Perioperative management of a child with glutaric aciduria type I undergoing cardiac surgery

Area of Science:

• Anesthesiology
• Pediatric Cardiology
• Metabolic Disorders

Background:

• Glutaric aciduria type I (GA-I) poses a risk of acute striatal injury, particularly under catabolic stress.
• Cardiac surgery with extracorporeal circulation presents significant perioperative challenges for GA-I patients.

Purpose of the Study:

• To describe the successful interdisciplinary anesthetic and perioperative management of a pediatric patient with GA-I undergoing cardiac surgery.
• To highlight strategies for preventing acute striatal injury in GA-I patients during major surgery.

Main Methods:

• Anesthetic strategy focused on avoiding high protein load and high-dose inotropics, particularly epinephrine.
• Deliberate hyperventilation and interventions triggering systemic inflammatory response were avoided.
• Comprehensive interdisciplinary perioperative care was implemented.

Main Results:

• Successful anesthetic and perioperative management was achieved in a child with GA-I undergoing cardiac surgery.
• The implemented anesthetic strategy aimed to mitigate risks associated with GA-I and cardiac surgery.
• Prevention of acute striatal injury was the central focus of the management plan.

Conclusions:

• Interdisciplinary anesthetic and perioperative management can be successfully applied to GA-I patients undergoing cardiac surgery.
• Careful anesthetic planning, avoiding specific triggers, is crucial for preventing adverse neurological outcomes in these patients.
• This case demonstrates a viable approach to managing complex pediatric surgical cases in the context of rare metabolic disorders.