M. Rolla

Thoracoscopic resection of mediastinal bronchogenic cysts in adults

OBJECTIVE Bronchogenic cysts are uncommon congenital anomalies of foregut origin usually located within the mediastinum and the lung and rarely diagnosed in adults. Surgical excision is recommended to establish diagnosis based on histologic examination, alleviate symptoms if present, and prevent future complications. Thoracoscopic approach is becoming the primary therapeutic option. METHODS Between January 1995 and July 2008, 30 patients with mediastinal bronchogenic cyst (MBC) underwent thoracoscopic operation (19 male, 11 female with a mean age of 39 years, range 19-59 years). Symptoms were present in 11 patients (37). RESULTS The cysts averaged 5.2 cm in their greatest diameter (range 3-10.5 cm). In two cases thoracoscopy was converted to thoracotomy because of major pleural adhesions. There were no operative deaths and no intra-operative complications. Postoperative course was uneventful in all cases and the 28 patients who underwent thoracoscopy were discharged after a mean of 3.7 days (range 2-5 days). CONCLUSIONS Considering the low conversion and complication rate, thoracoscopic excision of bronchogenic mediastinal cyst should be considered the primary therapeutic option.

Bronchoscopic lung volume reduction as a bridge to lung transplantation in patients with chronic obstructive pulmonary disease

BACKGROUND Chronic obstructive pulmonary disease (COPD) is the leading indication for lung transplantation; however, these patients rarely gain priority on the waiting list until very late. The clinical status can be improved by surgical lung volume reduction; this procedure, although carries significant morbidity, has been repeatedly advocated as a bridge. Recently, bronchoscopic lung volume reduction (BLVR) has been proposed to improve functional parameters in patients with emphysema; however, it has never been reported as a bridge to lung transplantation so far. METHODS We hereby report our experience with BLVR as a bridge to lung transplantation in four patients (males, mean age 51 years). RESULTS All patients underwent unilateral BLVR (two right upper lobe (RUL), one right lower lobe (RLL), and one left upper lobe (LUL); mean 3.5 valves per patient). No morbidity and mortality were observed. Three out of the four patients successfully reached transplantation after 6, 7, and 6 months, respectively. Two patients received single-lung transplantation and one sequential double-lung transplantation. The fourth patient died of respiratory failure 13 months after valve placement. BLVR was able to reduce the residual volume and improve the 6-min walking test and Medical Research Council (MRC) score. CONCLUSIONS BLVR allowed to improve the functional status and quality of life of these patients. In a selected group of COPD patients awaiting lung transplantation, the reported short- to medium-term objective improvement may play an important role to ameliorate the clinical status and reach the time of surgery.

Extracorporeal Circulatory Support for Lung Transplantation: Institutional Experience

Lung transplantation (LT) represents the only available therapy for selected patients affected by end-stage pulmonary disease. Cardiopulmonary bypass (CPBP) is used, when required, during single and sequential double lung transplantation; however, it increases the risk of bleeding, early graft dysfunction, failure, and other potential side effects. We report our experience with 145 patients who underwent lung transplantations, among whom 34 required intraoperative CPBP. The indications for LT among these 34 patients were cystic fibrosis (n = 22), chronic obstructive pulmonary disease (n = 3), bronchiectasis (n = 2), primary pulmonary hypertension (n = 1), fibrosis (n = 2), pulmonary microlithiasis (n = 1), and retransplantation for obliterative bronchilitis (n = 3). CPBP was planned in 12 cases (group I) and unplanned in 22 (group II). The main reason for planning CPBP was primary and secondary pulmonary hypertension (mean pulmonary artery pressure >or=25 mm Hg). Acute right ventricular failure, hemodynamic instability, arterial desaturation, and increased pulmonary artery pressure were mandatory for unplanned CPBP. Among the 34 CPBP patients, the 30-day mortality rate was 35% (12/34) including 9 (70%) in group II (unplanned CPBP). The leading cause of death was multiorgan failure. The 1-year survival rates were 67% and 36%, and the 3-year survival rates were 47% and 18% for groups I and II, respectively. In conclusion, even if it represents a useful tool in the management of critical events, the use of unscheduled CPBP during LT procedures is associated with an increased postoperative morbidity and mortality.

Cystic fibrosis and the thoracic surgeon

Indications for thoracic surgery in patients with cystic fibrosis (CF) are principally represented by pleural diseases including pneumothorax, pleural effusion, and empyema and by parenchymal lung diseases including bronchiectasis, hemoptysis, and pulmonary abscess. Moreover, lung transplantation has proved a viable therapeutic option for progressive respiratory failure due to end-stage CF. Main surgical experiences in this setting are reviewed and discussed.

Improved Results With Lung Transplantation for Cystic Fibrosis

YSTIC fibrosis (CF) is the most frequently inheritedlethal disorder among caucasians. Improvements intherapy have resulted in an average life span extending intothe third decade of life; however, no cure is available at thepresent time and 95% of deaths are related to chronicobstructive lung disease, bronchiectasis, and consequentrespiratory failure.

