CASE REPORT
A CASE OF CONGENITAL LOBAR EMPHYSEMA: CHALLENGES IN
DIAGNOSIS AND IN ANAESTHETIC MANAGEMENT
Swati Bisht1, Rashmi Dubey2, Bharath Narasimha3
HOW TO CITE THIS ARTICLE:
Swati Bisht, Rashmi Dubey, Bharath Narasimha. ”A Case of Congenital Lobar Emphysema: Challenges In
Diagnosis and in Anaesthetic Management”. Journal of Evidence based Medicine and Healthcare; Volume 2,
Issue 3, January 19, 2015; Page: 296-299.
ABSTRACT: Congenital lobar emphysema (CLE) is a rare clinical entity presenting as acute
respiratory distress in infants. Its diagnosis is difficult as clinically and radio logically it mimics
pneumonia or pneumothorax. Its early recognition and management with thoracotomy is
lifesaving. Inhalation induction is preferred and spontaneous ventilation should be maintained
until either the chest is opened or one lung ventilation of the contralateral lung is achieved. In
this case report, we describe the challenges faced in the anaesthetic management of this
condition.
KEYWORDS: Congenital lobar emphysema, hyperinflation, thoracotomy, endobronchial
intubation, positive pressure ventilation.
KEYMESSAGES: In a neonate presenting with respiratory distress, the possibility of congenital
lobar emphysema (CLE), a rare clinical entity should be kept in mind. Thoracotomy and complete
lobectomy are lifesaving in CLE. Inhalation induction with endobronchial intubation is the
preferred mode of anaesthetic management.
INTRODUCTION: Congenital lobar emphysema (CLE) refers to an idiopathic postnatal abnormal
over distension of anatomically normal lobe of lung characterized by expiratory air trapping within
the lobe producing compression and atelectasis of adjacent lobe.1-2 There occurs progressive
hyperinflation of the lobe/lobes with resultant compression atelectasis, mediastinal shift, impaired
venous return, secondary hypoxia and hypoventilation.3 It is a rare congenital malformation with
a prevalence of 1 in 20, 000 to 1 in 30, 000.4 Male babies are affected more than female babies
in the ratio 3:2.5 Its early recognition and surgical management can be lifesaving. Even today,
despite advanced diagnostic techniques, pitfalls in diagnosis and management are not
uncommon.3,6 The initial x-ray chest may not show typical appearance and may present with a
region of increased density rather than hyperlucency which can be mistaken for pneumonia.7 We
report a case of 3 month old female child who presented with respiratory distress and with a
radiological picture of pneumothorax. Eventually the diagnosis of congenital lobar emphysema
was made and emergency thoracotomy was done. Endobronchial intubation until the resection of
affected lobe and post-operative elective ventilation facilitated by infusion dexmedetomidine was
our line of action.
Case History: A 3-month old female child weighing 4.5 kg presented with cough, fever and
respiratory distress. On examination, the baby was tachypoenic, had flaring of alae nasi and
subcostal retraction. The heart rate was 155/ mt, respiratory rate was 60/ mt. On auscultation,
breath sounds were decreased on the left hemithorax. No gross cardiac anomaly was found.
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CASE REPORT
Oxygen saturation was 85 % with oxygen hood, but there was no visible cyanosis. An x-ray chest
revealed hyper inflated left lung with mediastinal shift to the right side. (Figure 1- Initial x-ray)
Fluid resuscitation was commenced with 5% Dextrose in normal saline at the rate of 10 ml/ kg/
hour.
A provisional diagnosis of left pneumothorax was made and the child put on IV antibiotics,
intermittent asthalin nebulization, oxygen via hood and intercostal chest drainage was inserted.
Minimal air was drained, but the distress continued. Senior consultant reviewed the x ray and the
diagnosis of CLE was considered. (Figure 2- x-ray after ICD insertion)
A CT thorax was done immediately. It showed a hyper inflated left upper lobe, and
collapse of left lower lobe and right lung, confirming CLE.
Routine hematological studies, biochemical parameters were normal. The child was posted for
emergency thoracotomy; left upper lobectomy. The child was posted under ASA III E. In OT,
ECG, oxygen saturation, temperature monitor, NIBP monitors were connected. Standard
precautions to avoid hypothermia were undertaken. A surgeon was scrubbed for an emergency
thoracostomy if required. The child was premedicated with Inj atropine 0.03 mg IV, Inj. fentanyl
10 mcg IV. Induction was done by a face mask with 100% oxygen and sevoflurane 4-6 % with
Jackson Rees circuit. After adequate depth, 4 mm endotracheal tube was inserted
endobronchially into the right lung and confirmed by auscultation. Gentle assisted ventilation was
done with hand. Baby was placed in the right lateral position and anaesthesia was maintained
with sevoflurane 2-3 % in 100% oxygen. Peak airway pressure was maintained at 20- 25 cm
H2O. Following thoracotomy, Inj. Atracurium 2.5 mg IV was administered. Left upper lobectomy
was performed with stable haemodynamic vitals. Then bilateral lung ventilation using N2O was
commenced after pulling out the endotracheal tube into the trachea.
