Air Cysts Research Articles

Tracheal Diverculum: A Rare Entity Diagnosed in the Context of Blunt Thoracic Trauma Report of A Case

Tracheal diverticulum (TD) is an outpouching or sac arising from the wall of the trachea. It can can be classified, based on etiology, in congenital and acquired. Congenital tracheal diverticulum is the consequence of a defect in endodermal differentiation of the membranous posterior tracheal wall and it contains all the layers of the airway wall (true diverticulum). Acquired diverticulum is the result of an abnormally increased pressure inside the airways pushing the respiratory mucosa out of a defect of the airway wall; it contains only mucosa (pseudodiverticulum). Both types are rare, accounting only for a limited percentage of the paratracheal air cysts (PTACs) from which TD should be differentiated in consideration of the similar basic pattern of “paratracheal air collection”. Diagnosis of TD is difficult because of its rarity, lack of symptomatology or specific symptomatology and low sensibility of the diagnostic tools available to date (chest CT and bronchoscopy). Apart from its intrinsic rarity, the case of TD we present here has the peculiarity of being diagnosed, differently from usual, by direct bronchoscopic visualization. The blunt thoracic trauma of which the patient was victim, gives more exemplarity to the diagnostic pathway of this entity. Indeed the detection of a para tracheal air collection on CT images could have been interpreted as a traumatic rupture of the trachea rather than a TD, leading to an unjustified surgical treatment. It was only clinical judgment combined with the use of the diagnostic imaging and bronchoscopy that conducted to the correct diagnosis.

Paratracheal air cysts are uncommon findings in the pediatric population.

To determine the prevalence of paratracheal air cysts (PACs) in the pediatric population. The chest CT images of pediatric patients between July 2007 and December 2014 were retrospectively reviewed for the presence and imaging findings of PACs. In addition, the association between PACs and the presence of bronchiectasis and air cystic pulmonary lesions were evaluated. Among a total of 819 pediatric patients (males=527, 64.3%), the overall prevalence of PACs was 1.3% (n=11; 1.7% for infants, 0.4% for children, and 1.9% for adolescents). The presence of PACs showed no association with gender, age groups, or the presence of bronchiectasis and air cystic pulmonary lesions (P=0.56, 0.88, 0.57 and 0.89, respectively). All PACs were located at the right side of the trachea at the thoracic inlet, and the median transverse diameter was 3.7mm (range 2.0-7.0mm); one PAC showed septation, and 27.3% of PACs had communication with the trachea. In the pediatric population, the prevalence of PACs is 1.3% as detected by chest CT. Knowledge of the prevalence and imaging findings of PAC would be useful to prevent confusion with pneumomediastinum or other cystic lesions in pediatric patients.

Chronic interstitial pneumonia in young patients undergoing lung transplantation or autopsy: clinico-radiologic-pathologic observations from a single institution.

To retrospectively evaluate high-resolution computed tomography (HRCT) findings and clinical diagnoses of chronic interstitial pneumonia (IP) with a poor prognosis in young patients (≤50years). HRCT images of 8 men and 7 women (mean age 34.8years) obtained before lung transplantation or autopsy were reviewed. After reviewing whole lung specimens and pathologic diagnoses, all patients were clinically diagnosed according to the 2010 idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) consensus statement. HRCT images revealed intralobular reticular opacity, air cysts, ground glass opacity, traction bronchiectasis, and interlobular septal thickening. Intralobular reticular opacity was the most extensive finding. Abnormal findings existed predominantly in both the peripheral and lower lung zones in only 1 patient. Classifications of HRCT patterns were "UIP" (n=2), "inconsistent with UIP" (n=11), and "indeterminate UIP" (n=2). Multidisciplinary diagnoses were "IPF/UIP" (n=1), "possible IPF/UIP" (n=1), "IP with connective tissue disease" (n=7), "fibrotic nonspecific IP" (n=1), and "unclassified IP" (n=5). The most extensive HRCT finding was intralobular reticular opacity. Most HRCT images differed from typical IPF/UIP, and IPF/UIP was uncommon in young patients with chronic IP with a poor prognosis.

Is There a Relationship Between Paratracheal Air Cysts and Upper Lobe Fibrosis?

