ANAESTHETIC MANAGEMENT OF A CASE OF CONGENITAL LOBAR EMPHYSEMA

1. Dr. V.Jayaraman, Professor, Department of Anaesthesiology, Saveetha Medical College, Chennai, Tamilnadu, India.

2. Dr.VR.Kalarani, 3rd year MD(Anaesthesia) PG, Department of Anaesthesiology, Saveetha Medical College, Chennai, Tamilnadu, India.

3. Dr.P.Manikandan, Senior Resident (NICU), Department of Pediatrics, Saveetha Medical College, Chennai, Tamilnadu, India.

ABSTRACT:

Congenital lobar emphysema (CLE) or congenital lobar over inflation is a

rare pulmonary anomaly that causes respiratory distress in neonates. CLE

presents significant challenges in diagnosis and management. We reported a

case of two-month old infant with respiratory distress and pneumonia related to

CLE affecting the left upper lobe. Lobectomy was performed under general

anaesthesia with spontaneous and controlled lung Ventilation. The case was

challenging, as it involved careful and planned anaesthetic management of lung

separation as well as prevention of hyperventilation of the un-involved lung.

KEYWORDS: Congenital Lobar Emphysema, Pneumonia, Respiratory

Distress, Lobectomy, Neonatal Intensive care Unit.

INTRODUCTION

Congenital lobar emphysema (CLE) is a rare congenital malformation of the

lung characterized by normal architecture with lobar over aeration and

subsequent respiratory distress secondary to partial obstruction of the bronchus

via the ball valve effect. It is also known as congenital lobar over inflation or

infantile lobar emphysema.1,2 The incidence is reported to be between 1 in

70,000 and 1 in 90,000 live births with male predominance.3 It presents as life

threatening respiratory distress especially within the first 6 months of life due to

compression atelectasis, hypoxia, mediastinal shift and associated

hypotension.4,5 Aetiology is unknown up to 50% cases, but several intrinsic and

extrinsic causes have been described. Chest radiograph shows mediastinal shift

and hyperinflation that is often confused with pneumonia and pneumothorax.6

Positive pressure ventilation or assist ventilation can worsen hyperinflation and

hemodynamic collapse can result. We discuss the anaesthetic management of a

2 months old infant scheduled for left lobectomy with emphasis on prompt

diagnosis and use of positive pressure ventilation in management of a case of

CLE.

CASE REPORT

A 60 days old boy baby born in an outside hospital at 36weeks(late

preterm) by normal delivery with meconium stained liquor, developed

respiratory distress, admitted and treated in NICU and discharged on 10th day of

life. However, mother noticed that he started developing respiratory distress

with suprasternal and intercostal recession, poor feeding since 25th day of life.

Baby was admitted in a tertiary care hospital, Air entry on the left side of the

chest was found to be decreased, heart sounds heard better on the right side.

Pulse oximeter showed saturation of 85% while using oxygenhood. X ray

chest showed hyper inflation of left upper lobe with pneumonitis and collapsed

lower lobe. CT chest confirmed the above findings and diagnosis of CLE with

pneumonia was made. Child was treated with IV antibiotics for pneumonia. In

view of persisting respiratory distress even after treating for pneumonia child

was admitted in our hospital for further treatment and surgical management.

On arrival, the child was found awake, underweight (wt- 3.9kg) and in

respiratory distress. There were marked intercostal and subcostal retractions.

Air entry was decreased in the left hemi thorax with expiratory wheeze noticed

on left upper lobe. Saturation was 85% on room air and 92% under oxygen

hood. There was no cyanosis. Routine haematological studies and biochemical

investigations were normal. X-Ray showed hyperlucency of left upper zone

with rightward mediastinal shift, and segmental collapse of left lower zone (Fig-

1). After admission, respiratory distress progressed to impending respiratory

failure. Child was intubated with 4 size endotracheal tube and fixed at 11 cm

and connected to mechanical ventilator with the optimal pressure settings. Child

had cardiac arrest but was effectively revived. Echo was done to rule out any

associated cardiac anomaly and it was normal.

The child was scheduled for emergency left lobectomy, blood was ordered and

no sedative premedication was prescribed. In the OT, basic monitoring like

ECG, NIBP, saturation and temperature monitoring were connected and 2

peripheral lines secured. Standard precautions to avoid hypothermia were taken

and premedication with iv atropine 0.1mg and fentanyl 4 mcg were given.

Induction was done with 100% O2 and sevoflurane. Gentle assisted ventilation

was done with hand using a Jackson Rees circuit, neonate placed in the right

lateral position and anaesthesia maintained with sevoflurane 2% in 100% O2.

Intraoperative analgesia was provided with intravenous fentanyl 4 mcg in

divided doses. A left upper lobectomy was performed through the 4th intercostal

space. The saturation rose up to 100% after the lobectomy. Hemodynamics

throughout the two hour surgical procedure (Fig-3) was stable. After lobectomy,

the left lower lobe was expanded using gentle manual recruitment maneuver.

