Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis: results from a multicentre experience.

OBJECTIVES: Tracheobronchial stenosis (TBS) is noted in 12-23% of patients with granulomatosis with polyangiitis (GPA), and includes subglottic stenosis and bronchial stenosis. We aimed to analyse the endoscopic management of TBS in GPA and to identify factors associated with the efficacy of endoscopic interventions. METHODS: We conducted a French nationwide retrospective study that included 47 patients with GPA-related TBS. RESULTS: Compared with patients without TBS, those with TBS were younger, more frequently female and had less frequent kidney, ocular and gastrointestinal involvement and mononeuritis multiplex. Endoscopic procedures included 137 tracheal and 50 bronchial interventions, mainly endoscopic dilatation, local steroid injection and conservative laser surgery, and less frequently stenting. After the first endoscopic procedure, the cumulative incidence of endoscopic treatment failure was 49% at 1 year, 70% at 2 years and 80% at 5 years. Factors significantly associated with a higher cumulative incidence of treatment failure were a shorter time from GPA diagnosis to endoscopic procedure [hazard ratio (HR) 1.08 (95% CI 1.01, 1.14); P = 0.01] and a bronchial stenosis [HR 1.96 (95% CI 1.28, 3.00); P = 0.002]. A prednisone dose ≥30 mg/day at the time of the procedure was associated with a lower cumulative incidence of treatment failure [HR 0.53 (95% CI 0.31, 0.89); P = 0.02]. CONCLUSION: TBS represents severe and refractory manifestations with a high rate of restenosis. High-dose systemic CSs at the time of the procedure and increased time from GPA diagnosis to bronchoscopic intervention are associated with a better event-free survival. In contrast, bronchial stenoses are associated with a higher rate of restenosis than subglottic stenosis.

Automatic administration of propofol and remifentanil guided by the bispectral index during rigid bronchoscopic procedures: a randomized trial.

BACKGROUND: Anesthesia for rigid bronchoscopic procedures is a demanding procedure. Automatic titration of propofol and remifentanil to maintain the bispectral index (BIS) within the recommended range (40-60) has been reported during routine surgical procedures. The aim of the present study was to evaluate its use during rigid bronchoscopy. METHODS: Patients were enrolled in a randomized study comparing manual target-controlled infusion of propofol and remifentanil (manual TCI group) with automatic titration guided by the BIS (dual-loop group). Categorical variables were compared by the Fisher's exact test, and continuous variables (median [interquartile range 25-75]) were compared by the Mann-Whitney test. RESULTS: Thirty-four patients were included in the manual TCI group and 33 were included in the dual-loop group. Baseline characteristics were well balanced between the groups. Intervention by the anesthesiologist in charge to modify propofol and/or remifentanil targets in the dual-loop group was not necessary. Percentage of time spent in the BIS interval (40-60) was similar in the manual TCI and dual-loop groups (69% [48-79] vs 70% [58-80], respectively). Durations of induction and of maintenance and propofol and remifentanil doses were also similar between groups, except for the amount of propofol needed for induction (P = 0.002). Time to tracheal extubation was also similar. No case of intraoperational awareness was detected. CONCLUSION: The present study could not establish the superiority of automatic system over manual adjustment for bronchoscopy. Further studies with a different design and a larger number of patients are required to establish the place of automatic delivery of anesthetic agents. This study was registered at ClinicalTrials.gov number, NCT00571181.

Presentation, Diagnosis, and Management of Subglottic and Tracheal Stenosis During Systemic Inflammatory Diseases.

BACKGROUND: Subglottic stenosis (SGS) and tracheal stenosis (TS) are characterized by a narrowing of the airways. The goal of this study was to describe the characteristics and prognosis of nontraumatic and nontumoral SGS or TS. RESEARCH QUESTION: What are the inflammatory etiologies of SGS and TS, and what are their characteristics and prognosis? STUDY DESIGN AND METHODS: This multicenter, observational retrospective study was performed in patients with SGS or TS that was neither traumatic nor tumoral. RESULTS: Eighty-one patients were included, 33 (41%) with granulomatosis with polyangiitis (GPA) and 21 (26%) with relapsing polychondritis (RP). GPA-related stenoses exhibited circumferential subglottic narrowing in 85% of cases, without calcifications. In contrast, RP-related stenoses displayed anterior involvement in 76%, in a longer distance from vocal cords (4 cm), with calcifications in 62%, and extension to bronchi in 86%. Other diagnoses included bullous dermatoses (n = 3), amyloidosis (n = 3), sarcoidosis (n = 2), and Crohn's disease (n = 2); the remaining stenoses (n = 15) were idiopathic. SGS/TS was the initial manifestation of the disease in 66% of cases, with a median interval from stenosis to disease diagnosis of 12 months (interquartile range, 0-48 months). Despite the use of glucocorticoids in 80%, combined with methotrexate in 49%, endoscopic procedures were required in 68% of patients. Relapses of stenoses occurred in 76% without any difference between causes (82% in GPA, 67% in RP, and 75% in idiopathic SGS/TS). Three patients died due to the stenosis, two of RP and one of GPA. INTERPRETATION: These data show that GPA and RP are the two main inflammatory diseases presenting with SGS/TS. GPA-related stenoses are mostly subglottic and circumferential, whereas RP-related stenoses are mostly tracheal, anterior, and calcified with a frequent extension to bronchi. Relapses of stenoses are common, and relapse rates do not differ between causes. Diagnosis and management of SGS/TS require a multidisciplinary approach.

