Anestesia e Pesquisa Clínica
Acesso livre
ISSN: 2155-6148
ISSN: 2155-6148
Shuji Kawamoto, Atsuko Shiraki, Chikashi Takeda, Tomoharu Tanaka, Kazuhiko Fukuda
Background: Kartagener syndrome (KS) is a partial disease of primary ciliary dyskinesia with defects in the fine structure of cilia and flagella. KS is an autosomal recessive inherited disease with three major features: visceral inversion, chronic sinusitis, and bronchiectasis. Lung transplantation is an option for end-stage KS, which results in respiratory insufficiency. However, there have been few reports on perioperative management of lung transplantation in patients with KS.
Case presentation: A 44-year-old female who was diagnosed with KS in infancy had tracheotomy at age 42 to prevent infection, and was placed on standby for lung transplantation under a ventilator. After a two-year wait, she underwent bilateral brain-dead lung transplantation. In parallel with induction of anesthesia, the left femoral artery and vein were taped to allow extracorporeal circulation in case of a sudden change, and extracorporeal circulation was started after opening the chest, as planned. As a general anesthetic, propofol, which has a lower inhibitory effect on airway ciliary movement, was mainly used. After both pneumonectomies were performed, the upper airway was washed. A donor left whole lung was then transplanted into the left chest cavity, and a donor right lung with resection of the middle lobe was transplanted into the right chest cavity. Pulmonary physiotherapy and bronchoscopic expectoration were encouraged, and the patient was weaned from the ventilator on postoperative day (POD) 13 and discharged from hospital without oxygen on POD 101.
Conclusion: Perioperative respiratory infection control is of paramount importance in bilateral lung transplantation for KS, and a good clinical course was achieved through careful infection control.