Blood and fluid loss monitored and replaced with 40 ml of warmed Ringer's

Lactate and 60 ml of fresh packed cells. At the end of the surgery, an intercostal

nerve block was given by the surgeon under direct vision using 4 ml of 0.25%

Bupivacaine. The baby was shifted to the Neonatal intensive care unit with ICD

and endotracheal tube insitu. Post operative analgesia was provided by rectal

paracetamol. Child was ventilated in the postoperative period using pressure

controlled ventilation for 36 hours and carefully extubated. Intercostal drain

removed on 5th postoperative day. Postoperative x ray revealed expanded left

lung and normal position of mediastinum (Fig-2). Child made a rapid

uneventful recovery and was discharged on the 7th post operative day.

Fig-1: Pre op Chest X-ray Fig-2: Post op Chest X-ray

Figure-1: Preoperative chest radiograph showing hyperlucency on the left

upper zone, rightward mediastinal shift and segmental collapse of left lower

zone

Figure-2: Postoperative chest radiograph AP view showing expanded left lung

and normal position of mediastinum.

Figure-3: Resection of left upper lobe and Specimen of the resected emphysematous left upper lobe

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.7,8 Other terms for CLE include congenital lobar overinflation

and infantile lobar emphysema.1,2,9 Progressive lobar hyperinflation is likely the

final common pathway that results from a variety of disruptions in

bronchopulmonary development. These result from abnormal interactions

between embryonic endodermal and mesodermal components of the lung.

Disturbances may leads to change in the number of airways or alveoli or

alveolar size.10 However, a definitive causative agent cannot be identified in 50

percent of cases.11

The most frequently identified cause of congenital lobar emphysema (CLE) is

obstruction of the developing airway, which occurs in 25 percent of cases.7

Airway obstruction can be intrinsic or extrinsic, the former being more

common. This leads to the creation of a "ball-valve" mechanism in which a

greater volume of air enters the affected lobe during inspiration than that leaves

during expiration, producing air trapping.

CLE can be hypo alveolar or polyalveolar, based on number of alveoli within

each acinus.12 Usually one lobe is affected, but bilateral involvement is seen in

20%.13,14 Congenital heart disease is associated in 12-14% of these patients.4

Clinical signs of CLE include tachycardia, tachypnea, and chest retractions

which progress to respiratory distress and respiratory failure. Asymmetric

expansion of the hemithorax, rhonchi, displacement of apical impulse, hyper

resonant percussion on affected side, and diminished breath sounds and heart

sounds may be noted. Lobar hyperinflation, atelectasis of contra lateral lung,

mediastinal shift, and flattening of ipsilateral diaphragm are typically seen on

CXR. Thus, the disease is often confused with pneumonia and pneumothorax, 15

even resulting in wrongful placement of a chest drain. CT and MRI help in

diagnosis of CLE, but the single photon emission tomography ventilation-

perfusion lung scan is confirmatory.14 This scan may reveal hypo perfusion of

affected lobe due to compression of vasculature and hyper perfusion of normal

lobes by shunted blood. While conservative management for CLE has been

described, lobectomy is the mainstay of treatment. The management of a child

with CLE with pneumonia or respiratory failure is controversial. Clearing the

infection prior to surgery can improve pulmonary mechanics but unrelieved

compression atelectasis is detrimental.

The physiological considerations for anaesthetizing these patients

include ventilation and perfusion impairment of the lung in an infant

undergoing thoracic surgery in the lateral decubitus position. Unlike adults,

infants with unilateral lung disease does not improve oxygenation when the

healthy lung is dependent and the diseased lung is nondependent.16 In infants,

oxygenation is optimized when the healthy lung is nondependent.17 This

difference is due to the more compliant chest wall of the infant, which cannot

completely support the dependent lung. The FRC is consequently closer to the

residual volume and the airway closure becomes more likely in the dependent

lung.18 The advantage of the abdominal hydrostatic pressure gradient to the

dependent diaphragm is lesser in infants. Due to their small size; the favourable

increase in perfusion to the dependent lung is also less. An infant's ability to

tolerate one lung ventilation (OLV) is affected by these considerations and the

position for thoracic surgery is unfavourable for them when compared to adults.

Inhalation induction is preferred because spontaneous ventilation should be

maintained until either the chest is opened or OLV of the contra lateral lung is

achieved.5

Positive pressure ventilation is due to a ball valve effect, which

leads to over distension of the emphysematous lobe, worsening of mediastinal

shift, reduction in cardiac output and hemodynamic collapse. However gentle

assisted manual ventilation may be needed if there is hypoventilation during

induction due to poor respiratory reserve. Nitrous oxide should be avoided.

