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CASE REPORT
Year : 2009  |  Volume : 53  |  Issue : 4  |  Page : 482-485 Table of Contents     

Congenital Lobar Emphysema


1 Department of Cardiac Anaesthesiology, Meenakshi Mission Hospital and Research Center, Lakearea,Madurai TN 625107, India
2 Department of Cardiothoracic and Vascular Surgery, Meenakshi Mission Hospital and Research Center, Lakearea,Madurai TN 625107, India
3 Department of Pediatrics, Meenakshi Mission Hospital and Research Center, Lakearea,Madurai TN 625107, India

Date of Web Publication3-Mar-2010

Correspondence Address:
Divya Chandran-Mahaldar
DM Cardio-thoracic and Vascular Anesthesia student, Sree Chitra Tirunal institute for Medical Science and Technology, Medical College PO, Thiruvananthapuram 695 011
India
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Congenital lobar emphysema (CLE) characterized by over distension and air-trapping in the affected lobe is one of the causes of infantile respiratory distress requiring surgical resection of affected lobe.
At induction, positive pressureventilation can expand the emphysematous lobe compressing the normal lung resulting in severe cardiovascular compromise. We report a case of 28 day old baby with CLE posted for emergency lobeetomy. Strategies to prevent hyperinflation and anaesthetic considerations of various techniques adopted for lung separation in infants have been reviewed.

Keywords: Congenitallobar emphysema, Neonatal anaesthesia, Hyperinflation of emphysematous lung, Positive pressure ventilation


How to cite this article:
Chandran-Mahaldar D, Kumar S, Balamurugan K, Raghuram AR, Krishnan R, Kannan. Congenital Lobar Emphysema. Indian J Anaesth 2009;53:482-5

How to cite this URL:
Chandran-Mahaldar D, Kumar S, Balamurugan K, Raghuram AR, Krishnan R, Kannan. Congenital Lobar Emphysema. Indian J Anaesth [serial online] 2009 [cited 2014 Jul 29];53:482-5. Available from: http://www.ijaweb.org/text.asp?2009/53/4/482/60322


   Introduction Top


Congenital lobar emphysema (CLE) character­ized by unilobar alveolar distension secondary to bronchomalacia or absent cartilage is a rare condition. This disease presents as respiratory distress due to the ventilation perfusion mismatch as aresult of the hyper inflated lung causing compression atelectasis on the ip­silateral orthe contralateral side with mediastinal shift. In 1954 Gross and Lewis, published the first case re­port of CLE. [1] Male babies are affected more often than female in the ratio of 3:2. [2]. The incidence of left upper lobe involvement is 43%, right middle lobe 32%, right upper lobe 20%, and bilateral involve­ment 20%. [3]

The exact etiology of the disease is not known, but several intrinsic and extrinsic causes have been postulated [4] . Presenting features in these infants can be dyspnea, tachypnea, retraction, wheezing, coughing, cyanosis, and asymmetric breath sounds. In these infants, there is increased intrathoracic pres­sure because of hyper inflation of one or more pulmonary lobes, leading to mediastinal shift and atelectasis of the ipsilateral or contralateral lobes of the lung. This causes displacement of heart sounds, decreased venous return, and varying degrees of ventilation-perfusion mismatch, which leads to hy­poxia. Chest radiographs help to diagnose but is not definitive [4]. A CT scan confirms the diagnosis and may rule out associated anomalous vascular slings. Associated congenital heart disease or vascular anomalies may occur in 12%-14% of these pa­tients [5] . Thus, all patients should have adequate preoperative cardiac evaluation by echocardiography and CT scan. Cardiac catheterization and angiogra­phy are necessary in children with known or sus­pected congenital cardiovascular lesions.


   Case report Top


A 28-day-old male baby weighing 3.4 Kg was referred as a case of perinatal asphyxia with respira­tory distress notresponding to medical management.

On examination, baby was tachypnoeic with flar­ing of alae nasi and sub costal retraction. The pulse rate was around 150/min. On examination of the respi­ratorysystem, decreasedbreath sounds on the left hemi thorax was noted. On examination of the cardiovascu­lar system, the heart sounds were shifted to the oppo­site side. No gross cardiac anomalywas found.

