Low Respiratory Function Research Articles

Dynamics of ventilation parameters in patients with bilateral destructive tuberculosis after staged surgical treatment using extrapleural pneumolysis with silicone plombage.

AimTo study the dynamics of respiratory function indicators in patients with bilateral widespread destructive tuberculosis during staged surgeryt using extrapleural pneumolysis with silicone plombageon the side of the smallest lesion
 Material and methods.The main group consisted of 14 patients with widespread bilateral destructive tuberculosis who underwent extrapleural pneumolysis with silicone plombage (ESP) in combination with lung resection on the opposite side. The control group included 29 patients who underwent lung resection operations on both sides. All patients underwent spirometry before and 3-5 weeks after each stage of surgery: FVC (functional vital capacity of the lungs) and FEV1 (volume of forced exhalation in 1 second) were determined.
 Results.The dynamics of the spirometric study after staged surgery in the ESP group was a decrease in FVC by 1.050.47, FEV1 by 0.950.6 l, and in the comparison group by 1.740.76 l and 1.330.5 l, respectively (p0.05).
 Conclusion.Staged surgery with the use of ESP is accompanied by a significantly smaller decrease in functional parameters and can be used in patients with widespread bilateral destructive tuberculosis with low respiratory function.

Centrilobular emphysema and airway dysanapsis: factors associated with low respiratory function in younger smokers.

Low respiratory function in young adulthood is one of the important factors in the trajectory leading to the future development of COPD, but its morphological characteristics are not well characterised. We retrospectively enrolled 172 subjects aged 40-49 years with ≥10 pack-years smoking history who underwent lung cancer screening by computed tomography (CT) and spirometry at two Japanese hospitals. Emphysema was visually assessed according to the Fleischner Society guidelines and classified into two types: centrilobular emphysema (CLE) and paraseptal emphysema (PSE). Airway dysanapsis was assessed with the airway/lung ratio (ALR), which was calculated by the geometric mean of the lumen diameters of the 14 branching segments divided by the cube root of total lung volume on a CT scan. Among the subjects, CLE and PSE were observed in 20.9% and 30.8%, respectively. The mean ALR was 0.04 and did not differ between those with and without each type of emphysema. Multivariable regression analysis models adjusted for age, sex, body mass index and smoking status indicated that CLE and a low ALR were independently associated with lower forced expiratory volume in 1 s (FEV1)/forced vital capacity (estimate -1.64 (95% CI -2.68- -0.60) and 6.73 (95% CI 4.24-9.24), respectively) and FEV1 % pred (estimate -2.81 (95% CI -5.10- -0.52) and 10.9 (95% CI 5.36-16.4), respectively). CLE and airway dysanapsis on CT were independently associated with low respiratory function in younger smokers.

Dynamics of respiratory indicators in patients with bilateral destructive tuberculosis after surgical treatment using staged bilateral extrapleural pneumolysis with silicone plombage

Introduce. Patients with a high prevalence of the process and extensive destruction of the pulmonary parenchyma have reduced lung reserves, which limits the use of resection interventions.Objective. To study the dynamics of respiratory function indicators in patients with bilateral widespread destructive tuberculosis during surgical treatment using staged bilateral extrapleural pneumolysis with silicone plombage (ESP).Object and methods. The main group consisted of 11 patients who underwent surgical treatment using staged bilateral ESP. The comparison group included 8 patients with bilateral widespread destructive pulmonary tuberculosis who underwent consecutive bilateral lung resections with a volume of more than 3 segments. The dynamics of respiratory function indicators were studied 3–5 weeks after surgery.Results. In the main group, after stage-by-stage surgical treatment, the FVC index decreased by 0.9 ± 0.6 liters, while in the comparison group by 1.3 ± 0.7 liters (p < 0.01). The index of FEV1 in patients of the main group decreased by 0.5 ± 0.5 liters, and in the comparison group by1 ± 0.3 liters (p < 0.01).Conclusion. Staged surgical treatment with the use of bilateral ESP is accompanied by a significantly smaller decrease in functional parameters and can be used in patients with widespread bilateral destructive tuberculosis with low respiratory function.

