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Background: Uniportal video-assisted thoracoscopic surgery (U-VATS) offers good cosmetic outcomes with minimal pain for the treatment of primary spontaneous pneumothorax (PSP). Moreover, the early removal of postoperative chest drains reduces postoperative pain and hospitalization duration for patients with PSP. We aimed to investigate the safety and feasibility of drainless U-VATS in patients with PSP and compare postoperative outcomes between specialists and residents., Methods: We retrospectively analyzed data obtained from the medical records of consecutive patients diagnosed with PSP who underwent surgery at Yamagata Prefectural Central Hospital and Tokyo Metropolitan Bokutoh Hospital between April 2023 and March 2024. Yamagata Prefectural Central Hospital and Tokyo Metropolitan Bokutoh Hospital initiated the drainless protocol in April and July 2023, respectively. All surgeries were performed using the U-VATS approach with a 1.8-2.0-cm incision., Results: We retrospectively reviewed the medical records of 54 patients who underwent U-VATS according to the protocol. Postoperative repeated drainage was not required for any patient. The median postoperative length of hospital stay was 1 day. No multiport conversions were required. The specialists performed significantly better than the residents based on operative time; however, the other perioperative outcomes were not significantly different., Conclusions: Protocol-compliant drainless surgery for PSP is safe and feasible. The results from the two institutions suggest that residents can adequately perform U-VATS for spontaneous pneumothorax with perioperative outcomes comparable to those of specialists., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jtd.amegroups.com/article/view/10.21037/jtd-24-972/coif). The authors have no conflicts of interest to declare., (2024 AME Publishing Company. All rights reserved.)

Objective: To introduce and evaluate the non-assistant help operation in dual-portal robotic-assisted thoracic surgery (neoDRATS), a novel technique designed to eliminate the need for skilled assistants by using all 4 robotic arms independently during anatomical lung surgery., Methods: Patients were placed in the lateral decubitus position under general anesthesia with single-lung ventilation. The da Vinci Xi Surgical System was used, with specific configurations for right- and left-side operations. The neoDRATS technique used a 4-cm working port and a 1.8-cm secondary port, with detailed guidelines for optimal setup and robotic arm manipulation., Results: The neoDRATS approach demonstrated successful surgical outcomes without the need for a skilled assistant. The use of a 0° camera and careful placement of instruments minimized interference within the thoracic cavity. The technique provided smooth operability and minimized postoperative discomfort. Video demonstrations of right and left upper lobectomies are provided to illustrate the approach., Conclusions: NeoDRATS offers a practical, safe, and minimally invasive alternative to conventional multiportal and uniportal robotic-assisted thoracic surgeries. This technique simplifies the surgical process, particularly in settings with limited availability of skilled assistants, and represents a significant advancement in robotic thoracic surgery. Further refinement and clinical integration of neoDRATS are anticipated as robotic innovations continue to evolve., Competing Interests: The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (© 2024 The Author(s).)

Background: Robotic-assisted thoracic surgery (RATS) has gained increasing interest in recent years, with most procedures performed using the conventional multiportal approach. Uniportal RATS (URATS) approaches have recently been reported in the pursuit of minimally invasive procedures. However, URATS requires specific skills. Herein, we introduce dual-portal RATS (DRATS) performed with two incisions., Methods: Data of DRATS procedures performed from December 2022 to May 2023 were retrospectively reviewed. Twenty patients with lung cancer underwent anatomical lung resections via DRATS performed by our group at three institutes., Results: Among 20 cases of planned DRATS for anatomical pulmonary resections, there were no conversions to thoracotomy and no need for extra ports. The mean surgery time was 121±60 minutes and mean console time was 91±47 minutes. The mean intraoperative blood loss volume was 9.6±12.1 g. The mean duration of chest tube drainage and hospital stay were 2±1 and 5±2 days, respectively. The mean numerical rating scale for pain was 2±1 on the first postoperative day, 1±1 on the third day, and 1±1 at discharge. There were no postoperative complications or mortalities., Conclusions: Our primary experience shows that DRATS is safe and feasible for anatomical lung resection. We consider DRATS to be a very good preliminary step in the future transition to URATS., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jtd.amegroups.com/article/view/10.21037/jtd-23-1141/coif). The authors have no conflicts of interest to declare., (2023 Journal of Thoracic Disease. All rights reserved.)

A male patient in his 60s at the time of the first medical examination had a smoking history of 50 years with 25 cigarettes a day. He was diagnosed with double urothelial cancers. In 200a, total left pelvic ureterectomy(pT2N0M0, Stage Ⅱ)and transurethral bladder tumorectomy(pTisN0M0, Stage Ⅰ)were performed. For his gastric cancer with malignant pleural effusion(cT3N0M1, Stage Ⅳb), in 200a plus 2, downstaging was acquired after chemotherapy. In 200a plus 5, subtotal gastrectomy D1 dissection was performed(W/D adenocarcinoma, pT2N0M0, Stage ⅠA, Ef 1). For the first lung cancer, in 200a plus 5, thoracoscopic lung wedge resection of the left lower lobe was performed(P/D adenocarcinoma, pT1aN0M0, Stage ⅠA1, R0, Ef 1). For the second lung cancer, in 200a plus 13, thoracoscopic lung wedge resection of the right upper lobe was performed after chemotherapy(P/D adenocarcinoma, pT1bN0M0, Stage ⅠA2, R0). For the third lung cancer, in 200a plus 17, immunotherapy was performed for the left upper lobe lung cancer(P/D adenocarcinoma, cT3N1M1a, Stage ⅢA). All the cancers were diagnosed as primary lesions by immunohistological examination. For the metachronous multiple cancers, multidisciplinary treatment was necessary for each cancer considering the patient's physical condition. Moreover, strict follow-up was necessary because of the high risk of carcinogenesis.

