Pseudo Cholinesterase Enzyme Deficiency: Two Rare Cases and Treatment

A deficiency of the enzyme pseudo cholinesterase (PChE) leads to slow hydrolysis of drugs having choline ester structure such as succinylcholine. The patients with this enzymatic disturbance should be provided long-term mechanical ventilation support until the return of normal muscle tonicity.
The current study was performed on two female patients with family relation (cousins). The first operation was an emergent transurethral lithotripsy (TUL), and the second operation was an elective ovarian cystectomy. All operations were performed under general anesthesia. At the end of operations, the patients still suffered from long-time apnea and muscle weakness such that complete muscle relaxation occurred 8 h after one dose of succinylcholine. No additional dose or use of another non-depolarization muscle relaxant was required. The investigation reported a deficiency in PChE. Therefore, the patients were treated with conventional therapies, including FFP injection and newly cross-matched blood (vessel-to-vessel).
PChE deficiency is a familial trait and can occur not only in the first but also in the second generation of a family. The best treatment includes supporting mechanical ventilation and patience until complete succinylcholine hydrolysis by PChE is achieved. It is recommended to avoid using succinylcholine except for emergency situations; instead, depolarization muscle relaxants should be utilized.