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 Table of Contents  
Year : 2022  |  Volume : 5  |  Issue : 2  |  Page : 77-80

Airway management of a giant thoracic ganglioneuroma causing airway obstruction in a 3-year-old child

1 Department of Anaesthesia and Critical Care, Army Hospital (Research and Referral), New Delhi, India
2 Department of Cardiothoracic Surgery, Army Hospital (Research and Referral), New Delhi, India

Date of Submission14-Mar-2022
Date of Acceptance15-May-2022
Date of Web Publication07-Jun-2022

Correspondence Address:
Dr. Simrandeep Singh
Department of Anaesthesia and Critical Care, Army Hospital (Research and Referral), Delhi Cantonment, New Delhi - 110 010
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/arwy.arwy_7_22

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Large tumours occupying one half of the thoracic cavity are rare and may cause life-threatening complications by compression or invasion of vital structures that results in cardiovascular and/or respiratory insufficiency. In-depth preoperative planning is necessary after assessment of perioperative risk of hemithoracic masses. By providing lung isolation, one-lung ventilation technique provides improved surgical conditions compared to conventional dual-lung ventilation for thoracic surgery. As double-lumen tubes are not available for smaller children, balloon-tipped bronchial blockers remain the technique of choice, especially under the age of 6 years. Huge intrathoracic masses in small children with radiological and clinical findings suggesting airway compression are high risk for airway complications. Fibreoptic bronchoscope-guided intubation should be preferred in view of tracheobronchial compression by the mass and placement of an endobronchial blocker helps in one-lung ventilation in small children. The management of these tumours is challenging and should be performed only in specialised centres.

Keywords: Arndt bronchial blocker, intrathoracic ganglioneuroma, paediatric

How to cite this article:
Kumar A, Singh S, Tiwari N, Joshi A. Airway management of a giant thoracic ganglioneuroma causing airway obstruction in a 3-year-old child. Airway 2022;5:77-80

How to cite this URL:
Kumar A, Singh S, Tiwari N, Joshi A. Airway management of a giant thoracic ganglioneuroma causing airway obstruction in a 3-year-old child. Airway [serial online] 2022 [cited 2023 Feb 1];5:77-80. Available from: https://www.arwy.org/text.asp?2022/5/2/77/346891

  Introduction Top

Intrathoracic ganglioneuroma (GN) is a rare, benign tumour arising from atypical cells of the sympathetic nervous system in the neural crest.[1] It is most often asymptomatic, but one of the feared presentations as a mediastinal mass in the paediatric age group is airway compression and cardiovascular collapse. The most common sites are posterior mediastinum and retroperitoneum (37.5% in each location).[2] We present a child with a massive tumour in the right hemithorax causing airway compression that proved to be a GN after surgical excision. In addition to this airway compression, the narrow and compressible airway due to more cartilaginous structures in paediatric age group makes it more challenging than the adult airway.[3]

  Case Report Top

A 3-year-old child presented with chronic dry cough for the past 1 year which was increasing progressively and got aggravated with exertion. A detailed history revealed symptoms suggestive of airway compromise such as dyspnoea while playing and orthopnoea for the past 6 months. The child preferred sleeping in the right lateral position. On auscultation, air entry was decreased over the right hemithorax. The chest radiograph was suggestive of a large, well-defined homogenous opacity, oval in shape and occupying most of the right hemithorax [Figure 1]. Non-contrast computed tomography revealed a large, minimally enhancing heterogeneous pleural-based soft-tissue mass lesion measuring 10.5 cm × 8.6 cm × 12 cm with patchy calcified foci in the right thoracic cavity, predominantly involving the upper two-third of the thoracic cavity. The mass formed an acute angle with the right lateral chest wall, compressing and displacing the adjacent lung parenchyma, compressing the trachea and right main bronchus and mildly shifting the mediastinal cardiovascular structures towards the left side [Figure 2]. As the intrathoracic mass was huge involving both anterior and posterior mediastinum, the surgical team decided to approach it through a median sternotomy. In view of the huge mass in the right hemithorax compressing adjacent lung parenchyma, trachea and right main bronchus, one-lung ventilation was chosen to provide a better surgical field. One-lung ventilation would also give the added advantage of avoiding lung injury and spillage of blood or surgical debris onto the left side.[4] The surgical plan was to perform a complete excision of mass through a median sternotomy using one-lung ventilation.
Figure 1: Chest X-ray showing large right hemithoracic mass with deviation and compression of large airway