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., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2023 Namba et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Competing Interests: Competing interests: None declared.

Background: The SedLine ® sensor processes (Masimo Corporation; Irvine, CA, USA) raw electroencephalogram (EEG) signals and displays the depth of sedation as a Patient State Index (PSi). Reliance on standard processed EEG data and failure to recognize age-related effects can lead to an erroneous interpretation that low-amplitude EEG findings in an older patient signify an insufficient depth of anesthesia presented as abnormally high PSi values (AHPSi). We hypothesized that the incidence of AHPSi would decrease with the use of the recently-updated version of the SedLine ® sensor, in which the Bispectral Index (BIS) values were used to titrate anesthesia., Methods: Thirty-three patients undergoing sevoflurane-remifentanil anesthesia were randomized into two groups. SedLine ® sensors designed based on an old (v.1203) or updated (v.2000) algorithms were used. The BIS (v.4.1) and absolute index of total EEG power (TP) were simultaneously recorded. The attending anesthesiologists titrated the anesthetics, and BIS was maintained at 40-60. The incidence of AHPSi (PSi>50 with BIS 40-60) was calculated during the first 30 min after the start of surgery., Results: Compared to the old algorithm group, the incidence of AHPSi was significantly lower in the updated algorithm group (26.7% vs. 4.2%, P<0.001). Lower TP values and the use of the old algorithm have significant effect on increased PSi values (P<0.001)., Conclusions: The incidence of AHPSi decreased with the use of the updated version of the SedLine ® algorithm.

Birt-Hogg-Dubé syndrome (BHDS), an autosomal dominant inheritance disease caused by folliculin (FLCN) mutations, is associated with lung cysts and spontaneous pneumothorax. The possibility of FLCN haploinsufficiency in pleural mesothelial cells (PMCs) contributing to development of pneumothorax has not yet been clarified. Electron microscopy revealed exposed intercellular boundaries between PMCs on visceral pleura and decreased electron density around the adherens junctions in BHDS. To characterize cellular function of PMCs in BHDS patients (BHDS-PMCs), during surgery for pneumothorax, we established the flow cytometry-based methods of isolating high-purity PMCs from pleural lavage fluid. BHDS-PMCs showed impaired cell attachment and a significant decrease in proliferation and migration, but a significant increase in apoptosis compared with PMCs from primary spontaneous pneumothorax (PSP) patients (PSP-PMCs). Microarray analysis using isolated PMCs revealed a significant alteration in the expression of genes belonging to Gene Ontology terms "cell-cell adhesion junction" and "cell adhesion molecule binding". Gene set enrichment analysis demonstrated that CDH1, encoding E-cadherin, was identified in the down-regulated leading edge of a plot in BHDS-PMCs. AMPK and LKB1 activation were significantly impaired in BHDS-PMCs compared with PSP-PMCs. Our findings indicate that FLCN haploinsufficiency may affect the E-cadherin-LKB1-AMPK axis and lead to abnormal cellular function in BHDS-PMCs.

Lymphangioleiomyomatosis (LAM) is a rare pulmonary disease characterised by the proliferation of smooth muscle-like cells (LAM cells), and an abundance of lymphatic vessels in LAM lesions. Studies reported that vascular endothelial growth factor-D (VEGF-D) secreted by LAM cells contributes to LAM-associated lymphangiogenesis, however, the precise mechanisms of lymphangiogenesis and characteristics of lymphatic endothelial cells (LECs) in LAM lesions have not yet been elucidated. In this study, human primary-cultured LECs were obtained both from LAM-affected lung tissues (LAM-LECs) and normal lung tissues (control LECs) using fluorescence-activated cell sorting (FACS). We found that LAM-LECs had significantly higher ability of proliferation and migration compared to control LECs. VEGF-D significantly promoted migration of LECs but not proliferation of LECs in vitro. cDNA microarray and FACS analysis revealed the expression of vascular endothelial growth factor receptor (VEGFR)-3 and integrin α9 were elevated in LAM-LECs. Inhibition of VEGFR-3 suppressed proliferation and migration of LECs, and blockade of integrin α9 reduced VEGF-D-induced migration of LECs. Our data uncovered the distinct features of LAM-associated LECs, increased proliferation and migration, which may be due to higher expression of VEGFR-3 and integrin α9. Furthermore, we also found VEGF-D/VEGFR-3 and VEGF-D/ integrin α9 signaling play an important role in LAM-associated lymphangiogenesis.

Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances present challenges to establishing the proper LAM diagnosis. The objective of this study is to accrue uncommon chest HRCT appearances, determine frequencies in both tuberous sclerosis complex (TSC)-associated LAM (TSC-LAM) and sporadic LAM (S-LAM) patients. 311 females referred to our hospital, including 272 S-LAM patients (mean age 39.2 years) and 39 TSC-LAM patients (mean age 38.3 years), were retrospectively evaluated. We found 2 types of radiologic findings likely to make HRCT cyst appearance atypical: characteristics of the cyst itself and uncommon findings in addition to cysts. We found that approximately 80% of LAM patients, whether TSC-associated or sporadic, showed typical HRCT appearance with mild to severe cystic destruction. The remaining 20% displayed unusual profiles in cyst appearance as well as additional findings aside from cyst: the former includes large cyst, thickened walls, and irregularly shaped whereas the latter includes ground glass attenuation and diffuse noncalcified nodules. It is important to be aware of various radiologic findings that make HRCT cystic appearance atypical of LAM.

