title: PERIOPERATIVE CARE OF A CHILD WITH CRISPONI SYNDROME.
abstract: Crisponi syndrome is an autosomal recessive disorder characterized by intermittent episodes of muscular contraction of the facial muscles with trismus and excessive salivation simulating a tetanic spasm. These episodes occur in response to tactile stimulation or during crying. Associated physical and constitutional findings include characteristic facial anomalies, camptodactyly, intermittent hyperthermia, and feeding difficulties. We present a 15-month-old girl who required anesthetic care during laparoscopic fundoplication and gastric tube insertion. The perioperative implications of the disorder are reviewed and suggestions for anesthetic management provided.
authors.forename: Mahmood
authors.initials: M
authors.lastname: Rafiq
authors.forename: Shadi
authors.initials: S
authors.lastname: Almasry
authors.forename: Anas
authors.initials: A
authors.lastname: Abdulrahman
authors.forename: Mazen
authors.initials: M
authors.lastname: Al-Sohabani
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Tobias
mesh_terms: D000758:Anesthesia; D003645:Death, Sudden; D019066:Facies; D005260:Female; D005334:Fever / Q000503:physiopathology*; D018662:Fundoplication; D006228:Hand Deformities, Congenital / Q000503:physiopathology*; D006801:Humans; D006945:Hyperhidrosis; D007223:Infant; D007441:Intubation, Gastrointestinal; D009119:Muscle Contraction; D019990:Perioperative Care; D014313:Trismus / Q000151:congenital* / Q000503:physiopathology