Year : 2010 | Volume
: 13 | Issue : 1 | Page : 53--58
Congenital lobar emphysema: Pitfalls and management
Deepak K Tempe1, Sanjula Virmani1, Swati Javetkar1, Amit Banerjee2, Sunil Kumar Puri3, Vishnu Datt1,
1 Department of Anaesthesiology and Intensive Care, G. B. Pant Hospital, New Delhi, India
2 Department of Cardiothoracic Surgery, G. B. Pant Hospital, New Delhi, India
3 Department of Radiology, G. B. Pant Hospital, New Delhi, India
Deepak K Tempe
Department of Anaesthesiology and Intensive Care, G. B. Pant Hospital, New Delhi - 110 002
Congenital lobar emphysema is a rare entity presenting in the first month of life. It appears with varying degrees of respiratory distress, clinical and radiological evidence of over-aeration of the upper and middle lobes, mediastinal shift and hypoxia. Its early recognition and surgical intervention can be life-saving. Even today, despite advanced diagnostic techniques, pitfalls in diagnosis and management are not uncommon and the condition may be mistaken for pneumothorax or pneumonia. This report elucidates the anesthetic management of three such cases with a review of literature.
|How to cite this article:|
Tempe DK, Virmani S, Javetkar S, Banerjee A, Puri SK, Datt V. Congenital lobar emphysema: Pitfalls and management.Ann Card Anaesth 2010;13:53-58
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Tempe DK, Virmani S, Javetkar S, Banerjee A, Puri SK, Datt V. Congenital lobar emphysema: Pitfalls and management. Ann Card Anaesth [serial online] 2010 [cited 2019 Sep 23 ];13:53-58
Available from: http://www.annals.in/text.asp?2010/13/1/53/58836
Congenital lobar emphysema (CLE) is a rare entity and usually presents as acute respiratory distress during infancy. The incidence is reported to be between 1 in 70,000 to 1 in 90,000 live births.  It is characterized by post-natal over-distension of one or more lobes of a histologically normal lung, usually presenting as respiratory distress in infancy. There occurs progressive hyperinflation of the lobe/lobes of the lung with resultant compression atelectasis, mediastinum shift, impaired venous return, secondary hypoxia and hypotension.  Its early recognition and surgical intervention can be life saving. Even today, despite advanced diagnostic techniques, pitfalls in diagnosis and management are not uncommon. , 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.  This article elucidates the pitfalls in diagnosis, and the anesthetic management of three patients with CLE who underwent surgical correction for the same.
A 35-day-old, term, male child, weighing four kg, presented with acute respiratory distress and suffered cardio-respiratory arrest in the casualty. He was successfully revived and positive pressure ventilation was instituted. Based on X-Ray chest findings (hyperlucency on the left side, mediastinal shift and compression atelectasis of the lung on the right side), a diagnosis of left-sided tension pneumothorax was done and an inter-costal chest drainage (ICD) tube was inserted [Figure 1]. A repeat X-ray of the chest did not show any improvement and thoracic surgeons were consulted. Based on a suspicion of CLE, a chest computed tomography (CT) scan [Figure 2] was advised, which revealed a hyper-inflated left upper lobe, and collapse of the left lower lobe and right lung. A diagnosis of CLE of the left upper lobe was made and a left upper lobectomy was planned. However, in the intervening period, extensive bronchopneumonia developed on the left side and right lower lobe and copious purulent secretions could be aspirated from the endotracheal tube and it was decided to delay the surgery. He maintained satisfactory arterial blood gases on bi-level positive airway pressure (BIPAP)-mode (respiratory rate of 15 to 20/min with inspiratory pressure of 10 to 15 cm H 2 O and expiratory pressure of 5-7 cm H 2 O). Antibiotic therapy and physiotherapy was continued during this period. The baby could be extubated after eight days of ventilatory support on BIPAP mode. The child was then subjected to left upper lobectomy. Anesthesia was induced with two to four % halothane in 100% oxygen, on mask, using Jackson-Rees circuit and an intravenous (IV) line was secured. Tracheal intubation was accomplished in the spontaneously breathing patient with the standby surgical team to do an emergency thoracotomy, if required. In addition to routine monitoring, invasive blood pressure (IBP) and central venous pressure (CVP) were also monitored. Spontaneous ventilation, with 100% oxygen, was continued until left thoracotomy was performed when the hyper-inflated lobe popped out. Pancuronium was then administered and positive pressure ventilation was instituted. Left upper lobectomy was performed uneventfully with stable hemodynamic parameters throughout the procedure. In the post-operative period, the baby was electively ventilated on synchronized intermittent mandatory ventilation (SIMV) mode for six hours. Post-operative X-ray of chest revealed expanded right lung and left lower lobe. The child was discharged on the seventh post-operative day.
