CASE 13153 Published on 03.12.2015

Kartageners syndrome


Chest imaging

Case Type

Clinical Cases


Dr. Apurva Kalra1 Dr Madan Manmohan Dr Thahir VU Dr Chitrangada Singh

Dr. D Y Patil Hospital and Research centre, Department of Radiology; Sector 5 Nerul 400704 NAVI MUMBAI , India

19 years, male

Area of Interest Lung, Abdomen, Ear / Nose / Throat, Spleen, Liver ; Imaging Technique Conventional radiography, CT-High Resolution, CT, Ultrasound
Clinical History
A 21 year-old male came with complaints of breathlessness since one month, chronic cough with expectoration and nasal congestion since 15 years. On physical examination bilateral wheeze and basal crackles were audible, with heart sounds being best heard on the right side of the chest.
Imaging Findings
Ultrasound revealed situs inversus with liver and IVC on the left and spleen and aorta on the right. There was mild ascites in the abdomen and the pelvic cavity [Figure 1]. Chest radiograph revealed dextrocardia, haziness with bilateral cystic bronchiectasis in the mid and lower lung zones [Figure 2]. HRCT Chest shows trilobed left lung and bilobed right lung [Figure 3]. Axial unenhanced CT image of the abdomen showed a situs inversus totalis- liver and IVC on the left and spleen and aorta on the right[ Figure 4]. HRCT chest image showed extensive cystic bronchiectasis distributed in both lungs with a predominance in the lower lobes. Few centrilobular micronodules representing mucoid impaction with few areas of ground glass opacities are seen in the left lower lobe [Figure 5]. CT paranasal sinuses revealed mucosal thickening in bilateral maxillary sinuses suggestive of sinusitis [Figure 6]
A. Kartagener's syndrome[KS] is a subset of primary ciliary dyskinesias and is a genetic condition with an autosomal recessive inheritance comprising a triad of situs inversus, bronchiectasis and sinusitis[1]. It has incidence of 1:20, 000 to 1:60, 000 live births[2]. Pathophysiology for recurrent sinusitis, bronchiectasis and situs inversus is the ultrastructural and functional defects of ciliary motility [3].Without functional nodal cilia in the KS patients during embryonic period, thoracoabdominal orientation is random [4].

B. Clinical findings are chronic recurrent rhino-sinusitis , productive cough, Nasal polyps , otitis media and significant shortness of breath. Some patients have severe obstructive impairment that is worse than patients with Cystic Fibrosis at a comparable age [4].

C. Imaging plays a vital role in diagnosing this condition. A set of radiological tests including chest Radiograph, HRCT Chest , CT Abdomen and CT PNS can lead us to conclusive diagnosis of Kartagener’s syndrome. Chest X-ray may reveal dextrocardia and bronchiectasis. To look for situs inversus ultrasound can be performed which shows spleen and aorta on the right and liver and IVC towards the left. Axial unenhanced CT of the abdomen can show situs inversus totalis with dextrocardia.
HRCT chest shows cystic bronchiectasis in lower lobes of both the lungs. It is important to differentiate it from cystic fibrosis as the thick walled bronchiectasis with intervening lung is often densely fibrotic and retracted in CF but not in KS. Bronchiectasis is usually present in the more dependent lobes such as the middle and lower lobes in KS , in contrast to the upper lobes most affected in CF [5]. CT PNS can reveal pansinusitis.
With this set of imaging findings which is more than conclusive electron microscopy can be done otherwise to reveal ciliary dyskinesia.

D.Screening tests like nasal nitrous oxide measurement, saccharin test and analysis of dynein proteins can be done. Electron microscopy (EM) is the more accredited test, even though it does not exist only one gold standard[6]. Treatment requires chest physiotherapy, antibiotics with good H.influenzae, S. aureus and S. pneumoniae coverage[7]. Lobectomy should be done in severe localised bronchiectasis. Lung transplantation is an option in end-stage lung disease[4].

E. Kartagener's syndrome is a rare condition and its proper diagnosis will result in early treatment and better prognosis of this disease. Prompt detection and treatment can prevent its complications as well[8].
Differential Diagnosis List
Kartagener's syndrome
Cystic fibrosis
Primary and secondary ciliary dyskinesias
Alpha-1-antitrypsin deficiency
Final Diagnosis
Kartagener's syndrome
Case information
DOI: 10.1594/EURORAD/CASE.13153
ISSN: 1563-4086