Study of human ribosomal gene complex in cystic fibrosis

Increasing the life expectancy of patients with CF is an urgent healthcare task all over the world. According to the Register of patients with cystic fibrosis in the Russian Federation (2020), the number of patients over 18 years of age is 26.5%. Assumably, cystic fibrosis can be used as a model of accelerated aging to study the aging process in general.Aim of the study was to analyze the number of rDNA copies in a sample of cystic fibrosis patients at different ages and with lethal outcome in relation to lung function, complications, and respiratory tract infections.Methods. We studied DNA samples isolated by the standard method from peripheral blood leukocytes of 277 patients diagnosed with cystic fibrosis. 998 DNA samples from healthy volunteers were used as a control group.Results. The study showed that the genomes of patients with CF contain more rDNA copies than those of control patients. The greatest number of copies of ribosomal genes was observed in DNA samples from deceased patients (p < 0.001) and was associated with more severe disease course. Among all CF patients, the largest number of rDNA copies in the genome was registered in patients with the lowest FEV1 values (less than 40%). It was found that patients with chronic Burkholderia cepacia complex infection had a significantly higher number of copies of ribosomal repeats than the total sample (p = 0.001) and the adults (p = 0.014). The number of ribosomal repeats did not differ between patients with other chronic respiratory tract infections.Conclusion. In the group of deceased patients, the patients with low respiratory function and Burkholderia cepacia complex infection had the highest number of rDNA copies in the genome, and the differences were significant. It can be assumed that the number of rDNA copies in the genome of CF patients is an additional prognostic marker that is associated with the patient’s life expectancy.

Association between sarcopenia and respiratory function in elderly orthopedic outpatients.

The relationship between sarcopenia and respiratory function has not been investigated in elderly Japanese orthopedic outpatients. This study aimed to assess the relationship between sarcopenia and respiratory function in elderly orthopedic outpatients in Japan.Elderly outpatients (n = 102; aged ≥65 years) with orthopedic diseases were enrolled in the study. Muscle mass was measured using the bioelectrical impedance analysis. Grip strength and walking speed were measured (2 trials). The respiratory function (e.g., percent vital capacity [VC] and percent forced expiratory volume in 1 second) was also measured. The association between sarcopenia and respiratory function was assessed using logistic regression analysis, adjusting for sex, comorbidities, and smoking status.The mean ages for men and women were 77.7 ± 8.3 and 76.5 ± 6.8 years, respectively, and the overall prevalence of sarcopenia was 25.5% (30.0% and 24.4% in men and women, respectively). The study demonstrated that low respiratory function, which was represented by decreased percent VC, was associated with sarcopenia in outpatients visiting an orthopedic department (odds ratio, 1.73; 95% confidence interval: 1.02–2.97).Sarcopenia was significantly associated with a lower percentage of VC in orthopedic outpatients after adjustment for sex, comorbidity, and smoking. Further studies are needed to determine the causality.

436 PRONE THORACOSCOPIC ESOPHAGECTOMY FOR PATIENTS WITH LOW PULMONARY FUNCTION

Abstract Thoracoscopic esophagectomy in the prone position (TEP) for esophageal cancer is reported to have superiority in preserving postoperative respiratory function and reducing postoperative respiratory complications. In Japan, the majority of patients with esophageal cancer are smokers and have obstructive ventilation disorders. But, the feasibirity and safety of TEP for patients with low respiratory function is unclear. Objectives To clarify the feasibirity and safety of TEP for esophageal cancer patients with obstructive respiratory function. Methods The 95 patients with obstructive respiratory disorder who underwent TEP and gastric tube reconstruction via posterior mediastinal route for esophageal cancer from January 2016 to April 2019 were divided into the two groups, low respiratory function (LRF) group and the control group. Short-term outcomes were compared between two groups. Results The control group was 73 cases, and the LRF group was 22 cases. Propensity score matching using age, gender, cT, and cN as covariates was used to identify matched patients (22 per group) in both groups. There were no differences in operation time of overall and intrathoracic part, or blood loss in each group. In the postoperative complications, pneumonia (13.6% vs. 9.1%), recurrent laryngeal palsy (18.2% vs. 22.7%), anastomotic leakage (13.6% vs. 13.6%) and hospital stay (36.3 days vs 27.5 days) were no differences in both groups. Conclusion TEP can be feasible and safe for the patients with obstructive ventilation disorder and low respiratory function.

T2-High Endotype and Response to Biological Treatments in Patients with Bronchiectasis.

