|Year : 2016 | Volume
| Issue : 1 | Page : 29-31
Anesthetic management of a child with autistic spectrum disorder and homocysteinemia
Deepak Choudhary, Ghansham Biyani, Pradeep Kumar Bhatia, Nikhil Kothari
Department of Anesthesiology and Intensive Care, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
|Date of Submission||08-Apr-2016|
|Date of Acceptance||14-Apr-2016|
|Date of Web Publication||17-Jun-2016|
Department of Anesthesiology and Intensive Care, 3rd Floor, OPD Block, All India Institute of Medical Sciences, Jodhpur, Rajasthan
Source of Support: None, Conflict of Interest: None
Autistic spectrum disorder (ASD) is a developmental disability of the central nervous system with rapid worsening. A subset of patients also has mitochondrial dysfunction leading to increased sensitivity to various anesthetic agents. Rarely, gene mutation in these patients results in homocysteinemia which causes higher incidences of thromboembolism, hypoglycemia, and seizures. Anesthetic management of ASD with homocysteinemia and refractory seizures has not been previously reported.
Keywords: Air-Q blocker, autistic spectrum disorder, etomidate, homocysteinemia, regional anesthesia
|How to cite this article:|
Choudhary D, Biyani G, Bhatia PK, Kothari N. Anesthetic management of a child with autistic spectrum disorder and homocysteinemia. Indian Anaesth Forum 2016;17:29-31
|How to cite this URL:|
Choudhary D, Biyani G, Bhatia PK, Kothari N. Anesthetic management of a child with autistic spectrum disorder and homocysteinemia. Indian Anaesth Forum [serial online] 2016 [cited 2020 Nov 30];17:29-31. Available from: http://www.theiaforum.org/text.asp?2016/17/1/29/183573
| Introduction|| |
Children with autistic spectrum disorder (ASD) have stereotypic behavior, poor personal and social communication, and lack of ability to understand. Various metabolic, genetic, and mitochondrial dysfunctions may also be present. Mutation in methylenetetrahydrofolate reductase (MTHFR) gene results in homocysteinemia. We report successful use of etomidate and air-Q blocker for general anesthesia (GA) in combination with regional anesthesia (RA) in a child suffering from autism with homocysteinemia.
| Case Report|| |
A 14-year-old boy, weighing 40 kg, was posted for correction of nonunion fracture neck of the femur which he sustained during an episode of seizure. He was a diagnosed case of ASD and seizure disorder since early childhood and was on oral sodium valproate 500 mg, clonazepam 10 mg, folic acid, Vitamin B1, B6, and B12. He was also taking oral aspirin 75 mg daily. In spite of multiple antiepileptic drug therapy, he frequently had seizure episodes. He was under regular follow-up by a pediatric neurophysician who diagnosed the child to have homocysteinemia and his heterozygosity for MTHFR genetic mutation was established.
Due to ASD, he had developmental delay, lack of social interaction, and abnormal aggressive behavior. He was not cooperative and his speech was difficult to understand. His hemodynamics was stable and systemic examination revealed no significant abnormality, except for increased tone in all the four limbs and contractures at various joints. Routine blood investigations were normal, and homocysteine level was 26.62 μmol/L (normal range 5–15 μmol/L). Magnetic resonance imaging of the brain revealed moderate size infarct in fronto-temporo-parietal area.
The child was put on low-molecular-weight heparin (LMWH) 40 mg subcutaneously once a day which was withheld 12 h prior to surgery. He was prescribed oral alprazolam 0.5 mg and pantoprazole 40 mg night before and on the morning of surgery and allowed to take clear liquids till 6 am. His morning blood glucose level was 126 mg/dl. A 20 gauge intravenous (IV) cannula was secured in the presence of parents, and dextrose-saline solution was started at 60 ml/h. Arterial blood gas analysis revealed raised serum lactate levels (1.9 mmol/L).
In the operation theater, in addition to the standard monitoring, bispectral index (BIS) and neuromuscular monitoring (NMM) were attached. The patient was premedicated with IV midazolam 1 mg, dexamethasone 8 mg, and fentanyl 60 μg. After preoxygenation, anesthesia was induced with IV etomidate 12 mg, and vecuronium 3 mg. The airway was secured with air-Q intubating laryngeal airway (ILA) blocker of size 2.5 and the esophagus was blocked with the use of blocker. Subarachnoid block (SAB) was performed in the lateral position at L3–L4 interspace, and injection bupivacaine heavy 7.5 mg and morphine 100 μg were administered. Pneumatic compression device was applied to the nonoperative limb.
