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G6PD Deficiency with Arnold-Chiari Malformation

Shilpi Verma1, Pradeep Kumar Bhatia1, Vandana Sharma1

  • 1Department of Anaesthesia, All India Institute of Medical Sciences (AIIMS), Jodhpur, India.

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
|December 17, 2016
PubMed

Insights

This case study details the anesthetic management of a neonate with glucose-6-phosphate dehydrogenase deficiency and Arnold-Chiari Malformation undergoing meningomyelocele repair. Careful anesthetic choices were made to avoid hemolytic complications in this high-risk infant.

Area of Science:

  • Neonatal Anesthesiology
  • Pediatric Neurosurgery
  • Clinical Genetics

Background:

  • Glucose-6-phosphate dehydrogenase (G6PD) deficiency presents a risk of hemolysis with oxidative stress.
  • Arnold-Chiari Malformation (ACM) type 2 involves altered cranio-spinal pressure dynamics.
  • Lumbar meningomyelocele (MMC) repair is a complex neonatal surgical procedure.

Observation:

  • A neonate presented with both G6PD deficiency and ACM type 2.
  • The infant required surgical repair of a lumbar meningomyelocele.
  • Anesthetic management aimed to mitigate risks associated with G6PD deficiency and ACM.

Findings:

  • General anesthesia was induced and maintained using propofol.
  • Fentanyl and an oxygen-nitrous mixture were utilized as adjuncts.
  • The anesthetic plan focused on avoiding oxidative agents to prevent hemolysis.

Implications:

  • This case highlights the importance of tailored anesthetic protocols for neonates with co-existing G6PD deficiency and ACM.
  • Safe anesthetic techniques are crucial for preventing hemolytic reactions during surgical repair in these patients.
  • Further research into anesthetic management for complex pediatric cases with rare genetic conditions is warranted.

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