Aim: To review the clinical features of the patient with cystic pulmonary light chain deposition disease(LCDD)and record high-resolution CT and histopathological findings., Patient: A 60-year-old woman who diagnosed multiple myeloma was admitted. There were diffuse proliferation of plasma cells by the bone marrow puncture that she received. And multiple cystic pulmonary tumors found in CT. We analyzed the method and result of the diagnosis. CT showed multiple cystic pulmonary tumors in the both lung which vessels traversing the cysts with thin wall ranged 5 to 30 millimeters. There were no abnormality in the heart, kidney and liver. Right upper lobe wedge resection was performed via video-assisted thoracic surgery( VATS)to establish a definitive diagnosis. Thoracoscopic findings revealed multiple white oval formed tumors in the visceral pleura. Histological HE staining findings of the surgical specimen revealed amyloid-like acidophilic material, and the immunohistochemical Congo red staining revealed monoclonal IgG with a kappa component. According to the above results these tumors had a diagnosis of pulmonary LCDD., Conclusion: VATS was effective to diagnose pulmonary LCDD in multiple cysts formation.

The Oxygen Reserve Index (ORi™) is a non-invasive variable that reflects oxygenation continuously. The aims of this study were to examine the relationship between arterial partial pressure of oxygen (PaO 2 ) and ORi during general anesthesia, and to investigate the usefulness of ORi as an indicator to avoid hyperoxia. Twenty adult patients who were scheduled for surgery under general anesthesia with arterial catheterization were enrolled. After induction of general anesthesia, inspired oxygen concentration (FiO 2 ) was set to 0.33, and arterial blood gas analysis was performed. The PaO 2 and ORi at the time of blood collection were recorded. After that, FiO 2 was changed to achieve an ORi around 0.5, 0.2, and 0, followed by arterial blood gas analysis. The relationship between ORi and PaO 2 was then investigated using the data obtained. Eighty datasets from the 20 patients were analyzed. When PaO 2 was less than 240 mmHg (n = 69), linear regression analysis showed a relatively strong positive correlation (r 2  = 0.706). The cut-off ORi value obtained from the receiver operating characteristic curve to detect PaO 2  ≥ 150 mmHg was 0.21 (sensitivity 0.950, specificity 0.755). Four-quadrant plot analysis showed that the ORi trending of PaO 2 was good (concordance rate was 100.0%). Hyperoxemia can be detected by observing ORi of patients under general anesthesia, and thus unnecessary administration of high concentration oxygen can possibly be avoided.

Purpose: To establish a method for quantitatively estimating the extent of the communication between the cyst and the airway in cystic lung diseases (CLDs) and evaluate its diagnostic utility in differentiating among CLDs., Materials and Methods: Seventy-one patients (mean age, 49.9 years; age range, 25-79 years) with CLDs who underwent paired inspiratory and expiratory CT between July 2015 and July 2018 were enrolled in this prospective study. Participants were divided into three groups based on their diagnosis: Birt-Hogg-Dubé syndrome (BHDS) group (15 participants), lymphangioleiomyomatosis (LAM) group (43 participants), and other diseases (OT) group (13 participants). Total lung volume (TLV) and low-attenuation area volume (LAAV) were calculated at inspiration and expiration. The collapsibility of the LAAV was determined as the expiration-to-inspiration (E/I) ratio of LAAV (E/I ratio LAAV). The cyst-airway communicating index (CACI), the ratio of the LAAV change between inspiration and expiration to the TLV change between inspiration and expiration, was also determined. Receiver operating characteristic (ROC) analysis was used to evaluate the diagnostic utility for differentiating diseases., Results: The E/I ratio LAAV was significantly higher in the BHDS group (0.69; 95% confidence interval [CI]: 0.61, 0.78) than in the LAM (0.33; 95% CI: 0.28, 0.38) ( P < .001) and the OT (0.51; 95% CI: 0.38, 0.64) ( P = .038) groups. The CACI was significantly lower in the BHDS group (0.89; 95% CI: 0.61, 1.17) than in the LAM (1.89; 95% CI: 1.76, 2.0) ( P < .001) and the OT (1.539; 95% CI: 1.21, 1.86) ( P = .003) groups. There was no significant difference in the area under the ROC curve of the CACI (0.881; 95% CI: 0.7749, 0.987) and the E/I ratio LAAV (0.877; 95% CI: 0.791, 0.963) for differentiating BHDS from other diseases., Conclusion: Quantitative analysis using paired inspiratory and expiratory CT for estimating the extent of cyst-airway communication in CLDs is useful when distinguishing BHDS from other diseases. Supplemental material is available for this article. © RSNA, 2020See also the commentary by Chung in this issue., Competing Interests: Disclosures of Conflicts of Interest: K. Suzuki disclosed no relevant relationships. K. Seyama disclosed no relevant relationships. H.E. disclosed no relevant relationships. T.K. disclosed no relevant relationships. R.K. disclosed no relevant relationships., (2020 by the Radiological Society of North America, Inc.)

