title: Anaesthetic Management of a Patient with Synchronous Kartagener Syndrome and Biliary Atresia.
abstract: Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is important to provide suitable anaesthetic management to avoid problems because of ciliary dysfunction in the perioperative period. Further, maintaining an effective pain control with regional anaesthetic methods reduces the risk of pulmonary complications. Infants with biliary atresia operated earlier have a higher chance of survival. Hepatic dysfunction and decrease in plasma proteins are important for the kinetics of drugs. In this presentation, the anaesthetic management of patients with synchronous Kartagener syndrome and biliary atresia, both of which are rare diseases, is evaluated.
authors.affiliation: Department of Anaesthesiology and Reanimation, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
authors.forename: Pınar
authors.initials: P
authors.lastname: Kendigelen
authors.affiliation: Department of Anaesthesiology and Reanimation, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
authors.forename: Ayşe Çiğdem
authors.initials: AÇ
authors.lastname: Tütüncü
authors.affiliation: Department of Anaesthesiology and Reanimation, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
authors.forename: Şafak Emre
authors.initials: ŞE
authors.lastname: Erbabacan
authors.affiliation: Department of Anaesthesiology and Reanimation, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
authors.forename: Güner
authors.initials: G
authors.lastname: Kaya
authors.affiliation: Department of Anaesthesiology and Reanimation, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
authors.forename: Fatiş
authors.initials: F
authors.lastname: Altındaş
keywords: Kartagener syndrome; anaesthesia; biliary atresia; infant