High-titer rheumatoid factor seropositivity predicts mediastinal lymphadenopathy and mortality in rheumatoid arthritis-related interstitial lung disease.

Rheumatoid arthritis-related interstitial lung disease (RA-ILD) is a common connective tissue disease-related ILD (CTD-ILD) associated with high morbidity and mortality. Although rheumatoid factor (RF) seropositivity is a risk factor for developing RA-ILD, the relationship between RF seropositivity, mediastinal lymph node (MLN) features, and disease progression is unknown. We aimed to determine if high-titer RF seropositivity predicted MLN features, lung function impairment, and mortality in RA-ILD. In this retrospective cohort study, we identified patients in the University of Chicago ILD registry with RA-ILD. We compared demographic characteristics, serologic data, MLN size, count and location, and pulmonary function over 36 months among patients who had high-titer RF seropositivity (≥ 60 IU/ml) and those who did not. Survival analysis was performed using Cox regression modeling. Amongst 294 patients with CTD-ILD, available chest computed tomography (CT) imaging and serologic data, we identified 70 patients with RA-ILD. Compared to RA-ILD patients with low-titer RF, RA-ILD patients with high-titer RF had lower baseline forced vital capacity (71% vs. 63%; P = 0.045), elevated anti-cyclic citrullinated peptide titer (122 vs. 201; P = 0.001), CT honeycombing (50% vs. 80%; P = 0.008), and higher number of MLN ≥ 10 mm (36% vs. 76%; P = 0.005). Lung function decline over 36 months did not differ between groups. Primary outcomes of death or lung transplant occurred more frequently in the high-titer RF group (HR 2.8; 95% CI 1.1-6.8; P = 0.028). High-titer RF seropositivity was associated with MLN enlargement, CT honeycombing, and decreased transplant-free survival. RF titer may be a useful prognostic marker for stratifying patients by pulmonary disease activity and mortality risk.

Minimally invasive surgical management of spontaneous esophageal perforation (Boerhaave's syndrome).

BACKGROUND: Spontaneous esophageal perforation (Boerhaave's syndrome) is a highly morbid condition traditionally associated with poor outcomes. The Pittsburgh perforation severity score (PSS) accurately predicts risk of morbidity, length of stay (LOS) and mortality. Operative management is indicated among patients with medium (3-5) or high (> 5) PSS; however, the role of minimally invasive surgery remains uncertain. METHODS: Consecutive patients presenting with Boerhaave's syndrome with intermediate or high PSS managed via a thoracoscopic and laparoscopic approach from 2012 to 2018 were reviewed. Demographics, clinical presentation, management, and outcomes were analyzed. RESULTS: Ten patients (80% male) with a mean age of 61.3 years (range 37-81) were included. Two patients had intermediate and eight had high PSS (7.9 ± 2.8, range 4-12). The mean time from onset of symptoms to diagnosis was 27 ± 12 h and APACHE II score was 13.6 ± 4.9. Thoracoscopic debridement and primary repair was performed in eight cases, with two perforations repaired primarily over a T-tube. Laparoscopic feeding jejunostomy was performed in all patients. Critical care LOS was 8.7 ± 6.8 days (range 3-26), while inpatient LOS was 23.1 ± 12.5 days (range 14-46). Mean comprehensive complications index was 42.1 ± 26.2, with grade IIIa and IV morbidity in 60% and 10%, respectively. One patient developed dehiscence at the primary repair, which was managed non-operatively. In-hospital and 90-day mortality was 10%. CONCLUSION: Minimally invasive surgical management of spontaneous esophageal perforation with medium to high perforation severity scores is feasible and safe, with outcomes which compare favorably to the published literature.

Primary and Rescue Endoluminal Vacuum Therapy in the Management of Esophageal Perforations and Leaks.

BACKGROUND: To investigate the efficacy of primary and rescue endoluminal vacuum (EVAC) therapy in the treatment of esophageal perforations and leaks. METHODS: We conducted a retrospective review of a prospectively gathered, Institutional Review Board (IRB) approved database of EVAC therapy patients at our center from July 2013 to September 2016. RESULTS: In all, 13 patients were treated for esophageal perforations or leaks. Etiologies included iatrogenic injury (n = 8), anastomotic leak (n = 2), Boerhaave syndrome (n = 1), and bronchoesophageal fistula (n = 2). In total, 10 patients underwent primary treatment and three were treated with rescue therapy. Mean Perforation Severity Scores (PSSs) in the primary and rescue treatment groups were 7 and 10, respectively. Average defect size was 2.4 (range: 0.5-6) cm. The rescue group had a shorter mean time to defect closure (25 vs. 33 days). In all, 12 of 13 defects healed. One death occurred following the implementation of comfort care. One therapy-specific complication occurred. Hospital length of stay (LOS) was longer in the rescue group (72 vs. 53 days); however, the intensive care unit (ICU) duration was similar between groups. Totally, 10 patients (83%) resumed an oral diet after successful defect closure. CONCLUSION: Utilized as either a primary or rescue therapy, EVAC therapy appears to be beneficial in the management of esophageal perforations or leaks.