The purpose of this study was to determine the characteristics of paratracheal air cysts (PACs) and their relationship with upper lobe pulmonary fibrosis. The routine thoracic computed tomography scans of 3549 patients carried out between January 2014 and April 2015 were retrospectively evaluated. The presence, location, structural characteristics (uniloculated or multiloculated), number of cysts, and anterior-posterior and transverse dimensions of the PAC and its communication with the tracheal lumen were evaluated. The presence of upper lobe fibrosis, emphysema, and bronchiectasis was also evaluated. The relationship between upper lobe fibrosis, emphysema, bronchiectasis, and the presence of paratracheal cysts was evaluated in all patients. An equal number of randomized patients with no paratracheal cysts were selected as a control group. A total of 190 PAC cases were diagnosed, with a prevalence rate of 5.35%: 146 (76.8%) of the cases were men, 44 (23.2%) were female, and the mean (SD) age was 53.79 (16.64) years (range, 12-89 years). The control group included 105 men (57.4%) and 78 women (42.6%), and the mean (SD) age was 53.87 (16.65) years (range, 13-87 years). The groups were similar in terms of age (P = 0.876), whereas the proportion of men in the PAC group was significantly higher (P < 0.001). Most of the PACs were located on the right side (n = 188, 98.9%). The incidence of fibrosis in the paratracheal cyst group was 45.8% (n = 87) compared with 19.5% (n = 37) in the control group; this difference was statistically significant (P < 0.001). The prevalence rates of emphysema in the cyst group and the control group were 29.5% (n = 56) and 11.6% (n = 22), respectively, and the difference was statistically significant (P < 0.001). When the groups were compared regarding bronchiectasis (scar and non-scar related), the rate of bronchiectasis in the paratracheal cyst group was 17.9% (n = 34), compared with 3.7% (n = 7) in the control group; this difference was statistically significant (P < 0.001). Paratracheal air cysts are relatively common, but the etiology is still unclear. We detected increased rates of cyst formation that may result from the traction effect on the tracheal wall in the presence of upper lobe fibrosis.

Pulmonary Lymphangioleiomyomatosis on a Post-Menopausal Woman with Chronic Lymphocytic Leukaemia

A 62 years old, post-menopausal female was admitted to the Internal Medicine Ward due to dyspnoea, cough and sputum of at least 2 months. Shortness of breath, cough and hypoxaemia persisted and the patient was submitted to a pulmonary angiogram ct which revealed numerous thin-walled air cysts affecting upper and medial zones of both lungs, typical images of pulmonary lymphangioleiomyomatosis. After discharge to Internal Medicine Consultation Service with Metilprednisolone, the patient was no longer hypoxaemic and remained asymptomatic, even after withdrawal of oral corticosteroid to inhalatory formulation. Further surveillance in short time was scheduled in order to implement rapid imunossupressive treatment when necessary.

