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

Kumar G. Belani, William Krivit, Becky L.M. Carpenter, Elizabeth Braunlin, Joseph J. Buckley, Ji Chia Liao, Thomas Floyd, Arnold S. Leonard, C. Gail Summers, Samuel Levine, Chester B. Whitley

Research output: Contribution to journalArticle

93 Scopus citations

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 ≤ .01) and smaller (12 v 15 kg, P ≤ .05). Preoperative obstructive breathing was associated with a significantly higher incidence of postextubation obstruction (P ≤ .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 C1C2 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.

Original languageEnglish (US)
Pages (from-to)403-410
Number of pages8
JournalJournal of Pediatric Surgery
Volume28
Issue number3
DOIs
StatePublished - Mar 1993

Keywords

  • Hunter syndrome
  • Hurler syndrome
  • Maroteaux-Lamy syndrome
  • Morquio syndrome
  • Mucopolysaccharidosis
  • Sanfilippo syndrome
  • bone marrow transplantation
  • coronary artery disease
  • hydrocephalus
  • obstructive apnea
  • odontoid dysplasia

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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  • Cite this

    Belani, K. G., Krivit, W., Carpenter, B. L. M., Braunlin, E., Buckley, J. J., Liao, J. C., Floyd, T., Leonard, A. S., Gail Summers, C., Levine, S., & Whitley, C. B. (1993). Children with mucopolysaccharidosis: Perioperative care, morbidity, mortality, and new findings. Journal of Pediatric Surgery, 28(3), 403-410. https://doi.org/10.1016/0022-3468(93)90240-L