Although bronchiectasis pathophysiology has been historically understood around the presence of airway neutrophilic inflammation, recent experiences are consistent with the identification of a type 2 inflammation (T2) high endotype in bronchiectasis. In order to evaluate prevalence and clinical characteristics of bronchiectasis patients with a T2-high endotype and explore their response to biologicals, two studies were carried out. In a cross-sectional study, bronchiectasis adults without asthma underwent clinical, radiological, and microbiological assessment, along with blood eosinophils and oral fractional exhaled nitric oxide (FeNO) evaluation, during stable state. Prevalence and characteristics of patients with a T2- high endotype (defined by the presence of either eosinophils blood count ≥300 cells·µL−1 or oral FeNO ≥ 25 dpp) were reported. A case series of severe asthmatic patients with concomitant bronchiectasis treated with either mepolizumab or benralizumab was evaluated, and patients’ clinical data pre- and post-treatment were analyzed up to 2 years of follow up. Among bronchiectasis patients without asthma enrolled in the cross-sectional study, a T2-high endotype was present in 31% of them. These patients exhibited a more severe disease, high dyspnea severity, low respiratory function, and high impact on quality of life. Among the five patients with severe eosinophilic asthma and concomitant bronchiectasis included in the series, treatment with either mepolizumab or benralizumab significantly reduced the exacerbation rate with an effect that persists for up to 2 years of follow up. If validated across different settings, our data suggest the need to design randomized controlled trials on biological treatments targeting the T2-high endotype in bronchiectasis patients.

Respiratory Sarcopenia and Sarcopenic Respiratory Disability: Concepts, Diagnosis, and Treatment.

The condition of muscle fiber atrophy and weakness that occurs in respiratory muscles along with systemic skeletal muscle with age is known as respiratory sarcopenia. The Japanese Working Group of Respiratory Sarcopenia of the Japanese Association of Rehabilitation Nutrition narratively reviews these areas, and proposes the concept and diagnostic criteria. We have defined respiratory sarcopenia as “whole-body sarcopenia and low respiratory muscle mass followed by low respiratory muscle strength and/or low respiratory function.” Respiratory sarcopenia can be caused by various factors such as aging, decreased activity, undernutrition, disease, cachexia, and iatrogenic causes. We have also created an algorithm for diagnosing respiratory sarcopenia. Respiratory function decreases with age in healthy older people, along with low respiratory muscle mass and strength. We have created a new term, “Presbypnea,” meaning a decline in respiratory function with aging. Minor functional respiratory disability due to aging, such as that indicated by a modified Medical Research Council level 1 (troubled by shortness of breath when hurrying or walking straight up hill), is an indicator of presbypnea. We also define sarcopenic respiratory disability as “a disability with deteriorated respiratory function that results from respiratory sarcopenia.” Sarcopenic respiratory disability is diagnosed if respiratory sarcopenia is present with functional disability. Cases of respiratory sarcopenia without functional disability are diagnosed as “at risk of sarcopenic respiratory disability.” Functional disability is defined as a modified Medical Research Council grade of 2 or more. Rehabilitation nutrition, treatment that combines rehabilitation and nutritional management, may be adequate to prevent and treat respiratory sarcopenia and sarcopenic respiratory disability.

Clinical characteristics and prognosis in patients with chronic thromboembolic pulmonary hypertension and a concomitant psychiatric disorder.

Chronic thromboembolic pulmonary hypertension (CTEPH) can cause right heart failure. A concomitant psychiatric disorder (PD) is thought to increase the risk of acute pulmonary thromboembolism; however, whether PDs are associated with deterioration in CTEPH pathophysiology is unclear. In this study, we evaluated the clinical characteristics and prognoses in patients with CTEPH and a co-existing PD. We retrospectively identified 229 consecutive patients (mean age = 58.7 ± 12.5 years; 160 women) with CTEPH and categorized them according to whether they had a PD (PD group; n = 22, 9.7%) or not (non-PD group; n = 207, 90.3%). We compared the clinical characteristics, respiratory function, hemodynamics, and clinical courses in the two groups. Those in the PD group had significantly lower exercise tolerance compared to the non-PD group (6-min walk test, 309.5 ± 89.5 m vs. 369.4 ± 97.9 m, P = 0.008, percent vital capacity 85.5% ± 17.3% vs. 96.0% ± 15.5%, P = 0.003) and partial pressure of oxygen (PaO2) (54.4 ± 8.6 mmHg vs. 59.3 ± 10.7 mmHg, P = 0.039). Three-year survival was significantly poorer in the PD group compared to the non-PD group (66.1% vs 89.7%, P = 0.0026, log-rank test), particularly in patients who underwent surgery (62.2% vs 89.5%, P < 0.001, log-rank test). A concomitant PD was associated with low exercise tolerance and impaired respiratory function in patients with CTEPH and predicted poor survival, especially in those who underwent a pulmonary endarterectomy.

