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Congenital, inflammatory
and infectious diseases of
the lung
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Most congenital diseases of the lung are recognized when
respiratory symptoms develop in the newborn or infant.
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Prenatal diagnosis
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Asymptomatic child, incidental radiograph of the chest
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Older child during evaluation for a respiratory tract infection
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Disease range: Simple abnormal bronchus ..... Complete
absence of one lung
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Radiologic evaluation :chest X-ray, ultrasonography,
computed tomography
Embryologic development of the lungs: 4 stages
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Embryologic phase (26th day – 5th week) : lung bud
disease: tracheo-esophageal fistula, bronchopulmonary
foregut malformations, tracheobronchial atresia, tracheal
bronchial diverticule
Pseudoglandular phase (5th week – 16 th week) :
disease: congenital cystic adenomatoid malformation
Canalicular phase (16th week – 25th week) :
Terminal saccule (alveolar period) (25th week – 40th
week) : saccular proliferation and progressive
vascularization. Disease: extreme proliferation and no
vascularization: congenital lobar emphysema
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TRACHEO-BRONCHIAL ANOMALIES - I
Tracheal Agenesia – Atresia: Fatal. Maternal
polyhidramnios and prematurity. Scream when born,
then progressive cyanosis, bradycardia, arrest. ETcan
not pass byond vocal cords. Esophageal entubation
(TEF).
Tracheal Malacia: elastic and connective tissue
degeneration. Structural deformation of the cartilage.
Tracheal wall collapse during inspiration. Cyanotic spells
in newborn, stridor. Recuurent pneumonia attacks. Asso.
With esophageal atresia and TEF. Dianosis:
bronchoscopy, CT, Virtual bronchoscopy and
MRI.Conservative treatment, surgery, intraluminal
absorbable stents
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TRACHEO-BRONCHIAL ANOMALIES - II
Tracheal Bronchus – Diverticula: Tracheal bronchus:
arise from trachea and either has a lobe or ends in
pulmonary parenchyma. Tracheal diverticula: arises
fromcervial or thoracic portion of trachea and ends
blindly or in a rudimentary lung. Treatment: if
asymptomatic, conservative. Recurrent infections:
resection
TRACHEO-BRONCHIAL ANOMALIES - II
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Laryngo-tracheo-esophageal Cleft: Fatal. Aspiration of
the ingested food. Chooking while feeding. Surgery.
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TRACHEO-BRONCHIAL ANOMALIES - III
Bronchial Atresia: ends blindly in lung tissue. At birth,
portion of lung adjacent to atretic bronchus is filled with
fluid. Fluid is absorbed. Retained secretions result in a
mucocele. Obstructive emphysema occurs. Recurrent
episodes of infection. Differential diagnosis: Congenital
lobar emphysema and bronchogenic cysts.
Bronchoscopy and CT. Surgery.
Anomalous Bronchi: most common communication
between trachea and another foregut derivative is
tracheoesophageal fistula. Pneumonia attacks
Tracheobiliary and bronchobiliary Fistula: bile-stained
secretions, respiratory distress. Bronchoscopy and
fistulography.
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TRACHEO-BRONCHIAL ANOMALIES - IV
Bronchogenic Cysts: Lined with ciliated columnar or
cuboidal epitheliumon a fibromuscuar base.cyst walls
are thin and may contain cartilage and bronchial glands.
Can be found in the hilum of the lung, posterior sucus
and pulmonary parenchyma. May cause pulmnary
sepsis. Dyspnea, stridor, wheezing, cyanosis. Air filled ,
air-fluid level. Surgery. Segmental or lobar resection.
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BRONCHIAL ANOMALIES
Accessory Lobe: Fissure anomalies result in accessory
lobes. Right and left posterior, right and left inferior, left
middle lobe and Azygos lobe. Azygos lobe is not a real
accessory lobe. Inverted comma shape.
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BRONCHIAL ANOMALIES
Pulmonary Agenesia – Aplasia - Hypoplasia: Range from
single hypoplasia of a segment or lobe to double lung
absence.
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Agenesia: absence of a structure.
Aplasia: defective development.