Background: Birt-Hogg-Dubé syndrome (BHDS) is a recently recognized inherited multiple cystic lung disease causing recurrent pneumothoraces. Similarly to the lesions in patients with lymphangioleiomyomatosis (LAM), the pulmonary cysts are innumerable and widely dispersed and cannot all be removed. We recently described a total pleural covering (TPC) that covers the entire visceral pleura with oxidized regenerated cellulose (ORC) mesh. TPC successfully prevented the recurrence of pneumothorax in LAM patients. The purpose of this study was to evaluate the effect of an ORC pleural covering on pneumothorax recurrence in BHDS patients., Results: This retrospective study enrolled a total of 81 pneumothorax patients with the diagnosis of BHDS who underwent 90 covering surgeries from January 2010 to August 2017 at Tamagawa Hospital. During the first half of the study period, a lower pleural covering (LPC) which covered the affected area with ORC mesh was mainly used to treat 38 pneumothoraces. During the second half of the study period, TPC was primarily performed for 52 pneumothoraces. All the thoracoscopic surgeries were successfully performed without serious complications (≥ Clavien-Dindo grade III). The median follow-up periods after LPC/TPC were 66/34 months, respectively. Pneumothorax recurrence rates after LPC at 2.5/5/7.5 years postoperatively were 5.4/12/42%, respectively; none of the patients who had underwent TPC developed postoperative pneumothorax recurrence (P = 0.032)., Conclusions: TPC might be an effective option for surgical treatment of intractable pneumothorax in patients with BHDS.

Purpose: The pleural covering technique, i.e., wrapping a part of or the entire surface of the lung with oxidized regenerative cellulose (ORC), reinforces visceral pleura through pleural thickening for patients with pneumothorax and cystic lung diseases. However, it remains undetermined how ORC induces pleural thickening., Methods: A histopathological examination was performed for lung specimens from patients who had recurrent pneumothoraces after pleural covering and re-operation (n = 5). To evaluate the influence of ORC on the pleura in vitro, we used MeT-5A cells (a human pleural mesothelial cell line)., Results: Pleural thickening was confirmed in all lung specimens examined. Three months after covering, the thickened pleura showed inflammatory cell infiltration, proliferation of myofibroblasts, and expression of fibronectin and TGF-β. However, after 1 year, those findings virtually disappeared, and the thickened pleura was composed mainly of abundant collagen. When MeT-5A cells were cultured in ORC-immersed medium, their morphology changed from a cobblestone to spindle-shaped appearance. The expression of E-cadherin decreased, whereas that of N-cadherin, α-smooth muscle actin, and fibronectin increased, suggesting mesothelial-mesenchymal transition (Meso-MT)., Conclusions: Our results suggest that Meso-MT may be involved as a mechanism of pleural thickening induced by pleural covering with ORC.

Background and Objective: Birt-Hogg-Dubé syndrome (BHDS) is a rare hereditary disease that presents with multiple lung cysts and pneumothorax (PTX). Although some reports propose that findings from chest computed tomography enable one to distinguish BHDS from primary spontaneous pneumothorax (PSP), it is still unclear whether clinical features are useful for identifying patients with suspicion of BHDS from those with PTX., Methods: We retrospectively reviewed the medical records of patients with PTX who underwent video-assisted thoracoscopic surgery at Nissan Tamagawa Hospital from January 2012 to December 2015., Results: We identified a total of 1141 patients with PTX, including 54 with BHDS and 517 with PSP. Among them, logistic regression analysis segregated five features that were significantly associated with BHDS: familial history of PTX, past history of bilateral PTX, age at the first episode of PTX (≥25 years old (y.o.)), body mass index (≥18.5) and gender (female). We assigned scores of 3, 3, 2, 2 and 1 to the five features, respectively, to establish a system with a calculated score from 0 to 11. The cut-off value of a calculated score ≥ 4 yielded the highest sensitivity of 93% and specificity of 86%. Receiver operating characteristic (ROC) analysis showed the area under the curve reflecting an accuracy of this diagnostic test as 0.953., Conclusion: BHDS has several clinical features distinct from PSP. Our scoring system consists of only five clinical variables that are easily evaluated and efficiently separate BHDS patients from those who have PTX without relying on an imaging study. Further prospective study is needed to confirm our findings., (© 2017 Asian Pacific Society of Respirology.)

Background: Birt-Hogg-Dubé syndrome (BHDS) (OMIM #135150) is an autosomal dominant disease, characterized by fibrofolliculomas (FFs) of the skin, pulmonary cysts with/without pneumothorax, and renal tumors. The prevalence of skin manifestations reported for Japanese BHDS patients is lower (<30%) compared with that of Western countries (75∼90%), which appear to be underestimated., Objective: To precisely examine the prevalence of skin lesions with dermoscopy and histopathology with reference to genetic analyses., Methods: We studied 31 patients (47.0±13.2years old, range 15-71) consisting of 26 unrelated families consecutively from May 2013 to June 2015 specifically for skin-colored papules on their faces and cervicothoracic regions. Patients initially suspected of BHDS from multiple pulmonary cysts that resulted in pneumothorax (30/31; 96.8%) received dermoscopic examinations and skin biopsies if applicable. The diagnosis of BHDS was established by folliculin (FLCN) genetic testing, and the results were compared to the histopathological findings of FFs or trichodiscomas (TDs)., Results: FLCN germline mutation was demonstrated in 25/26 (96.2%) unrelated families tested and 28/29 patients (96.6%) tested. Skin lesions were recognized in 26/31 patients (83.9%); skin biopsies were performed in 23 patients of whom FFs and/or TDs were histologically demonstrated in 17 (73.9%). Although our study population included patients whose skin manifestations were evaluated prior to or after FLCN genetic testing, skin lesions were clearly prevalent and recognizable irrespective of whether genetic testing was or wasn't done. When examined with dermoscopy, distinct FFs appeared as well-demarcated areas of pallor with central follicular openings in 13 of 15 FF-bearing patients (86.7%); however, those manifestations were not recognized for TD., Conclusions: Skin lesions appear to be more prevalent than previously reported (<30% vs.73.9%) in Japanese BHDS patients. Dermoscopy is a useful diagnostic aid for finding FFs., (Copyright © 2017 Japanese Society for Investigative Dermatology. Published by Elsevier B.V. All rights reserved.)

