We describe a patient with severe scoliosis for which corrective surgery was performed at the age of 12. During a previous caesarean section under general anaesthesia pseudocholinesterase deficiency was discovered. Ultrasound guided spinal anaesthesia was performed enabling a second caesarean section under loco-regional anaesthesia.
Pseudocholinesterase deficiency is an enzymatic abnormality resulting in abnormally slow metabolic degradation of exogenous choline-ester drugs such as succinylcholine. This requires prolonged ventilation with concommitant sedation. This is not always appreciated by the patient especially not by parturients with the desire to wake up as soon as possible to take care of their neonate. If combined with factors endangering the success of loco-regional anaesthesia, the management of such a patient becomes a challenge.
A 30-year-old parturient was born with congenital scoliosis, necessitating repeat surgical procedures in childhood and vertebral fusion osteosynthesis, extending up to the vertebral body of L4 (Figure
Radiography of the lumbal spine demonstrating osteosynthetic material until the border of L3.
The surgical procedure was uneventful and a healthy daughter was born with Apgar scores 9 and 9 at 1 and 5 minutes, respectively. At the end of the procedure the patient did not return to spontaneous breathing due to prolonged neuromuscular blockade, and cholinesterase deficiency was suspected. She remained mechanically ventilated for 6 hours after which she resumed spontaneous respiration. Postoperatively cholinesterase was determined in serum with spectophotometry (Vitros 5.1 FS Ortho Clinical Diagnostics) and the value was 1123 U/L (normal 4650–10440 U/L). There were no other complications in the postpartum period.
She consulted again for a second pregnancy three years later. No levels of cholinesterase in the interval between pregnancies were available. At a gestational age of 32 weeks serum cholinesterase level was 1479 U/L. It was decided to try spinal anaesthesia with placement of the needle under ultrasound guidance at the lumbar 4th–lumbal 5th level. At gestational age 39 weeks a repeat caesarean section was planned. As a preload 500 mL, 6% polyhydroxyethylstarch (Voluven, Fresenius Kabi, The Netherlands) was given. Ultrasound (Logiq-E, General Electrics with a 12 MHZ probe) was performed to locate the lower boarder of the osteosynthetic material and after ultrasound determination of the depth of the dura mater and localization of the intervertebral space, a 27-Gauge Whitacre Spinal Needle (Becton Dickinson, Madrid, Spain) was introduced successfully at first attempt in the lumbar interspace L5-S1 and 10 mg levobupivacaine (5 mg/mL) with 5
Pseudocholinesterase deficiency is an autosomal recessive disorder (OMIM 177400, synonyms: butirylcholinesterase deficiency, suxamethomiun sensitivity), different mutations of the gene located on chromosome 3q26 have been reported [
The condition is recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of succinylcholine. The mainstay of treatment is ventilatory supported until diffusion of succinylcholine out of the myoneural junction permits return of neuromuscular function. The diagnosis is confirmed by a laboratory assay demonstrating decreased plasma cholinesterase enzyme activity. Another proposed treatment is prophylactic transfusion of fresh frozen plasma which can augment the patients endogenous plasma pseudocholinesterase activity. This practice is not recommended because of the risk of iatrogenic viral infectious complications. However, perioperative transfusion of fresh frozen plasma administered to correct a coagulopathy may mask an underlying pseudo cholinesterase deficiency [
Different authors have demonstrated lower plasma cholinesterase activity in pregnant patients as compared to nonpregnant women. Blitt et al. could not demonstrate a correlation between plasma cholinesterase activity and duration of paralysis form succinylcholine [
We have been able to find 13 reports on cholinesterase deficiency during caesarean section [
Remifentanil is a rapidly metabolised opioid
We conclude that in patients with pseudo cholinesterase deficiency and difficulties for loco-regional anaesthesia ultrasound guided puncture can provide a possibility for loco-regional anaesthesia previously not possible, in a setting with facilities for ultrasound guided needle placement.