A&A practice
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A rare autosomal recessive disorder caused by mutations in the B3GALT6 gene on chromosome 1p36 results in deficiency of β-1,3-galactosyltransferase 6, an enzyme critical for glycosaminoglycan biosynthesis. Defects in this gene result in a phenotype that has features of both skeletal dysplasia and a connective tissue disorder. The anesthetic considerations for children with this disorder have not previously been described. We report a collaborative, multidisciplinary approach to the perioperative care of a child with B3GALT6 mutations with severe phenotypic expression.
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We report a rare case of iatrogenic arterial cannula transection during a dressing change and migration of the distal fragment within the radial artery. The retained intraarterial fragment was successfully located using ultrasound, enabling swift surgical removal. We discuss the risk factors associated with arterial cannula transection and the efficacy of imaging modalities in locating the intraarterial fragment.