This was confirmed by seeing the expansion of the left lower lobe. Blood and fluid loss
was monitored and replaced with 120 ml warmed ringer lactate and 20 ml fresh whole blood. At
the end of surgery intercostal block was given by the surgeon under direct vision using 3 ml of
0.25 % of bupivacaine. The child was electively ventilated on volume control mode in the PICU
for 12 hours. Intravenous dexmedetomidine infusion was started at 2.5 mcg/ hour and gradually
tapered over. The baby was successfully weaned off and extubated uneventfully. Postoperative
x-ray chest showed expansion of both the lungs with no mediastinal shift.
Fig. 1
Fig. 2
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DISCUSSION: CLE usually presents in full term infants between the newborn period and six
month of age. In 50 % cases, the exact etiology is unknown; but in 25% bronchial cartilage
dysplasia is postulated.6,7,8
Infants with congenital lobar emphysema present a diagnostic challenge. The clinical
picture in majority is chest retraction, tachypnea, tachycardia. If hypoxemia ensues, the infant
may become agitated, anxious and cyanosed. Physical examination shows asymmetric chest
expansion, focal hyperresonance and diminished breath sounds over the affected lobe. Chest
x-ray typically shows hyperinflation of the involved lobe, atelectasis of adjacent lung, mediastinal
shift and flattening of ipsilateral diaphragm.2 This picture is often confused with pneumothorax
leading to intercostals chest drain tube insertion. ICD insertion does not help; rather it aggravates
the respiratory distress.8 The correct diagnosis depends on a high level of suspicion. Chest
radiography is helpful; but not definitive.9,11 The typical appearance is that of pressure expansion
of a lobe of lung causing compression of adjacent lobe. A careful examination of the x-ray film by
trans illumination and observing the broncho vascular markings in the hyper inflated lobe can
help to differentiate congenital lobar emphysema for pneumothorax.9 CT scan, bronchoscopy and
angiography are also helpful in diagnosis.11, 12
Thoracotomy and complete lobectomy are lifesaving in CLE.12 Inhalation induction is
preferred and spontaneous ventilation should be maintained until either the chest is opened or
one lung ventilation of the contralateral lung is achieved.3 Positive pressure ventilation due to a
ball valve effect may cause over distension of emphysematous lobe, worsening of mediastinal
shift, reduction of cardiac output and hemodynamic collapse. In case of poor respiratory
response, if hypoventilation occurs during induction, gentle assisted manual ventilation may be
required. Airway pressure should be maintained at 20-25 cm H2O.3,13 Once chest is opened and
affected lobe is delivered, the patient can be paralyzed and controlled ventilation can be
initiated.14 Use of N2O before lobectomy should be avoided to prevent hyperinflation of
emphysematous lobe.15 If over inflation of the emphysematous lobe occurs during gentle manual
ventilation, then emergency thoracostomy and allowing the affected lobe to herniate through the
thoracostomy incision will help.
One lung ventilation in infants is challenging for anaesthesiologist. Positioning of an
endobronchial tube is difficult; also proper size is not available. Reports of endobronchial
intubation using single lumen endotracheal tube are described.11 The correct endobronchial
placement should be confirmed by fibreoptic bronchoscopy.
REFERENCES:
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Pathologic Basis of Disease, 7 th edition. Philadelphia, Pa; Saunders Elsevier, 2005, Chapter
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2. Tempe DK, Virmani S, Javetkar S et al. Congenital lobar emphysema: pitfalls and
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3. Cote CJ. The anesthetic management of congenital lobar emphysema. Anesthesiology 1978;
49:296-8.
4. Thakral CL, Maji DC, Sajwani MJ. Congenital lobar emphysema: Experience with 21 cases.
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[PubMed].
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case report. S Afr Med J 1984; 66: 421-2.
14. Hatch DJ, Summer E. Anesthesia – Specific conditions. In: Feldman SA, Scurr CF, editors.
Current Topics in Anesthesia No 5, Neonatal Anesthesia. 1st ed. London: Edward Arnold
Publishers Pvt Ltd; 1981. p.112.
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AUTHORS:
1. Swati Bisht
2. Rashmi Dubey
3. Bharath Narasimha
PARTICULARS OF CONTRIBUTORS:
1. Associate Professor, Department of
Anaesthesiology, Vydehi Institute of
Medical Sciences and Research Centre,
Bangalore.
2. Associate Professor, Department of
Anaesthesiology, Vydehi Institute of
Medical Sciences and Research Centre,
Bangalore.
3. Senior Resident, Department of
Anaesthesiology, Vydehi Institute of
Medical Sciences and Research Centre,
Bangalore.
NAME ADDRESS EMAIL ID OF THE
CORRESPONDING AUTHOR:
Dr. Swati Bisht,
SG 304, Shriram Spandhana Appts,
Challaghatta,
Banglaore-560037.
E-mail: swati_bisht@hotmail.com
Date
Date
Date
Date
of
of
of
of
Submission: 08/01/2015.
Peer Review: 09/01/2015.
Acceptance: 10/01/2015.
Publishing: 19/01/2015.
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