[Respiratory failure in cystic fibrosis: management in pediatric intensive care unit, lung transplantation recommendation]. / Les défaillances respiratoires dans la mucoviscidose: prise en charge en réanimation; indications de la transplantation.

Admission to the ICU for respiratory failure of a child with cystic fibrosis is a telltale sign of the severity of the disease. Bronchopulmonary exacerbation, pneumothorax and hemoptysis are the primary causes, for which respiratory assistance is indispensable in these life-threatening situations. Non-invasive ventilation (NIV) has enabled significant progress in improving patient survival. The modalities of NIV must be tailored to both the patient and the cause of respiratory failure. Invasive ventilation, on the other hand, should be a treatment of last resort, because often associated with high mortality. It must be adapted to the therapeutic strategy involving an impending transplantation, including in critical situations where placement on a high emergency list is a possibility. Since admission to ICU is at times the reflection of the terminal evolution of the disease, ongoing treatment must hence be adapted to the comfort of the child.

[How to increase availability of grafts in lung transplantion: ex vivo lung reconditioning - cardiac death donors - high emergency list]. / Comment élargir l'accès aux greffons : ex vivo reconditionnement - Donneurs décédés par arrêt cardiaque - Super-urgence pulmonaire (SU).

Lung transplantation has become an established treatment for end-stage pulmonary failure refractory to medical management. However, the scarcity of lung grafts and the growing number of candidates has led to an increase in deaths among patients on waiting lists. Despite improvements in donor management, organ preservation, and the use of marginal and cardiac death donors, only about 20% of candidate lungs are currently being transplanted. A new ex vivo "reconditioning" technique is opening up new perspectives. Indeed, a significant number of rejected lungs can now be retrieved and transplanted with acceptable results. Given the longer storage times provided by this technique, transplantation can be programmed, with better surgical efficiency. A new mobile organ-care machine is currently under evaluation. In near future, a pilot laboratory will be created and dedicated to ex vivo reconditionning of all lung grafts before transplantation and grafts will be sent to lung transplant centers after immunologic cross-matching.

Pulmonary vascular dysfunction in end-stage cystic fibrosis: role of NF-kappaB and endothelin-1.

Pulmonary hypertension is rare in chronic respiratory diseases but has a strong impact on the prognosis and is partly underlined by factors other than hypoxaemia. The aim of the present study was to assess the potential role of endothelin-1 (ET-1) and nuclear factor (NF)-kappaB vasoconstrictive pathways in pulmonary hypertension. The effects of ET-1 receptors blockers (BQ 123 and 788) and of genistein were assessed on response to acetylcholine of pulmonary vascular rings from cystic fibrosis (CF) lung transplant recipients (n = 23). NF-kappaB and ET-1 receptor expression was immunodetected in pulmonary arteries and quantitated using Western blotting. ET-1 vascular content was quantitated using ELISA. In total, 14 out of 23 subjects exhibited strongly impaired pulmonary vasodilation (p<0.01 versus nine out of 23 subjects with a normal response) associated with an activation of ET-1 receptors A and NF-kappaB pathways. Genistein restored vasodilation in subjects with an abnormal response. Pulmonary vascular dysfunction is frequent in end-stage CF, involving the NF-kappaB pathway and that of ET-1 through ET-1 receptor A (ETAR). These data leave a conceptual place for ETAR blockers and isoflavones in the management of the devastating vascular complication of chronic obstructive respiratory diseases such as CF.

Single-lung transplantation for cystic fibrosis and metachronus pneumonectomy: case reports.

Three patients with end-stage cystic fibrosis (CF) underwent single lung transplantation (SLT) with contralateral pneumonectomy. In the first case, contralateral pneumonectomy (CP) was performed before SLT. The patient is alive with no signs of infection or rejection. For the other 2 cases, CP was performed after SLT. One patient died 8 months later with septicemia; the other patient died after 10 days because of complicated bronchopleural fistula and infection of the pneumonectomy space. SLT can be a good option for some patients with CF. The outcome is good when CP is done before SLT. However, CP must be done simultaneously with SLTX.

