title: Difficult airway in a patient with Marshall-Smith syndrome.
abstract: Marshall-Smith syndrome is a rare clinical disorder characterized by accelerated bone maturation, dysmorphic facial features, airway abnormalities and death in early infancy because of respiratory complications. Although patients with Marshall-Smith syndrome have several features with potential anaesthetic problems, previous reports about anaesthetic management of these patients do not exist. We present a case, in which severe hypoxia developed rapidly after routine anaesthesia induction in an eight-month-old male infant with this syndrome. After several unsuccessful attempts the airway was finally secured by blind oral intubation. After 2 weeks, laryngeal anatomy was examined with fibreoptic laryngoscopy which revealed significant laryngomalacia. Laryngoscopy was performed without problems with ketamine anaesthesia and spontaneous breathing. The possibility of a compromised airway should always be borne in mind when anaesthetizing patients with Marshall-Smith syndrome. Anaesthesia maintaining spontaneous breathing is safest for children with this syndrome. If tracheal intubation or muscle relaxation is required, precautions are needed to maintain a patent airway. Muscle relaxants should possibly be avoided before intubation.
authors.affiliation: Department of Anaesthesiology, Turku University, Central Hospital, Finland.
authors.forename: H
authors.initials: H
authors.lastname: Antila
authors.forename: T
authors.initials: T
authors.lastname: Laitio
authors.forename: R
authors.initials: R
authors.lastname: Aantaa
authors.forename: P
authors.initials: P
authors.lastname: Silvoniemi
authors.forename: A
authors.initials: A
authors.lastname: Pakkanen
mesh_terms: D000015:Abnormalities, Multiple; D000769:Anesthesia, Inhalation / Q000009:adverse effects; D001848:Bone Diseases, Developmental / Q000151:congenital*; D005145:Face / Q000002:abnormalities*; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D007223:Infant; D007442:Intubation, Intratracheal / Q000009:adverse effects; D007818:Laryngeal Diseases / Q000151:congenital; D008297:Male; D008876:Middle Ear Ventilation; D012140:Respiratory Tract Diseases / Q000151:congenital*; D013577:Syndrome; D014133:Tracheal Diseases / Q000151:congenital