A 40-day-old male infant, weighing three kg, presented in a peripheral hospital with progressively increasing respiratory distress since birth. The mother gave a history of fatigability and diaphoresis while feeding, excessive cough following feeds, and fever for two days. Inter-costal and sub-costal recession was evident and he was kept under observation for four to five hours with supplemental oxygen using a hood. A chest X-Ray revealed a mediastinal shift to the left, opacity on the same side. A clinical diagnosis of the left lung collapse and pleural effusion was made. Antibiotic therapy was instituted and the infant was sent to a referral hospital. The infant had fever and respiratory distress worsened. On examination, the heart rate (HR) was 160 beats /min; respiratory rate (RR) was 46/min with sub-costal and inter-costal retraction and cyanosis. Trachea was shifted to the left and the air entry was restricted bilaterally, but more on the right than left. On the basis of X-ray findings, the treating physician made a diagnosis of right sided pneumothorax and inserted an ICD on the right side. Although there was some improvement in air entry, the patient showed persistent sub-costal and inter-costal retraction. An arterial blood-gas measurement showed respiratory acidosis. The child was intubated with number 4.0 uncuffed endotracheal tube and put on pressure controlled ventilation (Breath rate of 30 breaths/min, I:E ratio of 1:1, PEEP of 3 cm H 2 O, fractional inspired oxygen (FIO 2 ) 0.8 and positive inspiratory pressure of 20 cm H 2 O). The arterial blood-gas measurement revealed pH equal to 7.48, arterial oxygen tension (PaO 2 ) equal to141 mm Hg, arterial carbondioxide tension (PaCO 2 ) equal to 32.8 mm Hg and arterial oxygen saturation of 98%). The intubated child [Figure 3] was referred to our institute for further management. A CT scan of the chest [Figure 4] revealed right sided congenital lobar emphysema. A right pneumonectomy was planned and performed the next day. As the child was already intubated, anesthesia was induced and maintained with morphine 0.5 mg, pancuronium 0.5 mg, oxygen (FIO 2 :1) and isoflurane and gentle manual intermittent positive pressure ventilation (IPPV). In addition to routine monitoring IBP and CVP were also monitored. The peak airway pressure was maintained at 20 to 25 cm H 2 O by gentle manual ventilation until thoracotomy. Nitrous oxide was started after pneumonectomy. Intra-operatively the vital parameters were stable. Post-operatively, the child was electively ventilated for six hours and discharged on the eighth post-operative day.
A male child, two months and eight days old,, weighing six kg, was admitted in a peripheral hospital with a progressively increasing difficulty in breathing, irritability, decreased feeding and fever for four days. On examination, the patient had fever and the respiratory rate was 64/min with inter-costal retraction. A chest X-Ray revealed hyperinflated left lung and mediastinal shift to the right with bronchovascular markings barely visible in the peripheral two-third of the left hemithorax [Figure 5]. A provisional diagnosis of left pneumothorax was made and the child was treated with IV antibiotics, intermittent nebulization with asthalin, oxygen via hood and an ICD tube was inserted. Minimal amount of air was drained, but there was no improvement in the clinical condition of the patient. The patient developed respiratory distress and cyanosis. The treating physician reviewed the X-ray and the diagnosis was revised to CLE and. The ICD tube was removed and the patient was referred to our hospital. On examination, the child was conscious, irritable with a respiratory rate of 60/ min, nasal flaring, inter-costal and sub-costal retraction. Air entry was markedly decreased in the left hemithorax with crepitations in the bilateral axillary region and expiratory wheeze. There was subcutaneous emphysema on the left side near the site of insertion of the chest tube. A chest CT scan [Figure 6] revealed a large air-filled cystic space with multiple thin internal septae in the left upper lobe causing contra-lateral mediastinal shift and compression atelectasis. A diagnosis of CLE/ cystic adenomatoid malformation Type1 was made and the child was scheduled for left upper lobectomy. Anesthesia was induced with 100% oxygen and two per cent halothane in the spontaneously breathing child and an intravenous line was secured. Thereafter, fentanyl 7΅g, thiopentone 5 mg and succinylcholine 5 mg were administered. After gentle manual ventilation the trachea was intubated with a number 3 cuffed endotracheal tube. In addition to routine monitoring IBP and CVP were also monitored. Gentle manual ventilation was continued till thoracotomy using Jackson-Rees circuit. After induction, the blood pressure decreased from a baseline 100 mmHg systolic to 80 mm Hg systolic and recovered with volume replacement. Following thoracotomy, anesthesia was maintained with fentanyl (15 ΅g), pancuronium (1.5 mg) and halothane with FIO 2 of 1. After left upper lobectomy, the child was electively ventilated on volume control mode and extubated the next day.
