Penile erection during remifentanil anesthesia in children
oxidation of fatty acids (2) This disorder is often diagnosed because it can precipitate hypoglycemia, seizures and coma. Our patient was a 2 year old male who was presented for dental rehabilitation surgery. His past medical history was positive for hypoglycemia and seizures which led to the diagnosis of MCAD. He was being treated with carnitine (Carnitor) with good control. His past surgical history included bilateral myringotomy tubes and tooth extractions of which there were no anesthetic complications. He had a recent history of croup syndrome 3 weeks prior to this admission and was being treated with amoxicillin. As he had been NPO for 8 h prior to arriving at the hospital, an intravenous line was placed and 5% dextrose in lactated Ringer's was started in the day surgery unit. An accucheck done preoperatively revealed a blood glucose of 5.5 mmolAEl (100 mgAEdl) After premedication with 8 mg of oral midazolam, the patient was taken to the operating room where standard ASA monitors including electrocardiogram, noninvasive blood pressure, and pulse oximetry were placed. Anesthesia was induced with propofol and a nasotracheal tube was placed without difficulty and maintained with oxygen, air, and sevoflurane. Following an uneventful intraoperative course his trachea was extubated when he was fully awake. Postoperatively an accucheck revealed a blood glucose of 7.7 mmolAEl (140 mgAEdl) His postoperative course was uneventful and he was discharged home the same day. Clinical symptoms of MCAD often present in children 2 3 years of age and are often precipitated by periods of fasting greater than 12 h and/or viral infections. Vomiting and lethargy can progress to seizures, coma and in extreme cases, cardiorespiratory collapse (1) Liver enlargement may occur secondary to fatty deposition (1) Hypoglycemia is often present during acute episodes as well as low urinary ketone concentrations. A secondary carnitine deficiency may be seen when plasma and tissue concentrations of carnitine are reduced by 25 50% (1) Treatment of acute illnesses includes intravenous fluids containing dextrose. Chronic therapy includes prevention of prolonged fasting, restriction of dietary fat and carnitine (1) Review of the literature reveals no reports of anesthesia in children with MCAD deficiency. Although, treatment with carnitine is controversial and many times ineffective, with our patient carnitine therapy appeared to be therapeutic. MCAD is one of the many acyl CoA dehydrogenases, which are mitochondrial enzymes that are required for beta oxidation of fatty acids (2) The mechanism of hypoglycemia in these patients is evident during periods of starvation when fat becomes the body's major fuel source (1) Although our experience is limited with MCAD patients, we offer the following recommendations for anesthesia management: (i) limit NPO status to 2 4 h; (ii) preoperative intravenous catheter placement; (iii) 5% dextrose in lactated Ringer's solution preoperatively; and (iv) close perioperative blood glucose monitoring.
Paediatric anaesthesia