Anaesthetic Challenges and Difficult Airway Management in Noonan Syndrome

Abstract

Noonan’s syndrome was first described by Noonan and Ehmke in 1963. These patients have facial, cardiovascular, musculoskeletal and developmental abnormalities which may pose problems to the anaesthetist during surgery. It is a rare clinical entity representing the phenotype of Turner’s syndrome with normal chromosome studies. The most common congenital cardiac lesion is pulmonary stenosis, either alone or in combination with a septal defect, which is usually atrial. The potential anaesthetic problems presented by a patient with Noonan’s syndrome relate to impairment of cardiopulmonary function, the possibility of a difficult airway and the problem of technical difficulty with regional anaesthesia We reported a case of 21 years old male, weighing 30 kg, who was diagnosed clinically as a case of Noonan syndrome and had undergone balloon pulmonary valvotomy in past. Patient had severe kyphoscoliosis. This rare case report represents to the successful difficult airway and anaesthetic management due to existence of kyphoscoliosis. Problem with airway management may be fatal for patients.