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 Table of Contents  
Year : 2020  |  Volume : 3  |  Issue : 1  |  Page : 52-56

Anaesthetic management of a child for excision of extensive facial neurofibroma extending to lower neck with airway compression

Department of Anaesthesiology, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India

Date of Submission08-Apr-2020
Date of Acceptance12-Apr-2020
Date of Web Publication30-May-2020

Correspondence Address:
Dr. Sunil Rajan
Department of Anaesthesiology, Amrita Institute of Medical Sciences, Kochi, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ARWY.ARWY_10_20

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A 7-year-old girl presented with a large facial plexiform neurofibroma extending from the left infraorbital region downwards up to the manubrium sternum with laryngeal compression. The child was posted for excision of the tumour, which measured 30 cm × 20 cm. Following anaesthetic induction with inhalation agents, a videoscope-assisted nasal intubation was attempted but failed. Fibreoptic-assisted intubation also failed due to inability to railroad the tube into the trachea. Finally, a stylet was passed from the oral route into the trachea under videoscopic guidance, and a 5.5 mm ID endotracheal tube was passed over it. The intraoperative period was uneventful. The patient developed stridor on extubation in the intensive care unit (ICU) on the 1st postoperative day. Due to failed reintubation in the ICU and in view of need for a secure airway during the postoperative period, a tracheostomy was performed. Flexible bronchoscopy under general anaesthesia administered through the tracheostomy 1 week later revealed oedematous vocal cords, and hence decannulation was deferred. Repeat bronchoscopy after another week showed near-normal vocal cords, larynx and subglottic region. The child was then decannulated and had an unremarkable recovery.

Keywords: Airway management strategy, compromised airway, extensive facial neurofibroma, fibreoptic intubation

How to cite this article:
Babu KC, Mathew J, Rajan S, Paul J. Anaesthetic management of a child for excision of extensive facial neurofibroma extending to lower neck with airway compression. Airway 2020;3:52-6

How to cite this URL:
Babu KC, Mathew J, Rajan S, Paul J. Anaesthetic management of a child for excision of extensive facial neurofibroma extending to lower neck with airway compression. Airway [serial online] 2020 [cited 2021 Sep 27];3:52-6. Available from: https://www.arwy.org/text.asp?2020/3/1/52/285424

  Introduction Top

Children with large facial plexiform neurofibroma (neurofibromatosis type 1 [NF-1]) presenting for surgery pose significant perioperative challenges to anaesthesiologists due to various reasons. Difficult face mask ventilation and intubation are to be expected due to facial asymmetry and large size of tumour.[1] Difficulty in ventilation could be due to airway collapsibility following administration of neuromuscular blockers with an added risk of tracheomalacia in long-standing cases.[2] As these patients usually undergo multiple surgeries with prolonged postoperative ventilation, the risk of subglottic stenosis necessitating the use of smaller size endotracheal tubes (ETTs) should be anticipated.[3],[4] Because neurofibromas are very friable and vascular, intraoperative haemodynamic instability requiring massive blood transfusion and inotropic support are common.[5]

  Case Report Top

A 7-year-old girl, a known case of plexiform neurofibroma (NF-1), presented with recurrence of extensive facial plexiform neurofibroma extending from the left infraorbital region downwards up to the manubrium sternum. The tumour measured 30 cm × 20 cm [Figure 1]. This child had already been operated twice before in 2016 and 2017 for recurrent neurofibromata. There was a history of postoperative ventilation for 1–2 days following both the earlier surgeries. Following the second surgery, the child underwent 12 cycles of chemotherapy. Despite these measures, the tumour recurred and progressively increased to its current size, causing gross facial deformity. The child was able to talk properly, was taking food orally and had no change in voice. A history of snoring was present, and the child preferred to sleep in the prone position. As there was a fungal infection at the retroauricular area of the tumour with maggot infestation, the family wanted to get it operated at the earliest.
Figure 1: Extensive neurofibroma over the left side of the face and neck showing (a) right lateral view; (b) anterior view and (c) left lateral view

