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[Anesthetic management for a patient with citrullinemia and liver cirrhosis]
M Igarashi1, S Kawana, H Iwasaki
1Department of Anesthesiology, Sapporo Medical University, School of Medicine.
Insights
This study details successful anesthesia for a patient with hypercitrullinemia and liver cirrhosis undergoing hip surgery. Careful anesthetic management prevented hyperammonemia complications, ensuring a safe procedure.
Area of Science:
- Anesthesiology
- Hepatology
- Medical Biochemistry
Background:
- A 53-year-old female patient presented with hypercitrullinemia and severe liver cirrhosis, leading to chronic hepatic encephalopathy due to hyperammonemia.
- The hyperammonemia stemmed from reduced activity of argininosuccinate synthetase (ASS), a urea cycle enzyme.
- The patient required femoral neck fracture replacement arthroplasty.
Observation:
- Pre-operative consciousness disturbance due to hyperammonemia was managed with sodium benzoate.
- Spinal anesthesia was selected for minimal metabolic impact on the cirrhotic liver.
- Intraoperative prostaglandin infusion maintained hepatic blood flow, and acetated Ringer solution minimized liver metabolic load.
Findings:
- The patient received low-dose fentanyl and midazolam for analgesia and sedation, with antagonists naloxone and flumazenil administered post-operatively.
- Despite a temporary increase in serum ammonia post-surgery, the patient avoided loss of consciousness.
- The surgical procedure and anesthesia were completed without complications.
Implications:
- This case demonstrates the feasibility of managing complex anesthetic procedures in patients with severe liver dysfunction and urea cycle defects.
- The anesthetic strategy highlights the importance of minimizing metabolic burden and managing hyperammonemia proactively.
- Successful outcomes underscore the need for tailored anesthetic protocols in patients with rare metabolic disorders and liver cirrhosis.
Abstract:
We successfully anesthetized a 53-year-old female with hypercitrullinemia and severe liver cirrhosis. The hypercitrullinemia was accompanied with chronic hepatic encephalopathy due to hyperammonemia, which resulted from decreased activity of one of the urea cycle enzymes, argininosuccinate synthetase (ASS). She was scheduled for replacement arthroplasty of a fractured femoral neck. She suffered a consciousness disturbance due to hyperammonemia, which was successfully treated by oral administration of sodium benzoate before surgery. Spinal anesthesia was chosen because it would have the minimum metabolic load on the cirrhotic liver. During the operation, prostaglandin was continuously infused to maintain hepatic blood flow. Acetated Ringer solution was infused instead of lactated Ringer solution to reduce metabolic load on the liver. She was given a small dose each of fentanyl and midazolam for relief of pain and sedation. After the operation, naloxone and flumazenil were administered to antagonize the fentanyl and midazolam, respectively. Although the serum ammonia level temporarily increased during a postoperative interruption of oral administration of sodium benzoate, the patient did not develop loss of consciousness, which is a key sign of hyperammonemia. Surgery and anesthesia were uneventfully completed.