JIACM 2005; 6(3): 241-3

C A S E R E P O R T

Kartagener’s Syndrome: A Triad of Bronchiectasis,

Situs Inversus, and Chronic Sinusitis
AS Dabhi*, SR Chaudhari**, PB Thorat***, HB Pandya*, MC Shah**, UN Meswani**, PR Vaghela**

Abstract
Primary ciliary dyskinesia (PCD) is a syndrome characterised by productive cough with bronchiectasis and sinusitis in early life,
and reduced fertility later in life. PCD is a rare syndrome with an estimated incidence of 1: 20,000 to 30,000. Primary ciliary dyskinesia
is a genetic disorder with manifestations present from early life and distinguishes it from acquired mucociliary disorders. Here we
present one such case of Kartagener’s syndrome that presented with bronchiectasis, recurrent sinusitis with primary infertility,
and situs inversus, which fits in the triad of Kartagener’s syndrome.

Key words: Primary ciliary dyskinesia, Primary infertility, Acquired mucociliary disorders.

Case report small areas of consolidation in both lower lobes and apical
segments (Figure 2). USG abdomen showed liver and
A 45-years-old female presented with complaints of
inferior vena cava on left side; and spleen, stomach, and
productive cough with profuse yellowish expectoration
aorta on right side. X-ray PNS showed changes of chronic
with postural variation for 15 days, alongwith low grade
maxillary sinusitis bilaterally.
fever since 1 month. She had previous episodes of
recurrent rhinosinusitis and profuse expectoration for
many years. The patient had been married for 25 years
and she was infertile with regular menstrual periods
suggesting primary infertility.

On examination, the patient had temperature of 100° F,

normal pulse and blood pressure with respiratory rate of
20/min. Apex beat was localised to right fifth intercostal
space on mid-clavicular line; and on percussion, liver
dullness was on left side – all this suggestive of situs
inversus. On auscultating the respiratory system, there
were bilateral, lower zone, coarse leathery crepitations
present on inspiration as well as expiration.

On investigation, the patient had haemoglobin of 12 gm/

dl and total leucocyte count of 12,000/cmm of blood, and
ESR of 60 mm in 1st hour. Routine biochemical tests did
not reveal any significant abnormality. Sputum culture was
negative for acid-fast bacilli, but showed Gram-positive
Fig. 1: Chest radiography showing dextrocardia with aortic arch
cocci. Chest radiography confirmed dextrocardia with the lying on right side of trachea with cystic bronchiectatic changes.
aortic arch lying on right side of the trachea with cystic
bronchiectatic changes seen in both lower zones and mid- The patient was investigated for infertility in the form of:
zones (Figure 1). HRCT (High resolution computed
1. USG abdomen and pelvis
tomography) of chest showed bronchiectatic changes in
left upper lobe, lingula, and right middle lobe, with few 2. Laparoscopic examination of pelvis