Outcome of stent grafting for esophageal perforations: single-center experience.

BACKGROUND: Recent studies showed that stent grafting is a promising technique for treatment of esophageal perforation. However, the evidence of its benefits is still scarce. METHODS: Forty-three consecutive patients underwent stent grafting for esophageal perforation at the Oulu University Hospital, Finland. The main endpoints of this study were early and mid-term mortality. Secondary outcome endpoints were the need of esophagectomy and additional surgical procedures on the esophagus and extraesophageal structures. RESULTS: Patients' mean age was 64.6 ± 13.4 years. The mean delay to primary treatment was 23 ± 27 h. The most frequent cause of perforation was Boerhaave's syndrome (46.5%). The thoraco-abdominal segment of the esophagus was affected in 58.1% of cases. Minor primary procedures were performed in 25 patients (58.1%) and repeat surgical procedures in 23 patients (53.5%). Forty-nine repeat stent graftings were performed in 22 patients (50%). Two patients (4.7%) underwent esophagectomy, one for unrelenting preprocedural stricture of the esophagus and another for persistent leakage of a perforated esophageal carcinoma. The mean length of stay in the intensive care unit was 6.0 ± 7.5 days and the in-hospital stay was 24.3 ± 19.6 days. In-hospital mortality was 4.6%. Three-year survival was 67.2%. CONCLUSIONS: Stent grafting seems to be an effective less invasive technique for the treatment of esophageal perforation. Repeat stent grafting and procedures on the pleural spaces are often needed to control the site of perforation and for debridement of surrounding infected structures. Stent grafting allows the preservation of the esophagus in most of patients. The mid-term survival of these patients is suboptimal and requires further investigation.

The 30-day mortality and hospital mortality after chest surgery described in the annual reports published by the Japanese Association for Thoracic and Cardiovascular Surgery.

OBJECTIVES: The Japanese Association for Thoracic and Cardiovascular Surgery has conducted annual surveys of thoracic surgery throughout Japan. METHODS: The purpose of this study was to examine the 30-day mortality and hospital mortality after chest surgery per year to confirm the surgical outcomes of modern medical care in the area of respiratory surgery. RESULTS: The mean of the 30-day mortality/hospital mortality over a period of 16 years for the patients with lung cancer, metastatic pulmonary tumors, mediastinal tumors, inflammatory pulmonary disease, empyema, and spontaneous pneumothorax was 0.60/1.20, 0.26/0.41, 0.26/0.45, 0.32/0.50, 1.77/4.15, and 0.07/0.10, respectively. Undergoing thoracic surgery is therefore relatively safe in Japan. The death rates associated with lung cancer and mediastinal tumors have gradually decreased owing to therapeutic improvements in recent years. However, this tendency was not true of empyema. The 30-day mortality/hospital mortality rates for empyema were particularly high. CONCLUSIONS: These data suggest that the rates for empyema might have reached the limit for the current surgical techniques, and that there might be room for improvement by developing new techniques or management strategies. These data from the nationwide surveys can be useful for surgeons, because they can provide a better understanding of the present problems, as well as future prospects.

[Perforations near the cardia in benign diseases]. / Kardianahe Perforation bei gutartigen Erkrankungen.

Esophageal perforations nearby the cardia are a clinical disorder of various causes. Perforations occur most often following diagnostic or interventional endoscopy but spontaneous perforations (Boerhaave syndrome) are less frequent. Due to the heterogeneous etiology there is a broad range of therapeutic options. In most cases the esophageal perforation site can be covered by an endoscopic stent. Recent endoscopic procedures are the intraluminal application of an endoscopic vacuum-assisted closure system (endo-VAC) or clipping of the esophageal defect. Surgical procedures include direct suturing with external coverage of the defect or transhiatal blunt dissection of the esophagus without primary reconstruction. All endoscopic and surgical procedures often require an additional drainage of the mediastinum and if necessary of the thoracic and abdominal cavities. The clinical presentation ranges from a simple perforation without concomitant esophageal pathology to a defect of considerable length with pleural perforation and associated septic multiple organ failure. The severity of the septic course is the crucial parameter for the choice of the procedure. An early multiple organ failure indicates an insufficient drainage of the septic focus and is indicative for surgical resection. The overall mortality is given as 12 % in the current literature and primarily depends on the localization and the etiology of the perforation. The highest mortality rates are observed with Boerhaave syndrome. The most important prognostic variable is the time interval between perforation and initiation of therapy whereby the mortality rises up to 20 % if the interval exceeds 24 h. Due to the complex therapy and the poor prognosis esophageal perforations should be treated in specialized centers.

