DIFFICULT AIRWAY IN A PATIENT WITH MARSHALL-SMITH-SYNDROME

Marshall-Smith syndrome is a rare clinical disorder characterized by a ccelerated bone maturation, dysmorphic facial features, airway abnorma lities 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 anaesthet ic management of these patients do not exist. We present a case, in wh ich severe hypoxia developed rapidly after routine anaesthesia inducti on in an eight-month-old male infant with this syndrome. After several unsuccessful attempts the airway was finally secured by blind oral in tubation. After 2 weeks, laryngeal anatomy was examined with fibreopti c laryngoscopy which revealed significant laryngomalacia. Laryngoscopy was performed without problems with ketamine anaesthesia and spontane ous breathing. The possibility of a compromised airway should always b e borne in mind when anaesthetizing patients with Marshall-Smith syndr ome. Anaesthesia maintaining spontaneous breathing is safest for child ren 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.