Anesthetic Care of a Child With Merosin-Deficient Muscular Dystrophy

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,...

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Bibliographic Details
Published in:Journal of medical cases 2020, Vol.11 (5), p.115-119
Main Authors: Munlemvo, Dolly M., Kanaparthi, Anuradha, Tobias, Joseph D.
Format: Article
Language:English
Subjects:
Case Report
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Summary: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.
ISSN:1923-4155
1923-4163
DOI:10.14740/jmc3460