Study of hereditary and congenital chronic broncho- pulmonary
diseases in children is significant due to:
1. The overall
incidence of recurrent and chronic diseases of this system in children ranges from 0,85 to 1,45% and the proportion of malformations and hereditary diseases in the structure of
this pathology is quite high (from 4.6 to 20% according to different authors).
2. In 1-3% of deceased infants and 1/5-1/3 with
chronic bronchopulmonary pathology congenital anomalies of the lungs is diagnosed.
Moreover, with improved methods of diagnostics frequency of congenital and hereditary
pathology has a tendency to increase.
3. This group of diseases has usually
unfavorable forecast, because that leads to disability, reduced life expectancy, and often
to death in early age.
4. A
large number of pathological forms, lack of their study, low separate experience
for each pediatrician, rarity of a disease make it difficult to establish
diagnosis at time and prescribe treatment.
5. There are no effective preventive
measures.
Physicians
of different professions should be aware clinic of these diseases in order to
suspect congenital or hereditary pathology, to diagnose them in children and to
send a child to a specialist, as well as prevent these diseases.
Common
clinical symptoms of congenital lung diseases:
· debut of the disease at an early age
· prolonged, recurrent, chronic inflammation
in the lungs
· obstructive syndrome
· persistent prolonged wheezing in the lungs,
weakened breathing
· "drumsticks", pallor, cyanosis
· physical retardation
· "cor pulmonale"
· breathlessness
· bulging chest
· asphyxia
· loss of consciousness, convulsions
· prolonged cough
· pleural friction rub
· combination with other symptoms
Classification of hereditary and congenital bronchopulmonary
diseases
· Defects of the respiratory system:
· abnormalities of the trachea and bronchi
· anomalies of the lungs
· Hereditary lung diseases.
· Polyorganic hereditary disease with primary
lesion of the lungs.
General diagnostic criteria
§
Early emergence of diseases of the respiratory system
(the neonatal period, I year of life)
§
Physical retardation
§
Persistent obstructive syndrome
§
Recurrent nature of the respiratory system diseases
§
Increasing of sings of respiratory failure despite
treatment
§
Inefficiency therapy
ABNORMALITIES OF TRACHEA AND BRONCHI
ü
Anomalies of branching
tracheobronchial tree.
ü
Congenital
lobar emphysema (lobar, obstructive, hypertrophic, gigant) is a malformation
characterized by a sharp increase in the proportion of one lung due emphyzematous
change and blowing
ü
Traheobronhomegalia / Mounier-Kuhn syndrome –
1932, France/
– congenital anomaly of the system of the trachea and bronchi, with their
expansion due to underdevelopment of elastic cartilage and muscle structures of
the tracheobronchial tree.
ü
Syndrome
Williams-Campbell (I960) – congenital defect of the system due to generalized
hypoplasia of cartilage and segmental subsegmental bronchi from 3rd to 8 th caliber,
followed by the formation of secondary bronchiectasis, predominantly symmetric
in the lower lobes.
ü
Tracheo(bronchi)
- esophageal fistula – a severe malformation, which might result in a fatal
outcome already in the early periods after birth
ANOMALIES
OF LUNG
ü
Lung agenesia – absence of lung together with the main
bronchus.
ü
Lung aplasia – absence of lung with the presence of
rudimentary main bronchus.
ü
Pulmonary Hypoplasia – underdevelopment simultaneous
bronchi and lung parenchyma.
ü
Cystic hypoplasia of the lungs /polycystitis/ –
congenital malformation, which except reduction is accompanied by respiratory areas
of cystic like formation of cavities and bronchiectasis.
ü
Pulmonary sequestration – a section of lung placed
inside or outside the pulmonary lobe and does not participate in gas exchange.
Blood supply to the site is provided by the anomalous vessel from the thoracic
or abdominal aorta or intercostal arteries.
Malformations of
lung
Malformations
of lung frequently underlie the second place amount inflammatory processes in
the bronchopulmonary system and are evident more frequently in childhood and
adolescence. They include, first of all, various options of cystic hypoplasia
of the lung, congenital solitary cyst, pulmonary sequestration and rare
abnormalities of the trachea and bronchi.
Agenesia, aplasia
and hypoplasia of lungs
Agenesia is the absence of
lung together with the main bronchus.