Catamenial pneumothorax (CP) is generally caused by intraperitoneal air leaking from the uterus into the thoracic cavity via a defect in the endometrial tissue of the diaphragm and is usually detected in the right thorax. We report a case of left-sided CP caused by endometriosis in the visceral pleura and with no abnormal findings in the diaphragm. A 33-year-old female patient presented at the end of a course of low-dose contraceptive pills for pelvic endometriosis, with spontaneous pneumothorax in the left chest. Chest CT revealed a bulla in the left upper lung lobe. The patient underwent partial resection of the lung. Immunohistochemistry confirmed the presence of endometrial stromal tissue in the visceral pleura and confirmed this as the cause of pneumothorax since there were no observable abnormalities in the diaphragm. This case suggests that immunohistochemical examination of patients with spontaneous pneumothorax can detect alternative endometrial lesions.

Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant inherited disorder caused by germline mutations in the FLCN gene, and characterized by skin fibrofolliculomas, multiple lung cysts, spontaneous pneumothorax, and renal neoplasms. Pulmonary manifestations frequently develop earlier than other organ involvements, prompting a diagnosis of BHDS However, the mechanism of lung cyst formation and pathogenesis of pneumothorax have not yet been clarified. Fibroblasts were isolated from lung tissues obtained from patients with BHDS (n = 12) and lung cancer (n = 10) as controls. The functional abilities of these lung fibroblasts were evaluated by the tests for chemotaxis to fibronectin and three-dimensional (3-D) gel contraction. Fibroblasts from BHDS patients showed diminished chemotaxis as compared with fibroblasts from controls. Expression of fibronectin and TGF-β1 was significantly reduced in BHDS fibroblasts when assessed by qPCR Addition of TGF-β1 in culture medium of BHDS lung fibroblasts significantly restored these cells' abilities of chemotaxis and gel contraction. Human fetal lung fibroblasts (HFL-1) exhibited reduced chemotaxis and 3-D gel contraction when FLCN expression was knocked down. To the contrary, a significant increase in chemotactic activity toward to fibronectin was demonstrated when wild-type FLCN was overexpressed, whereas transduction of mutant FLCN showed no effect on chemotaxis. Our results suggest that FLCN is associated with chemotaxis in lung fibroblasts. Together with reduced TGF-β1 expression by BHDS lung fibroblasts, a state of FLCN haploinsufficiency may cause lung fibroblast dysfunction, thereby impairing tissue repair. These may reveal one mechanism of lung cyst formation and pneumothorax in BHDS patients., (© 2016 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of The Physiological Society and the American Physiological Society.)

Background: Spontaneous pneumothorax is a major and frequently recurrent complication of lymphangioleiomyomatosis (LAM). Despite the customary use of pleurodesis to manage pnenumothorax, the recurrence rate remains high, and accompanying pleural adhesions cause serious bleeding during subsequent lung transplantation. Therefore, we have developed a technique of total pleural covering (TPC) for LAM to wrap the entire visceral pleura with sheets of oxidized regenerated cellulose (ORC) mesh, thereby reinforcing the affected visceral pleura and preventing recurrence., Methods: Since January 2003, TPC has been applied during video-assisted thoracoscopic surgery for the treatment of LAM. The medical records of LAM patients who had TPC since that time and until August 2014 are reviewed., Results: TPC was performed in 43 LAM patients (54 hemithoraces), 11 of whom required TPC bilaterally. Pneumothorax recurred in 14 hemithoraces (25.9%) from 11 patients (25.6%) after TPC. Kaplan-Meier estimates of recurrence-free hemithorax were 80.8% at 2.5 years, 71.7% at 5 years, 71.7% at 7.5 years, and 61.4% at 9 years. The recurrence-free probability was significantly better when 10 or more sheets of ORC mesh were utilized for TPC (P = 0.0018). TPC significantly reduced the frequency of pneumothorax: 0.544 ± 0.606 episode/month (mean ± SD) before TPC vs. 0.008 ± 0.019 after TPC (P<0.0001). Grade IIIa postoperative complications were found in 13 TPC surgeries (24.1%)., Conclusions: TPC successfully prevented the recurrence of pneumothorax in LAM, was minimally invasive and rarely caused restrictive ventilatory impairment., Competing Interests: The authors have declared that no competing interests exist.

