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Sugammadex in Pediatric Congenital Myasthenia Syndrome
Poster Title: Sugammadex in Pediatric Congenital Myasthenia Syndrome
Submitted on 25 Jun 2018
Author(s): Carie Ann Sirmon, MD, Shivani Mukkamala, MD
Affiliations: Emory University School of Medicine
This poster was presented at Georgia Society of Anesthesiologist Summer Meeting Student Posters Georgia Society of Anesthesiologist Summer Meeting Student Posters
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Poster Information
Abstract: Congenital myasthenic syndrome (CMS) is an inherited neuromuscular disorder caused by a defect in the acetylcholine receptor in the neuromuscular junction. While CMS differs from myasthenia gravis (MG) both in age of onset (pediatric vs. adult) and etiology (congenital vs. autoimmune), both are characterized by 1) reduced acetylcholine activity at the NMJ, and 2) muscle weakness that gets worse with exercise. Due to their baseline restrictive lung pathology and muscle weakness, myasthenic patients are predisposed to respiratory complications following anesthesia. The use of rocuronium is controversial in myasthenic patients. Sugammadex is routinely used in adults and case reports show safety and efficacy in adults with myasthenia gravis. While there is a lack of evidence as to its effectiveness in children, we believe sugammadex offers an attractive alternative to reversal of neuromuscular blockade without the use of anticholinesterases and antimuscarinics.
Summary: Dosing muscle relaxants is complicated in myasthenic patients due to varied responses. This case describes the use of sugammadex in a pediatric congenital myasthenic syndrome patient. After surgery, our patient did not require extra time in the operating room, recovery room, or even an overnight stay in the ICU. Administration of sugammadex resulted in complete reversal of neuromuscular blockade, positive effects on respiratory status, and reduced length of stay in the ICU.References: 1. Benoît Plaud, Olli Meretoja, Rainer Hofmockel, Julien Raft, Peter A. Stoddart, Jacqueline H. M. van Kuijk, Yvonne Hermens, Rajinder K. Mirakhur; Reversal of Rocuronium-induced Meeting Abstracts with Sugammadex in Pediatric and Adult Surgical Patients. Anesthesiology 2009;110(2):284-294. doi: 10.1097/ALN.0b013e318194caaa.
2. Duke, James C., and Brian M. Keech, eds. Anesthesia Secrets. Fifth edition. Philadelphia, PA: Elsevier, Saunders, 2016.
3. Nag, Kusha et al. “Sugammadex: A Revolutionary Drug in Neuromuscular Pharmacology.” Anesthesia, Essays and Researches 7.3 (2013): 302–306. PMC. Web. 2 Mar. 2018
4. Sungur, Ulke, Z., Yavru, A., Camci, E., Ozkan, B., Toker, A. and Senturk, M. (2013), Rocuronium and sugammadex in patients with myasthenia gravis undergoing thymectomy. Acta Anaesthesiologica Scandinavica, 57: 745–748. doi: 10.1111/aas.12123
5. Palace, J., D. Lashley, J. Newsom-Davis, J. Cossins, S. Maxwell, R. Kennett, S. Jayawant, Y. Yamanashi, and D. Beeson. “Cli
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