[Lung transplantation: supply and demand in France]. / Transplantation pulmonaire: la satisfaction des besoins en France.

INTRODUCTION: For a decade lung transplantation has suffered from a lack of donor organs which aroused a national debate and led to planned action in collaboration with The French National Agency for Transplantation. BACKGROUND: Analysis of the stages of the process from potential donor to lung transplantation identified lung procurement as the main priority. An increase in the number of potential lung donors and revision of the acceptance criteria led to a doubling of the annual rate of lung transplantation in less than two years. VIEWPOINTS: In the near future we may solve the problem of donor family refusals and establish scientifically based criteria for lung acceptance to increase the rate of lung transplantation. Transplantation from non heart-beating donors and the reconditioning of ex vivo non acceptable lungs might supply additional organs to fulfill demand in the long term. CONCLUSIONS: The rate of lung transplantation activity in France doubled as the result of a dramatic increase of donor lung proposals. The current improvement in the results of lung transplantation might create new demands and generate future difficulties in the supply of donor lungs. New approaches, such as transplantation from non heart-beating donors and reconditioning ex vivo non acceptable lungs, should be examined in the near future.

Clinical outcome following lung transplantation in patients with cystic fibrosis colonised with Burkholderia cepacia complex: results from two French centres.

BACKGROUND: Infection with Burkholderia cepacia complex (BCC) is a life threatening complication of cystic fibrosis (CF), often seen as a contraindication for lung transplantation. METHODS: A long term retrospective study was conducted of all patients with CF undergoing lung transplants from January 1990 to October 2006 in two French centres allowing transplantation in patients colonised with BCC. RESULTS: 22 of the 247 lung transplant patients with CF were infected with BCC (B. cenocepacia genomovar III (n = 8), B. multivorans genomovar II (n = 11), B. vietnamiensis genomovar V (n = 2) and B. stabilis genomovar IV (n = 1)). BCC colonisation was not associated with any significant excess mortality (HR 1.5, 95% CI 0.7 to 3.2; p = 0.58). However, early mortality rates tended to be higher in the BCC group than in the non-BCC group (3 month survival: 85% vs 95%, respectively; log rank p = 0.05). Univariate analysis showed that the risk of death was significantly higher for the eight patients infected with B. cenocepacia than for the other 14 colonised patients (HR 3.2, 95% CI 1.1 to 5.9; p = 0.04). None of the other risk factors tested-primary graft failure, late extubation, septicaemia-had a significant effect. The 5 year cumulative incidence rate of bronchiolitis obliterans syndrome was not significantly higher in the BCC group than in the non-BCC group (38% vs 24%, respectively; p = 0.35). CONCLUSION: Our results suggest that BCC infection with a non-genomovar III organism may not be associated with excess mortality after lung transplantation in patients with CF and should not be seen as sufficient reason to exclude lung transplantation. However, colonisation with B. cenocepacia remains potentially detrimental.

[Lung cancer in women: surgical aspects related to gender]. / Cancer du poumon chez la femme: aspects chirurgicaux liés au sexe.

INTRODUCTION: Lung cancer is becoming more and more common in women where it presents significant differences at both clinical and therapeutic levels. Our purpose was to study those associated with surgical treatment. PATIENTS AND METHODS: 2972 patients were operated on between 1984 and 2002: 2480 men and 492 women. These two populations were compared (age, past history, investigations, interventions, TNM stage, long term survival and causes of death). RESULTS: The number of women increased with time; they were younger than the men, smoked less, had the same past history of cancer but less past medical history, and comorbidity. They underwent less pneumonectomies and had a lower postoperative mortality. Tumour size was smaller (39.5 vs 43.5cm, p=0.0001); N0 and stage I tumours were more frequent (52.6% vs 46% p=0.0074). Long term survival was better (48.6% vs 43.1%, p=0.016), particularly in stage I and with a past history of cancer. It was identical in stage III despite a higher incidence of multisite N2 disease. Smoking and adenocarcinoma were more frequent before the menopause and N2 prognosis deteriorated with age. CONCLUSION: These results confirm characteristics peculiar to lung cancer in women and warrant further investigation aimed at their better understanding. However, in multivariate analysis gender does not appear to be an independent prognostic factor.

