Ilaria Righi

Autoimmune hepatitis: an uncommon presentation of thymoma

Purpose In a substantial proportion of patients with thymoma, many different types of paraneoplastic syndromes are observed. The association between thymoma and autoimmune liver diseases, however, has been found in very few cases. Methods We report the case of a 31-year-old man affected by autoimmune hepatitis associated with myasthenia gravis and thymoma, successfully treated with extended thymectomy. Results The patient is free from neoplastic and hepatic disease 4 years after surgery. Eighteen months after thymectomy, an exacerbation of hepatitis was successfully treated with steroids. Conclusions To the authors’ knowledge, only 7 cases of myasthenia gravis associated with thymoma and autoimmune hepatitis have been reported in the English-language literature. The exact role of thymoma in immune-mediated hepatitis is unclear. It seems likely that thymoma-associated T-cell abnormalities, due to the presence of thymoma, may have a role in the development of this rare clinical triad of autoimmune hepatitis, thymoma and myasthenia gravis.

Color-Doppler sonography patterns in endobronchial ultrasound-guided transbronchial needle aspiration of mediastinal lymph-nodes

BACKGROUND Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is commonly used for the pathological diagnosis in patients with mediastinal lymphadenopathy. Even though the accuracy of EBUS-TBNA is considered high, several authors have tried to outline characteristic ultrasound criteria for malignant mediastinal lymph-node identification to maximize this accuracy. Recently, a color-Doppler patterns classification has been proposed by Nakajima to target suspected mediastinal lymph-nodes. The aim of this study was to assess such classification. METHODS Clinical, sonographic and pathological data of patients with suspected malignant mediastinal lymph-nodes on computed tomography (CT) and/or on positron emission tomography (PET) were prospectively recorded. On the basis of color-Doppler patterns, mediastinal lymph-nodes were classified in four grades and the bronchial-artery inflow sign was noted according to the Nakajima classification. The diagnostic sensitivity, specificity, positive predictive value (PPV), negative predicted value (NPV) and accuracy were calculated using standard definitions. RESULTS Seventy-five patients were enrolled in this prospective study, median age 66 years; 85% of patients had primary lung cancer and 15% had extra-thoracic malignancy. EBUS-TBNA was performed in 87 lymph-nodes (rate 1.16 per patient); 6 lymph-nodes had inadequate samples and were excluded from the analysis; 64 nodes were confirmed as malignant and 17 nodes as benign. Color-Doppler patterns classification was as follow: grade 0-I, 14 lymph-nodes; grade II-III, 67 lymph-nodes; bronchial-artery inflow sign, 8 lymph-nodes. The sensitivity, specificity, PPV, NPV, and diagnostic accuracy of the classification were 0.93, 0.64, 0.84, 0.6 and 0.81 respectively. CONCLUSIONS The color-Doppler sonography is a fast, reproducible and effective tool that could help in targeting suspected malignant lymph-nodes during EBUS-TBNA. The current study validates the efficacy of the color-Doppler patterns classification proposed by Nakajima.

Lung Allocation Score: A Single-Center Simulation.

BACKGROUND The lung allocation score (LAS) was introduced in the United States in May 2005 with the main goal of reducing the waiting list mortality of patients with end-stage lung diseases, but also to enhance the lung transplant benefit and improve the management of urgent candidates. Several papers have reported that LAS resulted in a reduction of the waiting list mortality but no significant survival benefit was noted. METHODS We evaluate the usefulness of LAS as a predictor for lung transplantation outcome in 123 patients listed for lung transplantation in an Italian center. Primary endpoints were waiting list mortality and posttransplant mortality at 1 year; secondary endpoints included perioperative circulatory support, cardiopulmonary bypass, primary graft dysfunction, and long-term survival after transplantation. RESULTS We observed the absence of correlation between LAS and waiting list mortality. The LAS did not affect the long-term survival in our population. CONCLUSIONS High LAS was predictive of primary graft dysfunction of grade 3 in the first 72 hours after transplantation.

Lung transplantation as successful treatment of end-stage idiopathic pleuroparenchimal fibroelastosis: a case report

Pleuroparenchymal fibroelastosis (PPFE) is a rare condition, characterized by predominantly upper-lobe pleural and subjacent parenchymal fibrosis, the latter being intra-alveolar with accompanying elastosis of the alveolar walls that leads a clinical progression to respiratory failure. This condition may not be as rare as it seems to be, because nowadays the increasing awareness among specialists is raising the number of new diagnoses. Limited data are available about the prognosis, both for secondary and idiopathic forms. Nevertheless, the idiopathic form seems to be rapidly progressive and no treatment can control the disease, which is why management is challenging. Since the disease was characterized, PPFE cases have been reported in the literature, but most have been secondary rather than idiopathic. Of these, few have successfully undergone lung transplantation as a treatment of end-stage respiratory failure. We here report a successful case of a 38-year-old man affected by idiopathic PPFE who underwent bilateral lung transplantation after extracorporeal membrane oxygenation bridging for an abrupt transition to critical clinical conditions. After a complex postoperative course and a first year characterized by acute rejection, the patient is alive at 5 years with a good quality of life. Our experience confirms that lung transplantation would be a valuable treatment option in case of end-stage idiopathic PPFE cases.

