This document reports on the case of a 26-day-old female neonate presenting with progressive respiratory distress who was diagnosed with congenital lobar emphysema (CLE). CLE is a rare developmental abnormality where one or more lung lobes are hyperinflated. The diagnosis of CLE in this patient was challenging as symptoms can be confused with other conditions. Chest X-ray and CT scan confirmed left middle lobe hyperinflation. Left middle lobectomy resolved the respiratory distress. CLE requires a high index of suspicion for diagnosis as early surgical treatment has an excellent prognosis.

1. Sreeram Reddy. / International Journal Of Advances In Case Reports, 2015;2(5):XX-XX.
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e - ISSN - 2349 - 8005
INTERNATIONAL JOURNAL OF ADVANCES IN
CASE REPORTS
Journal homepage: www.mcmed.us/journal/ijacr
CONGENITAL LOBAR EMPHYSEMA: A DIAGNOSTIC
CHALLENGE AND CAUSE OF PROGRESSIVE RESPIRATORY
DISTRESS IN A 26 DAY -OLD NEONATE
Sreeram Reddy
Professor & HOD, Department of Pediatrics, Prathima Institute of Medical Sciences, Nagnur, Karimnagar, Telangana
State.
Article Info
Received 15/02/2015
Revised 27/03/2015
Accepted 2/04/2015
Key words:
Congenital lobar
emphysema (CLE),
pneumothorax,
lobectomy.
ABSTRACT
Congenital lobar emphysema (CLE) is a developmental abnormality of lower repiratory tract with an
overall prevalance of 1 in 20,000 to 30,000. Males appear to be affected more than females, in a ratio
of 3:1. Relatively uncommon but an important cause of progressive respiratory distress.Mostly
presents during neonatal period.Here we report the case of a 26 days old female neonate presented
with progressive respiratory distress. Similar symptoms may occur in bronchopneumonia and
cyanotic congenital heart diseases. Chest x-ray showed lobar hyperinflation, mediastinal shift and
flattening of left hemidiaphragm. It can be confused with tension pneumothorax. Early suspicion of
CLE will prevent unnecessary insertion of intercostal drainage tube. CT scan confirmed the
diagnosis. Here we explore how the correct diagnosis of CLE had to be reached, highlighting key
clinical signs and investigations. Left middle lobectomy was performed with complete resolution of
his respiratory distress. Current literature concerning CLE is reviewed. Key Messages : Diagnosis of
CLE is a challenge, most often it is confused with tension pneumothorax .Similar symptoms may
occur in bronchopneumonia, cyanotic congenital heart diseases, and several congenital
abnormalities of the lung. There is every chance of placing an intercostals drain which can result in
devastating consequences Although rare clinical suspicion is must in every case with these clinical
and radiological features. Early diagnosis conservative management can be done but possibility of
recurrent infections and surgical correction has excellent prognosis.
INTRODUCTION
Congenital lobar emphysema (CLE) is a
developmental abnormality of lower repiratory tract with
an overall prevalance of 1 in 20,000 to 30,0000. Diagnosis
of CLE is a challenge. Most often it is confused with
tension pneumothorax the inexperienced may mistake it for
pneumothorax and put a chesttube into an emphysematous
lobe with disastrous result [1]
.Although rare ,clinical
suspicion is must in every case with these clinical and
radiological features. Early diagnosis and surgical
correction has excellent prognosis.
Case History
A 26 days old full term female neonate delivered
to primi by caesarean section from a + non
consanguineous marriage with an uneventful prenatal and
perinatal history and birth weight of 2.9 kg presented
with a history progressive respiratory difficulty. At 18 days
of age baby was admitted at outside Hospital and treated as
Bronchopneumonia with IV antibiotics. As the baby did
not Improve, was brought to our hospital. At admission,
the infant was cyanosed with a SaO2 of 72% on O2 with
nasal prongs.
Corresponding Author:- Sreeram Reddy
E-mail: jaganomc2k6@gmail.com

