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Background and Aims Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant disorder, with notable clusters in Portugal, Japan, and Sweden. FAP is characterized by the deposition of amyloid fibrils in multiple organs, leading to sensory, motor and autonomic polyneuropathy.

Methods Description of labor analgesia management in a pregnant woman with FAP.

Results A 35-year-old pregnant woman at 36 weeks gestation requested analgesia for spontaneous labor. The patient has FAP with sensory-motor and autonomic neuropathy, along with a permanent pacemaker. A multi-orifice epidural catheter was placed at the L3-L4 level. An initial bolus of 5mL of 0.1% ropivacaine with 10 mcg of sufentanil provided insufficient analgesia. Thirty minutes later, an additional 5 mL of the same solution was administered, providing adequate pain control for 3 hours. Due to recurrent pain, a total of 9 mL of 0.1% ropivacaine was administered over the following hour. Subsequently, eutocic delivery of a healthy newborn occurred. Throughout labor, the patient was advised against ambulation for safety. No neurological or hemodynamic changes were observed during labor. One month postpartum, the patient exhibited aggravated muscular weakness in left hallux extension (grade 0/5 versus 2/5 prepartum) and bilateral hallux flexion (grade 3/5 versus 4/5 prepartum) with no other new changes noted.

Conclusions Labor management for pregnant women with FAP should involve a multidisciplinary approach. There is no evidence that epidural analgesia exacerbates pre-existing neurological deficits, hence the recommendation to use less neurotoxic drugs. Further research is warranted to optimize anesthesia/analgesia techniques for pregnant FAP patients.