The optimum ventilatory technique has been discussed by many

authors. Tempe suggests that IPPV is best avoided as the critical airway

pressure is not known.16 Gentle manual ventilation keeping the airway pressure

at 20-25 cm of H2O before thoracotomy has been described.5 PRVC mode is an

attractive option for mechanical ventilation if available. High frequency

ventilation has been used successfully in the patients with CLE, as low airway

pressures are especially suitable.19 The risk of over distension leading to a

catastrophic hemodynamic crisis should never be underestimated and the

surgeon should be ready for immediate thoracotomy during induction. OLV is

not generally indicated for open thoracotomies in neonates and infants because

the surgeon is usually able to manually retract the lung. In neonates and infants,

the only options for lung isolation are either mainstem intubation or placement

of bronchial blockers. Use of double lumen Marraro tubes has been reported as

well.20

Maintenance of anaesthesia is usually done with inhalational agents. Opiates

like morphine are avoided due to the risk of postoperative respiratory

depression. Epidural analgesia with a caudal catheter inserted up to the thoracic

level and using local anesthetics has been described.21 Intravenous ketamine in

boluses of 1.5 mg/kg has been recommended for intraoperative analgesia.5, 20 In

most of the cases the trachea can be extubated at the end of the surgery. It was

decided to electively ventilate our patient in view of poor preoperative

respiratory condition.

CONCLUSION

An infant presenting in emergency with progressively increasing respiratory

distress should be viewed with a suspicion for CLE. Induction of anesthesia in a

patient with congenital lobar emphysema can be challenging for the

anesthesiologist because of the risk of overinflating the lung with either infant

induced airway Valsalva effects or positive airway pressure administered by the

anesthesiologist. 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 H2O 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 perfomed. The

successful outcome emphasizes the importance of use of low pressure manual

ventilation in management of such cases. The case highlights importance of

team approach in the difficult cases like this which yields a rewarding outcome.

REFERENCES

1. Stocker JT, Drake RM, Madewell JE. Cystic and congenital lung disease in

newborn. Perspect Pediatr Pathol 1978; 4:93.

2. Landing BH, Dixon LG. Congenital malformations and genetic disorders of

the respiratory tract (larynx, trachea, bronchi and lungs). Am Rev Respir Dis

1979; 120:151.

3. Ward CF. Diseases of infants. In: Katz J, Benumof JL, Kadis LB eds.

Anesthesia and uncommon diseases. Philadelphia: Saunders; 1990. 199.

4. Al-Salem AH, Adu-Gyamfi Y, Grant CS. Congenital lobar emphysema. Can

J Anaesth 1990; 37:377-9.

5. Cote CJ. The anesthetic management of congenital lobar emphysema.

Anesthesiology 1978; 49:296-8.

6. Franken EA, Buehl I. Infantile lobar emphysema: Report of two cases with

Unusual roentgenographic manifestations. Am J Roentgenol 1966; 98:354-7.

7. Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am

1994; 41:453.

8. Stanton M, Davenport M. Management of congenital lung lesions. Early

Hum Dev 2006; 82:289.

9. Hernanz-Schulman M. Cysts and cystlike lesions of the lung. Radiol Clin

North Am 1993; 31:631.

10. Tander B, Yalçin M, Yilmaz B, et al. Congenital lobar emphysema: a

clinicopathologic evaluation of 14 cases. Eur J Pediatr Surg 2003; 13:108.

11.Olutoye OO, Coleman BG, Hubbard AM, Adzick NS. Prenatal diagnosis and

management of congenital lobar emphysema. J Pediatr Surg 2000; 35:792.

12. Guidici R, Leao LE, Moura LA, Wey SB, Ferreira RG, Crotti PL.

Polyalveolosis: pathogenesis of congenital lobar emphysema? Rev Assoc Med

Bras 1998; 44:99-105.

13. Parray T, Apuya J, Abraham E, Ahsan F, Professor S. Anesthesiologist’s

dilemma in a patient with congenital emphysema. The Internet Journal of

Anesthesiology 2009; 24.

14. Tempe DK, Virmani S, Javetkar S, Banerjee A, Puri SK, Datt V. Congenital

lobar emphysema: pitfalls and managements. Ann Car Anaesth 2010; 13:53-8.

15. Basu S, Gupta AK, Kumar A. Congenital lobar emphysema mimicking as

persistent pneumonia in anewborn. Clin Pract 2011; 1:101.

16. Remolina C, Khan AU, Santiago TV, et al. Positional hypoxemia in

unilateral lung disease. N Eng J Med. 1981;304 (9):523–525.

17. Roysheinbaum, Gregory B, Hammer. Separation of two lungs. In: Carin A

Hagberg ed. Benumof's Airway Management. 2nded. Philadelphia: Elsevier;

2007.576-93

18. Mansell A, Bryan C, Levison H. Airway closure in children. J Appl Physiol.

1972;33 (6):711–4.

19. Goto H, Boozalis ST, Benson KT, et al. High frequency jet ventilation for

resection of congenital lobaremphysema. Anesth Analg. 1987; 66:684–6.

20. Lakshmi Vas. Congenital Lobar Emphysema. In: Litman RS, ed. Pediatric

Anesthesia Practice.Cambridge: University Press; 2007. p. 85–7.

21. Raghavendran S, Diwan R, Shah T et al. Continuous caudal epidural

analgesia for congenital lobar emphysema: a report of three cases. Anesth

Analg. 2001;93:348–50.