Editorial

Congenital Lobar Emphysema: An Anaesthetic Challenge

Anita R Chhabra¹, Vijay L Shetty¹, Hemalata R Iyer¹, Shivaji B Mane²

Keywords: Congenital Lobar Emphysema, Endobronchial intubation, Lobectomy

Congenital Lobar Emphysema (CLE) is a rare, life-threatening yet potentially curable cause of respiratory distress in neonates and infants presenting with varying degrees of severity. There is hyperinflation due to air trapping in the affected lobe(s) resulting in a mediastinal shift, hemodynamic instability, and hypoxia. We report a case of CLE in a 3-month-old male baby who was posted for thoracotomy and left upper lobe lobectomy. Here, we discuss the anaesthetic challenges and management including endobronchial intubation, controlled ventilation, and the successful outcome

Case Report

Abstract

Journal of Anaesthesia and Critical Care Case Reports 2019 Jan-April;5(1):11-13

Email: canita24@yahoo.com

Department of Anaesthesiology, Fortis Helth Care, Mulund, Mumbai.

Dr. Anita R Chhabra,Address of Correspondence

2Department of Paediatric Surgery, Fortis Helth Care, Mulund, Mumbai.

1Department of Anaesthesiology, Fortis Helth Care, Mulund, Mumbai.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

© 2019 by Journal of Anaesthesia and Critical Care Case Reports| Available on www.jaccr.com |

Journal of Anaesthesia and Critical Care Case Reports Volume 5 Issue 1 Jan-April 2019 Page 11-1311 | | || |

Dr. Anita R Chhabra Dr. Vijay L Shetty

Congenital Lobar Emphysema is a rare cause of sudden respiratory distress in a newborn or infant. It is a developmental anomaly of the lower respiratory tract system involving hyperinflation due to air trapping of the histologically normal, affected lobe(s) and resultant compression of the remaining lung t i s s u e [ 1 , 2 , 3 ] . C o n g e n i t a l l o b a r hyperinflation is the currently recommended term as it involves the pathologically healthy lung tissue[4]. It is the 2nd leading cause of early mortality in infants after anomalies of the cardiovascular system. The reported incidence is 1:20,000 to 1:30,000 live births with males being affected three times more than females[5,6]. The involvement of left upper lobe (LUL) is 43% followed by right middle lobe – 32% although bilateral involvement is unknown[7,8]. Etiology is unknown in 50% of cases but several intrinsic and extrinsic causes have been described[9]. Surgery is considered the first choice of treatment in severe cases[10]. We report the anaesthetic management in an infant diagnosed with CLE, posted for LUL lobectomy with a review of the literature.

A 3-month-old male baby weighing 5.4 kgs presented with symptoms of respiratory distress and fever and signs of lower respiratory tract infection(LRTI). Oxygen saturation was 93% on room air.The chest x-ray revealed hyperinflated LUL and mediastinal shift to the right (figure 1), which was further confirmed with high-resolution computed tomography (HRCT) (figure 2).A diagnosis of CLE was made, and treatment initiated with supplemental o x y g e n b y m a s k , a n t i b i o t i c s , b r o n c h o d i l a t o r s , n e b u l i z a t i o n a n d intravenous fluids. Surgery was scheduled after 3 days when the infective phase subsided. During the pre-anaestheticcheck-up, the baby was active, afebrile, and had an O2 saturation of 95% on room air. Air entry was decreased on the left upper zone and there were no other foreign sounds on auscultation. The routine lab findings were within normal limits.IV fluids (0.45% DNS) @ 20mls/hour was on flow and the baby’s hydration status was normal. The baby was premedicated using Atropine 0.1mg IV, Midazolam, 0.01mg/kg IV and Ketamine, 1mg/kg IV. In the operation theatre, standard monitoring (heart rate,

