[Show abstract][Hide abstract] ABSTRACT: 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.
Full-text · Article · Sep 2015 · Annals of thoracic and cardiovascular surgery: official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
[Show abstract][Hide abstract] ABSTRACT: The patient was a 63-year-old male. He was diagnosed with Langerhans cell histiocytosis (LCH) on CT examination in 2000. He underwent video assisted thoracoscopic surgery (VATS) for right pneumothorax in 2003. The pathological diagnosis was malignant melanoma of the lung. There was no other malignant melanoma noted on whole-body examinations, and so he was diagnosed with primary malignant melanoma of the lung. He thoracoscopically underwent both partial resection and pleural covering method with a regenerated oxidized cellulose mesh for left intractable pneumothorax in 2004. Regarding histopathological findings, the malignant melanoma cells invaded a thickened visceral pleura created by the covering. However, the malignant cells in the other covered areas had been contained below the thickened pleura. The pleural covering method was effective for pneumothorax due to invasive malignant cells. However, he subsequently died of brain metastasis in 2007.
Full-text · Article · Jan 2015 · The Journal of the Japanese Associtation for Chest Surgery
[Show abstract][Hide abstract] ABSTRACT: Purpose:
The aim was to characterize the computed tomographic (CT) findings from Japanese patients with lymphangioleiomyomatosis (LAM).
Materials and methods:
CT scans of the chest, abdomen, and pelvis from 124 patients with sporadic LAM (S-LAM, mean age, 37.4 years) and 14 patients with tuberous sclerosis complex (TSC)-LAM (mean age, 35.6 years) were analyzed.
Pulmonary nodules (18.8%) and hepatic angiomyolipoma (AML, 24.3%) were more common in our patients than those in previous reports. Compared with TSC-LAM, S-LAM group had a higher frequency of pulmonary nodules (28.6% vs 32.3%, P<0.01) and lower frequencies of air-space consolidation (21.4% vs 2.4%, P<0.01), pneumothorax (28.6% vs 8.1%, P=0.02), pulmonary hilar lymphadenopathy (14.3% vs 0.8%, P<0.01), renal AML (85.7% vs 17.4%, P<0.01), hepatic AML (71.4% vs 17.4%, P<0.01), and retrocrural lymphadenopathy (14.3% vs 1.4%, P=0.04). Axial lymphatic abnormalities (i.e., thoracic duct dilatation, lymphadenopathy, and lymphangioleiomyoma) were most common in the pelvis and tended to decrease in incidence with increased distance from the pelvis.
The incidence of some CT findings in Japanese patients differed from those in previous reports. Axial lymphatic abnormalities noted here suggest that the origin of LAM cells may be the pelvis.
No preview · Article · Dec 2014 · European Journal of Radiology
[Show abstract][Hide abstract] ABSTRACT: 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.
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.
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.
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.
No preview · Article · May 2014 · Beiträge zur Klinik der Tuberkulose
[Show abstract][Hide abstract] ABSTRACT: To characterize pathologic features of pulmonary cysts and to elucidate the possible mechanism of cyst formation in lungs of patients with Birt-Hogg-Dubé syndrome (BHDS), a tumor suppressor gene syndrome, we used histologic and morphometric analyses.
We evaluated 229 lung cysts from 50 patients with BHDS and 117 from 34 patients with primary spontaneous pneumothorax (PSP) for the number, size, location, and absence or presence of inflammation. These BHDS-cysts abutted interlobular septa (88.2%) and had intracystic septa (13.6%) or protruding venules (39.5%) without cell proliferation or inflammation. Frequencies of these histologic characteristics differed significantly from those of patients with PSP (P<0.05). Although the intrapulmonary BHDS-cysts were smaller than the subpleural BHDS-cysts (P<0.001), there was no difference in size between them when there was no inflammation. The number of cysts diminished logarithmically and the proportion of cysts with inflammation increased as their individual sizes enlarged (P<0.05).
These results imply that the BHDS-cysts are likely to develop at the periacinar region, an anatomically weak site in a primary lobule, where alveoli attach to connective tissue septa. We hypothesize that the BHDS cysts possibly expand in size as the alveolar walls disappear at the alveolo-septal junction and grow even larger when several cysts fuse. This article is protected by copyright. All rights reserved.
[Show abstract][Hide abstract] ABSTRACT: Objective:
Prolonged air leaks in patients with spontaneous pneumothorax are not infrequent. The aim of this study was to assess the duration of air leaks and define the clinical variables associated with the therapeutic success of chest tube drainage for spontaneous pneumothorax.
A total of 441 patients with spontaneous pneumothorax treated with chest tube drainage between 2008 and 2012 were retrospectively evaluated. The clinical differences between the patients successfully treated with drainage and those who required more invasive procedures were analyzed.
Invasive procedures, such as video-assisted thoracic surgery (n=121), fibrin glue administration through a chest tube (n=8) and pleurodesis with OK-432 (n=21), were performed in 34% (150/441) of the patients. The treatment rate of chest drainage alone was higher in the patients with initial pneumothorax (72%; 124/170) than in those with recurrent pneumothorax (62%; 167/271) (p=0.015). In addition, this rate was higher in the patients with moderate lung collapse (70%; 167/237) than in those with severe lung collapse (61%; 124/204) (p=0.032).
Patients with recurrent pneumothorax or severe lung collapse associated with prolonged air leakage are more likely to receive invasive procedures.
No preview · Article · Oct 2013 · Internal Medicine
[Show abstract][Hide abstract] ABSTRACT: Re-expansion pulmonary edema is an uncommon condition that occurs when a collapsed lung is expanded. The aim of the present study was to investigate the incidence and risk factors associated with re-expansion pulmonary edema which may occur as a complication when carrying out treatment for spontaneous pneumothorax.
