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.

Kartagener’s syndrome: A clinical reappraisal with two case reports

“…Lobectomy and pneumonectomy are advised for localised lung damage with recurrent haemoptysis or respiratory exacerbations. 9 Infertile patients can be treated by advanced micromanipulation techniques that permit nonmotile or poorly motile sperm to penetrate, or by in vitro fertilisation techniques. 14 Genetic testing carries the immense future potential for developing gene therapy and so genetic counselling can play an important role in addressing social and psychological aspects of fertility issues among young couples and possibility of genetic risks in children.…”

“…Delayed diagnosis culminates into progression of KS with complications such as bronchiectasis, pulmonary fibrosis, and finally impaired lung and/or heart function. 9 The diagnosis of KS is often made incidentally on routine radiological examination. Chest X-ray is usually the first imaging modality that reveals dextrocardia and situs inversus as an incidental finding on routine checkup.…”

Nasal Polyposis in Patients with Primary Ciliary Dyskinesia

“…Functional endoscopic sinus surgery (FESS) is frequently indicated in cases of failure of medical treatment. It may be useful to decrease or eliminate upper and lower airway infections in these patients [4]. PCD has an incidence of 1 in 20,000 with a prevalence ranging from 1: 4000 to 1: 50000.…”

“…Ciliary ultra structural abnormalities as seen on electron microscopy and video microscopy of nasal mucosa biopsies are useful adjunctive diagnostic modalities as the genetic mutations involved in PCD are further identified. Follow-up includes monitoring of lung function, imaging, upper airway microbiology, and early antibiotic treatment in cases of infection [1][2][3][4][5][6][7][8].…”

“…According to the literature we suggest to perform a wide mid-maxillary antrostomy, inferior meatotomy, and complete bilateral anteroposterior ethmoidectomy according to the compromise detected in the preoperative tomographic analysis. Following the procedure, postoperative debridement of the sinonasal cavity is important because of its role in the management of airway infections in patients with PDD due to the lack of mucociliary sweep [4].…”