Anaesthesia and orphan disease: rocuronium and sugammadex in the anaesthetic management of a parturient with Becker's myotonia congenita.
2016
Becker's Disease is an autosomal recessive type of myotonia
congenita. Worldwide prevalence is about 1/100000. It is linked
to mutations in CLCN1, the gene encoding skeletal muscle
chloride channel. It reduces flow of chloride ions during
repolarization and leads to sustained muscle contractions.
Typical clinical symptoms are myotonic stiffness and “warm-up”
phenomenon. 27 year old primipara with homozygote recessive
mutation in CLCN1 (c.1437_1450del,p.480HfsX24) was indicated to
elective caesarean section in 40 gestational week. In personal
history she had thoracic stabilisation for scoliosis and
hypothyreosis. We decided to provide the general anaesthesia
with propofol in TCI mode (Schnider mode, C e =effective
concentration 5 mcg/ml) and rocuronium 1 mg/kg IV for rapid
sequence induction, monitoring the depth of neuromuscular
blockade (NMB) on TOF WATCH SX device. The male newborn (APGAR
score 9-10- 10) with no signs of pathology in acid-base balance
in arterial umbilical blood was delivered. At the end of
surgery (C e =1 mcg/ml, TOF=0, PTC=0) we administered
sugammadex 4 mg/kg IV. It takes 2 min and 15 sec to reach TOF
ratio 90% and subsequent extubation. After extubation she was
breathing adequately, communicative, transferred to the
recovery room and after two hours with no signs of residual NMB
or respiratory problems back to the gynaecological ward to the
monitored postoperative room. She was discharged home on 4 th
day. This is the first report of anaesthetic management of a
parturient with Becker's myotonia congenita who underwent CS
under general anaesthesia. In this case we wanted to aware of
using malignant hyperthermia (MH) triggering drugs, though the
association with MH has been regarded as highly unlikely,
suxamethonium which can cause total body rigidity and
subsequent difficult airway management and neostigmine which
can cause myotonic response.
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