Airway problems in Larsen syndrome

Abstract

Larsen syndrome is a rare congenital connective tissue disorder which is caracterised with a typical facial appearance, and it is neurological and musculosceletal abnormality, Because of this special features, the patients are scheduled to have orthopedic and E-N-T region operations along their lifes. The patients have short stature, small chin, cervical instability that cause to diffucult intubation possibility during the operation. Airway problems caused by lack of rigidity of the upper airway may occur during intubation and extubation phases (1).A 4 month old baby with a know diagnosis of Larsen syndrome and motor retardation was scheduled for bilateral patellar lucsation repair. The baby was evaluated as a case for a difficult intubation before the operation. The intubation was performed with a guidewire at a second attempt. After the difficult intubation there were no problems observed during perioperative and postoperative 24h periods about anesthesia.Larsen syndrome is a rare disorder that needs a perfect preoperative preparation. Therefore it needs to review all alternative intubation techniques of elective difficult airway management.