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Year : 2021  |  Volume : 4  |  Issue : 1  |  Page : 54-56

Anaesthetic management of subglottic stenosis in granulomatosis with polyangiitis

Department of Anaesthesia, Kokilaben Dhirubhai Ambani Hospital and Research Institute, Mumbai, Maharashtra, India

Date of Submission11-Mar-2021
Date of Acceptance20-Mar-2021
Date of Web Publication29-Apr-2021

Correspondence Address:
Dr. Hemlata Kapoor
Department of Anaesthesiology, Kokilaben Dhirubhai Ambani Hospital and Research Institute, Mumbai - 400 053, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/arwy.arwy_9_21

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Granulomatosis with polyangiitis, earlier known as Wegener's granulomatosis, is an autoimmune vascular disorder in which small- and medium-sized vessels are affected. Anaesthetic management depends on the extent to which various organs are involved. Subglottic stenosis can complicate this autoimmune vascular condition which can become a challenging airway situation for anaesthesiologists, particularly when administering general anaesthesia.

Keywords: General anaesthesia, granulomatosis with polyangiitis, subglottic stenosis, Wegener's granulomatosis

How to cite this article:
Shah M, Kapoor H. Anaesthetic management of subglottic stenosis in granulomatosis with polyangiitis. Airway 2021;4:54-6

How to cite this URL:
Shah M, Kapoor H. Anaesthetic management of subglottic stenosis in granulomatosis with polyangiitis. Airway [serial online] 2021 [cited 2021 Oct 25];4:54-6. Available from: https://www.arwy.org/text.asp?2021/4/1/54/315172

  Introduction Top

Granulomatosis with polyangiitis (GPA) is a systemic autoimmune vascular disorder which was earlier known as Wegener's granulomatosis. It presents as systemic vasculitis involving small- to medium-sized blood vessels. The disease presents initially with symptoms and signs such as persistent rhinorrhoea, weight loss, fatigue, myalgia, fever, persistent upper respiratory tract and ear infections.[1] As the disease progresses, patients may also develop hearing loss, inflammatory lesions of eye, subglottic stenosis, pulmonary infiltrates and glomerulonephritis.

  Case Report Top

A 27-year-old woman, with known Wegener's granulomatosis under treatment, presented to the emergency room with breathlessness. She responded well to treatment including steroids, antibiotics, salbutamol and budecort nebulisations. However, the dyspnoea increased after 3 days.

Past history included recurrent sinusitis, epistaxis and stridor for which the patient received symptomatic treatment, including a tracheostomy. She was started on maintenance steroids, rituximab and steroid nasal drops, and the tracheal stoma closed at a later date. Two years prior, the patient had started developing hearing loss and was currently using a hearing aid. She also gave a history of recurrent conjunctivitis for the past 1 year. For the past 2 months, the patient was having pain in the frontal and maxillary sinuses, breathlessness while speaking and occasional biphasic stridor. Currently, the patient presented with severe dyspnoea. Fibreoptic laryngoscopy revealed subglottic stenosis [Figure 1].
Figure 1: View obtained on fibreoptic laryngoscopy

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The patient was scheduled for microlaryngoscopy and removal of granulation tissue. Laboratory investigations were within normal limits. High-resolution computerised tomography of the chest reported fibroatelectatic changes in the left lung, particularly the lingula and posterior basal segment of the left lower lobe. A two-dimensional transthoracic echocardiography reported normal ejection fraction of 60%, no regional wall motion abnormality and mild mitral and tricuspid regurgitation with a pulmonary artery pressure of 40 mm Hg. Electrocardiogram (ECG) was reported to be normal.

In the operation theatre, standard monitors including 5-electrode ECG, noninvasive blood pressure and arterial oxygen saturation (SpO2) were established. Difficult airway equipment was kept in readiness, and a tracheostomy cart was also kept ready with ENT surgeons standing by if required. In the past, we have used the Hunsaker Mon-Jet tube and jet ventilation for such cases; however, due to non-availability, our first plan was to attempt passing a smaller-sized endotracheal tube (ETT) and remove granulomatous tissue with coblation. Our alternate plan was to perform a tracheostomy before proceeding with definitive surgery. Titrated doses of anaesthetic agents used for induction included midazolam, fentanyl and propofol. Once bag mask ventilation without resistance was confirmed, atracurium was used to induce muscle relaxation. Direct laryngoscopy with a Macintosh blade provided a Cormack-Lehane grade 1 glottic view. However, the subglottic stenosis was severe. The first attempt to intubate with a 5 mm ID uncuffed ETT was unsuccessful. The patient was ventilated with #3 single-use laryngeal mask airway (Ambu® AuraGain™), and then intubation with a 4.5 mm ID cuffed ETT was tried without success. A 4 mm ID uncuffed ETT passed over a paediatric bougie created a snug fit [Figure 2]. The granulomata were removed with coblation [Figure 3]. After surgery was completed, a size 6.5 mm ID cuffed ETT could be placed without difficulty. After completion of surgery, trial extubation was performed uneventfully. The patient was nursed in a propped-up position and was given salbutamol and budecort nebulisation. She was kept under observation for a few hours in the postanesthesia care unit during which time she had an uneventful recovery.
Figure 2: Snuggly-fitting uncuffed 4 mm ID endotracheal tube

