NM-239
Scoliosis Repair in a Child with Mitochondrial DNA Depletion Syndrome and history of treatment for Malignant Hyperthermia
1Devlin C, 2Kars M
1Zucker School of Medicine at Hofstra Northwell, New Hyde Park, New york, United states; 2Cohen Children's Medical Center, New Hyde Park, NY, United states
Mitochondrial DNA Depletion Syndrome (MDS) is a group of rare disorders characterized by hypotonia, psychomotor retardation, seizures, and lactic acidosis which present within the first year of life.
We describe a 9 year old female who presented for a T11-T12 posterior spinal fusion & T11-L4 posterior vertebral tethering. Her history is significant for MDS, diagnosed via muscle biopsy, Episodic Ataxia Type 1, and essential hypertension. Although she had been exposed to general anesthetics in the past without complication, she recently presented to the hospital one day following an elbow ORIF with muscle rigidity, fever and perioral cyanosis, and was admitted to the PICU for a suspected Malignant Hyperthermia (MH). She was treated with dantrolene and improved clinically.
For her scoliosis repair, anesthetic considerations included both the avoidance of propofol, secondary to her MDS, and MH precautions.
The patient received 0.5mg/kg of PO versed prior to arrival in the OR. In addition, EMLA cream was applied in the holding area for IV placement. In the OR, standard ASA monitors were applied. Mask inhalation of nitrous oxide 50% was used for IV placement with her parent at bedside. IV induction was achieved with 2mg/kg of ketamine and 1mg/kg of rocuronium. A size 5.5 cuffed endotracheal tube followed by an arterial line catheter was then placed without difficulty. Anesthesia was maintained with IV infusion of remifentanil, dexmetetomidine, and ketamine. A baseline ABG at the beginning of the procedure was significant for a pH of 7.28, a base-deficit of -6.3mmol/L, and a sodium bicarbonate of 17. Despite initial treatment with IV fluids and sodium bicarbonate, her pH worsened to 7.08 and a base deficit of -7.4mmol/L, and the CO2 on the final ABG while awake and breathing spontaneously remained 75mmHg. We then decided to keep the patient intubated until electrolytes normalized. She was sedated with dexmetetomidine and transferred to the PICU. She was extubated approximately 12 hours after surgery once her electrolytes and ABG normalized, and remained stable.
MDS is a broad group of metabolic defects which present unique perioperative anesthetic considerations. This disorder most affects the central nervous system, muscles, and cardiovascular system, as well as other high-energy requiring tissues. The primary perioperative concerns include respiratory failure, cardiac depression, and conduction defects. General anesthetics may additionally depress mitochondrial function, most notably volatile anesthetics and propofol.
Despite the avoidance of propofol and MH precautions in our patient, she still demonstrated a profound metabolic acidosis during surgery. We attributed this to the stress response of surgery, in addition to her baseline metabolic acidosis on ABG. The worsening acidosis was self-limited and recovered within 24 hours after surgery. The history of both mitochondrial dysfunction and MH presented a unique complexity to this case.
References
Finsterer, J; Ahting, U "Mitochondrial depletion syndromes in children and adults" The Canadian journal of neurological sciences 40 (5): 635–44
Niezgoda J, Morgan PG. Anesthetic Considerations in Patients with Mitochondrial Defects. Paediatric anaesthesia. 2013;23(9):785-793
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