In lung agenesis, the entire lung
and bronchial tree may be absent on one side. The bronchial tree may form
without development of the alveoli. Pulmonary hypertension complicates lung
agenesis because of a combination of factors: normal blood volume passing
through reduced lung tissue, hypoxemia leading to pulmonary vasoconstriction,
and any associated left-to-right shunting cardiac lesion.
Aplasia is the absence of lung tissue in the presence of
rudimentary bronchus.
Hypoplasia is
a state when the main and lobar bronchi terminate functionally insolvent
rudiment, lung tissue is underdeveloped. Intrathoracic
or extrathoracic lesions can cause pulmonary hypoplasia. Therefore, prolonged
rupture of membranes, renal dysplasia, neuromuscular diseases, and congenital
diaphragmatic hernia can lead to lung hypoplasia. Reduced urine volume during
fetal life may retard lung growth. Pulmonary aplasia leads to respiratory
distress, which may vary according to the degree of alveolar involvement.
Pulmonary hypoplasia may be primary when the entire lung or when one lobe is
reduced in size.
Both pulmonary agenesis and
hypoplasia may be accompanied by renal anomalies, which are usually apparent
soon after birth and associated with respiratory distress. Cardiac defects
occur in 50% of patients.
Pulmonary agenesis is
differentiated from lung aplasia by the absence of the carina in the latter.
Lung agenesis is less common than aplasia, about 75% of cases affect the left
side, and it is lethal in half of all patients. It may be associated with other
manifestations of the syndrome of abnormalities of the vertebrae, anus,
cardiovascular system, trachea, esophagus, renal system, and limb buds (VACTERL
syndrome). The survival rate is better with left-sided lung agenesis than with
right-sided agenesis because the right lung is the larger of the two.
Clinic
Asymptomatic clinic is rare.
Children have physical retardation. Respiratory
failure is observed: dyspnea, cyanosis of varying severity. Cough and the
allocation of purulent phlegm are associated with the accession of the
inflammatory process. Sometimes there is a pain in the chest. On the lesion
side thorax is flattened, and the healthy half is convex. At the site of the
lesion there are observed shortening of percussion sounds, absent or weakened
respiratory noises. The heart is shifted toward the lesion, which may
erroneously be interpreted as dextracardia. When expressed hypoxia is observed for
a long time the nail phalanx become thickened as "drumsticks."
Clinic of hypoplasia is less
pronounced. The process proceeds by the type of chronic lung disease, vital lung
capacity, GLC are decreased.
X-rays
reveal the decrease of lung volume on the side of lesion, intense darkness,
highstanding of diaphragm. The heart and mediastinum organs are removed so that
the spine looks bare. However, there may be a "pneumocele" when
healthy lung is prolaboring through the anterior mediastinum in the other
direction.
The final diagnosis is based on
bronchography.
In pulmonary hypoplasia,
development of the distal lung tissue is incomplete. At earlier the delivery of
a child the incidence of lung hypoplasia is higher. In babies delivered before
28 weeks' gestation, the incidence approaches 20%. Pulmonary hypoplasia occur
as a result of conditions that restrict lung growth, such as oligohydramnios,
Potter syndrome (with bilateral renal agenesis or dysplasia), abnormalities of
the thoracic cage, Scimitar syndrome (right-sided pulmonary hypoplasia), and
diaphragmatic hernia (usually left-sided hypoplasia). More than 50% of patients
have associated cardiac, gut, or skeletal malformations. They may have a small
thoracic cage, decreased breath sounds on the affected side, and a mediastinal
shift to the side of the lesion. Therefore, aplasia of the right lung can be
confused with dextrocardia. Patients may present with lung infections, dyspnea
upon exertion, and/or scoliosis.
Radiography
of the newborn the first day of life with suspected hypoplasia of the left lung
(right lung volume more)
Hypovascularity of the entire left lung in a 16-year-old patient with
mild exercise intolerance. This patient had hypoplasia of the left lung
Bronchogram in simple
hypoplastic left lung: the left lung is reduced
in volume.
Displacement of mediastinum and heart to the left. Loculated
translucencies in right middle and lower lung fields with flattened right
diaphragm. (reprinted by permission from W. B. Saunders Company Ltd. Manual of
Neonatal Emergency X-Ray Interpretation, 1994.)
