Anaesthesia challenges of a parturient with paramyotonia congenita and terminal filum lipoma presenting for labour and caesarean section under epidural anaesthesia – a case report

Abstract

Background Paramyotonia congenita is a rare autosomal dominant myopathy which presents with periodic weakness due to cold and exercise. It is caused by mutations of the SCN4 gene which encodes the sodium channel in skeletal muscles. Case presentation We report a full term obstetric patient with both paramyotonia congenita and terminal filum lipoma who presents for induction of labour followed by an emergency caesarean section performed under epidural anesthesia. Her recovery is subsequently complicated by a 3-day history of postpartum paraparesis attributed to hypokalemic periodic paralysis. Conclusion We describe the perioperative anesthesia considerations and challenges in this case with a review of the current literature. This case report highlights the importance of early proactive and collaborative multidisciplinary approach, maintaining normal temperature and electrolytes with a heightened vigilance for muscle-related perioperative complications.

Anaesthetic management of a parturient with hypokalaemic periodic paralysis for caesarean section: A case report and review of the literature

“…13 –15,18,19 In contrast, in the case reports in which there was no preoperative replacement despite low K + levels or no intraoperative (or infrequent) 16 measurement of K + levels and no intraoperative replacement, in each case prolonged postoperative paralysis was reported. 11,16,17,24 –26…”

“…Considering this, epidural analgesia has been successfully used for many years to manage patients during labour. [11][12][13] Epidural and spinal anaesthesia have been used for many years for LSCS and other surgeries to avoid general anaesthesia because of concern regarding hypoKPP's association with malignant hyperthermia (MH), 11 to avoid a drop in K þ associated with muscle relaxants, 14 and to attenuate the stress response, minimising the sympathetic stimulation associated with labour and surgery. [12][13][14] General anaesthesia using both inhalational [15][16][17] and total intravenous anaesthesia (TIVA) 18,19 techniques has been reported in pregnancy.…”

“…[13][14][15]18,19 In contrast, in the case reports in which there was no preoperative replacement despite low K þ levels or no intraoperative (or infrequent) 16 measurement of K þ levels and no intraoperative replacement, in each case prolonged postoperative paralysis was reported. 11,16,17,[24][25][26] In view of this, every patient with hypoKPP should receive preoperative K þ replacement if it is in the low to low normal range. Intraoperative monitoring of serum K þ levels should be frequent, at least every 20 minutes and K þ should be replaced aggressively (20-30 mmol/hour) and without delay once the K þ begins to drop.…”