title: 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.
mesh_terms: D000293:Adolescent; D000768:Anesthesia, General; D000775:Anesthesia, Spinal; D001062:Appendectomy; D005260:Female; D006801:Humans; D007442:Intubation, Intratracheal; D009083:Mucopolysaccharidoses / Q000150:complications / Q000503:physiopathology*; D011183:Postoperative Complications; D013577:Syndrome