Patients with pulmonary
agenesis and pulmonary hypoplasia seem to have one of 3 presentations. The
first group consists of patients with insufficient lung tissue who may have
received mechanical ventilation for some time. However, ventilator-induced lung
injury results in slow decompensation and death. The second group of patients
is identified serendipitously when chest radiography is obtained to assess a
minor complaint. These patients require no intervention. The third group does
not have respiratory distress requiring mechanical ventilation, but they have
respiratory limitations to activity or kinking of the airway with shift of the
lung to the contralateral side of the chest. In addition to the aplasia or
hypoplasia, congenital narrowing of the upper airway also affects many
patients.
Cystic adenomatoid malformation
Cystic
adenomatoid malformation is a defect in the development of the terminal
bronchioles. A hamartomatous proliferation of cysts occurs and resembles
bronchioles (airways without cartilage).
Congenital Cystic Adenomatoid
Malformation was first identified in 1949 by Ch'in and Tang. Cystic adenomatoid
malformation accounts for 25% of all congenital lung malformations. Respiratory
distress occurs in the neonatal period, when collateral pores of Kohn ventilate
the alveolar tissue present. This process is responsible for the cystic
appearance on radiographs. Patients may have mediastinal shift and a
pneumothorax. The affected area is dull on percussion, and air entry is
decreased. The radiographic depiction of a solid or cystic mass on one side of
the thorax suggests the diagnosis. Cystic changes underdeveloped lungs or it lobe are a good ground
for the development of chronic suppuration, as the drainage function of bronchi
is severely disturbed.
Three
histologic categories of cystic adenomatoid malformation are described: (1)
macrocystic (13%), which has the best prognosis and in which one or more large
(>5 mm
on prenatal ultrasound) cysts are lined with normal pseudostratified ciliated
epithelium; (2) microcystic (73%), which has small cysts lined with ciliated
columnar or cuboidal epithelium; and (3) solid cystic adenomatoid malformation
(13%), which has the worst prognosis and is an airless tissue mass composed of
cuboidal epithelium-lined bronchioles. The difference in prognosis may be
because the solid and microcystic lesions involve a relatively large amount of
lung tissue. Macrocystic lesions are comprised of large, air filled,
nonfunctioning spaces involving smaller areas of lungs.
One lobe, multiple lobes, or
multiple segments on both sides may be affected. The upper lobes are usually
involved. The bronchiolar proliferation is terminal without much alveolar
development. The abnormal hamartomatous proliferation usually retains its
communication with the normal bronchiolar tree. However, no cartilage or
bronchiolar tubular glands are present in the malformation itself. Columnar
mucinous epithelium is present.
Polyhydramnios may be present if
the cystic adenomatoid malformation presses on the esophagus. Pressure on the
heart and large vessels may lead to hydrops fetalis.
Cystic adenomatoid malformation
results when the terminal bronchiolar component of the advancing endodermal
lung bud proliferates haphazardly because of disruption of humoral factors from
the surrounding mesenchyme. Apoptosis in the advancing lung bud is decreased.
Glial cell–derived neurotrophic factor is a growth factor that is abnormally expressed
in the epithelial cells of the cystic adenomatoid malformation. Cystic
adenomatoid malformations usually appear before 7 weeks' gestation but can
occur in the mid stage of lung development. The growth is thought to plateau at
28 weeks' gestation. Communication with the normal airways can lead to
overinflation and compression of the surrounding lung tissue. At the larger the
sonographic volume of cystic adenomatoid malformation in relation to head
circumference the chance for developing hydrops because of more severe central
venous compression is the greater.
Clinic
In approximately 60% of
patients, cystic adenomatoid malformation manifests soon after the neonatal
period. It results in recurrent infections because the mucociliary clearance is
poor. Malignancy can occur in the cystic adenomatoid malformation (pulmonary
blastoma, rhabdomyosarcoma, and bronchoalveolar carcinoma).
The disease
may occur immediately after birth or later, sometimes even in teens agers, depending on the join of
infection. Patients complain of a cough with purulent sputum, frequent colds, dyspnea
during physical exertion, fatigue. Early the developing phalanges and nail
changes in the form of drum sticks and watch glasses
occur.
As result of the decline in lung maturation asymmetry of the chest is observed.
Children suffer from malnutrition and have physical retardation. On
auscultation over the affected areas of lung there are constantly listened dry
and moist rales of various sizes.
X-ray
picture depends on the level and extent of damage of bronchial tree. In
hypoplasia of the lung there is marked shift of the mediastinum in the affected
side, the high standing of the diaphragm. Affected lung is reduced in volume,
sealed, sometimes with annular illumination. In hypoplastic lobe pattern
develops equity fibroatelectasia with a decrease and compaction percentage,
usually pressed against the mediastinum and therefore not always immediately
visible. Adjacent healthy areas of the lung appear to be more transparent in
comparison with the opposite lung due to hyperinflation.
