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CASE REPORT Table of Contents  
Ahead of print publication
Awake bronchoscopy-guided nasotracheal intubation in a child with treacher collins syndrome and obstructive sleep apnoea


 Department of Anaesthesiology and Critical Care, Valluvanad Hospital Complex LTD, Ottapalam, Kerala, India

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Date of Submission13-Jul-2022
Date of Acceptance08-Oct-2022
Date of Web Publication03-Nov-2022
 

  Abstract 


Flexible videobronchoscopy-guided awake intubation is the standard of care in adult patients with negligible mouth opening presenting for corrective surgeries. Although paediatric and neonatal flexible bronchoscopes are available, they are seldom used for awake intubation due to a lack of cooperation from children. A highly sensitive airway coupled with an increased risk for bronchospasm in children precludes attempts in performing bronchoscopy without any sedation. As a result, elective tracheostomy is often performed in such children with anticipated difficult airways. Our report describes the rationale, procedure and implications of videobronchoscopy-guided nasotracheal intubation under conscious sedation in a 5-year-old child with Treacher Collins syndrome or mandibulofacial dysostosis complicated by obstructive sleep apnoea.

Keywords: High-flow nasal cannula, obstructive sleep apnoea syndrome, paediatric awake bronchoscopy, rescue tracheostomy, temporomandibular joint ankylosis, Treacher Collins syndrome


How to cite this URL:
Elayat A, Krishnan V, Reddy RS, Dhaneesh CP, George S. Awake bronchoscopy-guided nasotracheal intubation in a child with treacher collins syndrome and obstructive sleep apnoea. Airway [Epub ahead of print] [cited 2022 Nov 28]. Available from: https://www.arwy.org/preprintarticle.asp?id=360438





  Introduction Top


Treacher Collins syndrome (TCS) is a developmental disorder characterised by unique facial traits acquired as a result of poorly developed first and second pharyngeal arches, groove and/or pouch with a prevalence of 1 in 25,000 to 50,000 live births.[1] Temporomandibular joint (TMJ) ankylosis is one of the few surgical problems which progresses with age requiring corrective surgery by adolescence. With the availability of flexible bronchoscopes, these patients undergo awake bronchoscopy-guided nasotracheal intubation for undergoing surgery under general anaesthesia (GA). Rarely, the fusion of these joints may be accelerated resulting in children with almost no mouth opening with associated feeding difficulties, making it a semi-urgent indication for corrective surgery.

Due to difficulty in a bag and mask ventilation, these patients are often tracheostomised electively with great difficulty ensuring airway security and facilitating surgery under GA. Although lifesaving in the event of a cannot ventilate – cannot oxygenate scenario, paediatric tracheostomy is almost impossible to perform without sedation and carries a significantly higher risk of morbidity and mortality.[2] We describe bronchoscopy-guided nasotracheal intubation of a 5-year-old boy with severe TMJ ankylosis and obstructive sleep apnoea (OSA) syndrome. Written informed consent was obtained from the parents before writing this report. This manuscript adheres to applicable EQUATOR guidelines.


  Case Report Top


A 5-year-old boy weighing 13 kg was admitted with complaints of progressive difficulty in feeding, talking and repeated apnoeic episodes during sleep for the past 3 years. Airway assessment revealed an interincisor gap of 0.4 cm with restricted tongue protrusion and a hypoplastic mandible [Figure 1]. A detailed preoperative evaluation including two-dimensional transthoracic echocardiography and a sleep study with fluoroscopy [Video Loop 1] [Additional file 1] was done which revealed severe joint fusion as well as a moderate degree of OSA.[3]
Figure 1: (a) Frontal view of the child demonstrating restricted mouth opening of 0.4 cm; (b) Child demonstrating maximum tongue protrusion; (c) Side profile of the child showing hypoplastic mandible

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After a multidisciplinary team meeting, the decision was taken to attempt bronchoscopy-guided nasotracheal intubation with rescue tracheostomy backup. The anaesthetic plan was formulated with clearly defined abort criteria for bronchoscopy and all essential equipment including a cuffed 4.5 mm tracheostomy tube was procured beforehand.

The parents were informed about the procedure and the risks involved. Our team visited the patient regularly during the preoperative period, familiarising the child with different airway equipment and nasopharyngeal airways. Xylometazoline nasal drops were administered into both nostrils eighth hourly to decongest the nasal passages.