All the children were males; two had CLE involving the left upper lobe while in one the right middle lobe was involved. It has been reported that the disease is more common in male children, usually unilateral, affecting more often the left upper lobe (43%) followed by right middle lobe (32%), although bilateral involvement is not unknown. ,
In 50% of cases the exact etiology of the disease is not known, but in 25%, bronchial cartilage dysplasia has been postulated. , The most recent theory proposes an increased number of alveoli within each acinus (polyalveolar lobe). 
Infants with CLE present a diagnostic challenge. There is over-inflation and air trapping in the affected lobe and the surrounding lung tissue gets compressed, mediastinum gets displaced, often herniating to the opposite hemithorax. Over-inflation and air trapping in the affected lobe/lobes can lead to disruption of anatomy, loss of elasticity, impairment in lung function and at times "tension emphysema" by compression of the opposite lung. Compression due to emphysema leads to decreased amount of functional lung tissue and an increase in the intra-thoracic pressure, which further decreases the respiratory reserve. Compression atelectasis on the ipsilateral or contralateral side and mediastinal shift produces ventilation /perfusion (V/Q) mismatch with resulting hypoxia.  The condition is most commonly confused with pneumothorax leading to ICD tube insertion. ICD insertion does not help; rather it may further increase the respiratory distress.  Mandelbaum et al.  in their series of eight patients of lobar obstructive emphysema have reported a patient similar to our first one in whom cardiac arrest occurred. The child was revived with external cardiac compression, IPPV through endotracheal tube and decompression of the cystic lobe by tube thoracostomy followed by lobectomy the next day. In the present series, all the three patients were misdiagnosed as tension pneumothorax and ICD tube was inserted as a part of initial treatment. One of them suffered cardiac arrest before the diagnosis of CLE was made. Thus, CLE should be suspected whenever there is respiratory distress, cyanosis, wheezing, asymmetrical breath sounds, displaced cardiac sounds and tympanitic chest percussion, all occurring within a few days of birth.
