Anaesthetic problems in Hurler-Scheie syndrome. Report of two cases

Abstract

Hurler-Scheie syndrome is an intermediate form of mucopolysaccharidosis. Affected patients characteristically present in infancy with serious abnormalities including the upper airways and the lungs. We present two patients with Hurler-Scheie syndrome and describe their anaesthetic management. One case was complicated by difficult endotracheal intubation and postoperative pneumonia. The second case was successfully managed using spinal anaesthesia and mild sedation.