Outpatient video-mediastinoscopy.

We reviewed our experience with video-mediastinoscopy as an outpatient procedure. From November 2002 to May 2003, 20 video-mediastinoscopies were performed on an out-patient basis. There were no operative deaths. No surgical complication is reported in a patient requiring readmission and antibiotic therapy for left pneumonia. In conclusion, video-mediastinoscopy can be safely performed on an out-patient basis in selected patients with mediastinal involvement.

Mediastinal lymphatic efferents from the diaphragm.

The prognosis of non small cell lung cancer (NSCLC) invading the diaphragm is poor, probably due to the richness of the lymphatic drainage of the diaphragm. The aim of this study was to determine mediastinal lymphatic efferents from the diaphragm. The diaphragms of 20 adult cadavers (77-104 years) were injected with a dye (modified Gerota's medium) to permit the lymph vessels to be catheterised and then dissected. Each stage of the dissection was described and photographed: 23 injections on the right and 25 on the left. Diaphragmatic lymph vessels passed to one of three lymph centres: posterior (paraaortic nodes, n = 16), anterior (juxtasternal nodes, n = 16) and mediastinal (visceral nodes, n = 16). From these lymph centres arose ascending lymph pathways: posteriorly to the thoracic duct (8/16), anteriorly along the internal thoracic vessels (10/16) and in the mediastinum to the peritracheobronchial nodes (6/10). Lymphatics from the diaphragm are abundant and drain towards mediastinal node lymph centres connecting to the blood stream via the thoracic duct. These lymph pathways are common with those of the pulmonary segments. Poor prognosis of NSCLC invading the diaphragm may be explained by the common lymphatic drainage of both the lung and diaphragm.

[Non-small cell lung cancer: surgical trends as a function of age]. / Cancer pulmonaire non à petites cellules: particularités chirurgicales en fonction de l'âge.

Most of the studies on lung cancer and age are usually done on very young or very old populations. We conducted a study of the evolution of surgical features over time in a population aged 24 to 89 years. The series included 1,809 men and 287 women (n = 2,096) who had undergone surgery between April 1984 and December 1990 (n = 1,026) and between January 1991 and December 1996 (n = 1,996). Patients were divided into 7 age groups with 2 subgroups for patients with or without prior medical history of cancer. We analyzed the type of surgery and pathology findings by age. Morbidity was recorded according to state of previous cardiovascular disease and long-term survival was analyzed. A significant increase in the number of female patients was noted during the last ten years. Adenocarcinomas were more frequent in young patients. Exploratory interventions, partial tumor resections and lung resections for metastasis were more frequently performed in young patients than in older patients. Excision of mediastinal nodes was less performed often in old patients. In case of curative resection, postoperative pTNM was not modified with age. Morbidity increased with age; mortality was more frequent in the elderly even when comorbidity was taken into account. Mortality was not related significantly with cardiovascular morbidity factors despite an increased frequency of previous cardiovascular disease with age. Survival according to age showed 3 main types of population: patients aged under 64 years, those between 65 and 74, and those over 75 years of age. Mortality increased with time but was less often related to recurrence of lung cancer. Nevertheless, survival for stage III and stage IV patients older than 75 years demonstrated that surgery was not indicated for these patients. This study shows that indications for surgery should not vary with age except for patients over 75 years who have locally advanced cancer and a risk of surgical death greater than the chances of survival. All non-small-cell lung cancers should be resected with no delay, even in the elderly population.