2. Sreeram Reddy. / International Journal Of Advances In Case Reports, 2015;2(5):XX-XX.
95
On examination baby was tachypnoeic with
severe chest indrawing and a respiratory rate of 80/min on
auscultation there was minimal air entry in left lung.
Downes score was 810.On physical examination chest was
asymmetric, had minimal expansion with respiration, the
trachea was shifted to the right, and had a hyperesonant
percussion note over the left anterior thoracic region. She
had no gross dysmorphic features, no clubbing, diaphoresis
or dependent oedema The radial pulse was regular, low
volume with a heart rate of 180/min though the intensity of
the heart sounds was not altered. Both the liver and spleen
were displaced 3 cm below the costal margin. As Downes
score was 8 baby was intubated on emergency basis with a
3.5mm endotracheal tube and connected to mechanical
ventilator. Baby was investigated with chest x-ray (CXR)
it revealed lobar hyperinflation, mediastinal shift and
flattening of left hemidiaphragm(figure 1). With this CXR
findings we suspected as congenital lobar emphysema and
CT scan was taken, it confirmed hyperinflated middlelobe
on the left with collapse of the adjacent leftlobes and
herniation of the left upper lobe (LUL) with mediastinal
shift, the inflated lobe herniated into the right side of the
chest and the grossly enlarged left upper lobe displaced the
mediastinum suggestive of CLE (figure 2). Other
investigations included CBC which showed hemoglobin
12.7g/dl total WBC count of 8300/mm3
with a differential
count of lymphocytes 32%, granulocytes 60% and
monocytes 8 %. The and the platelet count was 280,000
mm3
. Arterial blood gas showed evidence of respiratory
acidosis with a pH 7.26, pCO2 9.41 and base excess +.6.
and C-reactive protein (CRP) <1. As condition did not
improved with medical management, baby was shifted to
operation theatre and an emergency operation was
performed. Surgery was done using a left postero-lateral
thoracic incision. The pleura and vascular anatomy were
found to be normal.A left upper lobectomy was done . The
SaO2increased to 95% the same evening and no
complications developed. The respiratory distress resolved
Baby was discharged after a week. She remained
asymptomatic with a normal respiratory examination at 6
week follow-up.
Figure 1. XXXXXXXXXXXXXXXXXXXXXXXX Figure 2. XXXXXXXXXXXXXXXXXXXXXXXXXXX
Discussion
Congenital lobar emphysema (CLE) is a
developmental anomaly of the lower respiratory tract that
is characterized by hyperinflation of one or more of the
pulmonary lobes [2,3]. Males appear to be affected more
than females, in a ratio of 3:1 [2]. It mainly affects the
upper and middle lobes with the left lower lobe [LUL]
being the most often affected: LUL 40–50%; Right middle
lobe[RML] 25–30%; right upper lobe 20%; right lower
lobe 2–10%.9 [4,5,6] CLE is a rare congenital
malformation with a prevalence of 1 in 20,000 to 1 in
30,000 [2,4]. Failure to make a diagnosis of congenital
lobar emphysema at peripheral units as in this child or a
true reflection of the prevalence of the disease. Several
factors have been associated with the development of
congenital lobar emphysema. In 50% of cases there is
decreased bronchial cartilage tissue. This defect produces a
ball valve effect with consequent overinflation
[5,6]. Vascular abnormalities that produce compression
[7], bronchial stenosis. Concurrent congenital heart disease
(usually patent ductus arteriosus, or ventricular septal
defect, occurs in 15% of cases. [7], bronchogenic cysts [8],
and congenital cytomegaloviral infection have also been
associated [9]. Similar symptoms may occur in
bronchopneumonia, cyanotic congenital heart diseases, and
several congenital abnormalities of the lung. [6,10,11]
Congenital lobar emphysema has been described in twins
[12] but in up to 40% of cases, the cause is unclear. As in
this child, respiratory distress is the commonest mode of

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96
presentation [13]. There is dyspnoea, wheezing, grunting
respiration, tachypnoea and sometimes progressive
cyanosis. The basic investigation in congenital lobar
emphysema is the chest radiograph from which a diagnosis
can be made and is readily available. In an Indian series, a
correct diagnosis on chest radiography before referral was
made in only 4 out of 10 cases even though the diagnosis
could be arrived at in the 10 cases using the initial
radiographs [14]. The inexperienced may mistake it for
pneumothorax and put a chest tube into an emphysematous
lobe with disastrous result. In pneumothorax entire
ipsilateral lung is collapsed into the hilus [1]. In CLE the
pulmonary vessels extend to the periphery of the hyper-
inflated lobe and there is no visualization of a pleural line
unlike in a pneumothorax. Decubitus and cross lateral
radiographs may aid in distinguishing between CLE and a
pneumothorax. Controversy exists regarding surgical and
conservative management of this malformation.
There is no contentious opinion. One opinion is in
favor of conservative management for mild cases but
stringent follow up is necessary [15]. Antenatal diagnosis
can be done with Ultrasonography [16]. Operative surgery
with lobectomy is the commonest mode of treatment [3].
Depending on the symptoms, conservative measures are
sometimes taken but this may fail in the presence of inter-
current infections. Resection of vascular rings may be the
only surgical procedure where this is the cause [14]. In
over 85% of cases, the long term outcome after surgery is
excellent with complete cure [17].
Our patient improved immediately after surgery
with normalization of the SaO2 within 12 hours. In
summary, the diagnosis of CLE may present a diagnostic
challenge and a high index of suspicion in neonates with
progressive respiratory distress is important if the
diagnosis is to be made promptly. The outcome of surgery
is good in most cases.
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