Cem ÖZER, Buket KILIÇASLAN, Suna ASİLSOY

Burun Tıkanıklığı ile Gelen Kartagener Sendromu: Olgu Sunumu

Nazal polipler, nazal mukozanın kronik enflamasyonundan kaynaklanan oluşumlardır. Çocuklarda nadir görülmekle birlikte saptandığında eşlik edebilecek kistik fibrozis ve primer silier diskinezi açısından dikkat edilmelidir. Primer silier diskinezili olguların %50'sinde situs inversus gösterilebilir; böyle olgular Kartagener sendromu olarak adlandırılır. Kartagener sendromu, otozomal resesif geçişli, sinüzit, bronşektazi ve situs inversus ile karakterize, nadir görülen bir durumdur. Klinik olarak en sık nazal tıkanıklık ile Kulak Burun Boğaz uzmanlarına başvururlar. Pediatristler olarak biz de, çocukluk çağında nazal tıkanıklığın nadir bir sebebi olarak nazal polipleri akla getirmeli; poliplere eşlik eden tekrarlayan üst ve alt solunum yolu enfeksiyonlarının varlığında ise nadir olarak görülen Kartagener sendromu akılda tutulmalıdır.

Kartagener's Syndrome Presented with Nasal Obstruction: A Case Report

The nasal polyposis is a chronic inflammatory process of the nasal mucosa. Although it is rare in children, there may be also association with cystic fibrosis and primary ciliary dyskinesia. About 50% of primary ciliary dyskinesia patients develop situs inversus and it is known as Kartagener's syndrome. The Kartagener's sydrome is a rare autosomal recessive disorder characterized by sinusitis, bronchiectasis, situs inversus. Clinically, patients present to the otolaryngologist with nasal obstruction. We as pediatricians, should consider nasal polyposis as a rare cause of nasal obstruction in children. In the presence of recurrent upper and lower respiratory tract infections accompanying nasal polyposis, Kartagener's syndrome must be kept in mind as a rare reason.

PDF

___

1. Haro JI, Gavioli F, Junior VM et al. Clinical aspect of patients with nasal poylposis. Intl Arch Otorhinolaryngol. 2009;13: 259-63.
2. Bateman ND, Fahy C, Woolford J. Nasal polyps stil more questions than answers. T J Laring Otol. 2003;117:1-6.
3. Puwankar R. Nasal polyposis: an update. Curr Opin Allergy Immunol. 2003;3:1-6.
4. Drake-Lee AB, Lowe D, Swanston A et al. Clinical profile and recurrence of nasal polyps. J Laryngol Otol. 1984;98:783-93.
5. Settipane GA. Epidemiology of nasal polyps. Asthma Proc. 1996;17:231-6.
6. Kartagener M. Zur pathogenese der bronchiektasien bei situs viscerum inversus. Klin Tuberk. 1933;83:489-501.
7. Larsen K, Tos M. The estimated incidence of symptomatic nasal polyps. Acta Otolaryngol. 2002;122:179-82.
8. Pearlman AN, Chandra RK, Conley DB et al. Epidemiology of nasal polyps. Nasal Polyposis. Pathogenesis, Medical and Surgical Treatment. (Eds) Önerci TM, Ferguson BJ. Springer-Verlag Berlin Heidelberg, XI. 2010;311.
9. Bush A, Cole P, Hariri M et al. Primary ciliary dyskinesia: diagnosis and standarts of care. Eur Respir J. 1998;12:982-8.
10. Afzelius BA. A human syndrome caused by immotile cilia. Science. 1976;193:317-9.
11. Poduval J, Poduval M. Kartagener's syndrome- a case report. Medical Case Studies. 2011;2:15-8.
12. Nadel HR, Stringer DA, Levison H et al. The immotile cilia syndrome: radiological manifestations. Radiology. 1985;154:651-5.
13. Canciani M, Barlocco EG, Mastella G et al. The saccharin method of testing mucociliary function in patients suspected of having primary ciliary dyskinesia. Paediatr Pulmonol. 1988;5:210-4.
14. Wodehouse T, Kharitonov SA, Mackay IS et al. Nasal nitric oxide measurements for the screening of primary ciliary dyskinesia. Eur Respir J. 2003;21:43- 7.
15. Jorissen M, Willems T, Van Der Schueren B et al. Ultrastructural expression of primary ciliary dyskinesia after ciliogenesis in culture. Acta Otorhinolaryngol. 2000;54:343-56.
16. Rayner CF, Rutman A, Dewar A. Ciliary disorientation alone as a cause of primary ciliary dyskinesia syndrome. Am J Respir Crit Care Med. 1996;153:1123-9.
17. Bush A, Chodhari R, Collins N et al. Primary ciliary dyskinesia: current state of the art. Review. Arch Dis Child. 2007;1136-40.
18. Parsons DS, Greene BA. A treatment for primary ciliary dyskinesia: efficacy of functional endoscopic sinus surgery. Laryngoscope. 1993;103:1269-72