Cystic adenomatoid
malformation of
right
lung
Polycystiс malformation of left lung
Polycystiс malformation of the upper lobe of right lung
Most
demonstrably picture is given at bronchography, and computed tomography (CT). They
reveal multiple cystic cavities, which are the ends of lobe, segmental or
smaller bronchi. At angiopulmonography may be found signs of malformation of
the pulmonary blood vessels - their thinness, lack of contrasting small
branches, the expansion of branching angles.
Cystic
hypoplasia of right lung (bronchography in lateral projection)
Air cysts of right
lung
Changes in cystic
hypoplasia that develops in the later stages of embryogenesis are very similar
to those of acquired atelectasis bronchiectasia. In the absence of typical
radiological signs to determine the innate pathology is possible only by the
morphological study of resected preparates and even then not always.
Treatment
In cystic adenomatoid
malformation, resection of even asymptomatic masses is recommended because of
the risk for infection, hemorrhage, acute respiratory compromise (which may
occur anytime), and neoplastic transformation. This disease is usually
segmental; however, as noted for sequestration, lobectomy may reduce morbidity.
During surgery, lung cysts are often found to be cystic adenomatoid
malformations, though simple cysts do occur. Some lesions can be shelled out or
unroofed. If they are not congenital but related to barotrauma, they may
communicate directly with small bronchi. In this case, unroofing leads to major
air leaks. These lesions can sometimes be controlled with figure-8 sutures, but
wedge resection, segmentectomy, or even lobectomy may be required to avoid a
bronchopleural fistula.
Congenital (real) solitary
cysts
Bronchogenic cysts are also
known as foregut duplication. They arise from an abnormal budding of the
ventral foregut. Approximately 85% are mediastinal, and 15% are intrapulmonary.
The peripheral cysts are multiple and appear late in gestation. They may be filled
with air or fluid, or they may have air-fluid levels. The cysts can be central
or peripheral. Many are asymptomatic, but incidental findings may be observed
on chest radiography. Infection, hemorrhage, and, in rare cases, malignancy can
occur. Respiratory distress may result in a stridor or wheeze. Airtrapping may
lead to emphysema, atelectasis, or both. Dysphagia, chest pain, and epigastric
discomfort can occur.
Lung cysts are rare lesions
that may arise from any of the parenchymal tissues of the lung. They can cause
symptoms if they enlarge and occupy substantial space. Resection is performed
to diagnose lung cyst and to stop the progression of symptoms.
Bronchogenic cysts represent
outpouchings of the ventral foregut in the early part of gestation. These
outpouchings generally arise close to the bronchial tree. A cyst may become
infected, or it may compress adjacent structures to produce signs and symptoms.
Chronic infection and inflammation may predispose the patient to malignancy.
Peripheral cysts appear late in gestation and are multiple.
Bronchogenic
cysts are most commonly mediastinal in a pericarinal, paratracheal, or
retrocardiac location. The cysts are thin walled and lined with columnar
epithelium. The common central cysts represent outpouchings of the ventral
foregut in the early part of gestation.
Clinics
Many
cysts are asymptomatic, but incidental findings may be observed on chest
radiography. Infection, hemorrhage, and, in rare cases, malignancy can occur.
Respiratory distress may result in a stridor or wheeze. Airtrapping may lead to
emphysema, atelectasis, or both. Dysphagia, chest pain, and epigastric
discomfort can occur.
Unlike cystic hypoplasia solitary cysts
usually do not have widespread reports of bronchi and therefore become infected
less often and later than cystic. True cysts of the lung at birth are filled
with mucus, often having a dark brown color (so-called chocolate cysts). Communicating
with the small bronchi of the cyst may be complicated by valve mechanism (tense
cysts), a breakthrough in the pleural cavity or infection.
At infected
cysts on the first place there are the symptoms of purulent intoxication: high
fever, sweating, increasing weakness, lethargy, loss of appetite. For large
scale cysts symptoms of respiratory failure may appear: shortness of breath,
cyanosis of lips and limbs, which are more common in young children.
Characteristic is the appearance of dry
or wet cough. At percussion over the cavity of the cyst when it is of
sufficient size may be noted dullness of sound and auscultation with the
weakening of breath and wheezing of various sizes.