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On examination, the patient had noisy breathing. Airway assessment revealed that mouth opening was restricted to two finger breadths, Mallampati grade 4 with restricted neck extension. Preoperative evaluation showed that routine blood investigations were essentially normal with a haemoglobin content of 14.7 g/dL. Chest X-ray showed the tumour extending downwards on the left side up to the manubrium sternum, pushing the trachea to the right side. Computed tomography (CT) scan of the head and neck revealed the tumour extending from the left temporo-occipital region downwards up to the upper chest wall involving the muscles of mastication, retropharyngeal space, prevertebral and posterior pharyngeal muscles, sternocleidomastoid and strap muscles on the left side. The tumour was seen compressing the larynx and thyroid and pushing it to the right side, indicating a compromised airway [Figure 2]. The left internal jugular vein was compressed, and the left carotid artery was pushed medially. The left ramus of the mandible and condyloid process were not visualised. Chest CT suggested normal mediastinal structures with normal tracheobronchial tree.
Figure 2: Computed tomography‑neck axial cut at the level of vocal cords suggestive of massive tumour compressing and pushing the larynx to the right side

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The child was optimised and adequate blood products were arranged. Standard nil per oral guidelines were followed and high-risk consent. In view of the previous two successful intubations, the plan of anaesthesia was inhalation induction followed by videoscope-assisted nasal intubation, avoiding long-acting muscle relaxants as intraoperative facial nerve monitoring was planned.

On the day of the surgery, the operation theatre was prepared with all the paediatric difficult airway equipment. The C-MAC videolaryngoscope (Karl Storz-Endoskope 8403 ZX, Germany) and paediatric fibreoptic bronchoscope (FOB) were also kept ready. Standard preinduction monitors were attached and inhalational induction was performed with sevoflurane 8% in oxygen. The child was allowed to breathe spontaneously. A check scopy done with C-MAC videolaryngoscope revealed the epiglottis and vocal cords.

Then, glycopyrrolate 0.01 mg/kg, fentanyl 2 μg/kg and propofol 2 mg/kg were given, and after ensuring the ability to perform bag-mask ventilation, succinylcholine 2 mg/kg was administered. After 1 min of mask ventilation, C-MAC-assisted nasal intubation with 5.5 mm ID cuffed endotracheal tube (ETT) was attempted. There was difficulty in manipulating the Magill's forceps through the oropharynx to guide the ETT through the glottis due to lack of space secondary to restricted mouth opening. Even after reaching the level of the vocal cords, there was resistance to pass the tube distally across the glottis despite flexing the head. Intubation was then attempted with a size 5.0 mm ID cuffed nasal RAE tube which also failed. After 8 min of attempts at intubation, the child started to desaturate with the lowest recorded saturation of 70%. Spontaneous respiratory attempts had returned, and the child was ventilated with 100% oxygen through the nasal tube pulled back into the oropharynx. With jaw thrust and one provider occluding the mouth and nose to avoid air leaks, there was good transnasal ventilation with the appearance of capnographic waveform. Saturation also soon picked up to 100%.

As the epiglottis and vocal cords were visible during the initial check videolaryngoscopy and as we were finding it difficult to maintain depth of anaesthesia with the child breathing spontaneously, the decision to paralyse the child was taken especially because mask ventilation was possible earlier. We also suspected that respiratory attempts and efforts at coughing were contributing to the failure to pass the ETT below the level of vocal cords. Hence, the child was paralysed with atracurium 8 mg. Oxygenation and anaesthesia were maintained through a nasopharyngeal airway placed in the opposite nostril which was connected to the breathing circuit and 8% sevoflurane in oxygen administered using positive pressure ventilation. A paediatric FOB (2.9 mm OD) was reintroduced through the ETT. Although it was possible to pass the FOB up to the carina, railroading the ETT failed as there was some obstruction encountered below the vocal cords. Subsequent attempts with a 5.5 mm ID uncuffed nasal RAE tube followed by a 5.0 mm ID RAE tube also failed due to the same reason. Attempts at securing the airway lasted almost 35 min by then and during one attempt, accidental oesophageal intubation resulted in damage to the paediatric FOB.

