Kartagener´s syndrome was described for the first time in 1933, with its characteristic diagnostic triad: chronic paranasal sinusitis, bronchiectasis and situs inversus of the viscera (2, 3, 4). The disease is genetically determined, inherited recessive autosomal, and included in Primary Ciliary Dyskinesis Syndrome - PCDS. The first papers concerning the structure and function of the cilia appeared in the fifties (Fawcett and Porter). In 1962 Satir described abnormal cilia for the first time, and in 1976 McDowell drew up a classification of cilia defects in which congenital anomalies were distinguished from acquired ones. Genetically - determined cilia defects usually concern several organs, and often coexist with hearing impairment and male infertility. Conversely acquired cilia anomalies usually concern some loci in the respiratory epithelium. In the seventies Pedersen and Rebe revealed that the cause of cilia immobilization in Kartagener´s syndrome is a lack of dynein brachia in its structure. Recently, disorders of neutrophil, monocyte and fibroblast chemotactic effects in Kartagener´s syndrome have also been described (3). These can be additional causes of frequent and serious infections. Up to now, 20 different congenital cilia anomalies have been defined. Kartagener´s syndrome can be diagnosed on the basis of a classic triad of symptoms. There are many diagnostic tests confirming mucociliary transport disorders e.g. the saccharose nose clearance test, and the mucociliary clearance test with the use of particles of radioaerosols inhaled by the patient. The evaluation of ciliary motion frequency is done by the use of the phase contrast or light microscope and material is sampled by means of a brush from the trachea or bronchi.

This case is presented because of the rarity of Kartagener´s syndrome, and the treatment difficulties that we met because of a lack of mucociliary transport. An 8 year-old boy was referred to the Otolaryngology Department from a provincial lung disease out-patient clinic because of bilateral hearing loss, right otorrhea, and frequent infections of upper airways. In infancy the child was hospitalized several times in a Pediatric Department due to upper and lower respiratory tract infections. Kartagener´s syndrome was diagnosed on the basis of coexistence symptoms from airways and dextrocardia. The disease was confirmed in the Bronchology and Mucoviscidosis Clinic of the Tuberculosis and Lung Disease Institute, Pediatric Unit, in Rabka after brush sampling from the bronchi and cilia movement tests. The boy was examined three times. On the day of admission to our Department he presented vesicular murmur, dextrocardia (verified radiologically), a normal abdomen, and a left-sided liver. In laryngological examination by anterior rhinoscopy: both common nasal meatuses filled with mucopurulent contents. Otoscopy showed the left tympanic membrane preserved, thickened with multiple scars, reflex dilatation, and a bulge in the posteroinferior and inferosuperior quadrants was found. The right external ear canal was filled with mucopurulent debris. After removal of these masses by suction, a marginal perforation in the posterior quadrants was shown by microscopy. The tympanic mucosa was visible through the perforation and swollen and congested. The auditory tubes were patent. Ear roentgenograms according to Schiller and Stenvers were carried out - bilateral opacity of temporal bone pneumatization was noted. Threshold audiograms showed bilateral hearing loss of about 30-40 dB. The child was qualified to surgical treatment. In general endotracheal anesthesia, an incision was made on the right planum mastoideum. The external ear canal skin was preparated to the anulus tympanicus. The rest of tympanic membrane was put in front. Then, by means of fraise, pneumatic cells of mastoid process were obliterated. A large number of cells were found, even in temporal squama filled with mucous contents. The antrum mucosa was a little bit swollen. A very narrow epitympanic-mastoid canal blocked the movement of ossicles and secretion flow from antrum to tympanic cavity. After canal widening, a normal movement of chain of ossicles was achieved. A duramatral graft was inserted by the underlay technique. Then left paracentesis was carried out. Abundant, thick mucous contents were evacuated. During the postoperative period no complications were observed. After 7 days the internal dressing was removed. The graft was pink and situated properly. The boy was discharged from hospital 2 weeks after operation. He was admitted for the second time 10 months later because of right mucopurulent otorrhea. On the day of admission anterior rhinoscopy showed both common nasal meatuses filled with thick mucous contents. Microscopic otoscopy revealed that the left tympanic membrane was thickened and scarified, with no reflex. A bulge in the posteroinferior and inferosuperior quadrants was noted. After removal of mucous secretion from the right external ear canal by suction, a marginal perforation in was found posterior quadrants. The tympanic mucosa were pink and thin. The auditory tubes presented bilateral impaired patency. Audiograms were the same as before treatment. The child was qualified to nasopharyngoscopy in order to evaluate indications to surgical treatment (adenoidectomy). In general anesthesia, nasofiberoscopy showed an abundant amount of mucous secretion was found, and this was removed by suction. Then, after pulling away the soft palate in 70° the ceiling of the nasopharynx was shown - the pharyngeal tonsil was small, flat, and did not narrow outlet from the auditory tubes nor choanae. Surgical treatment in order to close the perforation was abandoned because of doubtful effects connected with the middle ear and upper airways ciliary system blockages.

Kartagener´s syndrome treatment is a symptomatic treatment mainly directed at the prevention of upper and lower respiratory tract infection. The roles of airway physiotherapy, inhalations with mucolytics, and procedures evacuating residual secretion are emphasized. It seems that early and effective treatment of acute otitis in these patients is also very important in order to avoid chronic disease which is very difficult to cure because of the lack of muco-ciliary transport (3).