Anaesthetic management and the role of sugammadex in a patient with Churg-Strauss syndrome.

Sir,

Churg-Strauss syndrome (CSS) is a rare vasculitic disorder affecting multiple organs, characterised by asthma, hypereosinophilia and necrotising vasculitis.[1] Patients are generally aged 20–40 years with no gender predominance. Scarce information is available in the anaesthetic literature, and the role of sugammadex for this group has not been reported. We describe our management of a patient with CSS and the successful use of sugammadex.

A 41-year-old female (96 kg) patient with large fibroid presented for abdominal hysterectomy and salpingo-oophorectomy. She had CSS diagnosed in 2004 with initial sinus involvement, haemoptysis, rash and pericarditis. She had well-controlled asthma and pansinusitis. Pre-operative chest X-ray was normal. Respiratory function test revealed mild obstructive ventilator defect (forced expiratory volume in 1 s 77%, forced expiratory ratio 76%). Transthoracic echocardiogram revealed normal left ventricular size and systolic function. There was mild eosinophilia (2.3 × 109/L), but other blood tests were normal. Since diagnosis, she had been on fluctuating doses of prednisolone, titrated to her flares and ceased when asthma symptoms resolved. On admission, she was on stable daily prednisolone 5 mg, mycophenolate 1 g, and regularly inhaled bronchodilator. After pre-medication with intravenous glycopyrrolate 0.2 mg, magnesium 8 mmol and midazolam 3 mg, anaesthesia was induced with propofol 130 mg, ketamine 20 mg, fentanyl 100 μg, rocuronium 50 mg and lignocaine 80 mg, followed by endotracheal intubation. Hydrocortisone 25 mg was administered after induction. Retrospectively, this could have been omitted as latest evidence suggests she had a low risk of hypothalamic-pituitary-adrenal-axis suppression and did not require perioperative stress-dose steroid.[2] Anaesthesia was maintained with sevofluorane. During surgery, further rocuronium bolus was given to target train-of-four count of '0' using a nerve stimulator for adequate paralysis. Fentanyl 100 μg/h, ketamine 20 mg/h and lignocaine 80 mg/h were infused for analgesia throughout the case (total: fentanyl 300 mcg, ketamine 60 mg and lignocaine 240 mg). Ropivacaine 200 mg was injected into the abdominal walls at the conclusion of surgery. Sugammadex 2 mg/kg (200 mg) was given followed by confirmation of neuromuscular blockade reversal using a nerve stimulator and successful extubation. Postoperatively, regular paracetamol, fentanyl-based patient-controlled analgesia and ketamine infusion of 0.1–0.2 mg/kg/h were commenced for analgesia.

Given the risk of CSS on airway hyperreactivity, anaesthetists should be prepared to prevent and manage a bronchoconstrictive response. We recognise that neuraxial techniques such as spinal or combined spinal and epidural are favourable alternatives, but undertook GA given her disease stability. We gave glycopyrrolate pre-induction to minimise airway secretions. Ketamine and magnesium are both bronchodilatory and analgesic through their actions on the N-methyl-D-aspartate receptors. Intravenous lignocaine attenuates reflex bronchoconstriction and decreases post-operative pain, ileus, hospital stay and nausea and vomiting.[3] We used 1 mg/kg bolus followed by 1 mg/kg/h infusion during the intraoperative period. Sevofluorane has bronchodilatory effects, minimises histamine-induced bronchospasm and decreases airway responsiveness.

We used fentanyl and rocuronium, avoiding histamine-releasing drugs such as morphine and atracurium. Few case reports described caution or complete avoidance of non-depolarising muscle relaxant to avoid using neostigmine and physostigmine as reversals which can cause bradycardia, increased secretions and bronchial hyperreactivity.[45] This strategy may not always be practical as in our case, which required deep paralysis for optimal operating conditions. We used sugammadex to overcome these problems associated with the standard reversal agents. This is advantageous for adequate paralysis throughout the case and subsequent rapid reversal.

Taylor et al. reported decreased plasma cholinesterase activity in two patients with CSS following suspected abnormal sensitivity to suxamethonium.[6] Without information regarding patient's plasma cholinesterase level or activity, caution should be exercised when using suxamethonium or mivacurium as their metabolism relies on this enzyme. Therefore, if a patient requires a rapid sequence induction, but abnormal sensitivity to suxamethonium is to be avoided, rocuronium can be considered the paralytic agent of choice, and sugammadex can be successfully used for reversal of neuromuscular blockade.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.