A total of 462 patients with spontaneous pneumothorax treated with chest tube drainage in inpatient settings at the Nissan Tamagawa Hospital during the 6-year period between January 2007 and December 2012 were retrospectively evaluated. The data were analyzed to identify any clinical differences between the patients with and without re-expansion pulmonary edema.
Re-expansion pulmonary edema occurred on 30 (6.5 %) of the 462 patients. The duration of lung collapse in the patients with re-expansion pulmonary edema was longer than that observed in the patients without re-expansion pulmonary edema. (7.7 ± 9.1 and 2.4 ± 4.6 days). This difference was statistically significant (P < 0.0001). The extent of lung collapse in the patients with re-expansion pulmonary edema was more severe than that observed in the patients without re-expansion pulmonary edema. This difference was also statistically significant (P = 0.004).
The results suggest that treating spontaneous pneumothorax using chest tube drainage requires careful consideration in view of the relatively high incidence of re-expansion pulmonary edema, especially in cases associated with long periods of lung collapse or large spontaneous pneumothoraxes.
[Show abstract][Hide abstract] ABSTRACT: Lymphangioleiomyomatosis (LAM) is a rare disease caused by dysregulated activation of the mammalian target of rapamycin (mTOR). Sirolimus, an inhibitor of mTOR, has been reported to decrease the size of angiomyolipomas and stabilize pulmonary function in patients with LAM. However, the optimal dose for the treatment of LAM remains unclear.
We conducted a retrospective, observational study of 15 patients with LAM who underwent sirolimus therapy for more than 6 months. The efficacy was evaluated by reviewing the patients' clinical courses, pulmonary function and chest radiologic findings before and after the initiation of sirolimus treatment.
All patients had blood trough levels of sirolimus lower than 5ng/mL. Sirolimus treatment improved the annual rates of change in FVC and FEV1 in the 9 patients who were free from chylous effusion (FVC, -101.0 vs. +190.0mL/y, p=0.046 and FEV1, -115.4 vs. +127.8mL/y, p=0.015). The remaining 7 patients had chylous effusion at the start of sirolimus treatment; the chylothorax resolved completely within 1-5 months of treatment in 6 of these cases. These results resembled those of previous studies in which blood trough levels of sirolimus ranged from 5 to 15ng/mL.
Low-dose sirolimus (trough level, 5ng/mL or less) performed as well as the higher doses used previously for improving pulmonary function and decreasing chylous effusion in patients with LAM.
[Show abstract][Hide abstract] ABSTRACT: Thoracic endometriosis is a rare disease responsible for catamenial pneumothorax. The immunohistochemical features of thoracic endometriosis are not well understood. An immunohistochemical examination of 84 diaphragmatic specimens of catamenial pneumothorax using antibodies against estrogen receptor (ER), progesterone receptor (PgR), CD10 and smooth muscle actin (SMA) was conducted. The endometrial tissue was small, and focally located around the chasm of the tendon on the side of the thoracic cavity. Endometrial stroma were detected in 84/84 (100%) of the specimens, endometrial glands were detected in 21/84 (25%) and smooth muscle was detected in 1/84 (1.2%). The endometrial stroma exhibited positive staining for ER in 74/84 (88.1%) of the specimens, PgR in 84/84 (100%), CD10 in 74/84 (88.1%) and SMA in 46/84 (54.8%). Because thoracic endometriosis is small in size, and only 25% of the resected tissue specimens were accompanied with the endometrial gland, an immunohistochemical analysis can be useful for their detection. The fact that over half of the thoracic endometrial stroma showed positive staining for SMA, and the existence of thoracic endometriosis accompanied by smooth muscle, indicated that some part of the thoracic endometriosis may have the ability to differentiate into smooth muscle, although further studies are needed to confirm this hypothesis.
Preview · Article · Sep 2013 · Pathology International
[Show abstract][Hide abstract] ABSTRACT: 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.
[Show abstract][Hide abstract] ABSTRACT: Backgrounds:
Lymphangioleiomyomatosis (LAM) is a destructive lung disease that share clinical, physiologic, and radiologic features with chronic obstructive pulmonary disease (COPD). This study aims to identify those features that are unique to LAM by using quantitative CT analysis.
We measured total cross-sectional areas of small pulmonary vessels (CSA) less than 5mm(2) and 5-10mm(2) and calculated percentages of those lung areas (%CSA), respectively, in 50 LAM and 42 COPD patients. The extent of cystic destruction (LAA%) and mean parenchymal CT value were also calculated and correlated with pulmonary function.
The diffusing capacity for carbon monoxide/alveolar volume (DL(CO)/VA %predicted) was similar for both groups (LAM, 44.4 ± 19.8% vs. COPD, 45.7 ± 16.0%, p=0.763), but less tissue damage occurred in LAM than COPD (LAA% 21.7 ± 16.3% vs. 29.3 ± 17.0; p<0.05). Pulmonary function correlated negatively with LAA% (p<0.001) in both groups, yet the correlation with %CSA was significant only in COPD (p<0.001). When the same analysis was conducted in two groups with equal levels of LAA% and DL(CO)/VA %predicted, %CSA and mean parenchymal CT value were still greater for LAM than COPD (p<0.05).
Quantitative CT analysis revealing a correlation between cystic destruction and CSA in COPD but not LAM indicates that this approach successfully reflects different mechanisms governing the two pathologic courses. Such determinations of small pulmonary vessel density may serve to differentiate LAM from COPD even in patients with severe lung destruction.
No preview · Article · Sep 2012 · European journal of radiology
[Show abstract][Hide abstract] ABSTRACT: 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.