PMID: 8468655Mar 1, 1993Paper

Children with mucopolysaccharidosis: perioperative care, morbidity, mortality, and new findings

Journal of Pediatric Surgery
K G BelaniS Levine

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.

References

Jan 1, 1991·Annual Review of Biochemistry·E F Neufeld
Apr 1, 1987·Acta Anaesthesiologica Scandinavica·P SjøgrenH Steinmetz
Jan 1, 1988·Canadian Journal of Anaesthesia = Journal Canadien D'anesthésie·I A Herrick, E J Rhine
Jul 1, 1988·Medicine·G L Semenza, R E Pyeritz
Feb 1, 1984·Anaesthesia·D H KingM B Barnett

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Citations

Feb 24, 2012·Journal of Inherited Metabolic Disease·Christian J HendrikszElizabeth Braunlin
Dec 1, 2012·Journal of Inherited Metabolic Disease·Robert WalkerBarbara Valdemarsson
Apr 27, 2005·International Journal of Pediatric Otorhinolaryngology·M A SimmonsM P Rothera
Nov 10, 2009·Bone Marrow Transplantation·P J OrchardJ Tolar
Jan 11, 2012·Rheumatology·Klane K White
Jan 11, 2012·Rheumatology·Kimberly Morishita, Ross E Petty
Mar 1, 1995·Archives of Disease in Childhood·J E Wraith
Nov 23, 2013·Current Rheumatology Reports·Rolando Cimaz, Francesco La Torre
Mar 19, 2011·Journal of Inherited Metabolic Disease·Marleen H van der LindenRené M Castelein
Dec 18, 2015·The Journal of Laryngology and Otology·A KeilmannA K Läßig
Jun 27, 2012·Journal of Cardiovascular Computed Tomography·B Kelly HanJohn R Lesser
Jan 30, 2010·Seminars in Hematology·Vinod K Prasad, Joanne Kurtzberg
Nov 26, 2008·Best Practice & Research. Clinical Rheumatology·Gregory M Pastores
Mar 10, 2016·Paediatric Anaesthesia·Elif C CingiKumar G Belani
Oct 4, 2015·International Journal of Obstetric Anesthesia·C DelgadoR Landau
Sep 6, 2012·Pediatric Pulmonology·Marianne S MuhlebachJoseph Muenzer
Feb 1, 2011·Pediatric Pulmonology·Nicolas LeboulangerBrigitte Fauroux
Jul 13, 2006·International Journal of Pediatric Otorhinolaryngology·Han-Sin JeongDong-Kyu Jin
Jul 22, 1998·The Journal of Pediatrics·C PetersW Krivit
Jun 6, 2014·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Joseph A ChiaroLachlan J Smith
Apr 16, 2008·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·Mieke AldenhovenTom J de Koning
Oct 24, 2006·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·John NeelyLucas Restrepo
Nov 26, 2009·International Journal of Pediatric Otorhinolaryngology·Stephen M WoldVirginia Proud
Aug 18, 2017·Anesthesia and Analgesia·Martina Richtsfeld, Kumar G Belani
Dec 17, 2016·Anesthesia and Analgesia·Benjamin Kloesel, Robert S Holzman
Apr 18, 2002·Bone Marrow Transplantation·S S GrewalC Peters
Jun 12, 2008·American Journal of Medical Genetics. Part a·Taiane VieiraRoberto Giugliani
Feb 22, 2012·European Journal of Anaesthesiology·Wilhlem A OsthausRobert Sümpelmann
Sep 15, 2017·Journal of Children's Orthopaedics·N WilliamsD M Eastwood
Jan 16, 2020·Diagnostics·Paulo Pires de MelloDafne Dain Gandelman Horovitz
Jun 29, 2021·Frontiers in Pediatrics·Nathan GrantSudhakar Vadivelu

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