports of a delayed presentation of a pneumothorax. This is the first report of a delayed pneumothorax followed by a recurrence of a pneumothorax due to the fractured ends of the clavicle. This case report describes a 49-year-old man who sustained a right-sided pneumothorax from a fractured clavicle several hours after a bus accident. His initial chest examination and radiographs showed no evidence of a pneumothorax. The pneumothorax resolved after 5 days of treatment with a thoracostomy tube. After removing the tube, the procedure was repeated later that day as he again developed a pneumothorax. Ten days later, the patient had surgical intervention of the clavicle due to the unresolved pneumothorax. The clavicle is usually managed conservatively in patients sustaining a clavicular fracture and pneumothorax, however, surgical intervention was mandatory based on failed conservative management. At 3 months follow up, the patient had normal shoulder function. Clinicians must be aware that fractured ends of the clavicle may cause repeated pulmonary damage resulting in a delayed and or a recurrent presentation of a pneumothorax.

Should surgical treatment of catamenial pneumothorax include diaphragmatic repair?

A best evidence topic in cardiothoracic surgery was written according to a structured protocol. The question addressed was whether treatment with hormones or pleural symphysis is better than operative procedures such as diaphragmatic repair with mesh to surgically manage recurrent pneumothoraces in patients with catamenial pneumothorax. Diaphragmatic repair with synthetic meshes, hormonal treatment and pleural symphysis are all accepted interventions for the treatment of recurrent catamenial pneumothoraces; however, there is uncertainty over the best combination of treatment. Altogether, 396 papers were found using the reported search, of which 13 represented the best evidence to answer the clinical question. However, it should be noted that the studies included were small in terms of sample size, and have demonstrated significant bias and surgical heterogeneity. Our literature review found that the recurrence rates of pneumothorax were greatly reduced in the treatment group where surgery and hormone therapy were combined (pooled average recurrence rate of 0%); however, the recurrence rates were significantly higher when these interventions were used alone: hormone therapy alone (58.5%), diaphragmatic repair alone (33.3%) and surgery alone (63.3%). Our results therefore demonstrate that a multimodality approach is required to reduce pneumothorax recurrence rates in patients with catamenial pneumothorax.

Linfagioleomiomatose pulmonar em paciente pediátrico: relato de caso / Pulmonar lymphagioleiomatosis in a pediatric patient: a case report

A linfangioleiomiomatose pulmonar (LAM) é considerada uma doença rara, com predileção de acometimento em mulheres durante período reprodutivo, principalmente entre a terceira e quarta década de vida. A doença é atualmente reconhecida como uma neoplasia de baixo grau e se caracteriza por proliferação de células musculares lisas atípicas no parênquima pulmonar. Dispneia, pneumotórax recorrente e tosse seca são as manifestações clínicas mais comuns. Em exames de imagem, é notada comumente a existência de hiperinsuflação, infiltrado intersticial reticulonodular e cistos de diferentes tamanhos em topografia pulmonar. A presença concomitante de angiomiolipomas pode ser verificada em alguns pacientes. Tais achados radiológicos, quando associados ao quadro clínico característico , falam a favor do diagnóstico de LAM, sendo possível a confirmação pela histopatologia. A despeito das diferentes formas de tratamento existentes para a LAM, ainda não existe medida curativa para a doença. Em geral, os pacientes evoluem com contínua deterioração da função pulmonar, com predomínio de um distúrbio ventilatório obstrutivo que deve ser diferenciado de outras afecções mais prevalentes, como asma e doença pulmonar obstrutiva crônica. Neste trabalho, relata-se um caso de paciente em idade pediátrica, com presença de achados clínicos e radiológicos compatíveis com o diagnóstico de LAM, ainda que fora da faixa etária usual de acometimento da doença.

Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease which affects women during reproductive age mainly between thirdies and forties. Currently the disease is recognized with a low grade neoplasm and it is characterized by proliferation of atypical smooth muscle cells in the lung parenchyma. Dyspnea, recurrent pneumothorax and dry cough are the most common clinical manifestations. On imaging studies, the presence of hyperinsuflation, interstitial infiltrates reticulonodular and cysts of different sizes in lung topography is common. The concomitant presence of angiomyolipoma can be recorded in some patients. When associated with the usual clinical condition, the radiological findings suggest the diagnosis of LAM and it can be confirmed by histopathology. Despite of different forms of treatment for LAM, there is still no curative method for it. In general, the patients develop continuous deterioration of lung function with a predominance of obstructive lung disorder which must be distinguished from other more prevalent diseases, such as asthma and chronic obstructive pulmonary disease. In this paper, we report a case of a child with the presence of clinical and radiological findings compatible with the diagnosis of LAM although it is outside the usual age of onset of the disease.

[Catamenial pneumothorax - case reports and literature review]. / Katameniální pneumotorax - kazuistiky a literální prehled.

AIM: Catamenial pneumothorax is menstrual cycle dependent and represents the most common form of thoracic endometriosis syndrome. Recurrences are very common even after surgical resection. DESIGN: Case reports and literature overview. SETTING: Department of Respiratory Medicine, Department of Surgery I, Department of Obstetrics and Gynecology, University Hospital, Palacky University, Olomouc. CASE REPORT: Two cases of catamenial pneumothorax are presented with repeated recurrences of right-sided pneumothorax even after surgical treatment. Histopathologic finding of pelvic endometriosis was shown in both patients. CONCLUSION: Catamenial pneumothorax is usually connected with thoracic endometriosis and also includes catamenial hemothorax, recurrent catamenial hemoptysis, catamenial pleural pain as well as endometriosis lung nodules. Multidisciplinary approach including pneumologist, thoracic surgeon and gynecologist with early postoperative hormonal treatment that deals with the main chronic systemic disease may lead to improved results, mainly reduced recurrence rates of catamenial and/or endometriosis related pneumothorax.