Endoscopic sinus surgery for treatment of kartagener syndrome: a case report

Balkan Med J. 2013 Jun;30(2):244-7. doi: 10.5152/balkanmedj.2013.8152. Epub 2013 Jun 1.

Abstract

Background: KARTAGENER SYNDROME (KS) IS A RARE CONGENITAL DISEASE CHARACTERISED BY A CLINICAL TRIAD OF SYMPTOMS: situs inversus, chronic rhinosinusitis, and bronchiectasis. Although congenital ciliary defect is recognised as the main cause of this syndrome, it remains difficult to treat the associated airway infection.

Case report: A 17-year-old female patient presented with repeated refractory airway infection. She also had bronchiectasis and situs inversus. Electron microscopic evaluation of her nasal mucosa revealed ciliary defect and confirmed the diagnosis of KS. She underwent functional endoscopic sinus surgery (FESS) followed by long-term postoperative debridement of the sinonasal cavity. This treatment reduced chronic rhinosinusitis and protected against subsequent airway infection in a 7-year follow-up.

Conclusion: FESS is effective for relieving both chronic rhinosinusitis and lung infection of KS in the long term.

Keywords: Kartagener syndrome; endoscopy; primary ciliary dyskinesia; sinusitis; surgery.