title: Children with mucopolysaccharidosis: perioperative care, morbidity, mortality, and new findings.
abstract: The perioperative care, morbidity, and mortality in 30 patients with mucopolysaccharidosis (MPS) are presented. They underwent a detailed preoperative assessment and were anesthetized 141 times. An intravenous induction technique was used in most patients. It was easier to see the vocal cords, during laryngoscopy, in children with Hurler syndrome (HS) when they were younger (23 v 41 months, P < or = .01) and smaller (12 v 15 kg, P < or = .05). Preoperative obstructive breathing was associated with a significantly higher incidence of postextubation obstruction (P < or = .05). A total of 28 children underwent bone marrow transplantation (BMT); this reversed upper airway obstruction and also reversed intracranial hypertension. In children with HS, the incidence of odontoid dysplasia was 94%; 38% demonstrated anterior C1-C2 subluxation. Head and neck manipulation was limited in children with cervical spine defects. None of the 30 patients experienced spinal cord morbidity. One child suffered an intraoperative stroke; another, pulmonary edema. Severe and extensive coronary obstruction was responsible for 2 intraoperative deaths. Coronary angiography underestimated coronary artery disease.
authors: Department of Anesthesiology, University of Minnesota, Minneapolis.
authors: K G
authors: KG
authors: Belani
authors: W
authors: W
authors: Krivit
authors: B L
authors: BL
authors: Carpenter
authors: E
authors: E
authors: Braunlin
authors: J J
authors: JJ
authors: Buckley
authors: J C
authors: JC
authors: Liao
authors: T
authors: T
authors: Floyd
authors: A S
authors: AS
authors: Leonard
authors: C G
authors: CG
authors: Summers
authors: S
authors: S
authors: Levine
mesh_terms: D000293:Adolescent; D000328:Adult; D000402:Airway Obstruction / Q000209:etiology; D000770:Anesthesia, Endotracheal; D000771:Anesthesia, Intravenous; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications / Q000401:mortality; D009017:Morbidity; D009083:Mucopolysaccharidoses / Q000150:complications / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D011300:Preoperative Care; D012189:Retrospective Studies; D014139:Tracheostomy