Neurofibromatosis With Recurrent Spontaneous Pneumothorax

SESSION TITLE: Disorders of the Pleura Global Case Reports SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM INTRODUCTION: Neurofibromatosis(NF); is a genetic heterogeneous disease of nervous system, skin, eyes, lungs and skeletal system. NF is classified in two groups of NF1 and 2. NF-1 is the most common form and affects 1 in about 4000 births in the world (1) CASE PRESENTATION: We present a patient from our clinic who is diagnosed with Neurofibromatosis and recurrent spontaneous pneumothorax. Our patient was 28 years old male who had received the diagnosis of pneumothorax for two times. He is an active smoker and suffering from chest pain, shortness of breath and trembling hands. His first degree family history revealed malignancies. His mother and sister have the similar skin lesions. Physical examination findings are decreased breath sounds, bilateral axillary freckling, café au lait spots from 6 to 15 mm and multiple neurofibromas. We performed Computed Tomography(CT) and revealed multiple air cysts measuring up to 25x13 mm in the upper lobes. Cranial CT revealed subcutaneous nodules on the right parietal and left frontal region. After the EMG VEP (visuel evoked potential), P100 latency in the study was normal. On the eye examination, bilateral Lisch nodules of the iris and congenital subcapsular cataracts were detected. Also he has a slight mix-type hearing loss in the right ear detected by audiometric evaluation. So we recommend smoking cessation and control chest CT scannings at regular intervals. DISCUSSION: Neurofibromatosis is a rare but important disease. Thoracic manifestations of the disease include intrathoracic neurogenic tumors, meningoceles, kyphoscoliosis and pulmonary fibrosis. Chest radiographic findings of these patients are large apical asymmetric thin-walled bullae (73%) and subpleural reticular abnormality (61%). There is a relationship between neurofibromatosis and interstitial lung disease. Honeycombing may be seen in neurofibromatosis rarely. Some investigators suggest that the pulmonary parenchymal disease of neurofibromatosis is attributed to a mesenchymal defect, resulting in primary deposition of collagen. Also, it has been suggested that neurofibromatosis may increase the sensitivity of the lungs to cigarette smoke which cause the early development of emphysema-like changes. So smoking is a risk factor for the development and severity of diffuse lung diseases in neurofibromatosis. CONCLUSIONS: As a result, because neurofibromatosis is a rare disease and may predispose to the tumor growth of the nervous system or other organs (2); careful long-term follow up after dignosis is therefore required (3). Reference #1: Pivnick EK, Riccardi VM. The neurofibromatosis. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, Fitzpatrick TB, eds. Fitzpatrick's Dermatology in General Medicine. 5 th ed. New York: Mc Graw-Hill; 1999. p.2152-8. Reference #2: Hirsch NP, Murphy A, Radcliffe JJ. Neurofibromatosis: Clinical presentations and anaesthetic implications. Br J Anaesth 2001; 86: 555-64 Reference #3: Gawlewicz-Mroczka A, Mastalerz L, Nizankowska-Mogilnicka E. Neurofibromatosis type 1 in an adult diagnosed by a pulmonologist. Pneumonol Alergol Pol 2009;77:474-8 DISCLOSURE: The following authors have nothing to disclose: Seher Satar, Aysenaz Ozcan, Tugba Kara, Sema Canbakan No Product/Research Disclosure Information

Paratracheal air cysts: prevalence and relevance to pulmonary emphysema and bronchiectasis using thoracic multidetector CT

We aimed to determine the prevalence of paratracheal air cysts (PTACs) and the relationship of PTACs with emphysema and bronchiectasis through retrospective analysis of multidetector computed tomography (MDCT) findings. MDCT findings of 1027 consecutive patients who underwent routine thorax examination between January 2012 and January 2013 were evaluated retrospectively for the presence of PTACs. Localization of the PTACs, as well as their size, shape, and relationship with the trachea were examined. Presence of emphysema and bronchiectasis was recorded, and bronchiectasis severity index was calculated when present. We randomly selected 80 patients who had no visible PTACs as the control group. The findings of patients with and without PTACs were compared. PTACs were determined in 82 of 1027 patients (8%), in 8.8% of females and 7.3% of males. The presence of PTACs was determined to be independent of gender (P = 0.361). Eighty-one PTACs (98.8%) were located in the right side of the trachea and 56.1% had a tracheal connection. The presence of PTACs significantly correlated with the presence and severity of bronchiectasis (P = 0.001 and P = 0.005 respectively). There was no significant relationship between the presence of PTACs and the presence of emphysema on CT images (P = 0.125). The prevalence of PTACs was determined as 8% in this study. There was significant association between PTACs and bronchiectasis.

The Use of Hyperbaric Oxygen Therapy in Dentistry.

