Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 11, Number 5, May 2020, pages 115-119

Anesthetic Care of a Child With Merosin-Deficient Muscular Dystrophy


Table 1. Reports of Anesthetic Care in Patients With Merosin-Deficient Congenital Muscular Dystrophy
ReferenceStudy cohort or demographicsFindings
TIVA: total intravenous anesthesia; MH: malignant hyperthermia; CVP: central venous pressure.
Scrivener et al [13]Ten children (average age 3.1 years, range 42 days to 11 years) requiring anesthetic care on 16 occasionsVolatile-based anesthetic agent in 13 and TIVA in three. No perioperative complications. No clinical signs of MH.
Shukry et al [20]A 7-year-old boy undergoing posterior spine fusion under with TIVAEndotracheal intubation reported as difficult during previous anesthetic so endotracheal intubation performed with a Shikani optical stylet. At the completion of the case, malignant hyperthermia suspected due to muscle rigidity, increase in body temperature, hypercarbia, metabolic acidosis and hyperkalemia. Treated with dantrolene with eventual resolution of symptoms. Muscle biopsy recommended, but results were not provided.
Jimenez et al [35]A 13-year-old adolescent undergoing posterior spinal fusion with TIVAFollowing prone positioning, hemodynamic instability was noted and the surgical procedure aborted. During second attempt at surgical procedure, following prone positioning, hemodynamic instability occurred again with CVP elevation. Surgery completed in lateral position. Intermittent right ventricular outflow obstruction thought to be cause of hypotension.
Pregardien et al [39]A 2-year-old boy for the placement of an implantable venous access systemBalanced technique with propofol, sevoflurane and alfentanil. Due to concerns of respiratory depression, pressure-support ventilation used intraoperatively.