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

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Case Report

Volume 6, Number 6, June 2015, pages 257-263

Perioperative Management of a Patient With Glutaric Aciduria

**Table 1.** Previous Reports of Anesthetic Care for Patients With Glutaric Aciduria
Authors and reference	Patient demographics	Intraoperative management	Postoperative problems and management
Ituk et al [24]	23 years for a scheduled cesarean section at 36 weeks’ gestation for marginal placenta previa.	Intravenous infusion of carnitine in 10% dextrose was started to prevent excess protein metabolism. Spinal anesthesia was performed for the cesarean delivery using hyperbaric bupivacaine. A prophylactic phenylephrine infusion was started along with volume loading to maintain blood pressure.	The carnitine and 10% dextrose infusion was continued perioperatively until a regular diet was resumed. No perioperative hemodynamic issues were noted. Male infant delivered without complications. The patient’s plasma carnitine, lactate, and electrolytes were within normal limits 24 h postoperatively. She was discharged home on postoperative day 3.
Teng et al [25]	37-month-old, 19 kg girl with macrocephaly and hypotonia for comprehensive dental surgery for treatment of multiple dental caries.	Anesthesia was induced with atropine, thiamylal (5 mg/kg), and fentanyl (2 µg/kg). Neuromuscular blockade with cis-atracuium (0.2 mg/kg). Maintenance anesthesia with sevoflurane. Blood levels of glucose and lactate, and arterial blood gas were monitored and maintained within normal range. Ketorolac was administered for postoperative analgesia. Neuromuscular blockade was reversed with neostigmine and the patient’s trachea was extubated.	The patient resumed a normal diet 8 h after the procedure and was discharged uneventfully the next day.
Farag et al [26]	11-year-old, 50 kg patient for closure of a perimembranous ventricular septal defect (VSD).	Anesthesia was induced with ketamine and fentanyl. Neuromuscular blockade with rocuronium. Maintenance anesthesia with isoflurane and nitrous oxide. During cardiopulmonary bypass, anesthesia was maintained with intermittent doses of ketamine. Remifentanil infusion throughout and morphine for postoperative analgesia.	During recovery, insulin was administered to maintain normoglycemia and sodium nitroprusside was administered to control blood pressure. The postoperative course was uneventful and the patient was discharged from the hospital on the postoperative day 3.
Hernandez-Palazon et al [27]	Two siblings: 17 and 30 months old sisters for VP shunt placement.	Perioperative dextrose and fluids were administered. Anesthesia was induced with atropine, propofol, and remifentanil. Neuromuscular blockade with rocuronium. Maintenance anesthesia with propofol and remifentanil. Dextrose infusion intraoperatively to prevent hypoglycemia.	The postoperative course was satisfactory and the patients were discharged home 7 days after the procedure.
Goktas et al [28]	Two siblings: 12 and 16 (51 kg) years, presented with macrocephaly and psychomotor delay. Sedation for MRI.	Peripheral intravenous infusion of glucose in normal saline started. Sedation achieved with propofol bolus dosing followed by an infusion. Spontaneous ventilation maintained with supplemental oxygen delivery via a facemask.	The procedures were completed uneventfully. No hemodynamic or respiratory problems were noted. The patients were discharged home 1 h after the procedure.
Tsiotou AG et al [29]	5 years old boy posted for surgery for neurogenic hip dislocation.	Induction of anesthesia with atropine, propofol, and fentanyl. Rocuronium for neuromuscular blockade. Maintenance anesthesia with sevoflurane and a remifentanil infusion. Intravenous paracetamol to supplement postoperative analgesia. D5¼NS was administered throughout the case.	Intravenous paracetamol continued every 6 h following the procedure. Water intake and a specific high carbohydrate solution were started at 2 and 4 h respectively. Discharged home on postoperative day 2.

Table 1. Previous Reports of Anesthetic Care for Patients With Glutaric Aciduria

Authors and reference

Patient demographics

Intraoperative management

Postoperative problems and management

Ituk et al [24]

23 years for a scheduled cesarean section at 36 weeks’ gestation for marginal placenta previa.

Intravenous infusion of carnitine in 10% dextrose was started to prevent excess protein metabolism. Spinal anesthesia was performed for the cesarean delivery using hyperbaric bupivacaine. A prophylactic phenylephrine infusion was started along with volume loading to maintain blood pressure.

The carnitine and 10% dextrose infusion was continued perioperatively until a regular diet was resumed. No perioperative hemodynamic issues were noted. Male infant delivered without complications. The patient’s plasma carnitine, lactate, and electrolytes were within normal limits 24 h postoperatively. She was discharged home on postoperative day 3.

Teng et al [25]

37-month-old, 19 kg girl with macrocephaly and hypotonia for comprehensive dental surgery for treatment of multiple dental caries.

Anesthesia was induced with atropine, thiamylal (5 mg/kg), and fentanyl (2 µg/kg). Neuromuscular blockade with cis-atracuium (0.2 mg/kg). Maintenance anesthesia with sevoflurane. Blood levels of glucose and lactate, and arterial blood gas were monitored and maintained within normal range. Ketorolac was administered for postoperative analgesia. Neuromuscular blockade was reversed with neostigmine and the patient’s trachea was extubated.

The patient resumed a normal diet 8 h after the procedure and was discharged uneventfully the next day.

Farag et al [26]

11-year-old, 50 kg patient for closure of a perimembranous ventricular septal defect (VSD).

Anesthesia was induced with ketamine and fentanyl. Neuromuscular blockade with rocuronium. Maintenance anesthesia with isoflurane and nitrous oxide. During cardiopulmonary bypass, anesthesia was maintained with intermittent doses of ketamine. Remifentanil infusion throughout and morphine for postoperative analgesia.

During recovery, insulin was administered to maintain normoglycemia and sodium nitroprusside was administered to control blood pressure. The postoperative course was uneventful and the patient was discharged from the hospital on the postoperative day 3.

Hernandez-Palazon et al [27]

Two siblings: 17 and 30 months old sisters for VP shunt placement.

Perioperative dextrose and fluids were administered. Anesthesia was induced with atropine, propofol, and remifentanil. Neuromuscular blockade with rocuronium. Maintenance anesthesia with propofol and remifentanil. Dextrose infusion intraoperatively to prevent hypoglycemia.

The postoperative course was satisfactory and the patients were discharged home 7 days after the procedure.

Goktas et al [28]

Two siblings: 12 and 16 (51 kg) years, presented with macrocephaly and psychomotor delay. Sedation for MRI.

Peripheral intravenous infusion of glucose in normal saline started. Sedation achieved with propofol bolus dosing followed by an infusion. Spontaneous ventilation maintained with supplemental oxygen delivery via a facemask.

The procedures were completed uneventfully. No hemodynamic or respiratory problems were noted. The patients were discharged home 1 h after the procedure.

Tsiotou AG et al [29]

5 years old boy posted for surgery for neurogenic hip dislocation.

Induction of anesthesia with atropine, propofol, and fentanyl. Rocuronium for neuromuscular blockade. Maintenance anesthesia with sevoflurane and a remifentanil infusion. Intravenous paracetamol to supplement postoperative analgesia. D5¼NS was administered throughout the case.

Intravenous paracetamol continued every 6 h following the procedure. Water intake and a specific high carbohydrate solution were started at 2 and 4 h respectively. Discharged home on postoperative day 2.