The role of esophageal stents in the management of esophageal anastomotic leaks and benign esophageal perforations.

OBJECTIVE: The aim of this review was to assess the safety and effectiveness of esophageal stents in the management of benign esophageal perforation and in the management of esophageal anastomotic leaks. BACKGROUND: Benign esophageal perforation and postoperative esophageal anastomotic leak are often encountered. Endoscopic placement of esophageal stent across the site of leakage might help control the sepsis and reduce the mortality and morbidity. METHODS: All the published case series reporting the use of metallic and plastic stents in the management of postoperative anastomotic leaks, spontaneous esophageal perforations, and iatrogenic esophageal perforations were identified from MEDLINE, EMBASE, and PubMed (1990-2012). Primary outcomes assessed were technical success rates and complete healing rates. Secondary outcomes assessed were stent migration rates, stent perforation rates, duration of hospital stay, time to stent removal, and mortality rates. A pooled analysis was performed and subgroup analysis was performed for plastic versus metallic stents and anastomotic leaks versus perforations separately. RESULTS: A total of 27 case series with 340 patients were included. Technical and clinical success rates of stenting were 91% and 81%, respectively. Stent migration rates were significantly higher with plastic stents than with metallic stents (40/148 vs 13/117 patients, respectively; P = 0.001). Patients with metallic stents had significantly higher incidence of postprocedure strictures (P = 0.006). However, patients with plastic stents needed significantly higher number of reinterventions (P = 0.005). Mean postprocedure hospital stay varied from 8 days to 51 days. There was no significant difference in the primary or secondary outcomes when stenting was performed for anastomotic leaks or perforations. CONCLUSIONS: Endoscopic management of esophageal anastomotic leaks and perforations with the use of esophageal stents is technically feasible. It seems to be safe and effective when performed along with mediastinal or pleural drainage. Esophageal stent can, therefore, be considered as a treatment option in the management of patients who present early after esophageal perforation or anastomotic leak with limited mediastinal or pleural contamination.

Boerhaave's syndrome: lessons learned from 83 cases over three decades.

The authors describe their experience in the treatment of 83 Boerhaave patients. During the last few years the mortality of the disease has decreased. A successful treatment requires good treatment resources and experienced team work. The tailored open primary repair technique with fundic reinforcement, developed by the authors, is described in detail. This technique has decreased the amount of postoperative fistulation and esophageal resection. The mortality after stenting was 20%.

Minimally invasive thymectomy and open thymectomy: outcome analysis of 263 patients.

BACKGROUND: An open thymectomy is a morbid procedure. If a minimally invasive thymectomy is performed without compromising the tenets of thymic surgery, it has the potential for decreasing morbidity and may offer similar clinical and oncologic results. METHODS: This is an institutional review board-approved, retrospective study of a single center's experience with both open (transsternal) and minimally invasive (video-assisted thoracoscopic surgery) thymectomy. Survival estimates and statistical comparisons were calculated using standard software. RESULTS: From 2000 to 2011, 263 patients (93 men; median age, 49 years; interquartile range, 37 to 60 years) underwent thymectomy for indications including myasthenia gravis (n=139) and mediastinal mass (n=108). Seventy-seven thymectomies were performed by minimally invasive approach. Both groups were equally stratified by sex, body mass index, World Health Organization and Masaoka-Koga staging, incidence of myasthenia gravis, and comorbidities except hyperlipidemia and diabetes. The minimally invasive thymectomy cohort had significantly shorter hospital (p<0.01) and intensive care unit lengths of stay (p<0.01) and a lower estimated blood loss (p<0.01). There was an insignificant difference in postoperative cardiac and respiratory complication rates as well as vocal cord paralysis (p=0.60). There was no difference in terms of operative room times (p=0.88) or volume of blood products transfused (p=0.16) between the two groups. Higher estimated blood loss was associated with higher intensive care unit admission rates (p<0.01). All minimally invasive thymoma resections were complete, with negative margins. CONCLUSIONS: Minimally invasive thymectomy is safe and achieves a comparable resection and postoperative complication profile when used selectively for all indications, including myasthenia gravis and small thymomas without vascular invasion.