The correct diagnosis depends on a high level of suspicion. Chest radiography is helpful, but not definitive. , The characteristic appearance is that of pressure expansion of a lobe of lung causing compression of adjacent lobes. A careful examination of the X-ray film by transillumination and observing the bronchovascular markings in the hyperinflated lobe can help to differentiate CLE from pneumothorax.  Lung cysts have a clearly demarcated edge best seen in lateral films, but may not always be distinguishable from lobar emphysema.  A single photon emission tomography V/Q lung scan is confirmatory and can differentiate lobar emphysema from compensatory emphysema by demonstrating impaired ventilation-perfusion ratio in the former as well as rule out associated anomalies. Other diagnostic modalities include CT scan, fluoroscopy, bronchoscopy, angiography, radio-isotopic evaluation, lung scintography and even thoracotomy. ,,
The key anesthetic concern in such patients is to avoid over-distention of the emphysematous lobe before the chest is opened. Over-distention of the lobe/lung is the root cause of respiratory and hemodynamic disturbances in these patients. Since IPPV can lead to over-distention of the lung, it is best to avoid it.  The critical airway pressure in such patients is not known. However, gentle manual ventilation maintaining the airway pressure at 20-25 cm H 2 O before thoracotomy, keeping an eye on the vital signs has been the method of induction used by Cote et al and Payne et al. in 1984. , Anesthesia ventilators with the facility of pressure controlled ventilation pressure regulated volume controlled (PRVC) can be very useful, but were not used due to unavailability. In addition, it is useful to avoid N 2 O as it can diffuse faster in a closed cavity leading to further compression and mediastinal shift.  In the present series (case 2) the child was already intubated and was successfully anesthetized and maintained with morphine and pancuronium bromide and gentle manual ventilation, while in case 3 succinylcholine was electively used for achieving tracheal intubation followed by gentle manual ventilation till thoracotomy because of the author's own experience with IPPV in the previous cases as well as the evidence in literature. , In addition, a surgical standby is desirable as a quick thoracotomy allows space for the over distended lung and reduces the pressure effect. One other technique that has been used effectively is selective endobronchial intubation of the healthy lung till the lobectomy/ pneumonectomy is performed, after which the endotracheal tube is withdrawn in the mainstem bronchus. ,, There are few other novel techniques described to manage such patients. Goto et al.  have described the use of high frequency jet ventilation. The dual challenge of safe induction and analgesia for thoracotomy was managed by placing thoracic epidural catheter via the caudal insertion site and retaining spontaneous ventilation until thoracotomy by Raghavendra et al.  Phillipos and colleagues  have managed a three kg neonate in severe respiratory distress who had CLE of the left upper lobe. A flexible ultra-thin bronchoscope was passed into the left upper lobe bronchus and this resulted in emergency relief of the initial respiratory distress. The left upper lobe lobectomy was performed three days later. In 1977 Campbell et al.  medically managed an infant with CLE for 23 weeks on continuous positive airway pressure (CPAP) to prevent episodes of respiratory failure.
A child with CLE may require ventilatory management before surgical correction can be undertaken either due to presence of infection or when the child presents with respiratory failure. The management of such a patient is controversial. Joosten et al. have suggested that one should wait till pneumonitis is resolved with selective endobronchial intubation and spontaneous deflation of the hyper-expanded lung, thereby improving the pulmonary mechanics, cardiovascular function and resorption of interstitial air.  On the other hand, waiting for the pneumonia to resolve may increase chances of compression atelectasis. This is due to residual pulmonary abnormalities such as pneumatoceles that can lead to localized areas of bronchial and alveolar necrosis. In ventilated infants with underlying bronchopulmonary dysplasia, these intra-pulmonary air pockets can collect large quantities of air that may evolve into either a localized lung hyperinflation or diffuse interstitial emphysema. 
Although gentle manual ventilation has been used in these children, how much positive airway pressure can be safely tolerated has not been addressed. In CLE the volume of abnormal lobe is determined by four factors:
Its pressure volume characteristic.The rate at which it empties and fills.The end-inspiratory transpulmonary pressure.The respiratory rate of the patient.  In the first case of the present report, the child was intubated and maintained for eight days on BIPAP mode with an inspiratory pressure of 10-15 cm of water, while in case 2 and 3 muscle relaxant and gentle manual ventilation was utilised, following induction, until thoracotomy. It seems, therefore, that assisted ventilation with airway pressure less than 20cm of water can be safely practiced in such patients. However, the risk of producing over-distension of the lung leading to cardiorespiratory complications should always be a consideration and a standby surgical team to perform an emergency thoracotomy should be present. In addition, cardiovascular problems such as decrease in cardiac output may occur, partly due to dehydration or decreased venous return (due to increase in intra-thoracic pressure) and mediastinal shift. Severe hypotension due to anesthesia related myocardial depression and/or vasodilatation may be refractory to volume replacement. Thus special attention should be paid to fluid balance during the pre-operative period.
An infant presenting in emergency with progressively increasing respiratory distress should be viewed with a suspicion for CLE. Anesthetic induction with inhalational agents is desirable; however, if IPPV is necessary, gentle ventilation or pressure controlled ventilation with a pressure limit of 20 cm of H 2 O should be carried out until thoracotomy. A standby surgical team should be available during induction of anesthesia and nitrous oxide should be avoided till lobectomy is performed.
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