Disadvantaged Early-Life Socioeconomic Circumstances Are Associated With Low Respiratory Function in Older Age.

Poor lung function in late life may stem from early-life risk factors, but the epidemiological evidence is inconsistent. We investigated whether individuals who experienced disadvantageous socioeconomic circumstances (SEC) in early life showed lower levels of respiratory function in older age, a steeper decline over time, and whether these relationships were explained by adult-life SEC, body mass index, and physical inactivity in older age. We used data from the Survey of Health Ageing and Retirement in Europe (2004-2015). Participants' peak expiratory flow (PEF) was assessed with a mini-Wright peak flow meter at second, fourth, and sixth waves. Confounder-adjusted linear mixed-effect models were used to examine the associations between early-life SEC and PEF in older age. A total of 21,734 adults aged 50-96 years (46,264 observations) were included in the analyses. Older adults with disadvantaged early-life SEC showed lower levels of PEF compared with those with advantaged early-life SEC. The association between early-life SEC and late-life PEF persisted after adjusting for adult-life SEC, smoking, physical inactivity, and body mass index. PEF declined with age, but the effect of early-life SEC on this decline was not consistent across robustness and sensitivity analyses. Early life is a sensitive period for respiratory health. Further considering the effect of SEC arising during this period may improve the prevention of chronic respiratory diseases.

Interaction of Calmodulin with the cSH2 Domain of the p85 Regulatory Subunit.

Calmodulin (CaM) is a calcium sensor protein that directly interacts with the dual-specificity (lipid and protein) kinase PI3Kα through the SH2 domains of the p85 regulatory subunit. In adenocarcinomas, the CaM interaction removes the autoinhibition of the p110 catalytic subunit of PI3Kα, leading to activation of PI3Kα and promoting cell proliferation, survival, and migration. Here we demonstrate that the cSH2 domain of p85α engages its two CaM-binding motifs in the interaction with the N- and C-lobes of CaM as well as the flexible central linker, and our nuclear magnetic resonance experiments provide structural details. We show that in response to binding CaM, cSH2 exposes its tryptophan residue at the N-terminal region to the solvent. Because of the flexible nature of both CaM and cSH2, multiple binding modes of the interactions are possible. Binding of CaM to the cSH2 domain can help release the inhibition imposed on the p110 subunit, similar to the binding of the phosphorylated motif of RTK, or phosphorylated CaM (pCaM), to the SH2 domains. Amino acid sequence analysis shows that CaM-binding motifs are common in SH2 domains of non-RTKs. We speculate that CaM can also activate these kinases through similar mechanisms.

Thoracoscopic right S6 sleeve segmentectomy for squamous-cell carcinoma arising from the B6 central bronchus

We describe a patient with low respiratory function who underwent thoracoscopic sleeve segmentectomy to preserve lung function as much as possible. The patient had already used home oxygen therapy because of chronic obstructive lung disease. There was a squamous-cell carcinoma at inlet of right B6 bronchus, and cT1aN0M0 disease was diagnosed. Because respiratory function was poor, right S6 segmentectomy was scheduled. Moreover, to preserve the respiratory muscles as much as possible, a thoracoscopic approach was selected. We performed S6 sleeve segmentectomy, and sutured the lower bronchus and basal bronchus. There were some limitations in handling needles during thoracoscopy. To resolve these difficulties, we devised two techniques. One was to suture the bronchus with continuous sutures on the mediastinal side and simple interrupted sutures on the other side. The other was to create a working space for handling the needles to avoid entanglement of the sutures. These techniques allowed us to suture the bronchi relatively easily.

Metachronous Lung Cancer Developing after 11 Years of Chemo-Radiotherapy in a Patient with Non-Small Cell Lung Carcinoma

The success rate of the chemo-radiotherapy is low for the patients with lung cancer. The risk of second primary malignancy (SPM) in lung cancer survivors is also not well reported in the literature. A 77-year-old male patient was given chemo-radiotherapy (KT_RT) 11 years ago because of the low respiratory function of the non-small cell lung cancer in the right lung. He applied to hospital for complaining cough. NSCLC was diagnosed and doctors performed left lower lobectomy to the patient. We presented the case in which the patient was cured after chemo-radiotherapy which is an extremely rare condition and the development of a second tumor 11 years later.