Birt-Hogg-Dubé syndrome (BHDS) is a rare hereditary disease that presents with multiple lung cysts and recurrent pneumothorax. These cysts occupy predominantly the lower-medial zone of the lung field adjacent to the interlobar fissure, and some of them abut peripheral pulmonary vessels. For the surgical management of pneumothorax with BHDS, the conventional approach of resecting all subpleural cysts and bullae is not feasible. Thus, after handling several bullae by using a stapler or performing ligation as a standardized treatment, we applied to a pleural covering technique to thicken the affected visceral pleura and then to prevent recurrence of pneumothorax. We herein report the successful application of a pleural covering technique via thoracoscopic surgery to treat the recurrent pneumothorax of a 30-year-old man with BHDS. This technique is promising for the management of intractable pneumothorax secondary to BHDS.

Lymphangioleiomyomatosis (LAM) is a rare neoplastic disease entailing cystic destruction of the lungs and progressive respiratory failure. LAM lungs are histologically characterized by the proliferation of smooth muscle-like cells (LAM cells) and an abundance of lymphatic vessels. To elucidate the pathophysiological processes of LAM, cell-type-specific analyses are required. However, no method exists for isolating the individual types of cells in LAM lesions. Therefore, we established a fluorescence-activated cell sorting (FACS)-based method for the direct isolation of LAM cells and other various cellular components from LAM-affected lung tissue. We obtained LAM-affected lung tissue from resections or transplant recipients and prepared single-cell suspensions. FACS, immunohistochemical, and molecular analysis were used cooperatively to isolate HMB45-positive LAM cells with tuberous sclerosis complex (TSC) 2 loss of heterozygosity (LOH). Using a combination of antibodies against an epithelial cell adhesion molecule (EpCAM) and podoplanin, we fractionated CD45-negative lung cells into three groups: lymphatic endothelial cells (LEC) (EpCAM(-)/podoplanin(hi) subset), alveolar type II cells (EpCAM(hi)/podoplanin(-) subset), and mesenchymal cells (EpCAM(-)/podoplanin(-/low) subset). During subsequent analysis of HMB45 expression, as a LAM-specific marker, we clearly identified LAM cells in the mesenchymal cell population. We then discovered that CD90(+)/CD34(-) cells in the mesenchymal cell population are not only positive for HBM45 but also had TSC2 LOH. These isolated cells were viable and subsequently amenable to cell culture. This method enables us to isolate LAM cells and other cellular components, including LAM-associated LEC, from LAM-affected lung tissues, providing new research opportunities in this field., (Copyright © 2016 the American Physiological Society.)

Background and Objective: A major pathogenic factor for catamenial pneumothorax is thoracic endometriosis. However, thoracic endometriosis-related pneumothorax (TERP) can develop as either catamenial or non-catamenial pneumothorax (CP). Therefore, the aim of this study was to elucidate the clinical differences between catamenial and non-catamenial TERP., Methods: The clinical and pathological data in female patients who underwent video-assisted thoracoscopic surgery at the Pneumothorax Research Center during an 8-year period were retrospectively reviewed. This study included 150 female patients with surgico-pathologically confirmed TERP. The subjects were divided into two groups, those having all of the pneumothorax episodes in the catamenial period (CP group) and those who did not (non-CP group). We compared the clinical characteristics and surgico-pathological findings between these two groups., Results: Of the 150 TERP patients, 55 (36.7%) were classified in the CP group, and 95 (63.3%) in the non-CP group. In regard to the locations of endometriosis, all TERP patients had diaphragmatic endometriosis, while pleural implantation was recognized in 34 of the 55 (61.8%) patients in the CP group and 42 of the 95 (44.2%) patients in the non-CP group (P < 0.05)., Conclusions: A significant difference in the proportion of patients with pleural endometriosis was observed between catamenial and non-catamenial TERP. The ectopic sites of the endometriosis may be responsible for the timing of the pneumothorax episodes., (© 2015 Asian Pacific Society of Respirology.)

We describe three cases of resected pulmonary metastasis from postoperative colorectal cancer after preoperative 5-fluorouracil, Leucovorin, oxaliplatin (FOLFOX) chemotherapy. The first case is a 38-year-old man who underwent low anterior resection for rectal cancer in 2003. Subsequently, left lung metastasis occurred in February 2009. FOLFOX chemotherapy was administered, resulting in a partial response (PR) of tumor size. Post-chemotherapy, segmental resection of he S1+2 segments of the left lung was performed in May 2009. A pathological diagnosis of Grade 1b was made. The patient remained recurrence-free 5 years post-surgery. The second case is a 68-year-old man who underwent left half colon resection for descending colon cancer with left lung metastasis in March 2006. FOLFOX chemotherapy was administered, with a PR of lung metastasis size. Post-chemotherapy, a segmental resection of the S4 segment of the left lung was performed in September 2006. A pathological diagnosis of Grade 1b was made. The patient was alive 8 years post-surgery. The third case is a 64-year-old man who underwent low anterior resection for rectal cancer in November 2007. A year later, bilateral lung metastases were detected. FOLFOX+bevacizumab was administered, with a stable disease effect on tumor size. Post-chemotherapy, partial resection of both lungs was performed in March and April 2010. A pathological diagnosis of Grade 1a was made. The patient was recurrence-free at the 4-year follow up. We believe that preoperative FOLFOX chemotherapy may be effective in treating lung metastasis from colon cancer.