Dear Editor, We appreciate the article “Hyperbaric Oxygen Therapy(HBOT) - Can It Be the New Era in Dentistry?” written by Devaraj et al., [1]. In present review, the authors have mentioned the history, mode of action, indications, contraindications, complications and the applications of hyperbaric oxygen therapy in dentistry. Thanks to the authors for their contribution on the literature. HBOT is increasingly used to treat the many conditons. In the present review, HBOT is defined as administration of 100% oxygen to a patient with a pressure greater than one atmosphere at sea level. According to the Undersea and Hyperbaric Medical Society (UHMS) committee report 2003 and most hyperbaric medicine specialists, the oxygen pressure should be applied 1.4 atmospheres absolute (ATA) as the minimum therapeutic dosage to gain benefit from hyperbaric oxygen therapy. Besides the mentioned 13 approved indications of hyperbaric oxygen therapy, sudden hearing loss is also approved by UHMS Board of Directors since October 8, 2011 [2]. The authors stated “Asymptomatic pulmonary lesions which are seen on chest. X- ray” as one of the contraindications of hyperbaric oxygen therapy. We agree with the authors. Air cysts or blebs in the lungs may predispose pulmonary barotrauma (PBT) by causing air trapping. Interestingly, Toklu et al., surveyed 98 European HBO centres for their attitude to patients with bleb and bullae. They found that a significant portion of the HBO centers accept patients with pulmonary bleb or bullae, X-ray is the mostly used screening tool for patients with a history of pulmonary disease and the prevalence of pulmonary barotrauma in these centers was found only 0.00045%, surprisingly [3]. These study results make us to question the outcome of asymptomatic pulmonary lesions. Furthermore X-Ray screening tool has a low sensitivity for this kind of lesions. The authors mentioned about the pressure equalization problems which predominantly affect the middle ear and the nasal sinus. We think that additionally to these concerns, barotraumatic lesions can occur in any trapped air e.g. dental abscess after failed filling or in periodontitis and subgingival anaerobes which may cause gaseous lesions. These lesions can cause a very strong sharp pain particularly in the ascent phase of the hyperbaric oxygen therapy when the pressure decreases and volume begins to re-expanse. As a conclusion, hyperbaric oxygen therapy provides the most benefit in tissues with vessels which have good blood flow, the anatomical structure of the mouth with its rich vascular beds is an advantage to benefit from this treatment. So we support the idea of using this treatment modality in dentistry.

Tracheal Diverticulum: A Unique Case with Intraoperative Morphologic Assessment.

There are rare case reports of tracheal diverticula or paratracheal air cysts. These cases, however, were reported mostly as incidental sonographic or radiologic findings without histologic confirmation. Furthermore, the handful of studies that describe this entity histopathologically report only cases in patients with prior respiratory symptoms. Here, we report a rare case of an asymptomatic 60-year-old female with no significant past medical history who presented with primary hyperparathyroidism. She was found to have an incidental right paraesophageal air-filled diverticulum with multiple thin septations on her imaging studies. She was taken to surgery and the histologic examination of the specimen revealed multiloculated cystic cavity lined by respiratory-type columnar epithelium with lymphocytic infiltrate and minor salivary glands within the surrounding stroma, rendering the diagnosis of tracheal diverticula.

An investigation into the prevalence of paratracheal air cysts revealed on multidetector computed tomography.

Paratracheal air cysts (PTACs) are frequently and incidentally found on computed tomography (CT) examinations of the chest, neck, and spine. Aetiology, pathophysiology, clinical and radiological relevance, and accompanying airway and parenchymal pathologies of PTACs are not known. A limited number of studies have discussed the association between PTACs and radiological or clinical abnormalities. Goo et al. and Kim et al. reported a relationship between emphysema and PTACs. However, Cheng et al., Buterbaugh et al., and Bae et al. found no association between emphysema and PTACs. Most patients are asymptomatic. The aim of this study was to investigate the prevalence of PTAC and the association between the PTACs and emphysema, bronchiectasis, pneumothorax, bullea, and sternotomies on multidetector CT (MDCT) examinations.

Maxillary Air Cyst

Pathologic dilatation of the maxillary sinus by air is a rare condition with unclear etiology. We present a case of a 17 year old male with a maxillary air cyst diagnosed by computed tomography. The CT demonstrated air-filled expansion of the maxillary sinus beyond the normal anatomical limits with associated cortical bone thinning. The case report highlights the pathognomonic computed tomography findings of this rare entity and discusses the perplexing nomenclature, proposed etiologies and various treatment options.

CT Findings of Thoracic Manifestations of Primary Sjögren Syndrome: Radiologic-Pathologic Correlation

