SynopsisCodesDifferential Diagnosis & PitfallsBest TestsReferences
Kartagener syndrome
Other Resources UpToDate PubMed

Kartagener syndrome

Other Resources UpToDate PubMed


An uncommon autosomal recessive inherited disorder causing abnormal function of lung cilia, which hinders mucosal clearance. A subtype of primary ciliary dyskinesia characterized by a triad of bronchiectasis, chronic sinusitis, and situs inversus. Patients typically present with chronic upper and lower respiratory tract infections, rhinitis, tachypnea, cough, chronic ear infections, and situs inversus. Respiratory distress begins in neonatal period. Adults may be diagnosed when infertility becomes an issue, along with respiratory symptoms.

Management of bronchiectasis is geared toward secretion clearance, use of antibiotics, influenza and pneumonia vaccinations, and utilizing many of the treatments of cystic fibrosis. Smoking cessation, treatment of rhinosinusitis, chest physiotherapy, and removal of nasal polyps may be employed. See nicotine dependence for information on smoking cessation. Bilateral lung transplant, if considered, may require modifications or heart-lung transplant due to situs inversus. Prognosis with appropriate therapies is generally good, despite repeated respiratory infections.


Q89.3 – Situs inversus

42402006 – Kartagener Syndrome

Differential Diagnosis & Pitfalls

At first presentation, chronic respiratory infections would suggest a differential including:
  • Alpha-1 antitrypsin deficiency
  • Chronic obstructive pulmonary disease
  • Cystic fibrosis
  • Immunodeficiency
  • Interstitial lung diseases
Radiologic studies showing situs inversus would narrow the differential considerably.

Best Tests

Subscription Required


Subscription Required

Last Updated:01/17/2022
Copyright © 2024 VisualDx®. All rights reserved.
Kartagener syndrome
A medical illustration showing key findings of Kartagener syndrome : Nasal polyps
Copyright © 2024 VisualDx®. All rights reserved.