(Primary ciliary dyskinesia type Kartagener, ciliary immotility type Kartagener, Afzelius syndrome, Siewert syndrome, Siewert-Kartagener syndrome)
Rare: 1/40,000. Autosomal recessive transmission with variable penetrance of mutations of the DNAI1 gene (9p21-p13) which codes for dynein. A mutation in the DNAH5 gene on (5p15-p14) has also been described. Represents 50 % of the cases of primitive ciliary dyskinesia.
Characterised by the triad:
- complete or partial situs inversus: dextrocardia; sometimes associated with a cardiac malformation (ASD, transposition of the great vessels)
- chronic sinusitis with nasal polyposis (30%), frontal sinus agenesis
- bronchiectasis: frequent respiratory infections
Frequent anosmia. Boys sterility.
Kartagener: dextrocardia, right aortic arch, right gastric air
Anesthetic implications:
situs inversus: adapt the positioning of the ECG electrodes, central vein catheters and double lumen endotracheal tube, etc. Risk of pulmonary complications (pre- and postoperative physiotherapy). Avoid intubation by the nasal route.
References :
- Sahajananda H, Sanjay OP, Thomas J, Daniel B.
General anaesthesia for lobectomy in an 8-year-old child with Kartagener's syndrome.
Paediatr Anaesth 2003; 13: 714-7.
- Cheng L, Dong Y, Liu S.
Anesthetic management of patients with Kartagener syndrome: a systematic review of 99 cases.
J Cardiothor Vasc Anesth 2023 ; 37 :1021-5
Updated: March 2023