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P218 Anesthetic management of familial amyloid polyneuropathy in elective c-section: a case report
  1. Inês Queiroz,
  2. Paulo Costa,
  3. Daniela Coelho and
  4. Óscar Camacho
  1. Anaesthesiology Department, ULSM – Hospital Pedro Hispano, Matosinhos, Portugal

Abstract

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Background and Aims Familial amyloid polyneuropathy (FAP) poses unique challenges in anesthetic management due to its multisystem involvement. We present a case of successful elective C-section in a patient with FAP, highlighting factors influencing anesthetic choice.

Methods A 40-year-old female (ASA III), 67 kg, 162 cm, with FAP diagnosed at 18, on tafamidis (suspended during pregnancy), presented with neuropathic pain, diarrhea and gastroparesis, anxiety/depression, lumbar disc herniation, and smoking history. Neurological examination revealed hypoesthesia below the knees, abolished Achillean and weak patellar reflexes (polyneuropathy disability score I). A normofunctioning DDD ADI pacemaker was implanted for 2nd degree AV block. No cardiomyopathy or orthostatic hypotension was evident. The airway had no signs of predictable difficulty. Coagulation tests were normal.

Results Following informed consent, general anesthesia with rapid sequence induction was performed, using etomidate, sevoflurane and rocuronium. Intubation was uneventful. The newborn’s APGAR scores were 9/10/10. Intraoperative analgesia included fentanyl, paracetamol, and ketorolac. Hemodynamic and electric stability was maintained. Postoperatively, an ultrasound-guided transversus abdominis plane (TAP) block was performed with ropivacaine. The patient had an uneventful recovery.

Conclusions In the absence of data supporting neuroaxial anesthesia safety in FAP, coupled with potential bleeding risks and disease progression due to medication interruption during pregnancy, general anesthesia in addition to a TAP block for postoperative analgesia was deemed the safest approach. This case contributes to the limited literature on FAP anesthetic management.

  • Familial amyloid polyneuropathy
  • Cesarean section
  • Anesthesia
  • General anesthesia
  • Regional anesthesia

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