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BULLOUS LUNG DISEASE

Emphysema
• Pulmonary emphysema is
described in clinical,
radiological and
physiologic terms, but the
condition is best defined
morphologically
• Definition:Abnormal
enlargement of airspaces
distal to the terminal
bronchioles with
destruction of their wall
• It is characterized by
destruction and
enlargement of alveoli
Cont.
• Although the normal lung has about
35,000 terminal bronchioles and their
total internal cross-sectional area is at
least 40 times as great as that of the
lobar bronchi but the bronchioles are
more delicate and vulnerable.
Cont
• Bronchioles may be obstructed partially
or completely, temporarily or
permanently, by thickening of their
walls, by collapse due to loss of
elasticity of the surrounding
parenchyma, or by influx of exudates.
Cont
• In advanced emphysema, the lungs are
large, pale, and relatively bloodless.
• They do not readily collapse.
• They many contain many superficial
blebs or bullae, which occasionally are
huge.
• The right ventricle of the heart is often
enlarged (cor pulmonale), reflecting
pulmonary arterial hypertension.
Emphysema: Pathophysiology
• Structural changes
– Hyperinflation of
alveoli
– Destruction of alveolar
& alveolar-capillary
walls
– Small airways narrow
– Lung elasticity
decreases
Emphysema

• Loss of Lung Surface Area for


Gas Exchange and Oxygen
Transport
• Loss of Lung Surface area is
due to death of Lung
Endothelial Cells
• Polycyclic aromatic
hydrocarbons (PAHs) in
Cigarette Smoke and in
Environmental Pollution may
cause endothelial cell death
Emphysema: Pathophysiology
• Mechanisms of
structural change
• Obstruction of small
bronchioles
• Proteolytic enzymes
destroy alveolar tissue
• Elastin & collagen are
destroyed
– Support structure is
destroyed
– “paper bag” lungs
Emphysema: Pathophysiology
• The end result:
• Alveoli lose elastic recoil,
then distend, & eventually
blow out.
• Small airways collapse or
narrow
• Air trapping
• Hyperinflation
• Decreased surface area for
ventilation
Emphysema:
Clinical Manifestations

• Early stages
– Dyspnea
– Non productive cough
– Diaphragm flattens
– A-P diameter increases
• “Barrel chest”
– Hypoxemia may occur
• Increased respiratory rate
• Respiratory alkalosis
– Prolonged expiratory phase
• Tripod position
Emphysema:
Clinical Manifestations

• Later stages
– Hypercapnea
– Purse-lip breathing
– Muscle retraction
– Use of accessory muscles to
breathe
– Underweight
• No appetite & increase
breathing workload
• Loss of subcutaneous fat
– Lung sounds diminished
Clinical Manifestations

• Pulmonary function
•  residual volume,  lung
capacity, DECREASED
FEV1, vital capacity
maybe normal
• Arterial blood gases
– Normal in moderate disease
– May develop respiratory
alkalosis
– Later: hypercapnia and
respiratory acidosis
• Chest x-ray
– Flattened diaphragm
– hyperinflation
Classification
• Anatomical Distribution
– Cetriacinar
– Panacinar
– Paraseptal
– Irregular
Pathogenesis
• Protease anti Protease Theory
– Hereditary deficiency of the major protease inhibitors
– Laurell and Eriksson –1963 – deficiency of α1-antitrypsin
and emphysema
• Pollutants in environment
– smoking of cigarettes
– exhalations from cars, dust from grain
• Recruitment of neutrophils
• stimulation of macrophages and epithelial cells to produce TNF-
α, IL-8 and LTB4
• Elastase release from leukocytes and tissue macrophages
• Inactivation of α1-antitrypsin by oxidants in tobacco smoke or
free radicals released from the neutrophils
Diseases in which misfolding of a given
protein results in improper trafficking
Diseases in which misfolding of a
given protein results in improper
trafficking

polymers of alpha1 antitrypsin molecules will form.


These will be retained in the liver (where alpha1
antitrypsin is produced) and will not reach the lungs
Copyright ©2005, The Regents
of the University of California
hyper aeration
increased
lung
lucency
decreased
vessel size

Emphysema – Total
Lung Capacity (TLC) &
Residual Volume (RV) are elevated. P = 2t/r.

Flattening of the Increased Lung


Decreased Peripheral
Diaphragm Height
Vascular Markings
Chest X-Ray Changes in Emphysema:

• The two primary findings are:


1. Increased Lung Volumes, i.e., hyperaeration or
overinflation.
2. Lung Destruction (bullae or decreased vascularity which
gives increased lung lucency and decreased vessel size ).
• In end-stage disease, emphysema and chronic obstructive
pulmonary disease can result in pulmonary arterial
hypertension and eventually cor pulmonale (cp) - which
causes an increase in the pulmonary arterial size, as well as
right ventricular enlargement and cardiomegaly.
Normal acinus
Morphology

Panlobular Emphysema Centrilobular Emphysema


Paraseptal
Peripheral alveolar
damage
© Univ of AL at Birmingham, Dept. of Path.

Bullous Emphysema
Other types of Emphysema
1 Bullous Emphysema (also known as
Bullous Lung Disease):

Bullous emphysema is so named when there


are multiple large bullae associated with a
compromise in pulmonary function. It is
usually associated with concomitant
emphysema, although occasionally, it can
be familial
Bullous Emphysema
• Subpleural Type: These subpleural bullae contain only gas
with no alveolar remnants or blood vessels. They are often
located in the apex of the upper lung zone, and along the
costophrenic rim of the middle lobe and lingua, but may be
seen in the vicinity of parenchymal scars.
• Superficial Type: These bullae are found along the anterior
edge of the upper and/or middle lung zones, or lingula, and
over the diaphragms. They contains blood vessels and
strands of partially-destroyed lung.
• Deep Type: These bullae are found within the lung substance
and contain strands of partially-destroyed lung tissue and
blood vessels.
2.Giant Bullous Emphysema: ("Vanishing Lung Syndrome"
or "Primary Bullous Disease of the Lungs")

• Itis usually associated with young males


who show large progressive upper lung
zone bullae that are often asymmetric.
• The giant bullous lesions occupy greater
than or equal to one-third of the hemithorax.
Cont.

3.Focal Dust Emphysema ( This is focal emphysema


surrounding silicotic nodules.
• 4.Congenital Lobar Emphysema (CLE)
5.Interstitial Emphysema
TREATMENT
• Bullae which enlarge enough to compress adjacent lung
tissue are best diagnosed by CT. A “double-wall sign” on
chest CT, demonstrating air on both sides of the bulla wall,
signifies an associated pneumothorax with the bulla. In
addition to chest CT, these patients should undergo ABGs
and PFTs, The decision to operate is often a challenging
one. Patients should undergo surgical resection when they
have incapacitating dyspnea with large bullae that fill more
than 30% of the hemithorax and result in the compression
of healthy adjacent lung tissue. In addition, operation is
indicated for patients who have complications related to
bullous disease such as infection or pneumothorax
CONT
• There are two surgical approaches for resecting giant lung
bullae. Stapling resection of the entire bulla, either through
a VATS or open approach, is the most common technique .
Pericardial strips can be used along the staple line to assist
in control of air leaks since the surrounding lung tissue is
often diseased. Another operative approach is the modified
Monaldi technique, which involves opening the bulla,
placing a purse-string suture at the neck of the bulla and
closing the overlying bullouss sac with a running back-
and-forth plicating stitch . Both techniques have been
shown to be effective. Smoking cessation and aggressive
pulmonary rehabilitation are also important for successful
treatment of patients with bullous lung disease.