BRONCHIECTASIS

IRREVERSIBLE abnormal dilation and anatomic distortion of the bronchial

tree
Most common cause of bronchiectasis: CYSTIC FIBROSIS
Lobe most commonly affected: lower lobes
Most common complaint: cough and production of copious purulent
sputum
Late finding: impaired diffusion capacity
Gold standard for diagnosis: thin-section HRCT san
Most frequent microorganism: H.influenza, P aeruginosa
Most severe form: sacular(cystic) bronchiectasis
Assoc. with more severe impairement of lung function: P.aeruginosa
Severe cases: dyspnea, hypoxemia
Prominent feature of chronic inflammatory disease: mucus
hypersecretion
Williams-Campbell Syndrome: absence of annular bronchial cartilage
Marnier-Kuhn syndrome: congenital tracheobronchomegaly,
connective tissue disorder
Right middle lobe syndrome: chronic extrinsic compression of right
middle lobe bronchus by hilar lymph node
Yellow Nail Syndrome: pleural effusion, lymphedema, discolored nail
Pathogenesis: Chronic inflammation, Obstruction, Congenital
Classification:
Cylindrical- bronchial outlines are regular, there is diffuse dilation of
bronchial unit
Varicose- local constriction cause an irregularity of outline, degree of
dilation is greater, small sacculation may be seen
Sacular(cystic)- results in ballooning that end in fluid or mucus-filled
sacs
Definition:
Prebronchiectatsis: chronic or recurrent endobronchial infection with
nonspecific HRCT changes, may be reversible
HRCT bronchiectasis: clinical symptom with HRCT evidence of bronchial
dilation, may persist, progress, improve or resolve
Established bronchiectasis: clinical symptom with HRCT evidence of
bronchial dilation, no resolution within 2 yrs
PE: area, wheezing, digital clubbing
crackles on affected

CX: increase in size and loss of definition of bronchovascular markings,

crowding of bronchi, loss of lung volume, honeycombing may occur,
compensatory overinflation of unaffected lung may be seen

CT Findings:
Cylindrical- “tram lines”, “signet ring”
Varicose- bronchi with “beaded contour”
Cyst- cyst in “strings and clusters”
Treatment: control infection, reduce inflammation, improve bronchial
hygiene

For acute axacerbation: parenteral antibiotic 2-4 weeks

Important adjunct to chest physiotherapy: nebulized with
hypertonic/NAC
Useful in preventing inspissated sputum retention: hydration with
oral/IV
Traditional method of facilitating mucus clearance: Chest percussion,
postural drainage
Bronchodilator: for airway obstruction and hyperactivity
Long term prophylaxis: macrolide
Surgical resection: for most severely affected segment
 LUNG ABSCESS
localized areas composed of thick-walled purulent material formed as a result of lung
infection that destroys the lung parenchyma, resulting in cavitations and central
necrosis
Most common cause in children: aspiration of infected materials or foreign
body
Most common symptom: cough, fever, tachypnea, dyspnea, chest pain,
vomiting, sputum production, weight loss, and hemoptysis
Most common aerobic bacteria: Streptococcus spp., Staphylococcus aureus,
E.coli, K.pneumoniae, P.aeruginosa
Most common anaerobic bacteria: Bacteroides spp., Fusobacterium spp.,
and Peptostreptococcus spp
Fungi: cause of lung abscess in immunocompronised patient
Diagnosis most commonly made by: Chest radiography
Treatment: conservative management
Predisposing condition: aspiration, pneumonia, CF, GER, TEF, post op
tonsillectomy&adenoidectomy, seizure
initial and, often, only intervention required: Minimally invasive
percutaneous aspiration
Negative prognostic factor: presence of aerobic organism
CT scan: can provide better anatomic definition of abscess
Pathogenesis: aspiration → pneumonitis(impairs drainage of fluid or
aspirated material → inflammatory vascular obstruction → tissue
necrosis → liquefaction → abscess formation

Which part is affected?

