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Detailed information of CONGENITAL LOBAR EMPHYSEMA
CONGENITAL LOBAR EMPHYSEMA
DEFINITION:
A congenital anomaly of the lung characterized by lobar
overaeration and subsequent respiratory distress.
EPIDEMIOLOGY:
- incidence: ?
- age of onset:
- risk factors:
PATHOGENESIS:
- Causative factors (which are often not found) are those
which cause a reduction in the calibre of the concerned lobar
bronchus resulting in a functional bronchial collapse and a
valvular arrangement producing a large, overdistended,
non-collapsible lobe and widespread alveolar emphysema and
rupture. Factors causing a bronchial obstruction may be
intrinsic or extrinsic.
- infection - TB, echovirus, retained secretions
- bronchial - adenoma, atresia, bronchomalacia, hypoplasia,
stenosis, absence of cartilage
2. Extrinsic Factors
- anterior mediastinal herniation
- pulmonary artery dilation
- vascular or lymph node compression
- Note: If you see CLE, try to determine what is the source
of obstruction of the bronchus -> intrinsic or extrinsic
CLINICAL FEATURES:
- respiratory distress (within the first 6 months)
- chronic cough, wheeze, dyspnea, stridor, intermittent
cyanosis exacerbated with feeds
- not associated with recurrent infections
2. Complications
- congestive heart disease
- tension emphysema/pneumothorax
INVESTIGATIONS:
- consistent with a check-valve mechanism
- hyperinflated lobe with vague lung markings
- usually unilobar: LUL > RML > RUL but can be segmental,
bilobar, or bilateral
- atelectasis of ipilateral lobes and contralateral lung
- ipsilateral diaphragmatic compression
- mediastinal shift towards contralateral lung field
- widened rib interspaces
2. V/Q Scan
- decreased V and Q in emphysematous lobe
3. Bronchogram
- incomplete distal filling of the affected bronchi
4. Angiogram
- poor and slow arterial filling
MANAGEMENT:
- respiratory distress - ventilation, thoracentesis
2. Surgery
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Pediatric Database - CONGENITAL LOBAR EMPHYSEMA
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