Primary hypoplasia and secondary hypoplasia (intrauterine
intrathoracic space problems: congenital diaphragmatic hernia,
congenital cystic adenomatoid malformation, hydrops fetalis with
pleural effusion, mediastinal neoplasms, cystic hygroma; Chest
wall anomalies resulting in thoracic volume decrement: thoracic
dystrohies (Jarco-Levin and Jeune synd), osteogenesis
imperfecta; oligohydramnios syndrome (uterine distress over the
fetus)
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BRONCHIAL ANOMALIES
Pulmonary Sequestration:
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Most common developmental lung disease
A segment or lobe of the lung tissue has no bronchial
communication with the normal tracheobronchial tree. Arterial
blood supply is from a systemic artery. This vessel arises from
abdominal aorta. Venous return is to systemic (azygos) or
pulmonary veins.
Extralobar sequestration: (25%) seperate from normal lung and
has its own visseral pleura. 90% on left side.
Intralobar sequestration: Within normal lung parenchyma.
Abscess formation
CT – angio and MRI
Surgery
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BRONCHIAL ANOMALIES
Pulmonary Sequestration:
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BRONCHIAL ANOMALIES
Congenital Lobar Emphysema:
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isolated hyperinflation of a lobe in the absence of extrinsic
bronchial obstruction.
Left upper lobe most commonly, second right middle lobe.
Defective cartilage structure results in bronchial wall weakness,
collapse of the bronchus during expiration and air-trap
Differential diagnosis: pneumothorax, tension cyst, congenital
diaphragm hernia and foreign body aspiration
Tachypnea, retraction of the chest wall, wheezing since birth
Tracheal and mediastinal shift to the contralateral hemithorax
Breath sounds are decreased on the affected side,
hyperresonance
X-ray: hyperaeration of the affected lobe with atelectasis of the
adjacent lobes and a mediastinal shift
CT: hyperlucent expanded lobe
Surgery
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BRONCHIAL ANOMALIES
Congenital Lobar Emphysema:
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BRONCHIAL ANOMALIES
Congenital Cystic Adenomatoid Malformation:
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Developmental pause at pseudoglandular phase, growth of
primitive structures.
Extreme proliferation of terminal bronchiolles, multiple cystic
structures lined with ciliated or cuboidal epithelium and nonfunctional lung tissue with high perfusion in between.
Prenatal USG
Neonatal respiratory distress
Emergency, Surgery
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BRONCHIAL ANOMALIES
Congenital Cystic Adenomatoid Malformation:
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CONGENITAL VASCULAR LESIONS
Agenesis and Stenosis of the pulmonary artery:
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Pulmonary Arteriovenous Fistulas
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Die at early age from right-sided ventricular failure and
hypertension in the pulmonary artery of the unaffected side
Most common anomaly of pulmonary vascular tree
Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber
syndrome
Exertional dypsnea, palpitations, easy fatigability
Hypoxia, blood gas analysis
CT-Angio
Surgery, embolic obliteration
Pulmonary artery Aneurysms
Abnormal pulmonary venous connection
Lymphangiectasia
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CONGENITAL VASCULAR LESIONS
Pulmonary Arteriovenous Fistulas
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INFLAMMATORY AND INFECTIOUS DISEASES
Cystic Fibrosis
Bronchiectasis
Lung Abscess
Right Middle Lobe Syndrome
Broncholithiasis
Pulmonary Tuberculosis
Mycotic and Actinomycetic Infections
Hydatid Disease of the lung
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INFLAMMATORY AND INFECTIOUS DISEASES
Cystic Fibrosis
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AR disease, mutations in a single gene that encodes CF
transmembrane conductance regulator (CFTR) protein
CF gene on long arm of chr 7
Chronic sinopulmonary infections, impaired exocrine pancreatic
function, elevated sweat chloride, male infertility
Most common lethal genetic disease in whites
Median predicted survival 36.5 years
X-ray: hyperinflation with flattening of domes of diapragm,
increase in postero-anterior diameter of chest
Diffuse cystic interstitial process
Surgery for pulmonary complicatios (pneumothorax, hemoptysis)
Lung transplantation
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INFLAMMATORY AND INFECTIOUS DISEASES
Bronchiectasis
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Abnormal permanent dilatation of subsegmental airways
Etiology:
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Congenital
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Congenital cystic bronchiectasis
Selective immunoglobulin A deficiency
Cystic fibrosis (most common)
Alpha-1 antitrypsin deficiency
Kartagener’s syndrome (most common)
Acquired
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Infection (most common)
Bronchial obstruction
Middle lobe syndrome
Scarring secondary to tuberculosis
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INFLAMMATORY AND INFECTIOUS DISEASES
Bronchiectasis
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Classification:
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Saccular (follows a major pulmonary infection or results from foreign