Multiple inflammatory myofibroblastic tumors involving lung and mediastinum: a rare clinical entity.

OBJECTIVE: Inflammatory myofibroblastic tumors (IMT) are a rare clinical entity. We retrospectively reviewed the clinicopathological characteristics and prognosis for all patients with surgically resected IMTs of the lung at our institution. MATERIAL AND METHOD: From January 1995 through February 2007, 16 patients, 9 men and 7 women ranging in age from 18 to 64 years with a median age of 46 years, were admitted to our hospital for IMT of the lung, mediastinum and thoracic outlet. Nine of them (56.3 %) had a history of pneumonia, while in the rest it was documented as an incidental finding on chest X-ray. Five of our patients (31.3 %) were under immunosuppressive therapy. CT scan was the diagnostic tool routinely used and PET performed turned out to be positive in 5 cases. Wedge resection was performed in the majority of cases along with 2 lobectomies and 2 segmentectomies. The resected lesions were studied histologically and immunohistochemically. There were no operative deaths. Follow-up was complete in all patients and ranged from 9 months to 135 months. No recurrence was observed. RESULTS: Overall 5-year survival was 93.8 %. Fifteen patients are still alive and the cause of death in one case was not related to the pseudotumor. Cox regression analysis was performed for different factors such as age, sex, previous pneumonia and immunosuppression. None of them was found to play a role in the development of an IMT. The type of intervention also did not seem to affect the prognosis in our series. CONCLUSION: IMTs are a rare clinical entity. An accurate preoperative diagnosis is difficult and complete resection remains the treatment of choice and leads to an excellent survival.

Advanced Hodgkin disease with large mediastinal involvement can be treated with eight cycles of chemotherapy alone after a major response to six cycles of chemotherapy: a study of 82 patients from the Groupes d'Etudes des Lymphomes de l'Adulte H89 trial.

BACKGROUND: The prognostic impact of large mediastinal involvement (mediastinum/thorax [M/T] ratio > 0.33) in advanced Hodgkin disease (HD) and the optimal treatment with chemotherapy or combined treatment remains controversial. METHODS: Among 533 assessable patients with Ann Arbor Stage IIIB/IV HD included in the H89 trial, 82 had large mediastinal mass defined on chest X-ray. All patients received induction with six cycles of chemotherapy (mechlorethamine, vincristine, procarbazine, prednisone-doxorubicin, bleomycin, vinblastine or doxorubicin, vinblastine, bleomycin, procarbazine, prednisone); then complete and good partial responders were randomized between two consolidation treatments: 2 cycles of the same chemotherapy or (sub)total lymph node irradiation. RESULTS: Among 82 patients with an M/T ratio greater than 0.33, 48 were very large (ratio > 0.45). A large mediastinal mass was associated with supradiaphragmatic disease, younger age, histologic nodular sclerosis, and different sex ratio compared with other H89 trial patients. Biologic parameters and prognostic factors were similar for both groups. Although the major response rate to induction chemotherapy (after 6 cycles) was lower for patients with large mediastinal mass (78% vs. 86%), the 5-year overall survival rate (80% vs. 79%) and event free survival rate (59% vs. 61%) were similar (P = 0.64 and 0.3, respectively). The outcome was the same for patients (74%) with a large mediastinal mass randomized to 1 of the 2 consolidation arms. Analysis of progression showed that 68% (21 of 31) of failures occurred early during treatment and involved the mediastinum in 86% of the cases. CONCLUSIONS: For patients with large mediastinal mass and advanced HD who achieved a major response of at least 75% after 6 cycles of chemotherapy, a consolidation radiation therapy can be replaced by 2 additional cycles of chemotherapy.

[Bleeding as a cause of mortality in thoracic surgery]. / Krovotechenie kak prichina letalnosti v torakalnoi khirurgii.

Analysis of hospital lethality showed that hemorrhage was the cause of lethal outcomes in thoracic surgery among 0.5% of all hospitalized patients, 0.8% of those who underwent operation, and 17.3% of all patients who died. Among all fatal hemorrhages 28.6% were surgical and 71.4% were erosional. Surgical hemorrhage was due to injury inflicted to the large vessels (atria) during the operation. Erosional hemorrhages were caused by postoperative infectious-septic complications, progressive malignant tumors and pyo-purulent diseases, and exacerbation of peptic ulcer or the formation of an acute gastric ulcer. Among the principal causes of fatal hemorrhages are defective treatment (32.9% of cases), methodical and technical errors during the operation (28.6%), erroneous diagnosis (25.3%), initially severe condition of patients (6.6%), and progressive malignant tumor (6.6%). Intraoperative prevention of fatal surgical hemorrhage is based on personal experience, knowledge and skill of surgeons, and the use of modern technology in the control of blood loss. The prevention of erosional hemorrhage in patients who are not operated on consists in timely surgical treatment and early diagnosis in the postoperative period and active treatment of infectious-septic complications.

