Anesthetic Implications of a Patient With Kniest Dysplasia and Mitochondrial Disease: A Case Report : A&A Practice

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Anesthetic Implications of a Patient With Kniest Dysplasia and Mitochondrial Disease: A Case Report

Salik, Irim MD; Kubal, Keshar MD; Barst, Samuel MD

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A & A Practice 12(8):p 267-269, April 15, 2019. | DOI: 10.1213/XAA.0000000000000906

Abstract

Kniest dysplasia, or metatropic dysplasia type II, is a rare chondrodysplasia caused by abnormal type II collagen. Clinically, it is characterized by dwarfism, deafness, skeletal derangements, and ocular abnormalities. Mitochondrial diseases produce a spectrum of abnormalities in affected individuals and predominantly impact organs of high energy utilization, including the brain, skeletal muscles, kidneys, and liver. We present the case of a 6-year-old boy with both Kniest dysplasia and underlying mitochondrial disease for examination under anesthesia before cataract surgery. Successful anesthetic management of a patient with Kniest dysplasia and a mitochondrial myopathy is discussed.

Copyright © 2018 International Anesthesia Research Society

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