congenital, inflammatory and infectious diseases of the lung

Congenital, inflammatory and infectious diseases of the lung

Most congenital diseases of the lung are recognized when respiratory symptoms develop in the newborn or infant.

Prenatal diagnosis

Asymptomatic child, incidental radiograph of the chest

Older child during evaluation for a respiratory tract infection

Disease range: Simple abnormal bronchus ..... Complete absence of one lung

Radiologic evaluation :chest X-ray, ultrasonography, computed tomography

Embryologic development of the lungs: 4 stages

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

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

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

Laryngo-tracheo-esophageal Cleft: Fatal. Aspiration of the ingested food. Chooking while feeding. Surgery.

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.

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.

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.

BRONCHIAL ANOMALIES Pulmonary Agenesia – Aplasia - Hypoplasia: Range from

single hypoplasia of a segment or lobe to double lung absence. 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)

BRONCHIAL ANOMALIES Pulmonary Sequestration:

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

BRONCHIAL ANOMALIES Pulmonary Sequestration:

BRONCHIAL ANOMALIES Congenital Lobar Emphysema:

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

BRONCHIAL ANOMALIES Congenital Lobar Emphysema:

BRONCHIAL ANOMALIES Congenital Cystic Adenomatoid Malformation:

Developmental pause at pseudoglandular phase, growth of primitive structures.

Extreme proliferation of terminal bronchiolles, multiple cystic structures lined with ciliated or cuboidal epithelium and non-functional lung tissue with high perfusion in between.

Prenatal USG Neonatal respiratory distress Emergency, Surgery

BRONCHIAL ANOMALIES Congenital Cystic Adenomatoid Malformation:

CONGENITAL VASCULAR LESIONS Agenesis and Stenosis of the pulmonary artery:

Die at early age from right-sided ventricular failure and hypertension in the pulmonary artery of the unaffected side

Pulmonary Arteriovenous Fistulas 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

CONGENITAL VASCULAR LESIONS Pulmonary Arteriovenous Fistulas

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

INFLAMMATORY AND INFECTIOUS DISEASES Cystic Fibrosis

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

INFLAMMATORY AND INFECTIOUS DISEASES Bronchiectasis

Abnormal permanent dilatation of subsegmental airways Etiology:

Congenital Congenital cystic bronchiectasis Selective immunoglobulin A deficiency Cystic fibrosis (most common) Alpha-1 antitrypsin deficiency Kartagener’s syndrome (most common)

Acquired Infection (most common) Bronchial obstruction Middle lobe syndrome Scarring secondary to tuberculosis


Classification: 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


Frequency of Distribution of bronchiectasis: 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:

Medical: preventive and control, antibiotics, postural drainage Surgical:

Unilateral, segmental or lobar distribution Persistent, recurrent symptoms when medication is discontinued Recurrent infection and hemoptysis Transplantation

INFLAMMATORY AND INFECTIOUS DISEASES Lung Abscess

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 :

Primary lung abscess (acute or chronic) Related to anaerobic aspiration Related to specific pneumonia

Secondary lung abscess With existing lung disease Metastatic from extrathoracic source Obstructing bronchial carcinoma Bronchoesophageal fistula Foreign body aspiration Pulmonary infarction Bullous emphysema


Most frequent anaerobic cause: Bacteroides Most frequent aerobic cause: staphylococcus aureus and

streptococcus pneumoniae Diagnostic techniques: H, PE, fever, purulent foul smelling-

sputum, FOB, CT Differential Diagnosis:

Cavitating carcinoma Tuberculous or other fungal diseases Pyogenic lung abscess Empyema with bronchopleural fistula


Principles of Therapy: Identification of etiologic organism Prolonged antimicrobial treatment Adequate drainage in acute stage Chest physiotherapy Bronchoscopy Percutaneous catheter drainage Emergency surgical treatment


Indications for Surgery: Acute Stage (emergency)

Complications Bronchopleural fistula Empyema Bleeding

Chronic Stage Persistent symptoms and signs Recurrent complications (empyema, fistula) Suspicion of carcinoma Persistence of lung abscess larger than 6 cm after 8 weeks of

treatment

INFLAMMATORY AND INFECTIOUS DISEASES Right Middle Lobe Syndrome

Episodes of hemoptysis, chronic cough and repeated pulmonary infections, atelectasis of right middle lobe

Bronchial obstruction CT

Broncholithiasis 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

INFLAMMATORY AND INFECTIOUS DISEASES Pulmonary Tuberculosis

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

INFLAMMATORY AND INFECTIOUS DISEASES Hydatid Disease of the lung

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.


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.


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)


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.

Surgery is the mainstay


SURGERY: lung-sparing surgery Enucleation (Barret) Pericystectomy +/- capitonnage (Perez-Fontana) Cystotomy + capitonnage Segmentectomy/lobectomy/pneumonectomy

Bilateral: larger cyst or multiple cystic side, intact cyst

congenital, inflammatory and infectious diseases of the lung

Documents

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tracheoesophageal fistula

esophageal atresia

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tracheal diverticula

rudimentary lung

lung bud disease