body or bronchial stricture and is the principal type of surgical
importance)
Cylindrical (consists of bronchi that do not end blindly but
communicate with lung parenchyma. Frequently asso. with
tuberculosisi and immune disorders)
Pseudobronchiectasis (cylindrical dilatation of a bronchus after an
acute pneumonic process that is temporary and disapperas in
weeks)
Posttuberculous bronchiectasis
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INFLAMMATORY AND INFECTIOUS DISEASES
Bronchiectasis
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Frequency of Distribution of bronchiectasis:
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Left lung more often than right lung
Left lower lobe: most frequently involved
Right middle lobe and lingula: next most frequently involved
Total left bronchiectasis, fourth most commonly involved
Right lower and total right are less often involved
Right upper lobe is involved more than left upper lobe (4:1)
HRCT
Treatment:
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Medical: preventive and control, antibiotics, postural drainage
Surgical:
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Unilateral, segmental or lobar distribution
Persistent, recurrent symptoms when medication is discontinued
Recurrent infection and hemoptysis
Transplantation
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INFLAMMATORY AND INFECTIOUS DISEASES
Lung Abscess
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Subacute pulmonary infection in which chest radiograph shows a
cavity within the pulmonary parenchyma
Localised collection of pus contained within pulmonary
parenchyma
An abscess is defined as acute when duration of symtoms is
<6 weeks
Classification :
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Primary lung abscess (acute or chronic)
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Related to anaerobic aspiration
Related to specific pneumonia
Secondary lung abscess
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With existing lung disease
Metastatic from extrathoracic source
Obstructing bronchial carcinoma
Bronchoesophageal fistula
Foreign body aspiration
Pulmonary infarction
Bullous emphysema
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INFLAMMATORY AND INFECTIOUS DISEASES
Lung Abscess
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Most frequent anaerobic cause: Bacteroides
Most frequent aerobic cause: staphylococcus aureus and
streptococcus pneumoniae
Diagnostic techniques: H, PE, fever, purulent foul smellingsputum, FOB, CT
Differential Diagnosis:
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Cavitating carcinoma
Tuberculous or other fungal diseases
Pyogenic lung abscess
Empyema with bronchopleural fistula
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INFLAMMATORY AND INFECTIOUS DISEASES
Lung Abscess
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Principles of Therapy:
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Identification of etiologic organism
Prolonged antimicrobial treatment
Adequate drainage in acute stage
Chest physiotherapy
Bronchoscopy
Percutaneous catheter drainage
Emergency surgical treatment
INFLAMMATORY AND INFECTIOUS DISEASES
 Lung Abscess
 Indications
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Acute Stage (emergency)
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for Surgery:
Complications
 Bronchopleural fistula
 Empyema
 Bleeding
Chronic Stage
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Persistent symptoms and signs
Recurrent complications (empyema, fistula)
Suspicion of carcinoma
Persistence of lung abscess larger than 6 cm after 8 weeks of
treatment
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INFLAMMATORY AND INFECTIOUS DISEASES
Right Middle Lobe Syndrome
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Episodes of hemoptysis, chronic cough and repeated pulmonary
infections, atelectasis of right middle lobe
 Bronchial obstruction
 CT
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Broncholithiasis
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Isolated area of bronchial compressive disease, calcified lymph node
eroded into the lumen of a bronchus, distortion or partial obstruction of
the tracheobronchial tree by calcified peribronchial lymph nodes
 Pathogenesis is caused by tissue response to a healing granulomatous
inflammation. Most frequent involved diseases are histoplasmosis and
tuberculosis
 CT, FOB
 Indications for surgery: intractable cough, recurrent hemoptysis, chronic
infection resulting from bronchostenosis or a secondary
esophagobronchial fistula
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INFLAMMATORY AND INFECTIOUS DISEASES
Pulmonary Tuberculosis
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Patients with lungs destroyed by MDR TB or cavitary disease
with or without positive sputum smears will require resection
Decortication alone for a trapped lung
Other patients who require surgical intervention are those with
bronchopleural fistulas, massive hemoptysis (>600ml in 24
hours), bronchostenosis or in whom there is a need to rule out
cancer
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INFLAMMATORY AND INFECTIOUS DISEASES
Hydatid Disease of the lung
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Endemic areas Middle East, Mediterranean countries, South
America, Australia, New Zealand, Central Asia, China, Alaska
and East Africa
Caused by Echinococcus granulosus
Primary host: Canidae family, dogs, wolves, coyotes.
