Endoscopic Sinus Surgery for Treatment of Kartagener Syndrome: A Case Report

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. Turkish Başlık: Kartagener Sendromu'nun Tedavisi için Endoskopik Sinüs Cerrahisi: Olgu Raporu Anahtar Kelimeler: Kartagener sendromu, primer silier diskinezi, sinüzit, cerrahi, endoskopi Kartagener sendromu (KS) üçlü klinik semptom – situs inversus, kronik rinosinüzit ve bronşiektazi – ile karakterize nadir bir konjenital hastalıktır. Kartagener sendromunun gerçek insidansı bildirilenden daha yüksek olabilir çünkü sıklıkla yanlış teşhis edilmekte veya geç teşhis edilmektedir. Konjenital siliyer defektin bu sendromun temel nedeni olduğu bilinmesine rağmen beraberindeki hava yolu enfeksiyonunun tedavisindeki güçlük devam etmektedir. Biz burada fonksiyonel endoskopik sinüs cerrahisinin ardından uzun süre post-operatif sinonazal kavite debritmanı yapılan KS'lu bir hasta bildirmekteyiz; bu tedavi kronik rinosinüziti azalttı ve sonraki hava yolu enfeksiyonuna karşı koruma sağladı.

Keywords

• Kartagener syndrome, primary ciliary dyskinesia, sinusitis, surgery, endoscopy

Endoscopic Sinus Surgery for Treatment of Kartagener Syndrome: A Case Report

Öz

Kaynakça

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