On plain radiograms and tomograms lung
festering cysts are seen as round or oval hollows formations with, as a rule,
the level of liquid and air over it. Unlike lung abscesses inner and outer
contours of the cyst are clear and smooth, perifocal reaction is expressed
slightly. Often cysts are a multi, and the upper edge of the liquid in
individual cells may be at different levels. Bronchi and vessels, clearly visible in
contrasting and tomograms uniformly bend around the contours of the cyst, which
is not at the case with lung abscess.
Bronchogenic cyst. Conventional radiographs demonstrate a subcarinal
mass.
Bronchogenic cyst. Media file shows a right paratracheal mass.
Bronchogenic cyst. CT scan demonstrates a thin-walled cyst in the right
upper lobe.
Review chest X-ray of the
child with a congenital air cyst of the right lung in
direct projection: annular shadow of the cyst is
indicated by arrows.
Review chest X-ray of the
child with a congenital air cyst of the right lung in
lateral projection: annular shadow of the cyst
is indicated by arrows.
In the differential diagnosis of
purulant cysts it is needed to remember about tuberculous cavity, the more so
that their localization (mainly in the upper lobes) usually coincides. For tuberculous
cavern characteristic features are rugged, "moth" inner contour and
fibrose focal shadows on the periphery of the cavity. In addition, the cavity is
characterized by the presence of shadows and enlarged lymph gland in the root
of the lungs and shadow of draining bronchus, well visible on the tomograms.
Pulmonary sequestration
Pulmonary
sequestration accounts for 6% of all congenital lung malformations and mostly
occurs in the lower lobes. A sequestration is a bronchopulmonary tissue without
a normal bronchial communication and with normal or anomalous vascular supply.
Sequestered lung may be intralobar or extralobar.
A pulmonary
sequestration there is a benign mass of non-functioning lung tissue that
appears during early lung development. This lesion has no connection with the
airway and receives its blood supply from the systemic circulation usually off
the abdominal or thoracic aorta. The most common type of BPS is formed within
the normal lung itself and is referred to as intralobar. The other type of BPS
is known as extralobar, and is formed outside the normal lung. This type of
lesion is usually found in the chest cavity although rarely it may be found in
the abdomen. There is a higher incidence of associated anomalies in babies with
extralobar BPS.
The involved lung
segments can be classified on the basis of their pleural coverage into
intrapulmonary or extrapulmonary types. Variants of pulmonary sequestration are
described as disconnected or abnormally communicative bronchopulmonary masses
with normal or anomalous vascular supply. The lesions may have some sort of
communication with the gut.
Clinic
About 50% of pulmonary
sequestration cases are intrapulmonic, and 60% of intrapulmonic cases occur in
the left lower lobe with equal sex distributions. Patients with intrapulmonary
sequestration usually present late. They may have a chronic cough, recurrent
pneumonias, or poor exercise performance. Systemic arterial flow may produce a
murmur, and shunts may lead to congestive cardiac failure. Squamous cell
carcinoma, adenocarcinoma, and rhabdomyosarcoma may arise in the sequestration.
Approximately 95% of
extrapulmonary cases are left sided. Most extrapulmonary cases are detected in
infancy, with boys affected 4 times more than girls. Infants usually present with
a chronic cough and recurrent chest infections. Radiographs may reveal signs of
consolidation. If communication with the gut is present, children may present
with vomiting, failure to thrive due to poor oral intake, and abdominal pain.
Clinical manifestations are due to
inflammatory changes: cough, fever, shortness of breath, over the lesions
listened small bubbling moist rales.
There are three forms of the
pathological process:
§
bronchiectasis,
in which repeated inflammation leads to
fusion of lung tissue and
secondary connection with
bronchial tree
§
pseudotumoral
that is characterized by small clinical symptoms
§
local abscess
formation or empyema
The main
distinguishing feature of sequestration of the lung is an additional large
vessel which deviates from the aorta and branching in the sequestered lung
tissue. This vessel can be identified at aortography, tomography and CT.
Sometimes it is finding during the operation as an
accident.
Diagnose of pulmonary sequestration
is based on angiography. Much less importance has bronchography. At radiograph
may be darkening of the affected segment inflammation.
Sequestration of the lung. There
is cystic changed area
of S10 of the lower lobe of
the left lung.