Assessment of Headache in Asthma Patients.

Background and Objective:Headache is a common health problem, which may present with neurological diseases and other chronic diseases, and has an adverse effect on the emotional status. We think that headache is a common disease in asthmatic patients. This study aims to evaluate the presence of headache and risk factors in patients with asthma.Methods:Ninety-three patients with asthma and 58 healthy control subjects were included in the study. The presence of headache was evaluated according to the revised criteria of the International Classification of Headache Disorders, 2nd edition (ICDH-II). Asthma control test (ACT) was performed to determine asthma control status. The Beck Depression Inventory (BDI) and Beck Anxiety Inventory (BAI) were performed in all participants. Demographic features, used medications, and presence of headaches were recorded.Results:Fifty-eight patients with asthma (62.4%) had headaches, whereas only 19 control subjects (32.8%) had headaches. Thirty-two patients (34.4%) had tension-type headache, 19 patients (20.3%) had migraine-type headache, and 7 patients (7.5%) had other types of headaches. The frequency of headaches was significantly higher in patients with asthma, compared to healthy control subjects (p=0.001). There was a significant correlation between migraine-type headache and inhaled steroid use, and presence of allergies.Conclusion:Migraine-type and tension-type headaches are more common in patients with asthma, compared to the overall population. The frequency of migraine-type headache is higher in patients with asthma who have allergies and low respiratory function test scores.

Efficacy of intensive immunosuppression in exacerbated rheumatoid arthritis-associated interstitial lung disease

Objectives: Acute or subacute exacerbations are recognized as a severe complication of rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Nevertheless, the role of intensive immunosuppression in RA-ILD remains elusive. We attempted to evaluate the clinical characteristics and efficacy of immunosuppressive treatment in exacerbated RA-ILD.Methods: Clinical data, including respiratory function, imaging, treatment, and prognosis, were retrospectively collected for 17 patients with RA-ILD who required hospitalization at the University of Tokyo Hospital due to an acute exacerbation (12 patients) or subacute exacerbation (5 patients).Results: Patients with RA-ILD demonstrated a significantly higher titers of anticyclic citrullinated peptide antibodies compared with RA patients in Japanese Ninja registry, suggesting the role of adaptive immunity. Immunosuppressive treatment suppressed the deterioration of pulmonary functions with improved ground grass opacity and consolidation. In particular, in patients with less fibrosis on computed tomography (CT) images showed a better response to treatment. Although five patients treated with combination therapy, including cyclophosphamide, showed a severely decreased lung volume, these intensive therapies provided a good prognosis without fatalities for the average observation period of 474 days.Conclusions: Immunosuppressive therapy is effective for exacerbations of RA-ILD. For severe cases with low respiratory function, intensive therapy, including cyclophosphamide, has a potential to improve the prognosis.

47. Autonomic dysfunction in ALS patients: An early phase study_preliminary data

Amyotrophic Lateral Sclerosis (ALS) is a fatal degenerative motoneuron disease. Since 40 years, study investigating a possible Autonomic Nervous System (ANS) involvement are reported. Although results are contradictory and studies difficult to compare, ALS appears not to be a single disease entity, but a spectrum of disorders involving mainly, but not only, motoneurons. The aim of the study was to investigate cardiovascular ANS function in ALS patients with low disability and normal respiratory function. We have included patients diagnosed with ALS according to El Escorial criteria, able to walk unaided. We excluded patients with swallowing difficulties, affected by autonomic dysfunction of other origin and taking ANS influencing drugs. We also excluded patients with respiratory insufficiency. Patients underwent cholinergic and adrenergic evaluations: SSR and standard autonomic tests during continuous monitoring of heart rate and blood pressure. We evaluated HRV, BRS and spectral analysis of HF and LF of RRI and BP. We compared patients with matched normal controls. We did not find any significant difference between groups, indicating a tendency toward a normal function of ANS in early phase of disease. We will expand our sample and possibily try a genotipic–phenotipic correlation, given the heterogeneity of ALS spectrum.