[Congenital esophagobronchial fistula in an adult]. / Fistule oesobronchique congénitale chez un adulte.

Congenital esophagobronchial fistulae are rare and are most often observed in neonatalogy. We present the case of a 21-year-old man with a history of chronic bronchorrhea due to middle and lower lung lobe destruction. This case demonstrates the diagnostic features in individuals with unexplained suppurating respiratory symptoms and the difficulty in identifying the congenital pathogenesis of this type of fistula.

Lung cancer occurring with Mycobacterium xenopi and Aspergillus.

Two cases which associate Mycobacterium xenopi pulmonary infection, aspergilloma and preoperative unsuspected lung cancer are related. To our knowledge, the association of these three pathologies has never been previously reported. These two cases, suggest that infected chronic lung lesions, especially in smokers, should be closely monitored and be surgically removed in order to prevent further complications.

Surgical treatment of active and sequelar forms of pulmonary tuberculosis.

BACKGROUND: The incidence of tuberculosis has risen since 1990, and in some countries, the resistant forms are becoming more and more frequent. Surgical treatment is once again needed to manage these problems. The purpose of this study was to analyze the indications and results of resection, which we performed for pulmonary tuberculosis. METHODS: From 1980 to 1997, 477 patients were operated on for thoracic or intrathoracic tuberculosis in Laennec Hospital, Paris (259 suffered lung diseases). There were 165 women and 94 men, aged 25 to 86 years (mean 46 years), from Europe (n = 148), North Africa (n = 65), Subsaharian Africa (n = 34), Asia (n = 7), and the West Indies (n = 5). This population was reviewed concerning the lung tuberculosis (sequelae or active lesions), the indications of lung resection, the type of resections performed, and the results at 1, 6, and 12 months. RESULTS: Active lesions were present in 97 cases and sequelae in 162. Surgery was performed for a therapeutic purpose in 104 patients with sequelae, and in 10 patients with active tuberculosis (pneumonectomy, n = 19; pleuropneumonectomy, n = 19; lobectomy, n = 54; and segmentectomy, n = 22). Surgery was performed for a diagnostic purpose in 54 patients with sequelae, and in 87 patients with active lesions (lobectomy, n = 32; segmentectomy, n = 19; wedge resection, n = 94, of which 11 performed by video-assisted thoracoscopy since 1991). One patient died after pleuropneumonectomy. We observed 25 complications: empyema, n = 7; hemothorax, n = 2; prolonged air leaks, n = 14; and pneumopathy, n = 2. All patients with active lesions subsequently were given antitubercular drugs. Follow-up was 100% at 1 month, 57% (n = 92) and 77% (n = 75) at 6 months for patients with sequelae and for patients with active lesions, respectively. All were asymptomatic with a normal chest roentgenogram. The number of operations for active lesions is increasing over the years, while it is decreasing for sequelar lesions. CONCLUSIONS: In our department, surgery is being performed more frequently to make a diagnosis in cases of active tuberculosis, and to treat complicated lesions in case of sequelae. Lung resection for active tuberculosis evolving under treatment or for drug resistance was rare. However, our study confirms the good results commonly obtained by surgery and supports the idea that surgery may help eradicate tuberculosis when social and economic circumstances render its medical management difficult or hazardous.

Lymphatic drainage of heart and lungs: comparison between pig and man.

In its anatomy and physiology the pig is comparable with humans and its organs can be considered for xenotransplantation. We have studied the lymphatic drainage of the heart and lungs in 15 pigs. A coloured mass was injected into the myocardium and/or beneath the visceral pleura. The first nodes coloured were directly injected again. No lymph node was observed inside the heart and lungs. The first lymph nodes coloured were the peritracheobronchial nodes. There was no node in front of the thoracic trachea (Barety's compartment in man). Left suprabronchial nodes were connected with the thoracic duct in the mediastinum. The lymphatics of the heart and lungs in the pig are similar to those of human. Phylogenesis explains "skipping" metastases and the significance of N1 disease in lung cancer, as well as chylothorax occurring after heart and lung surgery.

