Background and Aims A 30-year old patient at 39 week gestation,was scheduled for an emergency cesarean section. She had Thomsen myotonia- an autosomal dominant genetic disorder wich affects skeletal muscles leading to delayed relaxation after volountary contraction because it affects the CLCN gene located ond the 7th cromosome which encodes skletal muscle chloride channels. Patients with this desease are sentistive to anesthesia,especially depolarizing muscle relaxants and pregnancydeteriorates patient’s symptomrs so our prefered anesthesia method was neuraxial anesthesia-spinal block.
Methods The patient received the spinal block with 0,5%Bupivacaine combined with 2,5 mcg of Sufentanyl, but the block was inadequate, so we converted to general anesthesia without using any muscle relaxants, just propofol and sevoflurane for maintainamce and for airway management a LMA. After the delivery of the baby, we administered 25 mcg sufentanyl intravenously for the rest of the operation.
Results The c section was performed successfully resulting with a healthy baby, and the mother emerged without complications. For postoperative pain we gave an ultrasound guided Transversus abdominis plane Block using 0,25%Levobupovacaine 20 ml on each side.
Conclusions Thomsen myotonia is an anesthetic management chalenge. Complications are common in these patients especially using depolarizing muscle relaxants.Regional anesthesia is the prefered method for cesarean section. In this case general anesthesia was used without relaxants and the procedure finnished without complications.
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