Primary Sjögren syndrome is an immune-mediated exocrinopathy characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands. Various systemic extraglandular disorders are associated with primary Sjögren syndrome, and the thorax is commonly affected. The pulmonary manifestations of primary Sjögren syndrome may be categorized as airway abnormalities, interstitial pneumonias, and lymphoproliferative disorders; in each category, bronchiectasis or centrilobular nodules, nonspecific interstitial pneumonia, and lymphoid interstitial pneumonia are common. These manifestations do not usually occur in isolation; they are concomitantly seen with other types of lesions. Mucosa-associated lymphoid tissue (MALT) lymphoma and amyloidosis are key components of lymphoproliferative disorders, and MALT lymphoma should always be considered because its morphologic characteristics are similar to those of benign lymphoproliferative disorders. Amyloidosis is rare but important because it carries a risk for underlying MALT lymphoma or plasmacytoma, and it may lead to hemoptysis during biopsy. In addition, thin-walled air cysts are characteristic of primary Sjögren syndrome, irrespective of the main pulmonary manifestations. Lymphadenopathy and multilocular thymic cysts may be seen in the mediastinum. During the follow-up period, there is a risk for acute exacerbation of interstitial pneumonia and development of malignant lymphoma. Often, primary Sjögren syndrome is subclinical, but there are various underlying risks. Thus, imaging findings are important. In addition to the various types of interstitial pneumonia and airway abnormalities, air cysts and mediastinal manifestations may help diagnose primary Sjögren syndrome.

Formation of a parenchymal pseudocyst following a blunt thoracic trauma

Introduction Pulmonary pseudocyst is an uncommon condition that may occur after blunt thoracic trauma. Traumatic pulmonary pseudocysts develop in less than 3% of patients after blunt thoracic trauma. In this study we share a “formation of a pulmonary pseudocyst following a blunt thoracic trauma” case. Case presentation A 34-year-old male patient suffering from a motor vehicle accident, who had admitted to another medical unit, was referred to our ED for advanced treatment after the first intervention. Due to wide hemopneumothorax, bilateral tube thoracostomy had been performed when patient arrived. Thorax CT revealed a pulmonary pseudocyst in the right lung beside diffuse bilateral pulmonary contusions, hemopneumothorax and rib fracture. Following necessary medical treatments, patient was admitted to the intensive care unit. After three months with clinical and radiological follow-up, traumatic pulmonary pseudocyst in the right lung was fully healed. Discussion and Conclusion Pulmonary pseudocysts are air cysts that may occur in the lung parenchyma after blunt thoracic trauma. Around 85% of cases reported in the literature involved young and male patients. Tears occurring in the lung parenchyma are one of the underlying mechanisms of pseudocyst formation. In these cases there is no need for further treatment of these cysts which are known to recede spontaneously unless no complication occurs.

Atypical histiocytosis in the lung.

To report a rare case of atypical histiocytic tumor of the lung with a review of literature. The clinical materials were noted. Literature related to this condition from the past 50 years was reviewed from the group of histiocytic tumors. Clinical manifestations were non-specific. The imaging characteristics of our case were infiltrative lesions with multiple cysts in both lungs. Pathology showed nodular proliferation of atypical cells. Immunohistochemistry suggested a histiocytic origin of the infiltrating atypical cells. Because the pathological findings did not fall into any particular category of typical histiocytic tumors, the final diagnosis was atypical histiocytic tumor. The presentation of atypical histiocytic tumor of the lungs, only, with infiltrative lesions and multiple air cysts seems very rare, with pathological examination being "gold standard" for the diagnosis.

Paratracheal air cysts: Prevalence and correlation with lung diseases using multi‐detector CT

The purpose of this study was to determine the prevalence of paratracheal air cysts (PACs), their correlation with different lung diseases and their connection with the trachea by chest multi detector computed tomography (MDCT). We retrospectively reviewed chest MDCT images of 8240 consecutive patients obtained from January 2010 to December 2011 with a 16-detector multi-detector CT scanner. PACs were assessed for prevalence, location, level, size and the presence of visible communication with the trachea. MDCT diagnoses were classified as normal, primary or metastatic malignancies, chronic obstructive pulmonary disease (COPD), pneumonia and other lung diseases. We randomly selected 330 patients who had no visible PACs for the control group. We evaluated the associations between patients' demographic findings (age and sex), MDCT findings of lung and the presence of PACs. The findings of the PACs and control groups were compared. Statistical analysis used chi-squared test and Mann-Whitney U-test for evaluation. PACs were presented in 301 patients (4%); 204 men and 97 women, ranging in age from 14 to 91 years (median = 57 years). There was no significant difference in the presence of PACs by age (P > 0.05). Male subjects showed higher prevalence (P = 0.005). Fifty PACs (16.6%) showed communication with the trachea or main bronchus. Although the relation between COPD and PACs was statistically significant (P < 0.001), there was no relation between primary or metastatic malignancies, pneumonia and other lung diseases and PACs. PACs are common in MDCT and should not be misdiagnosed as pneumomediastinum. It should be kept in mind that PACs may be associated with COPD.