Purpose: Thoracic endometriosis-related pneumothorax (TERP) is a secondary condition specific for females, but in a clinical setting, TERP often is difficult to distinguish from primary spontaneous pneumothorax (PSP) based on a relationship between the dates of pneumothorax and menstruation. The purpose of this study was to clarify the clinical features of TERP compared with PSP., Methods: We retrospectively reviewed the clinical and histopathological files of female patients with pneumothorax who underwent video-assisted thoracoscopic surgery in the Pneumothorax Research Center during the 6-year period from January 2005 to December 2010. We analyzed the clinical differences between TERP and PSP., Results: The study included a total of 393 female patients with spontaneous pneumothorax, of whom 92 (23.4 %) were diagnosed as having TERP and 33.6 % (132/393) as having PSP. We identified four factors (right-sided pneumothorax, history of pelvic endometriosis, age ≥31 years, and no smoking history) that were statistically significant for predicting TERP and assigned 6, 5, 4, and 3 points, respectively, to establish a scoring system with a calculated score from 0 to 18. The cutoff values of a calculated score ≥12 yielded the highest positive predictive value (86 %; 95 % confidence interval (CI) 81.5-90.5 %) for TERP and negative predictive value (95.2 %; 95 % CI 92.3-98 %) for PSP., Conclusions: TERP has several distinct clinical features from PSP. Our scoring system consists of only four clinical variables that are easily obtainable and enables us to suspect TERP in female patients with pneumothorax.

In the past decade, the serum free light chain (FLC) immunoassays have become widely available enabling greater sensitivity in the diagnosis and management of monoclonal light chain diseases. Here, we describe a rare case of serum free light chain only myeloma with cytoplasmic IgM. A 75-year-old woman presented with a progressively worsening lumbosacral pain. FDG PET/CT images showed increased FDG uptake in the sacral mass, vertebral bodies, and ribs. Laboratory data found hypogammaglobulinemia and the bone marrow aspirate revealed only 2.2% of plasma cells. The serum and urine protein electrophoresis did not detect a monoclonal band. However, the serum FLC immunoassays reported an abnormal kappa/lambda ratio (0.001) indicating the presence of monoclonal lambda FLC. The sacral tumor biopsy revealed proliferation of plasma cells and immunohistochemical staining showed that the plasma cells were positive for CD138, IgM, and lambda light chain but negative for CD20. This case may have previously been described as a nonsecretory IgM myeloma but recently would be identified as free light chain only myeloma. The immunohistochemical and genetic features of the clonal plasma cells in free light chain only myeloma need to be further investigated to better understand the relevance and incidence of this myeloma type.

Purpose: Catamenial pneumothorax (CP) is classified as thoracic endometriosis syndrome. There are few reports of CP, and the clinical manifestations of this disease are unclear. The aim of the present study is to clarify the features of CP., Methods: The clinical and pathological files of the 92 female patients pathologically diagnosed with thoracic endometriosis are included in this study. The clinical data and pathological findings of the recurrent and non-recurrent groups are compared., Results: Thirty-six patients (39.1%) experienced recurrence, 37 (40.2%) patients did not, and 19 (20.4) patients could not be evaluated. The ratio of the endometrial gland in the diaphragm is significantly higher in the recurrent cases in comparison to non-recurrent cases (66.7% vs. 37.8%, P = 0.01)., Conclusions: The recurrence rate of CP is high. Further study of the optimal management strategies is needed, especially for CP cases with the endometrial gland in the diaphragm.

We describe 3 cases in which the pulmonary metastasis from the urothelial carcinoma of the bladder and upper urinary tract was resected. The duration from the operation of the primary lesion to the occurrence of the pulmonary metastasis was 19, 11, and 4 years in each of the 3 cases. Repeated treatment of the local recurrence was performed in all the 3 cases. Local recurrence in the bladder membrane was observed in 1 case. In 2 cases, computed tomography( CT) scans revealed that the cavitation had penetrated the tumor. Histological findings of the surgical specimen obtained from the 3 cases revealed severe tumor necrosis. Immunostaining of the surgical specimen resulted in a definitive diagnosis of pulmonary metastasis from the urothelial carcinoma in 2 cases in which a differential diagnosis could not identify the primary lung cancer from the pulmonary metastasis. The prognoses in terms of survival in each of the 3 cases were shorter than 32, 19, and 6 months from the operation of the pulmonary metastasis. However, the prognoses could be improved by multidisciplinary treatment, including the resection of the pulmonary metastasis.

Purpose: Primary spontaneous pneumothorax is believed to result from blebs, or from other abnormalities of the pleural surface. There is no consensus as to whether a change in weather conditions can precipitate spontaneous pneumothorax. The aim of the present study was to investigate the influence of weather conditions on the onset of primary spontaneous pneumothorax., Methods: The case histories of 1051 inpatients with primary spontaneous pneumothorax treated at Nissan Tamagawa Hospital between January 2006 and December 2011 were analyzed retrospectively. Data on weather conditions were collected daily throughout the 6-year period. The data were analyzed to determine differences in weather conditions between days on which primary spontaneous pneumothorax occurred and those on which it did not., Results: Primary spontaneous pneumothorax occurred on 819 (37.3%) of 2191 study days. On days before and the day of primary spontaneous pneumothorax onset, the difference in mean atmospheric pressure was 0.6 hPa lower than on days in which no primary spontaneous pneumothorax occurred. This difference was statistically significant (P = 0.015). There was no statistical difference in mean, maximum, and minimum temperature, hours of sunshine, amount of precipitation, and mean and minimum humidity between days with and those without primary spontaneous pneumothorax., Conclusion: This largest study of the literature showed decreased atmospheric pressure might play an important role in the occurrence of primary spontaneous pneumothorax.

