5. • CLE is characterized by respiratory distress due to overexpansion of
one or more pulmonary lobes of the histologically normal lung
without the destruction of alveolar walls with compression of
surrounding lung parenchyma; that is why it is also called congenital
lung overinflation
(CLE ditandai dengan gangguan pernapasan akibat ekspansi berlebihan
dari satu atau lebih lobus paru paru normal secara histologis tanpa
kerusakan dinding alveolar dengan kompresi parenkim paru di
sekitarnya; itu sebabnya disebut juga overinflasi paru bawaan)
6. • There is air trapping in the lung during the expiratory phase of respiration
due to deficient bronchial cartilage, which causes repeated episodes of
respiratory distress. The affected lobe is essentially non-functional because
of overdistention and air trapping. It is frequently recognized in newborns;
however, a few cases do not get evident until adulthood. This disease is
potentially reversible if diagnosed and treated on time
(Ada udara yang terperangkap di paru-paru selama fase ekspirasi karena
kekurangan kartilago bronkial, yang menyebabkan episode berulang dari
gangguan pernapasan. Lobus yang terkena pada dasarnya tidak berfungsi
karena overdistensi dan perangkap udara. Ini sering dikenali pada bayi baru
lahir; namun, beberapa kasus tidak terbukti sampai dewasa. Penyakit ini
berpotensi reversibel jika didiagnosis dan diobati tepat waktu)
7. • The incidence of CLE among live births is 1 out of 20,000-30,000. The
occurrence is more in males than females with a ratio of 3 to 1. CLE is
more common in infants and is extremely rare among adults. Thirty-
three percent of the cases are symptomatic at birth. Due to the
severity of symptoms, most are diagnosed within the first six months
of birth.
• The left upper lobe is most commonly involved (43%), followed by
the right middle lobe (32%) and right upper lobe (21%) involvement.
The involvement of the lower lobe is as rare as only 2% of the cases.
8. • The patient usually presents with difficulty in feeding and breathing,
wheezing, retractions, and cyanosis. There is typically a history of
chronic cough and recurrent respiratory infections. Due to
overlapping symptoms, some patients are wrongly diagnosed with
pneumonia or pneumothorax instead of congenital lobar
emphysema.[3] Lobes affected with CLE are overinflated; thus, there
is impaired ventilation and perfusion. Due to progressive
overinflation, there is compression of adjacent organs leading to
further impairment of ventilation/perfusion and, ultimately,
respiratory failure.
9. Plain radiograph
Immediate postpartum period
• The affected lobe tends to appear opaque and homogeneous because of
fetal lung fluid or it may show a diffuse reticular pattern that represents
distended lymphatic channels filled with fetal lung fluid
Later findings
• appears as an area of hyperlucency in the lung with oligemia (i.e. paucity of
vessels)
• mass effect with mediastinal shift and hemidiaphragmatic depression
• lateral decubitus film with the patient lying on the affected side will show
little or no change in lung volume
• lateral film may show posterior displacement of the heart
10. CT
• CT is usually performed to confirm the diagnosis, evaluate the
mediastinal vascular structures, and to rule out other
abnormalities.
• shows above features in greater detail
• attenuation of vascular structures in affected lobe 4
• may also show compressive atelectasis of adjacent lobes
11. Differential Diagnosis
• Congenital cystic adenomatoid malformation. Congenital lobar emphysema and congenital
cystic adenomatoid malformation type III have similar findings on ultrasound, which shows a
highly echogenic mass. Congenital lobar emphysema is devoid of cystic lesions (tidak punya
lesi kistik) as compared with cystic adenomatoid malformation.[13]
• Pneumonia. Patients with pneumonia present with fever, chest retractions, and cyanosis. The
affected lung on chest x-ray may present with collapse consolidation, and it usually improves
with antibiotics, unlike congenital lobar emphysema, the affected lung in pneumonia is not
hyperinflated.[11]
• Tension Pneumothorax. Like congenital lobar emphysema, patients with tension
pneumothorax also presents with respiratory distress, the physical examination will reveal
hyperresonance and decreased breath sounds on the affected lung, and the chest x-ray will
show that the mediastinum deviates to the contralateral lung. Tension pneumothorax is a
medical emergency, and patients have hypotension, and it needs prompt decompression.
The chest x-ray of the affected lung in tension pneumothorax shows depression of the
hemidiaphragm, which is not seen in congenital lobar emphysema.[14]
• Congenital diaphragmatic hernia. Congenital lobar emphysema and congenital
diaphragmatic hernia present with hyperlucent lung on chest x-ray. It can be differentiated
with congenital lobar emphysema because of the gas-filled loops of bowel in the chest.[15]