“Air cyst” of the neck

European Annals of Otorhinolaryngology, Head and Neck Diseases - Vol. 130 - N° 4 - p. 237-240

Unusual Neurological Syndrome Induced by Atmospheric Pressure Change

We describe a case of a 46-yr-old female who developed hypertension, tachycardia, dysarthria, and leg weakness provoked by pressure changes associated with flying. Typically during the landing phase of flight, she would feel dizzy and note that she had difficulty with speech and leg weakness. After the flight the leg weakness persisted for several days. The symptoms were mitigated when she took a combined alpha-beta blocker (labetalol) prior to the flight. To determine if these symptoms were related to atmospheric pressure change, she was referred for testing in a hyperbaric chamber. She was exposed to elevated atmospheric pressure (maximum 1.2 ATA) while her heart rate and blood pressure were monitored. Within 1 min she developed tachycardia and hypertension. She also quickly developed slurred speech, left arm and leg weakness, and sensory changes in her left leg. She was returned to sea level pressure and her symptoms gradually improved. A full neurological workup has revealed no explanation for these findings. She has no air collections, cysts, or other anatomic findings that could be sensitive to atmospheric pressure change. The pattern is most consistent with a vascular event stimulated by altitude exposure. This case suggests that atmospheric pressure change can produce neurological symptoms, although the mechanism is unknown.

Recurrent Spontaneous Pneumothorax during the Recovery Phase of ARDS Due to H1N1 Infection.

The pregnant patients are prone to influenza A (H1N1) virus infection, which may rapidly progress to lower respiratory tract infection and subsequent respiratory failure and acute respiratory distress syndrome (ARDS). Pneumothorax might develop in ARDS under mechanical ventilation. But post-ARDS pneumothorax in spontaneously breathing patient has not been reported in the literature. We report a 31-year old pregnant woman infected with influenza A (H1N1) virus and progressed to ARDS. Mechanical ventilation with high PEEP improved patient's gas exchange parameters within 3 weeks. However spontaneous pneumothorax was developed one week after she weaned off the ventilator. After successful drainage therapy, the patient was discharged. However she re-admitted to the hospital because of a recurrent pneumothorax one week later. She was discharged in good health after being treated with negative continuous pleural aspiration for 10 days. Influenza might cause severe pulmonary infection and death. In addition to diffuse alveolar damage, sub-pleural and intrapulmonary air cysts might occur in influenza-related ARDS and may lead to spontaneous pneumothorax. This complication should always be considered during the recovery period of ARDS and a long-term close follow-up is necessary.

Differential diagnosis of cystic lung diseases

A lung cyst is defined as a round parenchymal lucency or area of low attenuation with a thin wall. Although lymphangioleiomyomatosis (LAM) and Langerhans cell histiocytosis (LCH) are the most frequently encountered causes, the differential diagnosis for diseases characterised by diffuse lung cysts is broad ranging from isolated chest disorders to rare multisystem diseases. This article will illustrate and describe the spectrum of diseases associated with air cysts at high-resolution CT (HRCT), highlighting disorders in which the HRCT findings can be diagnostic.

Pulmonary lymphangioleiomyomatosis: a rare case

Lymphangioleiomyomatosis is an uncommon lung disease primarily affecting women of childbearing age. It is characterized by the progressive proliferation and infiltration of smooth muscle-like cells, which lead to cystic destruction of the lung parenchyma; obstruction of airways, blood vessels, and lymphatics; and loss of pulmonary function. We present the case of a 46-year-old female patient with chest pain, cough, sputum, and dyspnea on exertion for three weeks. Minimal pneumothorax was noted, and the patient was referred to our center for further investigation and treatment. High-resolution computed tomography revealed numerous bilateral thin-walled air cysts and interstitial thickening affecting the central and peripheral part of the upper zone of the lung. We performed an open-lung biopsy to confirm lymphangioleiomyomatosis. Our aim is to discuss the pathogenesis and other lesions noted in the differential diagnosis of this rare disease.