blood pressure, end-tidal Carbon Dioxide, temperature, Oxygen saturation, and urine output) was established. Surgical readiness for emergency thoracotomy at induction was ensured. Induction with spontaneous inhalation of Sevoflurane and Oxygen using Jack son-Rees circuit, aided with Atracurium (0.5 mg/kg) and gentle intermittent positive pressure ventilation was done. The baby was i nt u bated w i t h a 3 . 5 m m m i c ro - c u f f endotracheal tube (Haylard - Kimberly Clark) that was advanced into the right main bronchus by turning the baby’s head to the left side[11].The right main bronchus intubation and correct placement proximal to RUL branch was confirmed with a flexible endoscope. Ventilation of (RUL) bronchus were conf irmed intra-operat ively by auscultation. Standard precautions were taken for temperature control (ambient OT temperature maintained at 27⁰ Celsius, baby’s head and limbs were wrapped in Gamgee rolls; warming mattress, convective forced-air warmer, warm fluids, heat and moisture exchanging f i lters were also used).The preoperative maintenance fluids were continued @ 5 -7 ml/kg/hour with extra boluses of 10-20 ml whenever required, w i t h t h e c o n c o m i t a n t w a t c h o n

Case ReportIntroduction

Dr. Hemalata R Iyer Dr. Shivaji B Mane

Chhabra A R et al www.jaccr.com

Journal of Anaesthesia and Critical Care Case Reports Volume 5 Issue 1 Jan-April 2019 Page 11-1312 | | || |

The Preoperative evaluation aims at optimizing the cardio-respiratory status of the baby.

C L E i s a r a r e c o n g e n i t a l a n o m a l y characterized by hyperinflation of the

affected, histologically normal pulmonary tissue with compression atelectasis of the c o n t r a l a t e r a l l u n g a s s o c i a t e d w i t h amediastinal shift[12].

hemodynamics, urine output, and blood sugar levels.An a19-gauge caudal epidural catheter (Portex) was cited and threaded till thoracic (T4 segmental) level. Four mls of 0.25% b u p i v ac a i n e b o l u s w a s i n j e c te d f o r intraoperat ive pain rel ief . One lung ventilation (OLV) was maintained in the right lateral decubitus position using a closed circuit and pressure control mode with air, Oxygen (80%) and Sevoflurane (2%), Atracurium, 0.125mg/kg as top-up doses, maintaining the peak airway pressure no more than 20 mm of Hg. After the affected emphysematous lobe was excised, thorough e n d o - b r o n c h i a l s u c t i o n w a s d o n e . Subsequently, the ET tube was withdrawn into the trachea to faci l itate bi-lung ventilation, to recruit left lower lobe and to confirm theabsence of leak froma surgical stump. Inter-costal drain (ICD) was placed before closure. Hemody namics were maintained throughout and there was minimui blood loss. The residual neuro-muscular blockade was reversed using Atropine 0.02mg/kg IV and Neostigmine 0.05mg/kg IV and the baby was extubated, pain-free with good spontaneous efforts and shifted to (PICU) for further management. Post-operative analgesia was maintained through thecontinuous epidural infusion of 0.1% Bupivacaine @ 2ml/hour. Post-operative chest X-ray revealed acomplete correction of mediastinal shift and adequate lung expansion on the left side. The epidural catheter and ICD were removed on thethird and fifth day respectively. The rest of the postoperative period was uneventful, and the baby was discharged on day 12.

Discussion

The common diagnostic modalities include chest x-ray;[18] HRCT and (MRI) help to confirm the diagnosis. Bronchoscopy is increasingly being employed as a diagnostic as well as atherapeutic tool[19]. Single photon emission tomography V/Q lung scan reveals hypo-perfusion of affected lobe due to c o m p r e s s i o n o f v a s c u l a t u r e a n d hyperperfusion of normal lobe by shunted blood.

A definitive causative agent cannot be identified in approximately 50% of cases. Airway obstruction can be intrinsic or extrinsic with the former being more common. Common intrinsic causes are bronchomalacia, bronchial stenosis or cysts[13]. Bronchomalacia is reported in at least 25% cases resulting in a ball-valve mechanism wherein the air can go in during inspiration but cannot be expelled during expiration, causing air trapping in the affected lobe[14,15]. Extrinsic cause includes compression due to abnormal vessel[13].The resultant compression atelectasis due to the overinflated lobe causes ventilation-perfusion mismatch and hypoxia. There is increased intrathoracic pressure causing impaired venous return and hemodynamic instability[16].The baby usually presents with respiratory distress, signs of LRTI and displaced cardiac sounds. It is most commonly confused with pneumothorax with some reports having mentioned wrongful placement of ICD[17]. Other differential diagnoses of CLE are lung cysts, congenital diaphragmatic hernia, and c o n g e n i t a l c y s t i c a d e n o m a t o i d malformation.