Lung Transplantation for Cystic Fibrosis After Thoracic Surgical Procedures

During their life, cystic fibrosis (CF) patients may require thoracic surgical procedures for a number of reasons before undergoing lung transplantation. In the past, this has been considered to be a contraindication to lung transplantation. However, a meticulous surgical technique and careful intraoperative management allows one to perform the transplantation safely. Herein we have reported our experience with CF patients undergoing lung transplantation after previous surgical treatment for pneumothorax or bronchiectasis.

Lung Transplantation for Cystic Fibrosis: Ten Years of Experience

Lung transplantation represents the only therapeutic option for patients affected by end-stage cystic fibrosis (CF). We performed 76 lung transplantations in 73 patients from 1996-2007. The mean time on the waiting list was 10+/-6 months. The median follow-up after the transplantation was 69.3 months. Twenty-one transplants (27.6%) were performed under cardiopulmonary bypass. Perioperative mortality, excluding retransplants, was 16.4% (12 patients) and the causes of death were sepsis, primary graft failure, and myocardial infarction. The overall survival was 74.5%+/-5%, 62.9%+/-5%, 54.1%+/-6%, and 43.4%+/-6% at 1, 3, 5, and 10 years, respectively. The accurate selection of potential recipients and the correct timing of referral and transplantation are factors that play crucial roles to obtain satisfactory results in term of improvement of quality of life and long-term survival.

Lung Transplantation for Cystic Fibrosis in Italy

Lung transplantation (LT) is the only effective form of therapy for cystic fibrosis (CF) associated with end-stage pulmonary failure. In Italy, the management of CF is regulated by national law, which has instituted regional centers for care and follow-up of all CF patients. LT has been performed since 1992 in only nine LT certified centers. The structured national organization has led to a unified database for LT for CF. As of December 2006, 197 bilateral LT (96 male and 94 female patients; 7 retransplants) have been performed. Of these, four had also liver or heart and liver transplantation, and three are long-term survivors. Overall median survival is 7 years. Mean age at transplantation is 26.5 years, and the mortality on the waiting list is 33.6%. Patients listed for transplant either received a suitable donor within a mean of 10 months or died within a mean of 5.5 months. The most frequent cause of death is bronchiolitis obliterans syndrome (BOS). Our nationwide database indicates the excellent results obtained by LT in FC. Still, mortality on the waiting list remains a challenge and long-term outcome is limited by BOS.

Treatment of Complex Airway Lesions After Lung Transplantation With Self-Expandable Nitinol Stents: Early Experience

Airway complications (AC) are considered a serious cause of morbidity after lung transplantation (LT). Mechanical dilatation, laser vaporization, and silicone stent placement usually solve it. However, the use of self-expandable metallic stents (SENS) may be indicated in selected cases. Ten lung transplant recipients with AC were treated with SENS. Six patients underwent LT for cystic fibrosis, 2 for idiopathic pulmonary fibrosis, 1 for bronchiectasis, and 1 for emphysema. All patients received at least 1 treatment attempt with dilatation and silicone stent placement. The indications for SENS placement were the presence of a tortuous airway axis with stenosis and malacia of the right main bronchus in 5 patients; a long stenosis of the main and intermediate right bronchus involving the upper lobe orifice in 3 patients; or malacia that could not be stabilized with silicone stents in 3 cases. In 1 patient the procedure was bilateral. Functional improvement was immediate with a mean forced expiratory volume at 1 second (FEV(1)) gain of 35%. No stent dislocation was observed. Symptoms did not occur again in 5 patients with previous recurrent episodes of pneumonia. One stenosis, which was due to the ingrowth of granulation tissue occurred at 6 months after the procedure, was successfully treated with mechanical dilatation and laser vaporization. The deployment of SENS in a selected group of patients with AC after LT was easy, safe, and effective.

Idarubicin plus Cytosine-Arabinoside (ALL R-87 Protocol) in Advanced Acute Lymphoblastic Leukemia: The GIMEMA/AIEOP Experience

In our previous experience’, the combination of HDARA-C at a dosage of 3 g/m2/12 hours x 6 doses plus Idarubicin (IDA) (12mg/m2/day x 3 doses), follosed by bone marrow transplant (BMT) as intensive post-remission phase was employed in patients with advanced acute lymphoblastic leukemia (ALL). CR was achieved in 59% of these patients, however only 40% of them were elegible for BMT. With the aim to improve these results, we devised a new study, including induction with a different ARA-C plus IDA schedule, and a multidrug consolidation therapy, followed by BMT. The induction therapy consisted of ARA-C 1 g/m2 given as a continuous infusion for 6 hours daily for 6 days, plus IDA 5mg/m2/day as a 30-minute infusion administered 3 hours after ARA-C for a total of 6 doses (Figure 1). Responding patients received post-remission therapy, including vindesine (5 mg/m2/weekly x 2) followed by 2 courses of the L-VAMP regimen2. Intrathecal methotrexate (MTX) was given for 3 weekly doses during the postremission phase. Patients still in CR who were