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

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 8\10.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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