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Figure 3: Bronchoscopic view after coblation and removal of granulomatous tissue

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  Discussion Top

GPA is characterised by vasculitis, granulomatous inflammation and necrosis. Inflammation of the mucosa of the upper airway leads to granulation and gradually-progressive stenosis. Subglottic stenosis, reported to be 10%–16%, presents as cough, shortness of breath or stridor.[2] Systemic vasculitis can involve various organs, particularly the lungs and kidneys. During preanaesthetic assessment, pulmonary, renal and cardiac status should be thoroughly evaluated to grade the extent of pulmonary infiltrates and renal glomerular and myocardial involvement due to GPA. Dyspnoea can also be secondary to pulmonary fibrosis or due to cardiac involvement which indicates the chronicity of the GPA.[2]

Preoperative indirect laryngoscopy is a promising technique to assess involvement of larynx and degree of airway narrowing.[3] Quick accessibility to a difficult airway cart is crucial to avoid serious airway events such as hypoxia. We used a laryngeal mask airway in between attempts at intubation to provide adequate ventilation to avoid hypoxia. The laryngeal mask airway has been successfully used for ventilation for surgery in these patients.[4] Placement of an ETT should be gentle and undue force should be avoided as bleeding from granulomatous tissue or a portion of the ulcerated tissue can block the airway.[3] In GPA, obstruction is due to chronic vasculitis and granulomatosis. Hence, mucosal oedema during anaesthesia when surgical intervention to the stenosed area has not been done is uncommon, unlike epiglottitis and similar upper airway pathologies where the oedema might increase intraoperatively.[4] However, one should be watchful for risk of laryngeal swelling after extubation if the stenosed area has been operated.

Anaesthetic drugs which depend on renal excretion should be tailored according to the pre-existing renal function and profile of the patient.[1] Haemodynamic monitoring varies depending on the pre-existing cardiac profile of the patient, particularly chronic effects of hypoxia and hypercapnia leading to varying degrees of pulmonary hypertension and right ventricular dysfunction. As our patient had a good ejection fraction, we did not use invasive blood pressure monitoring.

The Hunsaker Mon-Jet tube is self-centring and provides adequate visual field to the operating surgeon,[5] allowing the anaesthesiologist to support oxygenation and ventilation unhindered. We have noted a rise in end-tidal carbon dioxide at the end of surgery with Hunsaker Mon-Jet tube from our earlier experience; however, this was not noted with the smaller-sized ETT. The heart has compensatory actions for mild hypercarbia and hypoxaemia. However, severe hypercarbia depresses cardiac contractility strongly by decreasing intracellular pH. Hypoxia causes peripheral vasoconstriction and bradycardia. Progressive hypoxaemia decreases cardiac contractility. Therefore, hypercarbia and hypoxaemia should be avoided in patients under anaesthesia.

  Conclusion Top

Although GPA is a rare disease, anaesthesiologists need to be aware of the airway, renal and cardiac effects of the condition. These patients can present with significant airway challenges, and a detailed preoperative airway assessment and appropriate perioperative management with good communication and a multidisciplinary approach involving ENT surgeons are paramount for success.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Dhansura T, Aasthaa, Chauhan S, Husein J, Agrawal P. Wegener's granulomatosis and subglottic stenosis – A case report. Acta Sci Otolaryngol 2020;2:23-5.  Back to cited text no. 1
Ohan F, Tsai E, Zheng-Ward F. A rare case of granulomatosis with polyangiitis and its anesthetic implications. Proc UCLA Health 2019;23.  Back to cited text no. 2
Sharma J, Lal J, Gehlaut P, Amanpreet, Dhawan G, Yadav A. Wegener's granulomatosis and anaesthetic implications: A case report. Int J Med Res Prof 2018;4:479-81.  Back to cited text no. 3
Slater RM, Bhatia K, Woolford TJ, Nirmalan M. Anaesthetic airway management of subglottic stenosis in Wegener's granulomatosis. Br J Anaesth 2011;107. Issue e Letters Supplement 23 December 2011.  Back to cited text no. 4
Davies JM, Hillel AD, Maronian NC, Posner KL. The Hunsaker Mon-Jet tube with jet ventilation is effective for microlaryngeal surgery. Can J Anaesth 2009;56:284-90.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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