Molecular imaging employing fluorescent proteins has been widely used to highlight specific reactions or processes in various fields of the life sciences. Despite extensive improvements of the fluorescent tag, this technology is still limited in the study of molecular events in the extracellular milieu. This is partly due to the presence of cysteine in the fluorescent proteins. These proteins almost cotranslationally form disulfide bonded oligomers when expressed in the endoplasmic reticulum (ER). Although single molecule photobleaching analysis showed that these oligomers were not fluorescent, the fluorescent monomer form often showed aberrant behavior in folding and motion, particularly when fused to cysteine-containing cargo. Therefore we investigated whether it was possible to eliminate the cysteine without losing the brightness. By site-saturated mutagenesis, we found that the cysteine residues in fluorescent proteins could be replaced with specific alternatives while still retaining their brightness. cf(cysteine-free)SGFP2 showed significantly reduced restriction of free diffusion in the ER and marked improvement of maturation when fused to the prion protein. We further applied this approach to TagRFP family proteins and found a set of mutations that obtains the same level of brightness as the cysteine-containing proteins. The approach used in this study to generate new cysteine-free fluorescent tags should expand the application of molecular imaging to the extracellular milieu and facilitate its usage in medicine and biotechnology.

A 75-year-old woman had an operation for gallstone ileus without cholecystectomy in other hospital and she was admitted to our hospital because of duodenal adenoma with severe atypia and small carcinoid in proximal duodenal wall. Distal gastrectomy and cholecystectomy were performed. Histological studies revealed the existence of cholecystoduodenal fistula and suggested the existence of gallbladder carcinoma progressed to the duodenal wall through the fistula. Cystic duct dissection and lymph nodes dissection were performed. It has been theorized that a cholecystoduodenal fistula may represent a significant risk factor in the development of gallbladder carcinoma because of the chronic reflux of duodenal contents which includes pancreatic juice. Our case may support this theory. In this case, we thought that the formation of gallbladder cancer could have been avoided if the cholecystectomy was performed in the first operation for gallstone ileus. It is very important that cholecystectomy should be performed when an existence of cholecystoduodenal fistula is highly suspected.

We report a case of late presentation of traumatic rupture of the diaphragm discovered incidentally on chest radiography (CXR) during an annual medical checkup. A 60-year-old man suffered severe blunt trauma from heavy steel frames collapsing against his back, resulting in pelvic and femoral fractures as well as pulmonary contusions. The patient recovered, but 10 months later CXR performed for lung cancer surveillance during an annual medical checkup revealed a traumatic rupture of the diaphragm. Video-assisted thoracic surgery was performed with reduction of the intestine and primary closure of the diaphragmatic defect. The patient recovered uneventfully. This report serves as a useful reminder that a medical history of severe blunt trauma should provoke a high index of suspicion for diaphragmatic rupture during annual medical surveillance.

The jejunostomy catheters were placed with needle catheter jejunostomy (NCJ) kits at the time of gastrectomy for the gastric cancer patients. When the oral intake of the post-operative patient is insufficient, home enteral nutrition (HEN) was introduced to the patient. Forty seven cases out of the 278 gatrectomy patients with jejunostomy were applied to HEN (400-1,200 kcal/day in the night). Twenty nine HEN gastric cancer cases received the chemotherapy in the outpatient clinic. The mean age was 71 years old, the number of total gastrectomy and distal gastrectomy were 21 and 8, respectively, and the number of Stage III and IV were 21 and 8, respectively. The chemotherapy for these 29 cases was continued for a long period unevenly in the outpatient clinic, even though the oral intake of the cases was insufficient. HEN is a good arm for keeping the chemotherapy secure and efficient in the outpatient clinic.

The intraperitoneal administration of anti-cancer drug is a rationale route to adjuvant chemotherapy. We applied adjuvant MTX-5-FU intraperitoneal chemotherapy for 60 advanced gastric cancer cases which had undergone gastrectomy (Stage II 18, Stage III A 19, Stage IIIB 13, and Stage IV 10 cases). A 5-year survival rate of Stage II, III A, IIIB and IV was 66.2%, 60.7%, 46.5% and 18.8%, respectively. Five-year survival rates of both Stage III A and IIIB on this study were likely to be higher than the rates of Stage III A and IIIB of other institutions. The 24 out of 42 cases with the serosal surface exposure of cancer demonstrated a cancer recurrence. Seventy percent (17 cases) of the 24 recurred cases developed a peritoneal recurrence, which means that the intraperitoneal chemotherapy did not touch a pattern of the recurrence of the gastric cancer with the serosal surface exposure.

Photo-induced isomerization of a newly synthesized surfactant, 1'(6-trimethylammoniododecyl)-3('),3(')-dimethyl-6-nitrospiro-(2H-1-benzopyran-2,2'-indoline) bromide (SP-Me-12), has been characterized on the basis of the UV-vis absorption spectra and the surface tension data. Visible light (lambda>420 nm) incident on the aqueous solution of SP-Me-12 results in the isomerization from the merocyanine (MC) form to the spiropyran (SP) form; this structural change was confirmed by a complete disappearance of a characteristic absorption peak of the MC form. When the surfactant solution is stored in the dark, the isomerized SP form reverts to the original MC form, however, the reverse isomerization rate is observed to be considerably slower than that seen for visible light irradiation (from the MC form to the SP form). A reversible change in the surface tension of the aqueous surfactant solution is observed for the photo-induced isomerization: the surface tension measured below the critical aggregation concentration decreases as a result of the visible light irradiation and it is gradually reversed to the original level during the equilibration in the dark.