Clinical manifestation of mediastinal fibrosis and histoplasmosis.

We treated 20 patients thought to have mediastinal fibrosis secondary to Histoplasma capsulatum. All but 1 were symptomatic. The most common symptoms were dyspnea (8), hemoptysis (6), postobstructive pneumonia (5), and superior vena caval obstruction (2). Nine patients had severe stenosis of the trachea, carina, or main bronchus. Special stains identified Histoplasma capsulatum in surgical specimens in 9 patients. Surgical procedures were done for 18 of 20 patients (resection of subcarinal mass, 6; right middle and lower lobectomy, 5; carinal pneumonectomy, 4; esophagoplasty, 4; sleeve resection, 3 (with right main bronchus in 1, right lower and middle lobectomy in 1, and carina in 1); right upper lobectomy, 1; middle lobectomy, 1; and bronchoplasty of left main bronchus, 1. There were 4 deaths, 3 after complications of carinal pneumonectomy and 1 in a patient with tracheobronchial obstruction that could not be dilated. Two patients were treated with amphotericin and 4 with ketoconazole. Sclerosing mediastinitis secondary to histoplasmosis presents tremendous surgical challenges because of the intense fibrosis encountered. Bronchoplastic procedures are possible in spite of the intense fibrosis. High mortality rates after carinal resection may be encountered. The exact role of antifungal therapy is as yet undefined.

[Indications for surgery and results of 207 thoracotomies in children with diseases of the lung, pleura and mediastinum]. / Indikation zur Operation und Ergebnisse bei 207 thorakotomierten Kindern mit Erkrankung der Lunge, der Pleura und des Mediastinums.

Between 1968 and 1988, 207 children with congenital, inflammatory, and neoplastic diseases of the lung, pleura, and mediastinum underwent thoracotomy. In 34 patients indication for operation was a therapy-resistent recurrent spontaneous pneumothorax, in 25 benign and malignant mediastinal tumors, in 26 pulmonary metastases of extrathoracic primary tumors, in 42 bronchiectasis and post-pneumonic empyema with callosity, in 21 bronchogenic and enterogenous cysts. 22 children had benign tumors of the trachea, bronchi, and lung, 5 malignant tumors of the lung and chest wall. In a smaller number of children congenital defects, parasitic cysts, and aspergillomas as well as foreign bodies, were present. The surgical procedure included anatomical and atypical resections, bronchoplastic interventions, exstirpation of tumors and cysts, decortications and partial resections of the parietal pleura. There was no perioperative mortality.

The pathology of combined heart-lung transplantation: an autopsy study.

Clinical heart-lung transplantation (HLT) began at Stanford University (Stanford, CA) in 1981, and since then, over 40 HLTs have been performed. There is now a worldwide total of 250 HLTs. While much of the pathology that occurs in patients receiving an HLT is similar to that which develops in patients with other transplanted organ systems, these patients also develop unique clinical complications and pathologic processes that deserve emphasis. We report the autopsy findings of 20 HLT recipients, of whom 12 died in hospital one day to 4 months post-HLT. A major contributing factor in five of these postoperative deaths was pleural hemorrhage from adhesions due to prior chest surgery. Overwhelming viral and fungal infections accounted for six deaths. The seventh patient died as a result of adult respiratory distress syndrome (ARDS). Two patients showed histologic evidence of the reimplantation response. Six long-term survivors died (mean survival, 22 months) with obliterative bronchiolitis (OB). In four patients, OB was the immediate cause of death, while one patient died of an intercurrent myocardial infarct, and the other patient died of complications from an appendectomy. Two long-term survivors died without OB, one of iatrogenic causes at 63 months and the second due to unexplained ARDS at 52 months. Both patients without OB had virtually normal underlying pulmonary parenchyma. All of the long-term survivors had either coronary arterial or pulmonary vascular intimal sclerosis, and renal lesions attributable to cyclosporine A toxicity. Although histologic features of mild acute pulmonary and cardiac rejection were observed in four patients overall, these did not contribute to the cause of death in any case. Although OB is a major threat to its success, HLT is a viable option for patients with endstage pulmonary disease.