Aspiration in recumbent position: dependent portion (right and left
upper lobe, apical segment of right lower lobe)
Aspiration in upright: posterior segments of upper lobe
Primary abscess: right side
Secondary abscess: left side
Primary vs Secondary
Primary lung abscesses: occur in previously healthy patients with no
underlying medical disorders and are usually solitary
Secondary lung abscesses: occur in patients with underlying or
predisposing conditions and may be multiple

Abscess vs. Pneumatocele

Abscess: thick-walled lesion with a low-density center progressing to an
air–fluid level
Pneumatocele: thin- and smooth-walled, localized air collections with or
without air–fluid level , resolve spontaneously
PE: tachypnea, dyspnea, retractions with accessory muscle use, decreased
breath sounds, and dullness to percussion in the affected area, crackles,
prolonged expiratory phase
CX: parenchymal inflammation with a cavity containing an air–fluid level
Antibiotic choice:
Antibiotic choice should be guided by results of Gram stain and culture but
initially should include agents with aerobic and anaerobic coverage.
S.aureus: penicillinase-resistant agent
anaerobic coverage: clindamycin or ticarcillin/clavulanic acid
Gram negative: aminoglycoside
Treatment:
Uncomplicated cases: 2-3wk course of parenteral antibiotic → oral
antibiotics to complete total of 4-6wk
Gram-negative bacteria: aminoglycoside
CT-guided percutaneous drainage: hasten the recovery and shorten the
course of parenteral antibiotic therapy needed
Surgical intervention: for severely ill patients or those whose status fails to
improve after 7-10 days of appropriate antimicrobial therapy
Complicated cases: thoracotomy with surgical drainage or lobectomy or
decortication

Prognosis:
Asymptomatic within7-10 days
Fever: can persist for as long as 3 wk.
Radiologic abnormalities resolve: 1-3 mo, can persist for years
 PNEUMOTHORAX
Accumulation of extrapulmonary air within the chest
Most common cause: leakage of air from within the chest
Most frequently seen in males who are tall, thin, thought to have
subpleural blebs: spontaneous pneumothorax
Familial cases of spontaneous pneumothorax: mutation in folliculin
gene
Unusually prone to dev’t of pneumothorax: Ehlers-Danlos disease and
Marfan syndrome
Diagnosis: radiographic exam
Reported after lung transplantation and with M. pneumoniae infection
and TB: bilateral pneumothorax
Expiratory views: accentuate the contrast between lung markings and
the clear area of the pneumothorax
Primary vs. Secondary
Primary pneumothorax: occurs without trauma or underlying lung
disease
Secondary spontaneous pneumothorax: arising as a complication of an
underlying lung disorder but without trauma, pneumonia with
empyema

Hydropneumothorax: serous effusion

Pyopneumothorax: purulent effusion
Hemopneumothorax: blood

Etiology: External chest or abdominal blunt or penetrating trauma,

Ecstasy, iatrogenic, during mechanical or noninvasive ventilation
• Clinical Mx: sudden-onset, unilateral, pleuritic chest pain, dyspnea,
cyanosis

PE:
tympanic percussion over the involved hemithorax
Larynx, trachea, and heart may be shifted toward unaffected side
Respiratory distress with retractions, markedly decrease breath sounds