Intraoperatively, anesthesia was maintained using isoflurane in 30% oxygen and air, titrated to BIS value of 50–70. Vecuronium was administered to keep train of four (TOF) count of 3–4. Blood sugar monitoring was done hourly and dextrose-saline infusion continued at 60 ml/h along with Ringer acetate as replacement fluid. The patient remained hemodynamically stable during the surgery which lasted for 3 h. Blood loss was approximately 300 ml. At the conclusion of surgery, neuromuscular blockade was reversed with IV neostigmine and glycopyrrolate and with the return of TOF ratio of 1, air-Q ILA was removed and the patient was shifted to intensive care unit for close observation. All his medications were restarted except for LMWH which was administered the next day. The patient remained pain-free for 24 h. Rest of his hospital stay was uneventful except for episodes of seizures for which he was referred back to pediatric neurophysician.
| Discussion|| |
The prevalence of ASD is on rise, particularly in the Western world. Approximately 7% of these children have associated mitochondrial dysfunction resulting in physical, behavioral, and cognitive impairment. There may be coexisting medical problems such as refractory seizures, tuberous sclerosis, and DiGeorge syndrome. Few children may develop regression of milestones following anesthesia. Random genetic mutations are common, and mutation in MTHFR gene causes abnormal homocysteine-methionine metabolism leading to homocysteinemia. Increased levels of homocysteine interfere with endothelial function leading to thromboembolism and increased insulin secretion causing frequent episodes of hypoglycemia.
Oral midazolam, ketamine, clonidine, and dexmedetomidine are reported to be safe for premedication. We used pantoprazole along with benzodiazepines as these patients are at risk of gastric aspiration. Intraoperatively, BIS and NMM were used as patients with ASD have increased sensitivity to anesthetic agents and muscle relaxants, probably due to mitochondrial dysfunction. Propofol and thiopentone interfere with mitochondrial functions and result in hemodynamic instability, hence we preferred etomidate for induction. Muravchick and Levy  cautioned on the use of propofol infusion in patients with ASD. However, safe use of propofol for induction has been documented in the literature. Nitrous oxide should be avoided as it inhibits the enzyme methionine synthase resulting in increased levels of homocysteine. Isoflurane was preferred over sevoflurane as our patient had refractory seizures and use of sevoflurane may result in increased epileptiform activity. Epidural catheter insertion was planned for postoperative analgesia; however, as the surgeons decided to put hip spica postoperatively, only SAB was performed using morphine for prolonged postoperative analgesia. Use of RA should be considered wherever possible as it decreases the requirement of systemic opioids to which these patients are sensitive and also reduces the incidence of thromboembolism.
Perioperative measures should be adopted to prevent thromboembolism. Pharmacological agents such as antiplatelets and anticoagulants should be considered. Intraoperative use of pneumatic compression device helps to prevent venous stasis. Adequate preoperative hydration, maintaining hemodynamics, and early postoperative mobilization can further prevent such episodes. Most of the measures were taken in our patient as described above.
Refractory seizures increase the risk of aspirations and hence antiepileptic medications should be continued perioperatively. We used air-Q ILA blocker for securing the airway as the anesthesia was maintained by SAB with lighter plane of GA. Endotracheal tube would have required deeper plane of GA. In addition, air-Q ILA blocker also prevents gastric aspiration and serves as a conduit for subsequent endotracheal intubation if required.
To prevent hypoglycemia, the preoperative fasting period should be kept to a minimum and frequent blood sugar monitoring should be done. We administered glucose infusion to maintain blood sugar levels. Ringer acetate was preferred over Ringer lactate as our patients had high serum lactate levels and lactate containing fluids may interfere with serum lactate measurements.
| Conclusion|| |
Patients with ASD have increased sensitivity to anesthetic agents and muscle relaxants. Combination of ASD with homocysteinemia makes anesthetic management even more challenging. Measures to prevent complications such as gastric aspiration, hypoglycemia, and thromboembolism should be addressed. Air-Q ILA blocker is an effective and alternative device to endotracheal intubation and should be considered in patients who are at increased risk for gastric aspiration. Drugs devoid of mitochondrial action and fluids lacking lactate should be preferred over routine ones.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Poling JS, Frye RE, Shoffner J, Zimmerman AW. Developmental regression and mitochondrial dysfunction in a child with autism. J Child Neurol 2006;21:170-2.
Crooke JW, Towers JF, Taylor WH. Management of patients with homocystinuria requiring surgery under general anaesthesia. A case report. Br J Anaesth 1971;43:96-9.
Herbert MR. Autism: A brain disorder or a disorder that affects the brain? Clin Neuropsychiatry 2005;2:354-79.
Oliveira G, Diogo L, Grazina M, Garcia P, Ataíde A, Marques C, et al.
Mitochondrial dysfunction in autism spectrum disorders: A population-based study. Dev Med Child Neurol 2005;47:185-9.
Darlong V, Shende D, Subramanian MS, Sunder R, Naik A. Oral ketamine or midazolam or low dose combination for premedication in children. Anaesth Intensive Care 2004;32:246-9.
Muravchick S, Levy RJ. Clinical implications of mitochondrial dysfunction. Anesthesiology 2006;105:819-37.
Badner NH, Beattie WS, Freeman D, Spence JD. Nitrous oxide-induced increased homocysteine concentrations are associated with increased postoperative myocardial ischemia in patients undergoing carotid endarterectomy. Anesth Analg 2000;91:1073-9.