title: Anesthetic and dental management of a child with IMAGe syndrome.
abstract: IMAGe syndrome (OMIM 300290) is a rare multisystem disorder that has a broad phenotypic presentation. Though variable, this disorder mainly consists of Intrauterine growth retardation, Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital abnormalities. Patients with IMAGe syndrome present as an uncommon yet important challenge for dentists and anesthesiologists due to their wide range of dysmorphic facial features, adrenal insufficiency, electrolyte imbalances, and need for steroid replacement. The purpose of this case report is to describe the successful anesthetic management of a pediatric patient diagnosed with IMAGe syndrome who presented for full mouth dental rehabilitation.
authors.affiliation: Associate Professor of Pediatric Dentistry, University of Pennsylvania School of Dental Medicine, Philadelphia, Pennsylvania, and Director, Pediatric Dental Residency Program, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Rochelle G
authors.initials: RG
authors.lastname: Lindemeyer
authors.forename: Stephanie E
authors.initials: SE
authors.lastname: Rashewsky
authors.forename: Phillip J
authors.initials: PJ
authors.lastname: Louie
authors.forename: Laura
authors.initials: L
authors.lastname: Schleelein
mesh_terms: D000309:Adrenal Insufficiency / Q000150:complications*; D000766:Anesthesia, Dental; D000768:Anesthesia, General; D018685:Anesthetics, Inhalation / Q000008:administration & dosage; D002675:Child, Preschool; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000628:therapy; D003777:Dental Prophylaxis; D003793:Dental Restoration, Permanent; D005260:Female; D005317:Fetal Growth Retardation; D005891:Gingivitis / Q000628:therapy; D006801:Humans; D007442:Intubation, Intratracheal / Q000379:methods; D009065:Mouth Rehabilitation; D010009:Osteochondrodysplasias / Q000150:complications*; D010508:Periodontal Abscess / Q000628:therapy; D014081:Tooth Extraction; D014094:Tooth, Deciduous / Q000473:pathology; D014564:Urogenital Abnormalities / Q000150:complications*
chemical_list: D018685:Anesthetics, Inhalation
keywords: Adrenal hypoplasia; Dental rehabilitation; General anesthesia; IMAGe syndrome