GA3-74
Sugammadex in Pediatric Congenital Myasthenic Syndrome
1Sirmon C, 2Mukkamala S
1Emory University, Decatur, GA, USA; 2Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, USA
Introduction: Congenital myasthenic syndrome can lead to respiratory complications due to muscle weakness. Dosing muscle relaxants can be complicated in myasthenic patients due to varied response to muscle blockers. There is no consensus on whether or not to reverse neuromuscular blockade in patients on chronic anticholinesterase therapy as traditional reversal agents can make it difficult to differentiate residual neuromuscular blockade from cholinergic crisis. Sugammadex offers an attractive alternative to reversal of neuromuscular blockade without the use of anticholinesterases and antimuscarinics. Case studies report the safe use of Sugammadex in adult myasthenia gravis patients; however, its use in pediatric anesthesia has lagged. Here, we present a case of Sugammadex use in a 6-year-old patient with congenital myasthenic syndrome who underwent major orthopedic surgery without the need for postoperative invasive mechanical ventilation or significant respiratory support.
Case: A 6-year-old boy with congenital myasthenic syndrome, DOK7 gene mutation, and bilateral congenital hip dysplasia presented for bilateral varus derotation osteotomies, bilateral acetabular osteotomies, and bilateral soft tissue lengthening. The patient had no history of anesthesia. His medication regimen included Firdapse (3,4-Diaminopyridine/amifampridine phosphate), pyridostigmine and albuterol. He was able to run, go to school, and play with family. At baseline, he required BiPAP on room air at night for sleep.
Anesthesia was induced via standard inhalational induction. A peripheral IV was inserted and fentanyl, propofol, and dexamethasone were given. Pt was easily masked and intubated. An arterial line was placed for close blood pressure monitoring and frequent blood draws. The acute pain team placed an epidural catheter for postoperative pain control. Pt was given rocuronium (0.3mg/kg) that achieved complete relaxation, which was assessed with an ulnar nerve twitch monitor. Patient was redosed twice. At the end of the case, he had return of twitches - 4/4 with fade. He was given Sugammadex 2mg/kg and subsequently had 4/4 twitches, and sustained tetany. He was extubated to home BiPAP and taken directly to intensive care unit (ICU) where he required oxygen via BiPAP for a few hours. Pt was then weaned to room air and transferred out of ICU that day. He remained stable throughout the remainder of his hospital course and was discharged home on postoperative day 3.
Discussion: Due to their baseline restrictive lung pathology, myasthenic patients are predisposed to respiratory complications following anesthesia. Chronic use of anticholinesterases can make relaxation and reversal challenging. Patients are often more sensitive to anesthetics and may require a stay in the ICU for careful monitoring of their respiratory status. These stays can lead to an accumulation of charges for both the patient and hospital. Following surgery, our patient did not require extra time in the operating room, recovery room, or an overnight stay in the ICU. While the cost comparison initially shows glycopyrrolate/neostigmine to be less expensive, the complete reversal of neuromuscular blockade and positive effects on respiratory status justify the cost of the medication.
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