They contract echinococcosis by ingesting mature, productive
echinococcal cysts in the viscera of an intermediate host (sheep,
goats, cattle, deer)
In the intestines of primary host, cyst develops into worm
containing 400 to 800 ova. Ova are released in the feces of the
primary host, introduced into intermediate hosts by ingestion of
contaminated grass, water, vegetables.
In GI tract of intermediate host ovum develops into embryo, with
hooklets penetrates mucosa od duodenum and jejenum, enters
mesenteric venules, proceeds to portal vein and liver.
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INFLAMMATORY AND INFECTIOUS DISEASES
Hydatid Disease of the lung
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Embryos <0.3 mm pass through snus capillaries of liver and by
hepatic veins and vena cava proceed to right side of heart and
pulmonary capillaries.
Alternative way: embryos enter lymphatics of small intestine,
proceed to thoracic duct, to central venous system, to right side
of heart and to lungs.
Another way: venovenous anastomosis in the liver
Most common infected organ : LIVER (60-80%)
Lung (10-30%)
Hydatid cyst : wall + hydatid fluid
Wall has 3 layers: outer laminated membrane and inner
germinative membrane derive from parasite and pericyst or
adventitia (ectocyst) is produced by host.
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INFLAMMATORY AND INFECTIOUS DISEASES
Hydatid Disease of the lung
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Germinative membrane is the living part.
Hydatid fluid: odorless, colorless, sterile, crystal-clear water.
Cyst may rupture during the growth period.
Rupture of germinative membrane toward the interior of the cyst
result in formation of daughter cysts.
Clinical presentation depends on whether cyst is intact or
ruptured.
Intact cyst : no symptom, cough, hemoptysis, pain
Ruptured: expectoration of salty sputum
Rupture into pleural cavity. 5%
X-ray: homogeneous spherical opacity with definite edges.
Change from spherical to an oval shape only during inhalation
(Escudero-Nemerow sign)
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INFLAMMATORY AND INFECTIOUS DISEASES
Hydatid Disease of the lung
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Crescent sign, moon sign, meniscus sign: clear crescent shadow
on the top or on one side: first sign of rupture
Double domed arch sign: small additional air enters in.
Camalote sign, water lily sign: enterance of free air into cyst after
complete rupture of the membrane. Floating membrane of the
cyst.
CT, MRI, USG, ECHO
Eosinophilia
IHA: 80-94% in liver, 65% in lung
Treatment:Albendazle, mebendazole: who cannot tolarate
surgery, numerous cysts, when complete removal is impossible.,
postoperative.
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Surgery is the mainstay
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INFLAMMATORY AND INFECTIOUS DISEASES
Hydatid Disease of the lung
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SURGERY: lung-sparing surgery
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Enucleation (Barret)
Pericystectomy +/- capitonnage (Perez-Fontana)
Cystotomy + capitonnage
Segmentectomy/lobectomy/pneumonectomy
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Bilateral: larger cyst or multiple cystic side, intact cyst
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INFLAMMATORY AND INFECTIOUS DISEASES
Hydatid Disease of the lung
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INFLAMMATORY AND INFECTIOUS DISEASES
Hydatid Disease of the lung