GA1-51
Anesthetic management of a pediatric patient with Leigh syndrome during magnetic resonance imaging
Rawtani N, Nelson J
Children's National Medical Center, Washington, DC, USA
A 4 year-old male with significant medical history including encephalopathy, hypotonia, and developmental delay presented to the MRI suite for diagnostic evaluation of suspected Leigh syndrome. Leigh syndrome, also known as subacute necrotizing encephalomyelopathy, is a rare progressive neurodegenerative disorder affecting only 1:40,000 and associated with mitochondial DNA mutations in about 20% of those diagnosed with the disorder. Research has led to limited case reports and few reviews discussing the anesthetic management of Leigh syndrome. The purpose of this report is to yield a comprehensive review of pediatric anesthetic management of a patient with suspected Leigh syndrome and particularly management during MRI since this imaging is used in diagnosis of the syndrome.
The patient has a medically complex history including hypoxic injury shortly after birth followed by possible seizures. Past medical history includes encephalopathy, progressive worsening developmental delay, and G tube dependence with increasing intolerance to feeds. The patient had no previous anesthetic record at the hospital but no history of problems with anesthesia per parents. Careful assessment of respiratory and cardiac function was conducted including a full history and exam. Information was limited since the patient has not received care at this hospital previous to the MRI.
He was a cooperative mask induction after the G tube was suctioned and vented. IV placement was difficult but obtained without any changes in hemodynamics during that time. The patient was then intubated and transported from the induction area to the MRI. In the MRI he was maintained on low dose sevoflurane at 0.5 MAC and a dextrose infusion of intravenous fluids. He was then extubated awake and spontaneously ventilating. He was transferred to recovery and discharged after checking glucose and when met criteria with no anesthetic complications.
Leigh syndrome is categorized as part of the mitochondrial myopathies, and patients can have episodes of lactic acidosis and respiratory failure with certain anesthetic triggers. It is important to obtain a comprehensive history and exam in order to understand the extent of the disease and whether the patient has any respiratory, cardiac or other concerns that may increase morbidity. Previous literature suggests avoidance of volatile agents, propofol, lactate containing fluids, and certain neuromuscular blocking agents. However, cases have shown mixed results when using these agents usually resulting in either no complications or respiratory complications. Recommendations of maintenance include Dexmedetomidine or remifentanil infusions. Further discussion should be considered to analyze the utility of small doses of volatile agents and propofol infusions for minor non-stimulating procedures such as a skin biopsy or imaging studies.
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