J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

Journal website http://www.journalmc.org

Case Report

Volume 6, Number 9, September 2015, pages 420-425

Anesthetic Care of a Child With Propionic Acidemia

**Table 1.** Previous Reports of Anesthetic Care for Patients With Propionic Acidemia
Authors and reference	Patient demographics	Intraoperative management	Postoperative problems and management
Harker et al [8]	A 4-month-old boy with propionic acidemia for urgent placement of a peritoneal dialysis catheter.	General anesthesia via an ETT. Intraoperative fluids consisted of 5% dextrose in ½NS with 20 mEq KCl/L and 27 mEq NaHCO₃/L at maintenance. The intraoperative care was uneventful during the 2 h procedure. During emergence, the patient exhibited spontaneous respirations with minimal gag in response to suctioning and was transported to the recovery room with the ETT in place.	The patient was admitted to the intensive care unit (ICU) and required mechanical ventilation for 2 days. After tracheal extubation, he immediately developed respiratory distress secondary to decreased clearance of secretions and required reintubation. Tracheal extubation 3 days later was complicated by stridor that responded to racemic epinephrine, heliox, and dexamethasone. Hemodialysis was started uneventfully and the remainder of his hospital stay was uneventful.
Kim et al [9]	Liver transplantation for propionic acidemia in a 14-month-old boy.	Prior to induction, midazolam, glycopyrrolate, and lidocaine were administered. Anesthesia was induced with propofol followed by neuromuscular blockade with pancuronium. Anesthesia was maintained with a sufentanil infusion, midazolam, and N₂O-isoflurane. Sodium bicarbonate was administered to treat acidosis while serum glucose was monitored intraoperatively.	Mechanical ventilation was continued postoperatively.
Karagoz et al [10]	A 2-year-old male with propionic acidemia for percutaneous cystolithotomy.	Induction with sevoflurane in N₂O-O₂. Ten percent dextrose in ⅓NS was administered to prevent hypoglycemia. Vecuronium was used for neuromuscular blockade. Maintenance anesthesia included sufentanil with N₂O-isoflurane. The surgical procedure lasted 30 min and was uneventful. Neuromuscular blockade was reversed by neostigmine and atropine. Bronchospasm and inadequate air exchange occurred immediately after tracheal extubation. Ventilation by face mask was inadequate resulting in hypoxemia and bradycardia. Atropine and tracheal intubation were required.	The patient was admitted to the intensive care unit (ICU) and his trachea was later extubated without complication.
Ryu et al [11]	Liver transplantation in a 22-month-old boy.	Anesthesia was induced with midazolam and thiopental followed by neuromuscular blockade with rocuronium. Maintenance anesthesia included desflurane and fentanyl with a vecuronium infusion. 5% dextrose was infused along with a continuous infusion of sodium bicarbonate. The total duration of operation was 8 h and 51 min.	The postoperative course was complicated by persistent metabolic acidosis and hepatic failure due to hepatic vein obstruction which required re-operation. The patient expired as a result of hepatic failure and subsequent severe metabolic acidosis.
Arcas-Bellas et al [12]	A 27-year-old woman for laparoscopic tubal ligation.	General anesthesia using a supraglottic airway (i-gel^®). Induction with thiopental and remifentanil followed by maintenance anesthesia with sevoflurane and remifentanil. Arterial cannula placed for monitoring. Duration of surgery was 30 min.	The patient progressed satisfactorily and was discharged to home 24 h after surgery.

Table 1. Previous Reports of Anesthetic Care for Patients With Propionic Acidemia

Authors and reference

Patient demographics

Intraoperative management

Postoperative problems and management

Harker et al [8]

A 4-month-old boy with propionic acidemia for urgent placement of a peritoneal dialysis catheter.

General anesthesia via an ETT. Intraoperative fluids consisted of 5% dextrose in ½NS with 20 mEq KCl/L and 27 mEq NaHCO₃/L at maintenance. The intraoperative care was uneventful during the 2 h procedure. During emergence, the patient exhibited spontaneous respirations with minimal gag in response to suctioning and was transported to the recovery room with the ETT in place.

The patient was admitted to the intensive care unit (ICU) and required mechanical ventilation for 2 days. After tracheal extubation, he immediately developed respiratory distress secondary to decreased clearance of secretions and required reintubation. Tracheal extubation 3 days later was complicated by stridor that responded to racemic epinephrine, heliox, and dexamethasone. Hemodialysis was started uneventfully and the remainder of his hospital stay was uneventful.

Kim et al [9]

Liver transplantation for propionic acidemia in a 14-month-old boy.

Prior to induction, midazolam, glycopyrrolate, and lidocaine were administered. Anesthesia was induced with propofol followed by neuromuscular blockade with pancuronium. Anesthesia was maintained with a sufentanil infusion, midazolam, and N₂O-isoflurane. Sodium bicarbonate was administered to treat acidosis while serum glucose was monitored intraoperatively.

Mechanical ventilation was continued postoperatively.

Karagoz et al [10]

A 2-year-old male with propionic acidemia for percutaneous cystolithotomy.

Induction with sevoflurane in N₂O-O₂. Ten percent dextrose in ⅓NS was administered to prevent hypoglycemia. Vecuronium was used for neuromuscular blockade. Maintenance anesthesia included sufentanil with N₂O-isoflurane. The surgical procedure lasted 30 min and was uneventful. Neuromuscular blockade was reversed by neostigmine and atropine. Bronchospasm and inadequate air exchange occurred immediately after tracheal extubation. Ventilation by face mask was inadequate resulting in hypoxemia and bradycardia. Atropine and tracheal intubation were required.

The patient was admitted to the intensive care unit (ICU) and his trachea was later extubated without complication.

Ryu et al [11]

Liver transplantation in a 22-month-old boy.

Anesthesia was induced with midazolam and thiopental followed by neuromuscular blockade with rocuronium. Maintenance anesthesia included desflurane and fentanyl with a vecuronium infusion. 5% dextrose was infused along with a continuous infusion of sodium bicarbonate. The total duration of operation was 8 h and 51 min.

The postoperative course was complicated by persistent metabolic acidosis and hepatic failure due to hepatic vein obstruction which required re-operation. The patient expired as a result of hepatic failure and subsequent severe metabolic acidosis.

Arcas-Bellas et al [12]

A 27-year-old woman for laparoscopic tubal ligation.

General anesthesia using a supraglottic airway (i-gel^®). Induction with thiopental and remifentanil followed by maintenance anesthesia with sevoflurane and remifentanil. Arterial cannula placed for monitoring. Duration of surgery was 30 min.

The patient progressed satisfactorily and was discharged to home 24 h after surgery.