[Purulent pleurisy and lung cancer. Non-iatrogenic forms and therapeutic management]. / Pleurésies purulentes et cancer du poumon. Formes non iatrogènes et prise en charge thérapeutique.

Thoracic empyemas may occur during the course of lung cancer as a post-thoracotomy complication, or after pleural drainage and/or chemotherapy in cases when surgery was unfeasible, or may complicate the natural history of the disease and appear as the clinical event that led to its discovery. This latter situation is a challenge requiring to cure the infection in order to further treat the underlying lung cancer. We reviewed the cases of 18 men aged between 46 and 79 years that were referred to our surgical department from 1984 to 1996 for management of a thoracic empyema with an underlying lung cancer. Initial presentation of empyemas, lung tumor characteristics, treatments performed and their results were analyzed so as to formulate guidelines if possible. Mean duration of 17 empyemas before arrival was 26 days (8 to 60 days) and in one case empyema occurred during diagnostic work-up of an excavated lesion. Frank pus was observed in all cases and micro-organisms were identified in 13 cases. Empyema and diagnosis of lung cancer were concomitant in 15 cases: in 3 cases lung neoplasia was already diagnosed but patients had refused surgery. Empyema was treated by under water-seal chest tube drainage with adjunct fibrinolytic therapy in all cases; 2 elderly and cachectic patients suffering metastatic diffusion died rapidly. The other 16 recovered within one month. In 7 cases management was limited to medical treatment (palliative n = 2, chemotherapy n = 1, chemo combined radiotherapy n = 2 and radiotherapy alone n = 2) but only short survivals were observed (inferior to 10 months). Surgery was possible in 9 (pneumonectomy n = 8, lobectomy n = 1); there was no death; postsurgical empyemas complicated the cause twice but were easily cured by drainage; long term survivals were observed in 3 cases that were p NO. Pleural empyema complicating lung cancer is a rare but challenging situation. Once the pleural empyema has been controlled, surgical resection must be performed when indicated: postoperative complications are rare and long-term survival is possible.

[Intrathoracic coelomic cysts]. / Kystes coelomiques intrathoraciques.

Intrathoracic coelomic cysts are benign embryonic tumors with a mesothelial lining. The aim of this work was to review possible localizations (pleuropericardic and other), the remaining surgical indications, and the current situation of minimally invasive techniques. We reviewed retrospectively, 28 cases of intrathoracic coelomic cysts in 12 men and 16 women, mean age 44 years. We recorded the cyst localization, clinical signs, indication for surgery, access routes used, and outcome. Twenty-one cysts were pleuropericardial cysts and 7 were ectopic mediastinal cysts. In all 7 of the ectopic mediastinal cysts and 4 of the pleuropericardial cysts surgery was indicated for diagnosis; for the other pleuropericardial cysts the indication was based on clinical signs (n = 4), large volume (n = 4), progressing volume (n = 7), no apparent reason (n = 1) and association with surgery for pneumothorax (n = 1). Assess was by mediastinoscopy (n = 1), mediastinotomy (n = 1), sub-xyphoid route (n = 1), thoracotomy (n = 18), and videothoracoscopy (n = 7). Long-term outcomes (mean follow-up 4 years 4 months) were good with no recurrences. Postoperative sequelae were observed in 6 cases after thoracotomy and in 1 case after videothoracoscopy. In summary, pleuropericardial cysts warrant surveillance without surgery unless their volume increases or clinical signs develop. Ectopic mediastinal cysts usually require surgery for diagnosis. It would appear advisable to prefer videothoracoscopy which allows diagnosis and excision of pleuropericardial cysts. Minimal thoracotomy may be helpful for ectopic mediastinal cysts.