Following investigations were carried out: com­plete blood count, blood glucose, bloodurea, serum creatinine and electrolytes and chest X-ray (PA and lateral view. Chest X-ray showed increased translu­cency onthe left side with tracheal and mediastinal shift to the right side. CT scan confumed the diagnosis of CLE of the left upper lobe[Figure 1] and [Figure 2].

The neonate was posted for left upper lobectomy.

Anaesthetic management:

Preoperative examination revealed tachycardia and tachypnoea with signs of respiratory distress. On auscultation, there were decreased breath sounds on the left hemithorax. The cardiovascular system was normal. Oxygen saturation (Sp0 2 ) was 84% in air, but there was no visible cyanosis. Routine hematological and biochemical investigations were within normal lim­its. Echocardiography ruled out any associated con­genital cardiac anomalies.

The baby was labeled as ASA III E. The baby was wrapped inwarm cotton wool gamgees and placed on the heating mattress. Cardioscope and pulse oxime­ter was attached to the baby. Ryle's tube was aspi­rated with a syringe. Before starting anaesthesia, a sur­geon was scrubbed to perform emergency thoracos­tomy if required.

Antisialogogue atropine 0.0lmg.kg -1 and fenta­nyl 3 mcg was given intravenously and rectal paracetamol suppository 80mg was placed. The baby was pre oxygenated for 5 minutes and then gradually sevoflurane was started. Gentle manual ventilation was performed via the facemask.

After introducing laryngoscope, a 3.5 size endot­racheal tube was inserted. The baby was connected to anaesthesia machine through Jackson Rees modifica­tion ofAyre's 'T-piece'.

Spontaneous ventilation was maintained using 100% oxygen, l-2% sevoflurane with gentle manual ventilation. Saturation on pulse oximeter was 98% fol­lowing intubation

The neonate was placed intrue rightlateral position

Monitoring included electrocardiogram, invasive blood pressure, Sp0 2 , ETCO 2 and rectal temperature. IV fluids were titrated accordingto Holiday Segar for­mulate replace fasting and maintenance requirements. Blood loss was replaced. Vital signs were maintained in normal range throughout surgery.

Once resection of the affected lobe was com­pleted, controlled lung ventilation with atracurium as the neuromuscular blocking agent was started. Nitrous oxide was added thence.

Blood gases intraoperatively and postoperatively were within normal limits. At the end of operation, in­tercostal block was given with 3 ml of 0.125% bupivacaine, and residual neuromuscular block was reversed with neostigmine 0.15 mg along with atropine 0.03 mg I V The infant was extubated when spontane­ous respiration was sufficient to maintain Sp0 2 >90% in air. Later, the child was kept in an oxygen-enriched environment in the pediatric intensive care unit under continuous SpO Z and EKG monitoring. At 72 hours the chest drain was removed after full expansion of the re­sidual lung. Rest of the postoperative period was un­eventful, and the child was discharged after 7 days.

Lung biopsy of the resected segment showed lung parenchyma with atelectatic changes and emphysema­tous dilatation of alveolar spaces in the surrounding zone. [Figure 3] and [Figure 4].


   Discussion Top


Controversy still exists concerning the diagnosis and treatment of congenital lobar emphysema (CLE). Although surgical removal of the affected lobe is the most commonly accepted form oftreatrnent, there is a small place for conservative therapy in patients who are not clinically inrespiratory distress and able to feed and grow. Maintaining ventilatory pressures and vol­ume as low as possible avoids producing ventilator­related hyper expansion of the affected lobe. Manage­ment by more conservative, gentle ventilation technique if successful will result in fewer emergency surgeries with CLE. Operative mortality mte is 3 to 7% whereas with conservative therapies itis SOto 75%. Hence con­servative management should be reserved only for pa­tients with milder symptoms or no distress at all. [6]