Lung transplantation from donor with tracheal bronchus: case report and literature review

Donor lung abnormalities are quite rare; one of them is the presence of bronchial anomalies, whose incidence range is from 0.1% to 0.5%. The upper right tracheal bronchus is one of the most frequent anatomic variations. We present a case of successful double lung transplant in a young female patient affected by cystic fibrosis from a donor with upper right tracheal bronchus, emerging 2 rings before the tracheal carina. During implantation of the left lung, we performed a double apical segmentectomy on back table; therefore, the right lung was implanted with the standard technique. Four cases of graft transplant characterized by the presence of tracheal bronchus are reported in the scientific literature; the authors report 4 different technical solutions to tackle the problem of anatomic anomaly. We report the first case of graft segmentectomy at back table suggesting a simple, safe, and time-sparing procedure. In conclusion, provided that the team has sufficient skill in reductive surgery at the back table and the anthropometric data are permissive, we stress the opportunity to downsize the graft in order to minimize anastomotic risks and save time.

Prophylactic continuous positive airway pressure after pulmonary lobectomy: a randomized controlled trial

Background Despite advances in perioperative care and surgical techniques, patients undergoing pulmonary lobectomy are still at high risk for postoperative complications. Among interventions expected to reduce complications, continuous positive airway pressure (CPAP) is a discussed option. This trial aims to test the hypothesis whether prophylactic application of CPAP following pulmonary lobectomy can reduce postoperative complications. Methods The study was designed as a prospective, randomized, controlled trial. Patients with clinical stage I non-small cell lung cancer scheduled for pulmonary lobectomy were eligible and were trained for the use of CPAP interface. The control group received standard postoperative pain management and physiotherapy; in addition, the study group received CPAP (PEEP 8-12 cmH2O, 2 hours thrice daily for three days). Results After the appropriate selection, 163 patients were considered for the analysis: 82 patients constituted the control group, 81 the study group. The two groups were substantially comparable for preoperative parameters. The rate of postoperative complications was lower in the study group (24.7% vs. 43.9%; P=0.015) as well as the hospital stay (6 vs. 7 days; P=0.031). The stepwise logistic regression model identified: CPAP [odd ratio (OR): 0.3026, CI: 0.1389-0.6591], smoke habits [OR: 2.5835, confidence interval (CI): 1.0331-6.4610] and length of surgery in minutes (OR: 1.0102, CI: 1.0042-1.0163) as regressors on postoperative complications. Conclusions The present trial demonstrated that prophylactic application of CPAP during the postoperative period after pulmonary lobectomy for stage I non-small cell lung cancer was effective in prevent postoperative complications.

Lobar Lung Transplantation From Deceased Donor: Monocentric Experience

INTRODUCTION Lung transplantation is considered a therapeutic option in selected patients affected by end-stage pulmonary disease. The mortality on the waiting list is mainly attributed to the shortage of the donor pool available for transplantation. There are various strategies to overcome this shortage; one of them is lobar transplantation. METHODS The aim of the current study was to analyze the outcome of lobar lung transplantation from deceased donors in our Lung Transplant Center. Overall survival, perioperative mortality and morbidity, problem on bronchial anastomosis, and chronic rejection were prospectively recorded in a 5-year time-frame. RESULTS From November 2010 to October 2015, we performed 100 lung transplantations; 6 of which (6%) were lobar transplantations from deceased donors. Three recipients were on an emergency list due to preoperative extracorporeal support. The causes of lobectomy leading to lobar transplantation were: size mismatch (3), iatrogenic vascular damage (2), and chronic atelectasis (1). One patient died 5 months after surgery for sepsis; and 5 patients were alive at the study end (median follow-up: 17.5 months). Prevalence of grade 3 primary graft dysfunction at 72 hours was 50%. One patient developed bronchial stenosis. No cases of chronic rejection were recorded. CONCLUSIONS Lobar transplantation can be considered a valid tool to overcome the donor pool shortage in selected cases; such a technique has proved particularly useful in critically ill patients who were scheduled in an emergency transplant program.