Anesthetic management with remimazolam for a pediatric patient with Duchenne muscular dystrophy

Abstract

Rationale: With Duchenne muscular dystrophy (DMD) being the most common and most severe type of muscular dystrophy, DMD patients are at risk for complications from general anesthesia due to impaired cardiac and respiratory functions as the pathological condition progresses. In recent years, advances in multidisciplinary treatment have improved the prognosis of DMD patients, and the number of patients requiring surgery has increased. Remimazolam is a benzodiazepine derivative similar to midazolam. Its circulatory stability and the fact that it has an antagonist make it superior to propofol. There are no reports of pediatric patients with DMD undergoing total intravenous anesthesia with remimazolam. Patient concerns: A 4-year boy was scheduled for single-incision laparoscopic percutaneous extraperitoneal closure for inguinal hernia under general anesthesia, but the surgery was postponed because his serum creatine phosphokinase level was extremely high. Diagnosis: He was diagnosed with DMD. According to the results of the genetic test, exon deletion of the DMD gene was detected using multiplex ligation-dependent probe amplification, although he had no symptoms of DMD except for elevated serum levels of creatine phosphokinase, etc. Intervention: He was admitted for the same surgical purpose. Anesthesia was induced with 3 mg of intravenously administered remimazolam. He lost the ability to respond to verbal commands. After the intravenous administration of 100 μg of fentanyl, a continuous infusion of remifentanil (1.0 μg/kg/min) and remimazolam (15 mg/h) was started, and the endotracheal tube was inserted smoothly after the administration of 10 mg of rocuronium with which the muscle twitches disappeared in train-of-four monitoring. At the end of the surgery, 15 mg of flurbiprofen was administered intravenously. After surgery, we injected 40 mg of sugammadex to confirm a train-of-four count of 100%. Outcomes: Although the dose of remimazolam was reduced to 5 mg/h 30 minutes before the end of the surgery, it took 20 minutes after the discontinuation of remimazolam for the patient to open his eyes upon verbal command. On postoperative Day 2, he was discharged from the hospital without any complications. Lessons: Remimazolam was shown to be safe to use for general anesthesia in a pediatric patient with DMD.

Remimazolam for Sedation During Upper Gastrointestinal Endoscopy in an Adolescent

“…To date, there are only three previous reports regarding the administration of remimazolam in pediatric-aged patients ( Table 1 ) [ 16 - 18 ]. These reports describe the use of remimazolam in combination with remifentanil or propofol for TIVA during surgical procedures with endotracheal intubation, rather than as a primary agent for procedural sedation with a native airway as in our case report.…”

Novel anesthetics in pediatric practice: is it time?

“…Remimazolam, like midazolam, is a potent GABA A receptor agonist [23], which is the hallmark property of many neurotoxic anesthetics [4,[35][36][37][38][39]. Interestingly, there is a report suggesting that remimazolam may negatively affect cognition in the elderly [40 ]. On the basis of the pharmacodynamic properties of remimazolam, there is currently no evidence to suggest it may be any different than other GABA A -agonists with regard to anesthesiainduced neurotoxicity.…”

Remimazolam in a Pediatric Patient With a Suspected Family History of Malignant Hyperthermia

“…The patient did not display any signs of acute MH crisis through the second postoperative day. Additional anecdotal clinical work has suggested the safety and efficacy of remimazolam in various myopathic conditions including myotonic dystrophy and Duchenne muscular dystrophy [18,19]. Similar to MHsusceptible patients, TIVA is generally chosen in these patients due to concerns regarding the administration of volatile anesthetic agents.…”

“…Despite these potential advantages, remimazolam does not hold FDA approval for use in children and, to date, there has been only one previous anecdotal report of its use in a pediatric-aged patient. Horikosi et al reported the use of remimazolam as part of TIVA for a 4-year-old, 16 kg boy with Duchenne muscular dystrophy during inguinal herniorrhaphy and umbilicoplasty [18]. After the intravenous administration of fentanyl (100 µg), continuous infusions of remifentanil (1 µg/kg/min) and remimazolam (15 mg/h) were started.…”