Kartagener’s syndrome: A case report

Introduction. Kartagener’s syndrome is a recessive autosomal disease which is mainly seen to affect ciliary movement. The symptoms of the syndrome are the consequence of the defective motility of the cilia found in the respiratory tract and that results with recurrent lung infections caused by mucus stasis in the bronchi. Case report. A 37-year-old married male, father of one child, presented with the history of productive cough, wheezing, dispnea, headache, temporary fever. In his 9th year of age, in 1986, situs inversus, sinusitis and pectus excavatum were diagnosed. In 1994 he was operated for correction of pectus excavatum. Bronchial asthma was diagnosed in 2008 when he was 31. In the last 2 years he had episodes of breathlessness, wheezing, cough, expectoration, headache, fever and fast declining lung function. The patient was treated with combination of inhaled bronchodilatators (inhaled corticosteroids + long-acting β-2 agonist), and occasional administration of antibiotics, oral prednisolone, mucolytics in episodes of exacerbations of disease over a period of 7−14 days. Conclusion. Treatment for patients with this syndrome has not been established yet, but it is important to control chronic lung infections and prevent declining of lung function.


Introduction
Kartagener's syndrome is a recessive autosomal disease which is mainly seen to affect ciliary movement 1 .The incidence of Kartagener's syndrome is 1-2/30,000 births.Siewert first described the combination of situs inversus, chronic sinusitis and bronchiectasis in 1904 2 .Manes Kartagener, a pulmonologist in Zurich, first recognized this clinical triad as a distinct congenital syndrome in 1933.Kartagener described this syndrome in detail, so it bears his name 2 .The symptoms of the syndrome are the consequence of the defective motility of the cilia found in the respiratory tract and that results with recurrent lung infections caused by mucus stasis in the bronchi [1][2][3] .In older children and adults with primary ciliary dyskinesia, 3 diseases of the lower Correspondence to: Djordje Taušan, Clinic for Lung Diseases, Military Medical Academy, Crnotravska 17, 11 000 Belgrade, Serbia.Phone: +381 11 3608 102.E-mail: tausandjordje@gmail.com respiratory tract have been described: pneumonia, bronchiectasis and asthma 4 .Patients with Kartagener syndrome may have immotile spermatozona as well 5,6 .Treatment for patients with this syndrome has not be established, but it is important to control chronic lung infections and prevent declining of lung function 7,8 .

Case report
A 37-year-old male, married, father of one child, presented with the history of productive cough, wheezing, dispnea, headache, occasional fever.In 1986 situs inversus, sinusitis and pectus excavatum were diagnosed.In 1994 the patient was operated for correction of pectus excavatum.In 2008 bronchial asthma was diagnosed.In the last 2 years he had episodes of breathlessness, wheezing, cough, expectoration, headache, fever and fast declining lung function.
At clinical bilateral examination, predominantly on the left side, crepitations and rhonchi were evidenced.
A chest X-ray showed dextrocardia, signs of pneumonia in lower pulmonary field on the left side (Figure 1).
Chest and abdominal CT revealed dextrocardia, bronchiectasis in the lower lobes, dominantly on the left side, left liver, gastric bubble and spleen on the right (Figures 2−5).It was difficult to obtain correct images during echocardiography because of the chest abnormality (after the operation of pectus excavatum), but transthoracic echocardiogram and transesophageal echocardiography confirmed dextrocardia, too.
As the patient was diagnosed with congenital Kartagener's syndrome 28 years ago, in the last two years he was treated with a combination of inhaled bronchodilatators (inhaled corticosteroids+long-acting beta-2 agonists), and occasional administration of antibiotics, oral prednisolone, mucolytics in episodes of exacerbations of disease over a period of 7-14 days.In the last year the patient was admitted to hospital 4 times because of exacerbations of bronchial asthma and inflmmation bronchiectasis.

Discussion
Kartagener's syndrome is seen in 50% of patients with primary ciliary dyskinesia.Kartagener's syndrome is characteri-zed by situs inversus, bronchiectasis, sinusitis and otitis media.In some cases it is inherited with bronchial asthma.Our case is unusual for 2 reasons.Firstly, the presented patient was fertile and had a child.He refused to give a spermogram.Munro et al. 9 their show that 30% of their patients with primary ciliary dyskinesia have normal spermatozoa and that 2 patients are fertile.Secondly, the presented had pectus excavatum.In a study by Kennedy et al. 10 it was found that 10% patients with Kartagener's syndrome had pectus excavatum.
The presented clinical case demonstrated a progressive course of bronchiectasis, declining lung function, because of recurrent infections which were treated inappropriately in recents period.

Conclusion
We presented this case because the Kartagener's syndrome is a very rare condition.
The prognosis is generally considered favorable, and life expectancy is usually normal.An important part of the clinical visits at regular intervals should be monitoring the progression of the lung disease.

Fig. 1 -Fig. 2 -
Fig. 1 -Chest radiograph shows dextrocardia and features of pneumonia in the left lower lung field.