* Assistant Professor, ** Resident, *** Associate Professor and Unit Head, Department of Medicine,
Medical College and SSG Hospital, Vadodara - 390 001, Gujarat.
3. Hormonal assay for FSH, LH, and prolactin levels.
4. Chromopertubation.
All reports were normal.
Fig. 2: HRCT of chest showing bronchiectatic changes in left upper
lobe and lingula, and right middle lobe, with few small areas of
consolidation in both lower lobes and apical segment.
Pulmonary function tests (PFT) showed FEV1/FVC to be
62% of predicted values, suggestive of obstructive pattern
with minimal improvement in PFT after bronchodilators.
The ‘nasal saccharin transit time’ test gave mucociliary
clearance time of 60 minutes (Normal < 30 minutes)1.
However, due to lack of the facility of electron microscopy
of the nasal cilia, we were not able to study the
ultrastructural defect.
Thus, Kartagener’s syndrome was diagnosed clinically. She
was treated with intravenous antibiotics and mucolytic
agents with chest physiotherapy. The majority of
symptoms rapidly improved and she was discharged on
low dose antibiotics in rotation.
Discussion
The triad of bronchiectasis, sinusitis, and situs inversus was
first described by Siewert in 1903, although its usual
eponym is Kartagener’s syndrome – after the Swiss
paediatrician who described four cases with similar
features in 19332.
242 Journal, Indian Academy of Clinical Medicine
By 1960, 300 cases had been reported and concept of a
disease with congenital and generalised non-functioning
of the cilia evolved. About 50% of the people affected with
primary ciliary dyskinesia have situs inversus, so fit in the
criteria of Kartagener’s syndrome.
The clinical features of PCD have been described in
primary ultrastructural defects in cilia. Ultrastructurally,
cilia and spermatozoa tail are similar. The axoneme is the
key component of the cytoskeleton and has a
characteristic nine plus two arrays of microtubules (Figure
3).The nexin links and spokes seem to provide structural
rigidity to the axoneme. Dynein arms extend from one
side of a doublet in a clockwise direction, when viewed
from the tip of the cilium. They contain most of the ATPase
activity of the axoneme, and are important in releasing
energy for sliding and bending of microtubules and ciliary
motion3.
The tracheobronchial tree is ciliated to the level of the
respiratory bronchioles, each ciliated cell having about 200
cilia. Mucociliary transport in the respiratory tract is
important for normal respiratory function and resistance
to respiratory infection4.
The typical clinical picture of PCD is a chronic productive
cough which can usually be traced back to early childhood
or infancy, chronic rhinitis often with nasal polyposis,
chronic or recurrent maxillary sinusitis, and frequent ear
infections in childhood. Bronchiectasis is not present at
birth, but may develop early, sometimes even in childhood.
The most common respiratory pathogens are
Haemophilus Influenzae and Streptococcus pneumoniae.
Most males are sterile, but many females have a lowered
fertility. About 50% of patients have situs inversus
viscerum5.
Inner dynein arm
Outer dynein arm
Nexin link
Cell membrane
Central microtubule pair
Axis of
effective Microtubule B
stroke Microtubule A
Radial spoke
Fig. 3: Cilia axoneme diagram.

Vol. 6, No. 3
July-September, 2005
Patients with suspected primary ciliary dyskinesia should
have their mucociliary clearance measured, and the cilia
should be examined by microscopy. Nasal mucociliary
clearance can be measured by the ‘nasal saccharin transit
time’ test in which a saccharin pellet is placed on the
anterior end of the inferior turbinate and the time taken
for the subject to notice the taste is recorded. This test
requires the patient’s co-operation and is not reliable in
children under 10 years of age. Nasal cilia are easily
accessible, and can be obtained from the inferior turbinate
without anaesthesia by a non-invasive brush technique.
Ciliary beat frequency can then be assessed by light
microscopy and photometric techniques, and cilia fixed
on electron microscopy6. In males, the motility of sperm
can be examined, and electron microscopy may show the
characteristic ultrastructural defects.
Rationale of treatment
Treatment of PCD is aimed at relieving symptoms and
preventing complications. Early recognition of the disease
and prompt antibiotic treatment are the keys to minimise
the irreversible lung damage. Physiotherapy with postural
drainage and cessation of smoking are also important.
Coughing should not be suppressed since it acts as a
substitute for mucociliary clearance. Huffing from mid to
low lung volume with forced expiratory manoeuvre helps
to improve clearance7.
Despite this being a chronic respiratory disease, life span
seems to be normal. Infertile patients benefit from
advanced micromanipulation techniques that allow non-
Journal, Indian Academy of Clinical Medicine
Vol. 6, No. 3
motile or poorly motile sperm to penetrate, or by in vitro
fertilisation techniques8.
Thus, we should remember that any patient with a history
of recurrent cough and cold, and bronchiectasis with
infertility should be examined for Kartagener’s syndrome
which is a part of PCD.
References
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method for testing mucociliary function in patients
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2. Siewert A. Uber einen Fall von Bronchiektasie bei einem
Patientem mit Situs inversus viscerum. Berlin Klin
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3. King SM. The dynein microtubule motor. Biochim Biophys
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4. Braunwald,Fauci AS, Kasper DL et al. Bronchiectasis.
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5. Anthony Seaton, Douglas Seaton, A.Gorden Leitch. Crofton
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