Low respiratory function co-existing with smoking habit is associated with elevated morning BP -The J-HOP study

The clinical characteristics of the tenth decile group in the standard deviation for awake systolic blood pressure in the korean ambulatory blood pressure monitoring registry data Sang-Hyun Ihm, Wook Bum Pyun, Soon Kil Kim, Kwang-il Kim, Sungha Park, Jinho Shin. Ewha Women University of Korea, Seoul, Republic of Korea; Hanyang University, Seoul, Republic of Korea; Seoul National University, Bundang, , Republic of Korea; The Catholic University of Korea, Bucheon St. Mary’s Hospital, Bucheon, , Republic of Korea; Yousei University, Seoul, Republic of Korea

PO-0132 Acid Reflux Into The Oesophagus And Exercise: A Prospective Study In Children

<h3>Background and aims</h3> It has been reported that gastro-oesophageal reflux (GOR) can be induced by exercise, as described in adult subjects; studies in children are lacking. We sought whether the presence of acid in the oesophagus may increase with exercise and its potential relationship with atopy and lung function in children. <h3>Methods</h3> We recruited 45 patients (M/F: 30/15) aged 11 ± 2.7 years with reported exercise-induced respiratory symptoms; subjects were asked for frequency of gastrointestinal symptoms. All patients did lung function before and after 24-h gastro-oesophageal (GO) pH monitoring; they also underwent exercise testing (treadmill) before removing GO catheter. GO-pH was also analysed for 6 min intervals before, during and after exercise. The gastro-oesophageal reflux disease (GORD) was defined as a 24-hour reflux index (IR) ≥ 4.5% and/ or symptom index ≥ 50%. Total serum IgE levels were also assessed. <h3>Results</h3> GORD was found in 11/45 (24.4%) of our patients; these children had also a higher IR score during exercise than patients without GORD (7.1 ± 18.5 vs 0.5 ± 2.3, p &lt; 0.05). A fall of GO-pH was recorded during exercise, greater in children with GORD as compared with those without (17.2 ± 42.2 vs 0.9 ± 6.4, p = 0.03). The exercise-induced fall in GO-pH was associated with frequent gastrointestinal symptoms and correlated with IgE levels and baseline FEV₁% (IgE: r = -0.37, FEV1%: r = -0.31, p &lt; 0.05 for both). <h3>Conclusions</h3> Our results suggest that oesophageal acidity increase with exercise, particularly in atopic children with frequent gastrointestinal symptoms and low baseline respiratory function.

Bone loss in adults with cystic fibrosis: Prevalence, associated factors, and usefulness of biological markers

Osteoporosis is a common complication of cystic fibrosis. To determine the prevalence of osteopenia and osteoporosis in adults with cystic fibrosis and to look for factors associated with low bone mineral density (BMD) values. During this prospective 2-year study, we collected clinical data (history, body mass index, and treatments) and laboratory data (nutritional and hormonal status, markers for inflammation and bone turnover, and calcium and phosphate levels). Lung function tests and a 6-meter walking test were performed. BMD was measured at the spine, total hip, and femoral neck. We included 55 patients (31 women) with a mean age of 31.9 years; 14 were homozygous and 25 heterozygous for the deltaF508 mutation and 20 (36%) had a history of fractures. BMD values indicated osteopenia in 32 (58.2%) patients and osteoporosis in 11 (20%) patients at one or more measurement sites. At all three sites, BMD values correlated with the body mass index, forced vital capacity as % predicted, and forced expiratory volume in 1 s as % predicted; at the femoral neck and total hip, BMD values correlated with the 6-meter walking test result and grip strength. BMD was low in 78.2% of adults with cystic fibrosis. Three factors reflecting lung disease severity were associated with low BMD values, namely, respiratory function, physical function (walking test and grip strength), and nutritional status.

Cystoperitoneal Shunt for a Giant Intrathoracic Meningocele Under Local Anesthesia

Giant intrathoracic meningoceles are extremely rare, and the standard treatment for giant intrathoracic meningoceles remains controversial. We present the case of a patient with giant intrathoracic meningoceles associated with neurofibromatosis type I. Our patient had poor respiratory function because of the giant intrathoracic meningocele, so we performed a cystoperitoneal shunt under local anesthesia. We describe our cystoperitoneal shunt technique using an adjustable-pressure valve. This simple, minimally invasive treatment is a valuable alternative treatment option in patients at high operative risk, especially those with low respiratory function.