C-MAC videolaryngoscopy was performed again, and a paediatric stylet with a soft tip was passed through the glottis following which a normal 5.5 mm ID cuffed ETT was railroaded over the stylet and fixed at 16 cm after ensuring bilateral equal air entry and square wave capnographic waveform. Although it took 40 min of repeated attempts to secure the airway, the saturation was always maintained >95% with the help of oxygenation through the nasopharyngeal airway while maintaining continuous jaw thrust. After securing the airway and placement of a bite block, the child was positioned with head tilted to the right side. The ventilator was set to pressure-controlled ventilation (pressure setting of 19 cm H2O which resulted in a tidal volume of 130 mL), respiratory rate of 20 breaths/min and a positive end - expiratory pressure of 5 cm H2O. A mixture of nitrous oxide and oxygen (1:1) with sevoflurane (1.5% to 2%) was used, targeting an end-tidal minimum alveolar concentration of 1.

Three large-bore intravenous (IV) accesses were secured, one each over the right and left saphenous veins and the third over the right cubital region. IV hydrocortisone (50 mg) was given, and dexmedetomidine infusion was started at a rate of 0.5 μg/kg/h. A 22-SWG cannula was placed in the left dorsalis pedis artery.

The haemoglobin dropped to 5.2 g/dL after 1 h of surgery; 300 mL of packed red blood cells were transfused intraoperatively and adequate crystalloid replacement was also given. Intraoperatively, the peak airway pressure spiked on a few occasions, probably due to the pressure on or kinking of the tube as the tumour was very large. These episodes resolved through communication with the surgeon and repositioning of the ETT.

Four hours into surgery, there was sudden hypotension to 60/40 mm Hg. This was caused by bleeding from inadvertent injury to the vertebral artery which was managed with 100 mL of a colloid infusion and a bolus of 50–60 mL of packed red blood cells. An arterial sample drawn at that time showed a haemoglobin of 8.6 g/dL. The tumour weighing 2.6 kg was removed. As a check videolaryngoscopy at the end of surgery showed oedema of left arytenoid, epiglottis and false vocal cords, it was decided to electively ventilate the child. Dexamethasone 2 mg IV was given 8 hourly for 1 day.

On the 1st postoperative day, the child was weaned from mechanical ventilation, was fully awake and taking adequate breaths. The child was extubated and remained stable for 2 h post-extubation after which stridor developed gradually with difficulty to swallow. Repeated attempts at intubation failed, and a tracheostomy was initiated during which the child desaturated to 20% with sudden bradycardia. During tracheostomy, as difficulty was encountered in introducing a 5.0 mm ID ETT, an uncuffed 4.5 mm ID ETT was inserted and ventilation was initiated until the saturation picked up. The ETT was then replaced with a 4.5 mm ID tracheostomy tube. The child was sedated, paralysed and ventilated. Hypothermia with ice packs over the major blood vessels was initiated as a brain-protective strategy. The child woke up after 2 h without any neurological deficit and was weaned off the ventilator and placed on a T-piece for 6 h. She was shifted out of the intensive care unit (ICU) after 5 days and was able to maintain saturation on room air.

A flexible bronchoscopy was performed 1 week later under inhalational anaesthesia administered through tracheostomy. The FOB passed through the oral cavity showed oedematous vocal cords and hence decannulation was deferred. Repeat bronchoscopy after another week showed near-normal cords, larynx and subglottic structures. The child was decannulated successfully at this point and made an unremarkable recovery.

