Propofol administration in patients with methylmalonic acidemia and intracellular cobalamin metabolism disorders: a review of theoretical concerns and clinical experiences in 28 patients

Abstract

Background

Methylmalonic acidemia and intracellular cobalamin metabolism disorders represent a heterogeneous group of inborn errors of metabolism. Most patients will require diagnostic and/or therapeutic procedures frequently requiring sedation or anesthetic management due to neurological and neurocognitive impairments. It has been stated that propofol is contraindicated in this population. We report our experience with propofol administration in a large series of patients.

Methods

28 patients (14 mut, 7 cblC, 3 cblA, 3 cblB, 1 cblG) aged 2–35.6 years enrolled in a natural history study ( ClinicalTrials.gov identifier: NCT00078078) and required anesthetics for 39 diagnostic or therapeutic procedures. Data were collected on the anesthetic technique, perianesthetic course and adverse events related to propofol.

Results

Propofol was used as the sole induction agent in most cases (36/39) and as the primary maintenance agent in all cases. Infusion rates were 100–400 mcg.kg ⁻¹.min ⁻¹ (mean=214). Infusion duration was 60–325 min (mean=158) and total doses ranged between 270–3610 mg (mean=1217). Adverse events were recorded in 2 cases; neither appeared to be related to propofol administration.

Conclusions

Propofol is an effective, safe induction and maintenance agent for elective short procedures requiring anesthesia in patients with MMA and cobalamin metabolism disorders. Despite multiple comorbidities and propensity toward instability, those affected can receive anesthesia with an acceptable safety profile, if metabolically and hemodynamically stabilized prior to the event.