ANESTHESIA FOR LIVER-TRANSPLANTATION IN CYSTIC-FIBROSIS PATIENTS

Introduction: Cystic fibrosis (CF) is a disease caused by an inherited genetic defect. While pulmonary and pancreatic abnormalities predomin ate the clinical spectrum, other organ involvement is common. includin g liver. The severity of liver disease does not appear to be related t o the severity of exocrine pancreatic or lung function. We discuss ana esthesia in four CF patients undergoing liver transplantation. Methods : We studied haemodynamic and oxygenation modifications during anaesth esia in four patients affected by CF with end-stage liver disease and mild to moderate pulmonary abnormalities. The patients received pancre atic enzyme prior to transplantation and two had insulin-dependent dia betes mellitus. All patients were treated with broad-spectrum antibiot ic therapy. After a waiting time ranging one week to three months, all patients were successfully transplanted. General anaesthesia was indu ced with fentanyl, thiopental and pancuronium, and maintained with iso flurane supplemented by fentanyl in O-2:air. Haemodynamic and oxygenat ion evaluations were made during the main phases of the transplant. Af ter the intubation and at the end of the procedure all patients receiv ed a broncho-alveolar toilet through fiberoptic bronchoscopy. Results: During anaesthesia for liver transplantation, PaO2 increased proporti onally to the decreasing of Qs/Qt. In postoperative follow-up, Fev1 an d FVC improved from preoperative time in all patients. In conclusion, even if cystic fibrosis is a multisystem disease, liver transplantatio n can be offered to CF patients with endstage liver disease and mild t o moderate pulmonary function abnormalities. The four patients are sti ll alive, enjoying good health. The improved respiratory function and quality of life of these children is remarkable.