title: The effect of rocuronium and sugammadex on neuromuscular blockade in a child with congenital myotonic dystrophy type 1.
abstract: Myotonic dystrophy type 1 (MD1) is the commonest muscular dystrophy found in adults; however, it may present in the neonatal period with hypotonia, talipes, poor feeding, and respiratory failure. Inheritance is autosomal dominant with a defect in the DMPK gene found on the long arm of chromosome 19 with variable expansion of the cytosine-thymine-guanine (CTG) triplet repeat. A 14-month-old boy with congenital MD type 1 was scheduled for percutaneous endoscopic gastrostomy (PEG) insertion, orchidopexy, and division of tongue-tie. Following induction of anesthesia, acceleromyography was used to monitor neuromuscular function. This revealed a very rapid onset of profound neuromuscular block which lasted significantly longer than would be expected in a child without MD1. Sugammadex reversed the block rapidly. The anesthetic management of children with MD1 has been well described but not the acceleromyographic monitored use of rocuronium and its subsequent reversal with the new cyclodextrin sugammadex.
authors.affiliation: Department of Anaesthesia, Bristol Royal Hospital for Children, Bristol, UK. ameliapickard@googlemail.com
authors.forename: Amelia
authors.initials: A
authors.lastname: Pickard
authors.forename: Clinton
authors.initials: C
authors.lastname: Lobo
authors.forename: Peter A
authors.initials: PA
authors.lastname: Stoddart
mesh_terms: D000732:Androstanols / Q000037:antagonists & inhibitors; D000758:Anesthesia; D004562:Electrocardiography; D005774:Gastrostomy; D006801:Humans; D007223:Infant; D008297:Male; D016343:Monitoring, Intraoperative; D009213:Myography; D009223:Myotonic Dystrophy / Q000601:surgery*; D019148:Neuromuscular Blockade / Q000379:methods*; D003473:Neuromuscular Nondepolarizing Agents / Q000037:antagonists & inhibitors; D056126:Orchiopexy; D000077123:Rocuronium; D000077122:Sugammadex; D014059:Tongue / Q000601:surgery; D047408:gamma-Cyclodextrins
chemical_list: D000732:Androstanols; D003473:Neuromuscular Nondepolarizing Agents; D047408:gamma-Cyclodextrins; D000077122:Sugammadex; D000077123:Rocuronium
keywords: infant; myotonic dystrophy; neuromuscular blockade; neuromuscular monitoring; rocuronium; sugammadex