On the day of surgery, the patient was brought to the preoperative holding area, a 22 SWG intravenous cannula was placed and 0.05 mg glycopyrrolate was administered intravenously. The patient was then given nebulisation with 3 mL of 2% lignocaine-adrenaline for 15 min. The patient was successfully taken into the operation theatre and a well-lubricated 4.5 mm nasopharyngeal airway was placed through the left nostril. Standard monitors consisting of an electrocardiogram, noninvasive blood pressure and pulse oximeter were attached and the surgical team was scrubbed and ready to perform a tracheostomy.

Ketamine 0.25 mg/kg was administered and humidified oxygen through a high-flow nasal cannula (HFNC) was attached to the nasopharyngeal airway with a flow rate of 25 L/min (2L/kg) and an FIO2 of 1.0 (Airvo™ 2, Fisher and Paykel Healthcare Ltd, East Tamaki, New Zealand). An additional 0.25 mg/kg of ketamine was administered and a well-lubricated paediatric bronchoscope (Ambu aScope 4 Broncho Slim, Denmark) was negotiated through the right nostril. The routine technique of spraying lignocaine on the vocal cords was avoided due to a high risk of laryngospasm.[4] Instead, a prophylactic dose of succinylcholine 0.1 mg/kg was administered to prevent airway spasm while still allowing spontaneous ventilation.[5]

An additional 0.25 mg/kg of ketamine was administered and the bronchoscope was negotiated without difficulty into the trachea. Propofol (30 mg) was administered and a preloaded 4.5 mm cuffed endotracheal tube was railroaded over the bronchoscope and advanced through the right nostril until it was placed above the carina under vision [Video Loop 2].[Additional file 2] Anaesthesia was induced and the patient was paralysed after confirming the capnography trace. No laryngospasm or bronchospasm was encountered during this manoeuvre. The surgery proceeded uneventfully. Mouth opening improved following the procedure to an interincisor distance of 3.2 cm [Figure 2]. The nasopharyngeal airway that was placed at the time of anaesthetic induction was still in situ and appropriate-sized supraglottic airways were kept ready for an emergency. The patient was extubated smoothly on the table. The postoperative period was uneventful and airway obstruction was also relieved to a significant extent due to more space now available following the release of TMJ ankylosis.
Figure 2: (a) Postoperative picture showing improved mouth opening; (b) Improved interincisor distance of 3.2 cm; (c) Intraoperative photo showing incision site

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  Discussion Top


Recommendations for diagnostic paediatric airway endoscopy have been published but a clear-cut anaesthesia protocol encompassing the entire spectrum of cases is still lacking.[6] The near-zero mouth opening with hypoplastic mandible complicated by OSA presenting at such a young age made airway management a unique challenge compared to other cases of TCS described earlier.[7],[8]

Achieving bag and mask ventilation proved difficult due to abnormal facial contour. Hence every effort was made to preserve spontaneous ventilation as far as possible. Since there was no scope for rescue ventilation using any supraglottic airway devices, the only available choices were elective tracheostomy or bronchoscopy-guided intubation. Since the complications of paediatric tracheostomy are high, an attempt was made to spare the child from the same, and bronchoscopy was performed while a rescue team was on standby to perform a tracheostomy.

The focus of the entire anaesthetic was on two-key elements – the first was to preserve spontaneous ventilation until the bronchoscope was introduced just above the carina and the second was to prevent bronchospasm while negotiating the bronchoscope in between the vocal cords while the patient was still breathing spontaneously. The first objective was achieved using a subanaesthetic dose of ketamine to maintain conscious sedation while supplementing humidified oxygen with the help of HFNC at 25 L/min (2 L/kg).[9] The cyclical airway collapse on account of OSA was reduced to some extent as a result of the placement of a 4.5 mm nasopharyngeal airway and continuous positive airway pressure (CPAP) generated by HFNC.[10],[11]

The low level of CPAP of 2.7-7.4 cm H2O generated by the HFNC reduced the nasopharyngeal dead space and airway resistance while improving alveolar recruitment and work of breathing. Its role in extending the safe apnoea period during airway instrumentation has been demonstrated in previously published literature in both adults and children. Excessive hypercapnoea detected at the end of the procedure indicated the limitation of HFNC in carbon dioxide clearance especially in children, partly due to maximum allowable flow rates in children (2 L/kg) and also due to higher metabolic demands in children.

