KS may be suspected because of respiratory disease or mirror image arrangement (conventional diagnostic clues).
Diagnosis is frequently made late,
in part because it presents with symptoms (rhinitis,
secretory otitis media,
cough) which are common in children.
The common diagnostic features of KS are: mirror image arrangement ,
continuous rhinorrhoea from the first day of life,
respiratory distress or neonatal pneumonia with no obvious predisposing cause,
chronic productive or “wet” cough, atypical “asthma”,
non-responsive to treatment,
“idiopathic” bronchiectasis,
rhinosinusitis (daily rhinitis is typical,
without remission,
and sometimes in older children severe sinusitis despite multiple surgical procedures ),
otitis media with effusion (OME).
In adolescence and adult life other possible clues are ectopic pregnancy and subfertility in women,
and male infertility.10
These chronic infections of the upper respiratory tract,
as well as chronic infections of the lower respiratory tract can be caused by multiple factors,
such as fibrocystic disease of the pancreas,
hypoproteinemia,
avitaminosis,
and congenital/secondary bronchiectasis,
as well as viral or bacterial infections that were not treated efficiently or were secondary to the aspiration of foreign bodies.
When the results of the various clinical,
laboratory,
and imaging approaches are negative for these etiologies,
other less prevalent clinical entities should be considered.11
When,
in addition to the clinical profile,
patients present situs inversus,
they are classified as having Kartagener syndrome (KS).
It has been proposed that normal ciliary beating is necessary for visceral rotation during embryonic development.
Abnormal ciliary motility results in general impairment of respiratory defense mechanisms due to problem with bacterial clearance leading to recurrent upper and lower respiratory tract infections.
Patients with Kartagener’s syndrome may have either situs solitus i.e dextrocardia only or situs inversus totalis where all the viscera are on the opposite side.12
Demonstration of abnormal ciliary movement needs electron microscopic studies of biopsies obtained from the nasal mucosa or trachea.
However these procedures are invasive and available only at specialized centers,
therefore the diagnosis of Kartagener’s syndrome may be clinical,
supported by imaging studies.
As previously stated,
the initial diagnostic hypothesis of KS,
was formed based on simple chest X-rays and on the clinical history and approach,
complemented by the following tests: X-ray of the facial sinuses; high-resolution computed tomography of the chest,
abdomen,
and sinuses.
The radiological findings reported here are also of great value in cases of suspicion of KS and are in concordance with the data in the literature.7-8
An X-ray of the sinuses may sometimes be used to confirm a suspected diagnosis of acute sinusitis.
Standard X-rays are commonly used to help distinguish uncomplicated sinusitis from other problems that may cause similar symptoms,
such as problems with the jaw joint,
dental infections,
or headache.
The findings are often not reliable,
though,
so they should be evaluated with caution.
The X-rays of the paranasal sinuses of all cases revealed findings suggestive of sinus disease,
in some cases accompanied by nasal polyps,
hypoplasia/agenesis of the frontal sinus,
opacifications/air-fluid levels,
or turbinate hypertrophy.
X-rays are fairly good at showing the frontal and maxillary sinuses (those in the cheek and forehead).
They do not show the ethmoid and sphenoid sinuses as well.
A sinus X-ray is less expensive than a CT scan,
but it will also provide less detail.
There is a slight risk of exposure to radiation.
Sinus CT scans provides greater definition of the anatomy and abnormalities of the paranasal sinuses and it is more sensitive than plain radiography for detecting sinus pathology,
especially within the sphenoid and ethmoid sinuses.
Today,
CT is the radiologic examination of choice in evaluating the paranasal sinuses of a patient with sinusitis.
Many nonspecific CT findings,
including thickened turbinates or diffusely thickened sinus mucosa,
opacified air cells,
bony remodeling may be associated with several sinusal conditions.13-14
Standard chest x-rays show dextrocardia with the stomach bubble and aortic arch on the right side (situs inversus totalis) ,
increased bronchovascular markings from peribronchial fibrosis and intrabronchial secretions,
crowding from an atelectatic lung,
tram lines (parallel lines outlining dilated bronchi due to peribronchial inflammation and fibrosis),
areas of honeycombing,
or cystic areas with or without fluid levels.
CT scan of the chest,
particularly high-resolution CT (HRCT) scanning,
has gained importance in severity grading and monitoring of KS lung disease for clinical management and intervention studies.
Consideration should be given to this imaging technique early in the presentation of Kartagener syndrome,
when a chest radiograph may not be sensitive enough to identify disease processes or when another differential is being considered.
Bronchiectasis,
mucous plugging,
peribronchial thickening and tree-in-bud pattern were the most frequent lung changes and showed the highest scores in the entire KS study population,
and in affected children and adults.15
As expected from the underlying pathophysiologic characteristics of KS severity of bronchiectasis correlate with severity of pulmonary function (worsening forced expiratory volume in 1 second (FEV1)) and age at CT.
The distribution of bronchiectasis was central or diffuse ,
rarely peripheral.
Peribronchial thickening was identified in almost all patient.
The anatomic distribution of bronchiectasis in KS identified on high-resolution CT also was similar to that described in reports of studies in which middle-lobe-predominant disease was identified.7-16 The diagnosis of KS is less likely in any adult or pediatric patient with upper-lobe-predominant bronchiectasis,
in contrast to bronchiectasis related to cysticfibrosis.
Our findings are consistent with those of previous radiographic studies of KS in which radiographic manifestations of chronic airway disease progressing from bronchial wall thickening to bronchiectasis.
Because of the chronic failure of mucociliary defense in KS,
both the number of involved lobes and the severity of bronchiectasis increase as age progresses.
Peribronchial consolidation,
mucous plugging,
atelectasis,
and nonspecific infiltrates have been associated with bronchiectasis.
To find emphysematous changes is unusually.8
Situs inversus totalis was identified in our patients,
it was related to disorganized left-right axis asymmetry caused by embryonic nodal ciliary dysfunction.
Cases of heterotaxy are reported in some studies,
these subgroups included situs inversus with congenital heart disease, polysplenia with cardiovascular anomalies,
polysplenia alone,
asplenia with vascular anomalies,
and abdominal situs inversus with polysplenia.
Pectus excavatum is another possible aberration in patients with Kartagener syndrome.9-18