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

摘要

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 Ba?l?k: Kartagener Sendromu'nun Tedavisi i?in Endoskopik Sinüs Cerrahisi: Olgu Raporu Anahtar Kelimeler: Kartagener sendromu, primer silier diskinezi, sinüzit, cerrahi, endoskopi Kartagener sendromu (KS) ü?lü klinik semptom – situs inversus, kronik rinosinüzit ve bron?iektazi – ile karakterize nadir bir konjenital hastal?kt?r. Kartagener sendromunun ger?ek insidans? bildirilenden daha yüksek olabilir ?ünkü s?kl?kla yanl?? te?his edilmekte veya ge? te?his edilmektedir. Konjenital siliyer defektin bu sendromun temel nedeni oldu?u bilinmesine ra?men beraberindeki hava yolu enfeksiyonunun tedavisindeki gü?lük devam etmektedir. Biz burada fonksiyonel endoskopik sinüs cerrahisinin ard?ndan uzun süre post-operatif sinonazal kavite debritman? yap?lan KS'lu bir hasta bildirmekteyiz; bu tedavi kronik rinosinüziti azaltt? ve sonraki hava yolu enfeksiyonuna kar?? koruma sa?lad?.