Anaesthesia Management and Use of Sugammadex in a Patient with Ullrich’s Disease

Abstract

Case Report / Olgu Sunumu Here, we report anaesthesia management and the successful use of total intravenous anaesthesia and sugammadex in a patient with Ullrich's disease. Propofol and remifentanyl infusion was used for anaesthesia. After the end of the surgery, when the train-of-four value was 0%, 4 mg kg −1 sugammadex was administered, and the patient was successfully extubated after 36 s. No adverse effects or safety concerns were observed. In conclusion, we suggest that the use of propofol infusion to avoid the use of inhalation anaesthetics and the use of sugammadex for the reversal of the effects of rocuronium is safe in patients with Ullrich's disease.Keywords: Sugammadex, muscular dystrophy, total intravenous anaesthesia Bu olgu sunumuzda Ullrich hastalığı olan bir hastada başarılı total intravenöz anestezi ve sugammadeks kullanımını bildirmeyi amaçladık. Anestezi idamesi için propofol ve remifentanil infüz-yonu kullanıldı. Cerrahi bitiminde train of four (TOF) değerleri %0 iken, 4 mg kg -1 sugammadeks verildi ve 36. saniyede hasta başarıyla ekstübe edildi. Herhangi bir yan etki ile karşılaşılmadı. Sonuç olarak, Ullrich hastalığı olan hastalarda inhalasyon anestetiklerinin kullanılmasından kaçınmak adına propofol infüzyonun kullanımının ve roküronyumun etkilerinin sonlandırılması için sugammadeks kullanımının güvenli olduğunu bildirmekteyiz.Anahtar kelimeler: Sugammadeks, musküler distrofi, total intravenöz anestezi Abstract / Öz

Perioperative Pulmonary Optimization With Average Volume-Assured Pressure Support of a Pediatric Patient With Ullrich Congenital Muscular Dystrophy: A Case Report

“…For avoidance of narcotic respiratory depression, we chose to use precedex, ketamine, IV acetaminophen, and surgical site local anesthetic infiltration for pain control and sedation. Although use of neuromuscular blockade was avoided in this case, a case report by Erbabacan et al 14 has reported the safe use of rocuronium and sugammadex in a 7-year-old male with Ullrich disease during urologic surgery. Sugammadex has also been used safely in patients with Duchenne and Becker muscular dystrophies.…”

“…Sugammadex has also been used safely in patients with Duchenne and Becker muscular dystrophies. 14 Thus, neuromuscular blockade and reversal with sugammadex may be an option in muscular dystrophies if needed for surgical exposure.…”

Sugammadex: Applications in Pediatric Critical Care

“…Anecdotal experience has demonstrated the use of sugammadex to reverse neuromuscular blockade in patients with neuromyopathic conditions and weakness that may predispose to adverse effects related to the residual effects of NMBAs. [36][37][38][39][40][41] Even with intermediate acting NMBAs such as atracurium and vecuronium, prolonged neuromuscular blockade may occur with a single dose and reversal with acetylcholinesterase inhibitors may be ineffective. These anecdotal reports have included patients with various neuromyopathic conditions including muscular dystrophies (Duchenne, Becker, and Ullrich), myotonic dystrophy, spinal muscular atrophy, and myasthenia gravis.…”

Anesthesia and Ullrich Congenital Muscular Dystrophy: Comment

“…1 This disease is well known to carry a risk of difficult intubation. [2][3][4][5][6] The authors nicely described how they used a nasopharyngeal airway to administer a volatile anesthetic and oxygen through one nostril while performing nasotracheal fiberoptic intubation via the other. I am however surprised that the choice of the anesthetic agent(s) used was not discussed.…”

“…Very few reports on the anesthetic management of patients with this type of myopathy have been published so far and all except two 5,6 report using total intravenous anesthesia. [2][3][4] Carefully titrated intravenous anesthesia to maintain spontaneous ventilation and using either dexmedetomidine, propofol, and/or ketamine could therefore be a safe alternative.This letter was sent to the author of the original article referenced above, who declined to respond.…”