Showing posts with label BRONCHIECTASIS. Show all posts
Showing posts with label BRONCHIECTASIS. Show all posts
Kartageners Syndrome
By Dr Deepu
See the question
See the question
The answer is b."Kartagener syndrome"
Kartagener syndrome is the inheritable disorder of
dextrocardia, chronic sinusitis (with the formation of nasal polyps), and
bronchiectasis. Patients may also present with situs inversus.The disorder is
due to a defect that causes the cilia within the respiratory tract epithelium
to become immotile. Cilia of the sperm are also affected
The HRCT findings of Bronchiectasis.
Pulmonary Medicine Blog By Dr Deepu
Does this look like a gloved finger???
CT findings in bronchiectasis include the
following:
· Let me first describe the specific findings in the bronchiectasis
Has parallel tram track lines, or
It may have a signet-ring appearance,
Let me make it more clearer, it is composed of a dilated bronchus cut in a horizontal section representing the golden ring; with an adjacent pulmonary artery representing the stone of the Ring. Take a look at the signet ring to get the idea.
· The diameter of the bronchus lumen is normally 1-1.5 times that of the adjacent vessel; a diameter greater than 1.5 times that of the adjacent vessel suggests bronchiectasis. Let us correlate it in the CT scan
Second Sign is lack of bronchial tapering, Normally as we move to the peripheral parts of the HRCT, the Bronchus should start tapering, as the diameter of the bronchus progressively decreases, whereas in the patients with bronchiectasis this tapering is not seen as the bronchial wall is destroyed and dilated , see the images below
NORMAL BRONCHIAL TAPERING |
LACK OF BRONCHIAL TAPERING |
We can also see the abnormal bronchial contour due to the tractional forces applied by the fibrosed and diseased lungs
Visibility of peripheral airways within 1 cm of the pleura
Normally airways are not seen upto 1 cm from the pleura but with bronchiectasis we may be able to see the dilated peripheral airways
ARROWS POINTING TO THE DILATED PERIPHERAL AIRWAYS WITHIN 1 CM OF PLEURA |
Its time to describe the non specific findings in the bronchiectasis
•Peribronchial cuffing (thickened hazy bronchial wall).
•Finger in glove opacities
(mucus filled bronchi).
•Multiple air fluid levels
(fluid filled bronchi).
Peripheral cuffing- here we have a thickened bronchial wall due to constant underlying inflammatory process in the bronchial wall
PATIENT WITH LONG STANDING ABPA SHOWING BRONCHIECTASIS IN THE RIGHT LUNG, WITH BRONCHIAL WALL THICKENING |
Finger in glove opacities( Mucus filled bronchi)
mucus plugging of the bronchus causes bronchus to appear as a gloved hand
let us see how it appears in the CT Scan
Next sign is multiple air fluid levels
Along with these specific and non specific finding we will be able to see few ancillary findings associated with bronchiectasis
•Mosaic perfusion.
•Air trapping.
•Tree in bud opacities.
Mosaic Perfusion
Air Trapping, to see air trapping specifically ask for expiratory film, the air gets trapped in the blocked small airways causing dark areas, whereas the air is squeezed out from normal lung.
Tree in Bud Appearance-
occurs due to active infection, study the image and find tree in bud, I have marked it in 2nd image
occurs due to active infection, study the image and find tree in bud, I have marked it in 2nd image
Image shows bronchiectasis and tree in Bud |
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Suggested Reading
The Rings !!!The Trams!!!, Chest X Ray Findings in Bronchiectasis
My Next post will be on "SOLITARY PULMONARY NODULE"
The Rings !!!The Trams!!!, Chest X Ray Findings in Bronchiectasis
Pulmonary Medicine Blog By Dr Deepu
Bronchiectasis is an abnormal and permanent distortion of one
or more of the conducting bronchi or airways.
Types of bronchiectasis
Cylindrical Bronchiectasis
Mild Form shows Tram Track Appearance
Varicose Bronchiectasis
Moderate Form appears as string of pearls
Cystic/ Saccular Bronchiectasis
Severe Form appears like Bunch of Grapes
Chest
radiography Chest radiography (CXR) is usually the initial study performed in
both suspected bronchiectasis and the evaluation of nonspecific respiratory
symptoms, such as dyspnoea and haemoptysis, when bronchiectasis may be
identified incidentally.