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Figure 2: Non-contrast computed tomography of chest showing (a) large mass in right hemithorax and (b) compression of trachea

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Standard monitoring consisting of a 5-electrode electrocardiogram, noninvasive blood pressure, pulse oximeter and temperature probe were initiated. The child was premedicated with intravenous (IV) fentanyl (2 μg/kg) and midazolam (0.1 mg/kg). The left radial artery was cannulated under local anaesthesia with a 22-SWG cannula for invasive arterial blood pressure monitoring as we anticipated haemodynamic perturbations during anaesthetic induction. After preoxygenation with 100% oxygen, the child was induced with IV ketamine (1 mg/kg) and sevoflurane (MAC 0.6-1). A 2.8-mm fibreoptic bronchoscope (FOB) was introduced into the trachea to evaluate the extent of airway compression [Figure 3]. A fibreoptic-aided intubation was performed with a 4.5-mm ID cuffed armoured endotracheal tube under spontaneous ventilation. Anaesthesia was maintained with 1.0 to 1.5 MAC sevoflurane and infusion of vecuronium at 1 μg/kg/min. For intraoperative pain management, IV paracetamol 15 mg/kg was given in addition to an infusion of fentanyl at 2 μg/kg/h that was stopped 30 min before extubation. A 5-Fr triple-lumen central venous catheter was placed in the right femoral vein under ultrasound guidance.
Figure 3: Fibreoptic image revealing (a) external compression of trachea and (b) bronchial blocker in right main bronchus

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A 5-Fr Arndt bronchial blocker (Cook Critical Care, Bloomington, IN, USA) was advanced through a 'multiport adapter' into the right main bronchus under fibreoptic guidance. With the blocker placed in the right main stem bronchus, the bronchoscope was withdrawn and the cuff inflated with 2 mL of air under direct vision. Separation of lungs by the inflated balloon was visualised using the FOB and confirmed by differential auscultation of both the lungs. The wire loop was retracted so that the open-tipped blocker could be utilised for suction or application of continuous positive airway pressure.

The intraoperative course was uneventful. However, during dissection of the mass, there was frequent variation in arterial blood pressure due to compression of the aorta. Intraoperative blood loss was 16 mL/kg; no major vessel was injured. Intraoperative one-lung ventilation was aimed at haemodynamic stability of patient and to provide a 'quiet' surgical field with adequate exposure while simultaneously avoiding any hypoxaemia. The Arndt bronchial blocker was deflated after debulking of tumour to achieve dual-lung ventilation. A repeat fibreoptic bronchoscopy revealed no residual tracheal compression and ruled out the presence of any tracheobronchomalacia. Bronchoscopy showed a normal calibre trachea at the level of the carina and a normal calibre left main stem bronchus.

The child was extubated with simultaneous removal of the Arndt bronchial blocker after suctioning and cuff deflation. Postoperative pain management included paracetamol 15 mg/kg 8 hourly for 5 days and IV boluses of fentanyl 2 μg/kg given as required. The postoperative period was uneventful, and the patient was discharged on the 7th postoperative day. Follow-up over 12 months showed the absence of recurrence or metastasis. Histopathological examination revealed mature ganglion cells and Schwannian stroma with interspersed lymphoid follicles. A small focus of immature cells was seen, immunopositive for chromogranin and synaptophysin and negative for leucocyte common antigen, calretinin and NeuN MIB-1. Overall features were suggestive of GN (intermixed type).