Evidence of tension:
Shift of mediastinal structure away from the side of air leak
Evidence of circulatory compromise
Hearing a “hiss” of rapid exit of air under tension with the insertion of
thoracostomy tube
Treatment:
Open thoracotomy: effective for recurring pneumothorax (chest tube can be
removed in 24-48hr)
Closed Thoracotomy: adequate to reexpand the lung in most patient,
minimum 72hr
VATS: preferred therapy for blebectomy, pleural stripping, pleural brushing
and instillation of sclerosing agent
Needle aspiration: emergency basis for tension pneumpthorax
CTT: emergency managemnet for primary spontaneous pneumoyhorax
CT Drainage: if pneumothorax is recurrent, secondary, or undertension or
there is >5% collapse
Resolve without treatment(within 1 wk): small <5% or moderate sized
pneumothorax in normal child
Analgesia: for pain
Chemical pleurodesis: indicated when there have been previous
pneumothorax, to induce the formation of strong adhesions between the
lung and chest wall by sclerosing procedure to prevent recurrences
 INTERSTITIAL LUNG DISEASE
A group of uncommon, heterogenous, familial, or sporadic diseases that
cause disruption of alveolar interstitium and sometimes involve airway
pathology
Injuries occur during: process of lung growth and differentiation
Initial injury causes: damage to the alveolar epithelium and capillary
endothelium
abnormal healing of injured tissues: more prominent than inflammation in
the initial steps of development of chronic ILD
frequent cause of chronic lung disease in childhood: aspiration
Late complication: hypoxia, hypercarbia
most common: tachypnea, dyspnea, cough, failure to thrive
pattern of symptoms: insidious, occur in continuous, not episodic pattern
usually the final step and is often necessary for diagnosis: lung biopsy
It can be diagnostic for disorders such as pulmonary alveolar proteinosis:
Broncheoalveolar lavage
May demonstrate hypersensitivity pneumonitis: children experiencing an
exaggerated immunologic response to organic dust, molds, or bird antigens
Predominant histologic pattern seen in SP-C mutations: chronic pneumonitis
of infancy
Classification and Pathology
Diffuse developmental disorder of the lung: due to primary aberration in lung and
or pulmonary vascular development
Growth abnormalities reflecting deficient alveolarization: secondary to impaired
prenatal or postnatal alveolarization from restriction of fetal thoracic space,
limitation of pulmonary blood supply, or chronic lung disease of prematurity (BPD)
Neuroendocrine cell hyperplasia infancy: hyperplasia of neuroendocrine cells
within the bronchioles and the lung histologic background is nearly normal
Pulmonary interstitial glycogenosis: diffuse accumulation of mesenchymal cells in
the alveolar interstitium with accumulation of monoparticulate glycogen in the
interstitial cell cytoplasm that is confirmed by ultrastructural examination
Severe surfactant dysfunctions (surfactant protein (SP-B) mutations): manifest as
respiratory failure in neonates
Diffuse ILD: can occur without known immunodeficiency or systemic disorder but it
can also be seen as a pulmonary manifestation of other systemic disease process,
such as collagen vascular disorders and sarcoidosis
Clinical Mx:
cyanosis, prominent 2nd heart sound: suggestive of severe disease
anemia, hemoptysis: pulmonary vascular disease or pulmonary
hemosiderosis
Rashes, joint complaint: consistent with an underlying connective tissue
disease
PE: tachypnea, crackles, and retractions (auscultation findings: normal)
CX: reticular pattern, honeycombing
Dx:
Noninvasive test: initially used to determine the extent and severity of the
disease
High resolution CT: better defines the extent and distribution of disease, can
provide specific information for selection of a biopsy type
Serial HRCT scan: may be of benefit in monitoring disease progression and
severity
Pulmonary function test: important in defining the degree of
pulmonary dysfunction and in following the response to treatment
Exercise testing: may detect pulmonary dysfunction
Bronchoalveolar lavage: provide helpful information regarding
secondary infection, bleeding and aspiration, and allows cytologic and
molecular analysis
Genetic testing: for surfactant dysfunction mutational analysis
Supportive care: essential and includes supplemental oxygen for
hypoxia and adequate nutrition for growth failure
Antimicrobial therapy: for infection
Prednisone: 1-2mkd for 6-8wk with tapering of dosage dictated by
clinical response
Lung transplantation: for progressive or end-stage ILD
Preventive measurements: avoidance of all inhalation irritants