Monitoring of the vital parameters, during neona­tal surgery is a must. During thoracotomy, the baby is at great risk. On induction of anaesthesia if positive pressure ventilation is applied before opening of the chest, it may cause rapid inflation of emphysematous lobe or cystwith sudden mediastinal shift and cardiac arrest [7] . Therefore, induction of anaesthesia should pro­vide adequate spontaneous ventilation with minimal air­way pressure. Occasional gentle assistance is neces­sary. Once the chest is opened andthe affectedlobe is delivered, the patient can be paralyzed and the lungs ventilated by controlled ventilation [8] . Hyperinflation of the emphysematous lobe or cyst can be prevented by avoiding the use of nitrous oxide be­fore the delivery of the affected lobe, as it diffuses faster in a closed cavity and expands the cavity, leading to further compression of normal lung andmore mediasti­nal shift [9] . In our patient nitrous oxide was started only after the affected lobe was resected. [10],[11]

Isolated case reports of endobronchial intubation using single lumen endotracheal tube with gentle ventila­tion as an alternative to spontaneous bilaterallung venti­lation are also described. [12] However endobronchial in­tubation of the normal side leads to temporary collapse of the affected lobe with elimination of ventilation to the non perfused lung segment on the diseased side is an ever-present risk. Lack of double lumen tubes in this age group makes things difficult. Pediatric fibreoptic bron­choscope to confirm properplacement of the bronchial blockerwas not available in our institute.

The technique of caudal thoracic epidural catheter­ization provides a stable cardiovascular profile and ex­cellent analgesia without depressing respiration. How­ever kiniking and doubling backof cathetermay prevent the catheter from reaching the midthoracic segments. [13]



 
   References Top

1.Irving IM Malformations and acquired lesions of lungs, pleura and mediastinum. In: Lister J, Irving IM. Eds. Neonatal Surgery London: Butterworth& CoLtd 1990, pp 265-271.  Back to cited text no. 1      
2.Al-Salem AH, Gyamfi YA, Grant CS. Congenital lobar emphysema. Can JAnaesth 1990;37:377-9.  Back to cited text no. 2      
3.Monin I', Didier F, Vert I', et al. Giant lobar emphy­sema: neonatal diagnosis. PediatrRadiol 1979; 8:259­60.  Back to cited text no. 3      
4.Berlinger NT Porto DP, Thompson TR. Infantile lobar emphysema. Ann Otol Rhinol Laryngol 1987;96:106-11.  Back to cited text no. 4      
5.Raynor AC, Cap MI', Scaly WC. Lobar emphysema of infancy: diagnosis, treatment and etiological aspects. Ann Thorac Surg 1967;4:374-85.  Back to cited text no. 5      
6.Holli MA, Segar WE. The maintenance need for water inparenteral fluidtherapy. Pediatrics 1957;19:823-832.  Back to cited text no. 6      
7.Tander B, Yalgin Y, Yilm az B, Ali Karadao C, Bulu M. Congenital lobar emphysema: a clinicopathologic evalu­ation of 14 cases. Eur JPeciatr Surg 2003;13:108-11.  Back to cited text no. 7      
8.Hatch DJ, Summer E. Anesthesia - Specific conditions. In: Feldman SA, Scun CF, editors. Current Topics in Anesthesia No 5, Neonatal Anesthesia, 1st ed. London: Edward Arnold Publishers Pvt Ltd 1981:112.  Back to cited text no. 8      
9.Raynor AC, Cap MP, Scaly WC. Lobar emphysema of infancy: diagnosis, treatment and etiology aspects. Ann Those Surg 1967; 4:374-375.  Back to cited text no. 9      
10.Brown TCK, Fisk GC. Anesthesia for thoracic surgery. In: Anesthesia for children, 2nd ed. Oxford: Blackwell Scientific Publications 1992:177.  Back to cited text no. 10      
11.Morray J. Anesthesia for thoracic surgery. In: Gregory G Ed. Pediatric Anesthesia, vol 2. New York: Churchill Livingstone 1983:662.  Back to cited text no. 11      
12.Gupta R, Singhal SK, Rattan KN, Chhabra B, Manage­ment of congenital lobar emphysema with endobron­chial intubation and controlled ventilation. AnesthAnalg 1998;86:71 3.  Back to cited text no. 12      
13.Raghavendran S, Diwan R, Shah T, Vas L. Continuous caudal epidural analgesia for congenital lobar emphysema: areportof three cases. AnesthAnalg2001 ;93:348-50.  Back to cited text no. 13      


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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