The second objective was accomplished by a carefully timed prophylactic administration of 0.1 mg/kg of succinylcholine about 30-40 seconds before negotiating the bronchoscope through the glottis.[12] As a result, there was neither drop in saturation nor any event of bronchospasm despite a longer duration (nearly 14 min) for the entire procedure.

Clearly defined abort criteria were established before starting the anaesthetic and the following were ensured in that direction. A cuffed tracheostomy tube was procured beforehand and an experienced paediatric surgeon was scrubbed and ready with important surgical landmarks marked for immediate identification in case of emergency to perform a tracheostomy. The desaturation limit was set to 85% below which no further drop in saturation was entertained and a rescue tracheostomy was to be done. The pharmacological endpoint was defined as the upper limit of dosage of drugs, particularly ketamine which was limited to 0.8 mg/kg, exceeding which the primary plan was to be abandoned and the procedure would either be aborted or rescue tracheostomy performed.

While it is always advisable to play safe while handling paediatric airways, in the light of overwhelming evidence and success rates in paediatric bronchoscopy and increasingly convincing results with HFNC in the operative setting, an argument against tracheostomy for the provision of GA is no longer irrelevant. In conclusion, a well-planned and rehearsed anaesthetic strategy catering to specific patient requirements with an emergency backup made airway management in this case successful.

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 identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Goel L, Bennur SK, Jambhale S. Treacher-Collins syndrome – A challenge for anaesthesiologists. Indian J Anaesth 2009;53:496-500.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Watters K, O'Neill M, Zhu H, Graham RJ, Hall M, Berry J. Two-year mortality, complications, and healthcare use in children with medicaid following tracheostomy. Laryngoscope 2016;126:2611-7.  Back to cited text no. 2
    
3.
Kapur VK, Auckley DH, Chowdhuri S, Kuhlmann DC, Mehra R, Ramar K, et al. Clinical practice guideline for diagnostic testing for adult obstructive sleep apnea: An American Academy of Sleep Medicine Clinical Practice Guideline. J Clin Sleep Med 2017;13:479-504.  Back to cited text no. 3
    
4.
Hampson-Evans D, Morgan P, Farrar M. Pediatric laryngospasm. Paediatr Anaesth 2008;18:303-7.  Back to cited text no. 4
    
5.
Chung DC, Rowbottom SJ. A very small dose of suxamethonium relieves laryngospasm. Anaesthesia 1993;48:229-30.  Back to cited text no. 5
    
6.
Schramm D, Freitag N, Nicolai T, Wiemers A, Hinrichs B, Amrhein P, et al. Pediatric airway endoscopy: Recommendations of the Society for Pediatric Pneumology. Respiration 2021;100:1128-45.  Back to cited text no. 6
    
7.
Hosking J, Zoanetti D, Carlyle A, Anderson P, Costi D. Anesthesia for Treacher Collins syndrome: A review of airway management in 240 pediatric cases. Paediatr Anaesth 2012;22:752-8.  Back to cited text no. 7
    
8.
Kim JS, Park SY, Min SK, Kim JH, Lee SY, Moon BK. Awake nasotracheal intubation using fiberoptic bronchoscope in a pediatric patient with Freeman-Sheldon syndrome. Paediatr Anaesth 2005;15:790-2.  Back to cited text no. 8
    
9.
Drake MG. High-flow nasal cannula oxygen in adults: An evidence-based assessment. Ann Am Thorac Soc 2018;15:145-55.  Back to cited text no. 9
    
10.
Renda T, Corrado A, Iskandar G, Pelaia G, Abdalla K, Navalesi P. High-flow nasal oxygen therapy in intensive care and anaesthesia. Br J Anaesth 2018;120:18-27.  Back to cited text no. 10
    
11.
Humphreys S, Lee-Archer P, Reyne G, Long D, Williams T, Schibler A. Transnasal humidified rapid-insufflation ventilatory exchange (THRIVE) in children: A randomized controlled trial. Br J Anaesth 2017;118:232-8.  Back to cited text no. 11
    
12.
Gavel G, Walker RW. Laryngospasm in anaesthesia. Contin Educ Anaesth Crit Care Pain 2014;14:47-51.  Back to cited text no. 12
    

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Correspondence Address:
Anirudh Elayat,
Department of Anaesthesiology and Critical Care, Valluvanad Hospital Complex LTD, Kanniyampuram, Ottapalam - 679 104, Kerala
India
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Source of Support: None, Conflict of Interest: None



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