Other diagnostic modalit ies include

fluoroscopy, angiography, radio-isotope evaluation, and lung scintigraphy [11,18,20,21]. Its prenatal diagnosis is difficult due to its low prevalence; and also, the requirement of ventilation for it to manifest. Fetal MRI is helpful to establish a diagnosis. Surgical excision of the affected lobe is the commonest mode of treatment in severe cases[10].

The anaesthetic challenges in a case of CLE comprise of the venti lator y management of a baby undergoing

thoracic surgery in the lateral decubitus position. Induction is crucial as the patients are in respiratory distress; the crying and struggling can worsen the emphysema. Nitrous oxide must be avoided as it can rapidly diffuse into closed cavities leading to further compression and mediastinal shift. A High degree of suspicion of pneumothorax w ith surgical standby for emergenc y thoracostomy at induction is prudent to deal with any hemodynamic catastrophe. We intubated with the aid of muscle relaxant using gentle intermittent positive pressure ventilation, keeping airway pressures below 20-25 mm of Hg[17]. This helped to deliver less tidal volume at a controlled rate thereby avoiding over-aeration of the lungs. Pressure regulated volume control (PRVC) mode is desirable if available[17].One lung ventilation is desirable, although lung isolation is technically difficult in small infants. Moreover, double lumen tubes are not freely available. We achieved OLV by doing selective right sided endobronchial intubation[11]. The RUL blockage was avoided by endoscopic guided placement and fixation of the tube. Kamra et al have reported rigid bronchoscopic placement of Fogarty catheter as abronchial blocker in the diseased lung[22]. However, it may pose the risk of proximal displacement in trachea and inability to apply suction or continuous positive airway pressure (CPAP) as it is a c l o s e d t i p. In t h e pa s t , a n ae s t h e t i c management of CLE has been described with tracheal intubation and spontaneous ventilation till lobectomy. However, it demands deeper planes of anaesthesia which may aggravate hypoxia and hypotension. Whereas, our approach of gentle manual venti lat ion al lows control over lung expansion and venti lator y rate while

Figure 1: Preoperative Chest X-ray PA view showing hyperinflated left upper lobe and mediastinal shift to right.

Figure 2: CT scan showing hyperinflated left lobe, mediastinal shift to right and midline shift to right side.

www.jaccr.comChhabra A R et al

Chhabra A R, Shetty V L, Iyer H R, Mane S B. Congenital Lobar Emphysema: An Anaesthetic Challenge. Journal of Anaesthesia and Critical Care Case Reports Jan-April 2019;5(1):11-13.

How to Cite this ArticleConflict of Interest: Nil

Source of Support: None

Journal of Anaesthesia and Critical Care Case Reports Volume 5 Issue 1 Jan-April 2019 Page 11-1313 | | || |

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References

maintaining hemodynamic stability.In our case, intraoperative and postoperative analgesia was provided through thethoracic level placement of epidural catheter via caudal insertion site. Raghavendra et al. have also reported similar placement while retaining spontaneous ventilation until thoracotomy[23].Other methods of providing intra-op and post-op analgesia include parenteral narcotics/ketamine, non-steroidal anti-inflammatory drugs, intercostal nerve blocks under direct vision and rectal suppositories.Most cases get extubated postoperatively in OT. Elective ventilation is indicated when

more than one lobe is excised or if the infant is very sick preoperatively and has poor spontaneous efforts. Conser vative management has a role especially when the patients are mildly sy mptomatic and have tolerated the respiratory exacerbations adequately. Use of flexible bronchoscope has been described to directly and dynamically evaluate the airway anomalies and identify the reversible cause in patients with suspected CLE[19]. Use of flexible ultra-thin bronchoscope passed into affected bronchus for emergency relief of ini t ia l respirator y di stress has been reported[24].

ConclusionIn conclusion, an infant presenting with respiratory distress should be viewed with ahigh degree of suspicion of CLE. Early detection and prompt intervention can treat this potentially life-threatening yet reversible illness. Anaesthetic management pertaining to varying modes of ventilation, lung i s o l a t i o n t e c h n i q u e s a n d a d e q u a t e postoperative analgesia is crucial for the successful outcome of the case.