Optimal Management With Radical Resection of Obstructive Primary Chondrosarcoma of the Trachea.

We report 2 cases of chondrosarcoma of the trachea. This etiology of tracheal tumors is exceptional, and only a few cases have been reported so far. The optimal management for these 2 cases was challenging. First an interventional bronchoscopy was required for biopsy and to prevent airway obstruction. Second a radical en bloc resection with free margins was performed through a sternotomy in the first case and by a cervicotomy in the second case. Fifty and 6 months after surgery the 2 patients are alive with no local or distant recurrence.

Radical Carinal Resection for a Glomic Tumor.

We report the case of a 33-year-old woman who presented with increasing dyspnea secondary to a tumor arising from the carina. After desobstruction by bronchoscopy, the pathologic analysis revealed a glomic tumor. Carinal resection and reconstruction were performed with venoarterial extracorporeal membrane oxygenation support. The patient's postoperative course was uneventful, and the long-term result was excellent.

A new endoscopic standardized grading system for macroscopic central airway complications following lung transplantation: the MDS classification.

OBJECTIVES: After lung transplant, between 9 and 13% of bronchial anastomoses develop complications severe enough to warrant therapeutic intervention. These complications include stenosis, dehiscence, granulation tissue, bronchomalacia and fistula. Most of these have already been included in a classification or another, but none of these have been universally accepted. Moreover, no grading system has integrated all of these complications. The Groupe Transplantation (GT) (Transplant Group), from the Société de Pneumologie de Langue Française (SPLF) [French Language Pulmonology Society], maintains a prospective national registry of lung transplants performed in France. The GT has mandated the Groupe d'Endoscopie de Langue Française (GELF), also from the SPLF, to develop an endoscopic classification, in order to describe the macroscopic aspect of the bronchial anastomoses, and downhill airways, using a standardized and exhaustive grading system. METHODS: An endoscopic classification that would take into account the three major aspects of the description of bronchial anastomoses was elaborated. The first parameter is the macroscopic aspect (M), the second, the diameter (D) of the anastomosis and the third, the sutures (S) of the anastomosis. This classification was then submitted to expert bronchoscopists from nine centres, responsible for lung transplants in France, for their opinion, using a five-item questionnaire, according to the Delphi methodology. RESULTS: After the first round of consultation, all experts (100%) agreed on Questions 1 and 4. Answers were positive for Questions 2 (59%), 3 (56.25%) and 5 (70%). A modified classification, incorporating propositions from the first round, was then submitted. This second round allowed a consensus to be reached between all experts: the MDS classification. Each parameter (M, D and S) can be classified from 0 to 3. For M and D, it is possible to determine the extent of abnormalities downhill from the anastomosis into four subgroups (a, b, c or d). For S, the localization of abnormalities can be divided between two subgroups (e and f). CONCLUSION: The MDS classification, established by a consensus of French experts in bronchoscopy, could represent a standardized, universally acceptable system to describe central airway complications after lung transplant.

The management of bronchus intermedius complications after lung transplantation: a retrospective study.

BACKGROUND: Airway complications following lung transplantation remain a significant cause of morbidity and mortality. The management of bronchial complications in Bronchus Intermedius (BI) is challenging due to the location of right upper bronchus. The aim of this study was to analyze the results of BI Montgomery T-tube stent in a consecutive patients with lung transplantations. METHODS: Between January 2007 and December 2010, 132 lung transplantations were performed at Foch Hospital, Suresnes, France. All the patients who had BI Montgomery T-tube after lung transplantation were included in this retrospective study. The demographic and interventional data and also complications were recorded. RESULTS: Out of 132 lung transplant recipients, 12 patients (9 male and 3 female) were entered into this study. The indications for lung transplantation were: cystic fibrosis 8 (67%), emphysema 3 (25%), and idiopathic pulmonary fibrosis 1 (8%). Most of the patients (83%) had bilateral lung transplantation. The mean interval between lung transplantation and interventional bronchoscopy was 11.5 ± 9.8 (SD) months. There was bronchial stenosis at the level of BI in 7 patients (58.3%). The Montgomery T-tube number 10 was used in 9 patients (75%). There was statistically significant difference in Forced Expiratory Volume in one second (FEV1) before and after stent placement (p = 0.01). The most common complication after stent placement was migration (33%). CONCLUSION: BI complications after lung transplantation are still a significant problem. Stenosis or malacia following lung transplantation could be well managed with modified Montgomery T-tube.

Hyperacute rejection after lung transplantation caused by undetected low-titer anti-HLA antibodies.

Hyperacute rejection is an early complication of transplantation, caused by the presence in the recipient of pre-formed donor-directed antibodies (Ab). We report a case of fatal early graft dysfunction after lung transplantation in a female recipient with no anti-HLA Ab detected before transplantation. Further immunologic studies revealed the presence, in the pre-transplant serum, of low-titer anti-HLA Ab directed against the donor's HLA, detectable only by flow-cytometry screening for panel-reactive antibodies. This observation emphasizes the importance of sensitive Ab detection in patients awaiting lung transplantation. Women should be specifically targeted for such sensitive Ab detection.