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.
From the Department of Pediatric Anesthesiology at Westchester Medical Center, New York Medical College, Valhalla, New York.
Accepted for publication August 28, 2018.
The authors declare no conflicts of interest.
Address correspondence to Keshar Kubal, MD, Department of Pediatric Anesthesiology at Westchester Medical Center, New York Medical College, 100 Woods Rd, Valhalla, NY 10595. Address e-mail to firstname.lastname@example.org.