Signs on CXR are the
identification of
Read This X Ray Before Proceeding Further
1. Parallel
linear densities, tram-track opacities.
what was seen on the chest X ray, it is nothing but the tram line appearance, unable to spot it, here comes the Modified image
Now Compare the previous X Ray with the one above , Here are few examples of tram line shadows
The black arrows points towards tram line and the white to shadows which will be discussed below
Read this X ray before proceeding
What Can we see here
if you have got it proceed further
What we see here is the ring shadows, there are many other ring shadows in the x ray , only a few are marked
One More X ray below showing the ring shadows in Cystic Fibrosis Patient
2. Ring
shadows reflecting thickened and abnormally dilated bronchial walls. These
bronchial abnormalities may vary from subtle
or barely perceptible 5-mm ring shadows to obvious cysts.
3. Fluid or mucous filling of bronchi is seen and
leads to Tubular branching opacities conforming to the expected bronchial branching
pattern.
4. The Definition
of vessel walls is lost due to peribronchial
fibrosis.
5. Signs
of complications/exacerbations, such as patchy densities due to mucoid
impaction and consolidation
6. Volume loss secondary to bronchial mucoid
obstruction or chronic cicatrisation, are also seen.
7. In the
more diffuse forms , such as cystic fibrosis (CF), generalised hyperinflation
and oligaemia are often present, consistent with severe small airways
obstruction.
The radiograph may raise the initial suspicion of
bronchiectasis, triggering more definitive imaging.
CXR also plays a role in the follow-up of bronchiectasis and management of exacerbations.Although CXR has limitations in specificity in diagnosing bronchiectasis and in detecting early or subtle changes, it is useful for assessing more florid cases of bronchiectasis, in CF and in follow-up of bronchiectatic patients. Computed tomography.
suggested Reading
CXR also plays a role in the follow-up of bronchiectasis and management of exacerbations.Although CXR has limitations in specificity in diagnosing bronchiectasis and in detecting early or subtle changes, it is useful for assessing more florid cases of bronchiectasis, in CF and in follow-up of bronchiectatic patients. Computed tomography.
suggested Reading
BRONCHIECTASIS
BRONCIECTASIS
Definition
Bronchiectasis is a severe, chronic infection of the lung causing
pronounced permanent abnormal dilatation of bronchi and
bronchioles.
Pathology
- Chronic, necrotizing
infection of bronchi in a segment or multiple segments of the lung.
- Infection damages and
weakens walls of
bronchi so that these structures become markedly dilated and filled with mucous and pus.
Pathogenesis
- The major factors which
contribute to the development of bronchiectasis are loss of
muco-ciliary clearance system and infection.
- The loss of muco-coliary
escalator system can follow either obstruction (foreign
body) or due to defect in dynein arm of cilia (Kartagener's
syndrome) or due to abnormal mucus (Cystic fibrosis).
- Defect in the clearance
mechanism is followed by accumulation of secretions, bacterial overgrowth,
infection and atelectasis.
- The chronic infection
damages the integrity of the bronchial wall causing dilatation.
- Temporary (6 Weeks) tubular
bronchial dilatation occur in all pneumonias.
- Saccular follows destructive
inflammation of the bronchial wall.
Pathophysiology
- The muco-ciliary escalator
system is destroyed in bronchiectatic segments perpetuating accumulation
of secretions and infection.
- The bronchiectatic segments
are also insensitive thus do not provoke cough until the
pus spills over to normal bronchi.
- Airway obstruction follows diffuse
bronchiectasis.
- If there is significant
recurrent pneumonias and peribronchial fibrosis a
restrictive defect can be seen.
Localized vs. Diffuse Bronchiectasis
- Localized bronchiectasis is encountered under
the following clinical circumstances:
- Following necrotizing
pneumonia
Obstructive lesion
Tuberculosis (dry bronchiectasis)
Allergic bronchopulmonary aspergillosis - Diffuse bronchiectasis is encountered in:
- Cystic fibrosis
Immobile Cilia Syndrome (Kartagener's Trial)
IgA deficiency
Hypogammaglobulinemia
Childhood infections
Clinical Features
- Clinically characterized by postural
cough and expectoration of large quantities of foul
smelling sputum with three characteristic layers. Foul smell is
due to anaerobic infection. The three layers are purulent
sediment, clear middle liquid and top foamy layer. Bronchiectatic segments
are insensitive and do not provoke cough. When the patient bends down the
gravity moves the pus to proximal normal bronchi evoking a cough response,
thus explaining the postural cough.
- Clubbing, coarse persistent leathery rales
are seen.
- Chest x-ray is often normal, but can show thickened
bronchial walls, multiple cystic spaces with air fluid levels.
- Characteristic dilated
bronchi can be demonstrated with high resolution CT. Bronchogram is no longer necessary.
- A combined obstructive and
restrictive defect can be seen in PFT's.
Therapy
- Localized bronchiectasis can
be cured with resection, if indicated.
- Cyclical antibiotics and postural
drainage are the mainstay of therapeutic measures for diffuse
bronchiectasis.
- Lung transplant is an option for far
advanced cases.
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