Open AccessAnesthetic Care of a Child With Merosin-Deficient Muscular Dystrophy
Author(s) -
Dolly M Munlemvo,
Anuradha Kanaparthi,
Joseph D. Tobias
Publication year - 2020
Publication title -
journal of medical cases
Language(s) - English
Resource type - Journals
eISSN - 1923-4163
pISSN - 1923-4155
DOI - 10.14740/jmc3460
Subject(s) - medicine , hypotonia , scoliosis , muscular dystrophy , congenital muscular dystrophy , anesthetic , muscle contracture , weakness , anesthesia , muscle weakness , joint contracture , surgery , pediatrics , contracture
Merosin-deficient congenital muscular dystrophy (MDCMD) is a progressive autosomal recessive disorder caused by the lack of expression of the α 2 -chain of laminin-211 glycoprotein. The defect results in skeletal muscle dysfunction with severe muscle weakness, hypotonia, proximal joint contractures, facial dysmorphism, and late or failed ambulation. Given the progressive neuromuscular involvement and the potential for neuromuscular scoliosis, patients frequently require anesthetic care during surgical procedures to correct orthopedic deformities. We present a 9-year-old girl with MDCMD who required anesthetic care during insertion of growing rods to correct neuromuscular scoliosis. Previous reports of anesthetic care for patients with MDCMD are presented and options for perioperative care are reviewed.