Aortogramme at sequestration
of the lower lobe of right lung: the additional vessel (2) is going from the
aorta (1) to the sequestered part of lung.
Review X-ray of the child with
intrapulmonary sequestration in low medium sections of the right lung: in the
zone sequestration there is shading lung tissue (indicated by arrow).
Surgical
treatment: resection of the sequestered area.
Resection is recommended, even
in asymptomatic patients, to prevent infection, hemorrhage, shunting from
arteriovenous anastomoses, or compression of normal lung mass leading to
respiratory distress. Lobectomy can usually be performed. For patients with
intralobar sequestration, segmentectomy may suffice. Segmentectomy is
relatively difficult, but preserves additional functioning lung tissue.
Prognosis is favorable.
Malformations of trachea and bronchi
Among the rare malformation of the trachea
and bronchi, facilitating the development of intrapulmonary suppuration, should
first be called traheobronhomegalia usually combined with tracheomalacia (so
called Moanier-Kuhn syndrome), and
congenital stenosis of the bronchi, as described in the form of casuistic
observations.
Traheobronchomegalia
(Moanier-Kuhn
syndrome)
Tracheobronchomegalia (Moanier -Kuhn
syndrome (P. Moanier-Kuhn, modern French otolaryngologist), 1932) - congenital
enlargement of the trachea and bronchi.
At tracheobronchomegalia and malacia
softened and lost their resilience cartilages of the trachea and major bronchi
make in the airways the flaccid, extended tube, decreasing with exhalation and
cough. Bronchial drainage function is disrupted, and delayed sputum, rapidly
infecting, promotes suppuration in the distal lung. A similar mechanism of
development of septic complications arise at bronchial stenosis, which disturb
the self-cleaning of bronchi.
Pathomorphologic changes are due to
the diffuse extension of the trachea, sometimes the main bronchi. At this
anomaly atrophy of the longitudinal elastic fibres and thinning of the muscle
bundles occur.
There are the following types of a
defect: an isolated tracheomegalia (pathological enlargement of the trachea
with a normal value of the lumen of the bronchi), isolated bronhomegalia
(enlargement of one or both main bronchi with normal width of the trachea) and
traheobronhomegalia (enlargement of the trachea and one or both main bronchi).
There is a rare involving in
pathological process the segmental and smaller bronchi, although in the distal
bronchial tree in some cases may be secondary changes.
Clinic
Clinically there is the recurrent
tracheobronchitis, in the course of which predominates bitonal irritating cough
with prolonged sputum discharge.
Abnormalities of the trachea and
bronchi may be suspected by the presence of the characteristic cough with
metal, vibration shade, hard forced exhalation and the presence of hard
discharged purulent sputum in children. On auscultation over the lungs there
are listened many variegated wheezing, which do not disappear after cough.
Radiographic examination often reveals signs of bronchiectasis, and in patients
with acute inflammation the massive bilateral pneumonia, often with abscess
formation.
Frequent symptoms are chronic intoxication and hypoxemia, pallor,
retarded physical development, the deformation of the fingers on the type of
drum sticks. In the lungs, there are different changes in percussion sounds, a
variety of wheezing.
Studies of lung function and blood
gas composition reveal pronounced degree of combined ventilatory insufficiency
and hypoxemia.
Bronchofibroscopy helps to establish
the correct diagnosis. At tracheobronhomegalia there are determined a
significant expansion of the lumen of the trachea and main bronchi, deformation
of their walls with the curvature of the cartilaginous rings and deep, sac
interchondral intervals, a thickening of the mucous membrane in the form of
circular folds and the almost total collapse of the lumen by coughing and
forced expiration, expiratory stenosis and the presence of inflammatory
changes in the trachea and bronchi. Congenital stenosis of the major bronchi is
defined as smooth wall sunken or membranous narrowing with a small round hole
at the center.
Bronchoscopy - the examination of the
bronchi (the main airways of the lungs) using a flexible tube (bronchoscope).
Bronchoscopy helps to evaluate and diagnose lung problems, assess blockages,
obtain samples of tissue and/or fluid, and/or to help remove a foreign body.
Вronchoalveolar lavage - to remove cells from
lower respiratory tract to help identify inflammation and exclude certain
causes.
lung biopsy - to
remove tissue from the lung for examination in the pathology laboratory.
Bronchoscopical sings at tracheobronchomegalia (Moanier-Kuhn syndrome)
Treatment
is provided as at endobronchitis.
Prognosis
for life is favourable.
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