Anesthetic management in children with Hurler's syndrome undergoing emergency ventriculoperitoneal shunt surgery

  • Gupta N
  • Rath G
  • Bala R
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BACKGROUND/PURPOSE: Mucopolysaccharidosis I (MPS I) is a rare lysosomal storage disorder caused by deficiency of α-L-iduronidase, which results in progressive multisystemic disease. Patients with MPS I often require multiple common and uncommon surgeries and are at risk for surgical and anesthetic complications because of respiratory and cardiac disease. Surgery often precedes diagnosis; thus, surgeons and anesthesiologists may be unaware of potential risks. METHODS: We analyzed data from the MPS I Registry, a voluntary observational database, for deaths occurring within 1 month of a surgical procedure among the 932 patients enrolled as of July 2010. RESULTS: Among the 196 deceased patients, 186 reported 1 surgery or more, and 32 had 1 surgery or more within 1 month of death, including 20 who had 1 surgery or more within 10 days of death. Surgeries before death included hernia repair, central line placement, spinal surgery, tracheostomy, and ventriculo-peritoneal shunt. Most patients (28/32) had severe MPS I (Hurler), and 20 of 32 patients (all Hurler) died at 3 years or younger. In 6 of 32 patients, surgery was directly noted in the cause of death, including 4 patients with an attenuated form of MPS I. CONCLUSIONS: Patients with mucopolysaccharidosis have a high postoperative mortality because of underlying respiratory and cardiac diseases.

Author-supplied keywords

  • Adolescent
  • Adult
  • Cause of Death
  • Child
  • Female
  • Humans
  • Infant
  • Intraoperative Complications
  • Intraoperative Complications: mortality
  • Male
  • Mucopolysaccharidosis I
  • Mucopolysaccharidosis I: complications
  • Mucopolysaccharidosis I: mortality
  • Mucopolysaccharidosis I: surgery
  • Postoperative Complications
  • Postoperative Complications: mortality
  • Preschool
  • Registries
  • Young Adult

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  • N Gupta

  • GP Rath

  • R Bala

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