  Discussion Top

Anaesthetic management of patients with intrathoracic mass has always been challenging, especially when it causes compression of the larger airways and major vessels. Intraoperative risk of cardiorespiratory complications has been reported in the paediatric age group due to the smaller calibre and compliant nature of major airways and inability to cooperate for awake intubation.[1] Diagnostic workup including fibreoptic airway examination and pulmonary function testing can also difficult due to lack of patient cooperation, making management in children even more challenging.[5]

One-lung ventilation provides improved surgical conditions compared to conventional dual-lung ventilation for thoracic surgery. With advancement in technology and improvement in surgical equipment, there have been parallel advancements in paediatric airway equipment. Options for lung isolation in the paediatric population include main stem bronchial intubation, bronchial blockers, Univent tubes and double-lumen endobronchial tube.[3] We used Arndt bronchial blocker for lung isolation as it is one of the most common blockers used in the paediatric age group. The Arndt bronchial blocker has a 'high volume-low pressure' cuff and causes minimal cuff-related complications in the paediatric airway. Balloon-tipped bronchial blockers are preferred in children under the age of 6 years and remains, apart from endobronchial intubation, the technique of choice for lung separation in this age group. This is because the diameters of the Univent™ 3.5 uncuffed tube (recommended for 6 to 8 year olds) and double-lumen endobronchial tube (recommended for 8 to 10 year olds) are not appropriate for the aforementioned age group.[6] For children, the Arndt endobronchial blocker is only suitable if the endotracheal tube to be used is greater than 4.5-mm internal diameter, as the available 5-Fr catheter has a diameter of 2.5 mm and requires a small bronchoscope of 2.2–2.8 mm for positioning.[7]

Ventilation and perfusion (V/Q) are greatest in the dependent regions of lung due to gravitational forces. During thoracic surgery, there are several factors that cause V/Q mismatch and hypoxaemia. These include surgical retraction of lung on operated side or single-lung ventilation resulting in collapse of operative lung, decrease in functional residual capacity and impairment of hypoxic pulmonary vasoconstriction due to anaesthetic agents.[3] The duration of single-lung ventilation should be minimised by deflating the cuff of the bronchial blocker following tumour debulking to avoid complications related to prolonged single-lung ventilation.

Patients with radiological and clinical findings suggesting airway compression are at high risk for airway complications.[8] Fibreoptic bronchoscope-guided tracheal intubation under spontaneous ventilation is preferred in view of tracheal and bronchial compression by mass and placement of endobronchial blocker.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Sonawane NB, Gaiwal S. Anaesthetic management of posterior mediastinal mass in a child: A case report. Pediatr Anesth Crit Care J 2015;3:89-91.  Back to cited text no. 1
Geoerger B, Hero B, Harms D, Grebe J, Scheidhauer K, Berthold F. Metabolic activity and clinical features of primary ganglioneuromas. Cancer 2001;91:1905-13.  Back to cited text no. 2
Fitzgerald J, Evans F. Techniques for single lung ventilation in infants and children: WFSA - Resources. WFSA Resource Library. Available frm: https://resources.wfsahq.org/atotw/techniques-for-single-lung-ventilation-in-infants-and-children/. [Last accessed on 2020 Sep 10].  Back to cited text no. 3
Letal M, Theam M. Paediatric lung isolation. BJA Educ 2017;17:57-62.  Back to cited text no. 4
Au V, Marsh B, Benkwitz C. Resection of a posterior mediastinal mass in a 4-year-old child complicated by difficult airway management and emergent use of extracorporeal membrane oxygenation. Semin Cardiothorac Vasc Anesth 2020;24:349-54.  Back to cited text no. 5
Fabila TS, Menghraj SJ. One lung ventilation strategies for infants and children undergoing video assisted thoracoscopic surgery. Indian J Anaesth 2013;57:339-44.  Back to cited text no. 6
[PUBMED]  [Full text]  
Bird GT, Hall M, Nel L, Davies E, Ross O. Effectiveness of Arndt endobronchial blockers in pediatric scoliosis surgery: A case series. Paediatr Anaesth 2007;17:289-94.  Back to cited text no. 7
Datt V, Tempe DK. Airway management in patients with mediastinal masses. Indian J Anaesth 2005;49:344-52.  Back to cited text no. 8
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