G6PD Deficiency with Arnold-Chiari Malformation

J Coll Physicians Surg Pak. 2016 Nov;26(11):931-933.

Abstract

A neonate with glucose-6-phosphate dehydrogenase (G6PD) deficiency and Arnold-Chiari Malformation (ACM) type 2 underwent lumbar meningomyelocele (MMC) repair. Patients with G6PD deficiency are prone to develop haemolysis following any kind of oxidative stress and in ACM, there is a disturbed cranio-spinal pressure relationship. The neonate was managed under general anaesthesia with propofol for induction as well as for maintenance along with fentanyl and oxygen-nitrous mixture.

Publication types

  • Case Reports

MeSH terms

  • Anesthesia / methods*
  • Anesthetics, Inhalation
  • Anesthetics, Intravenous
  • Arnold-Chiari Malformation / physiopathology
  • Arnold-Chiari Malformation / surgery*
  • Cerebral Ventriculitis
  • Decompression, Surgical*
  • Fentanyl / administration & dosage
  • Glucosephosphate Dehydrogenase Deficiency / blood*
  • Hemolysis
  • Humans
  • Hydrocephalus / surgery*
  • Infant, Newborn
  • Male
  • Meningomyelocele / physiopathology*
  • Meningomyelocele / surgery*
  • Nitrous Oxide
  • Oxidative Stress
  • Oxygen
  • Propofol / administration & dosage
  • Treatment Outcome

Substances

  • Anesthetics, Inhalation
  • Anesthetics, Intravenous
  • Nitrous Oxide
  • Oxygen
  • Fentanyl
  • Propofol