  Discussion Top

Current literature mentions awake fibreoptic bronchoscopy as the gold standard for all patients with large facial neurofibromas.[1] However, in this setting of a 7-year-old child, that option was not possible. As it was an anticipated difficult airway, all attempts at securing the airway were performed by senior anaesthesiologists. Even though the vocal cords and epiglottis were clearly visible at all the attempts, there was difficulty in passing the tube across the vocal cords, eventually leading to damage of the paediatric bronchoscope. The possible causes could be acquired subglottic stenosis due to multiple previous intubations, multiple failed attempts and a history of prolonged ventilation in the past. Compression and pressure effect of the large tumour which was acutely displacing the larynx and trachea to the right side might have also contributed to the difficulty. In view of evidence of a compromised airway in the preoperative CT, a three-dimensional reconstruction could have been done to obtain a virtual bronchoscopic picture for identifying areas of narrowing of the tracheobronchial tree. This would have provided an airway map for easy navigation later.

Nasotracheal intubation was planned on the request of the surgeons as they wanted to assess the symmetry of the face intraoperatively while debulking the tumour. Though we finally secured the airway through the oral route after several attempts at nasotracheal intubation failed, it would have been safer to achieve control of the airway using an orotracheal tube first and then electively switched over to a nasotracheal tube in an unhurried manner.

Extubation on the 1st postoperative day was performed under the assumption that removal of tumour must have relieved the airway compression. An imaging or ultrasonography could have been done to identify any subglottic obstruction. As the child was fully awake, a check scopy to assess airway oedema was not possible. Cuff leak test[6] to rule out tracheomalacia was inconclusive as the child was struggling. Extubation in the operation theatre would have been safer as more equipment and expertise would have been available in the operating room as compared to the ICU. In such cases, extubation over a tube exchanger in the operation theatre would have been a safer and wiser option. Post-extubation stridor could have been due to increased laryngeal oedema, subglottic stenosis or tracheomalacia.[7] This called for bedside emergency surgical tracheostomy. Multiple failed traumatic airway attempts might have resulted in arytenoid, vocal cord and epiglottic oedema, which persisted even after a week and necessitated abandoning the decannulation attempt initially. Resolved airway oedema led to successful decannulation after 2 weeks.

  Conclusion Top

Preoperative CT is useful to assess the diameter of subglottic tissues and grading of the same by Myer Cotton grading.[8] Tracheomalacia should be anticipated with long-standing large tumours, leading to inevitable pressure effects. Extubation in such patients may be attempted after a due course of steroid therapy to reduce airway oedema, which can be ensured by pre-extubation check scopy and a leak test without fail to rule out possible extubation failure. It may be safer to do elective tracheostomy prior to induction of anaesthesia in select patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the parents of the child have given consent for the child's images and clinical information to be reported in the journal. The parents understand that the name and initials of the child will not be published and due efforts will be made to conceal the child's identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Mendonça FT, de Moura IB, Pellizzaro D, Grossi BJ, Diniz RC. Anesthetic management in patient with neurofibromatosis: A case report and literature review. Acta Anaesthesiol Belg 2016;67:48-52.  Back to cited text no. 1
Tripathi D, Kumari I. Tracheomalacia: A rare complication after thyroidectomy. Indian J Anaesth 2008;52:328-30.  Back to cited text no. 2
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Misra S, Gogri P, Misra N, Bhandari A. Recurrent neurofibroma of the orbit. Australas Med J 2013;6:189-91.  Back to cited text no. 3
Eid EA. Anesthesia for subglottic stenosis in pediatrics. Saudi J Anaesth 2009;3:77-82.  Back to cited text no. 4
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Kotamarti VS, Feintisch AM, Datiashvili RO. Large neurofibroma of the face. Eplasty 2015;15:ic36.  Back to cited text no. 5
De Backer D. The cuff-leak test: What are we measuring? Crit Care 2005;9:31-3.  Back to cited text no. 6
Pluijms WA, van Mook WN, Wittekamp BH, Bergmans DC. Postextubation laryngeal edema and stridor resulting in respiratory failure in critically ill adult patients: Updated review. Crit Care 2015;19:295.  Back to cited text no. 7
Myer CM, O'Connor DM, Cotton RT. Proposed grading system for subglottic stenosis based on endotracheal tube sizes. Ann Otol Rhinol Laryngol 1994;103:319-23.  Back to cited text no. 8


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