title: The anesthetic management of a patient with Dorfman-Chanarin syndrome.
abstract: Dorfman-Chanarin syndrome (DCS), which is also known as neutral lipid storage disease, is a rare autosomal recessive inherited lipid storage disease with congenital ichthyotic erythroderma. Since the Dorfman-Chanarin syndrome is a multisystemic disease the choice of drugs and the conduct of anesthesia in these patients are important. Preoperative evaluation should be performed in detail and anesthetic method and drugs to be used should be chosen carefully in accordance with affected system, overall state of patient and characteristics of the operation, in order to decrease perioperative morbidity rates in these patients. We report the anesthetic management of a child with DCS operated for correction of strabismus under general anesthesia.
authors.affiliation: Firat University School of Medicine, Department of Anesthesiology and Reanimation, 23119-Elazig, Turkey. abestas@firat.edu.tr
authors.forename: Azize
authors.initials: A
authors.lastname: Beştaş
authors.forename: Esef
authors.initials: E
authors.lastname: Bolat
authors.forename: Mustafa K
authors.initials: MK
authors.lastname: Bayar
authors.forename: Omer L
authors.initials: OL
authors.lastname: Erhan
mesh_terms: D000768:Anesthesia, General / Q000379:methods*; D002675:Child, Preschool; D004562:Electrocardiography; D005260:Female; D006801:Humans; D016113:Ichthyosiform Erythroderma, Congenital / Q000150:complications*; D008052:Lipid Metabolism, Inborn Errors / Q000150:complications*; D016343:Monitoring, Intraoperative; D009135:Muscular Diseases / Q000150:complications*; D013508:Ophthalmologic Surgical Procedures; D013285:Strabismus / Q000601:surgery