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Obstetric anesthesia for a parturient with congenital insensitivity to pain: a case report
Abstract Number: RF6BI-396
Abstract Type: Case Report Case Series
Introduction: Hereditary sensory and autonomic neuropathy type IV (HSAN IV) or congenital insensitivity to pain with anhidrosis (CIPA syndrome) is a rare phenotypic disease stemming from a number of genetic mutations. It may present in infancy as self-inflicted cuts, bruises, or burns due to insufficient temperature and pain perception. Anhidrosis may lead to altered thermoregulation and often death. We present a cesarean delivery case with CIPA that was successfully managed with neuraxial anesthesia.
Case: A 26-year-old G2P1 female at 37 weeks of gestation presented for a scheduled repeat cesarean delivery. Her medical history included CIPA, diagnosed at 2 months of age. She exhibited decreased pain perception and hyperthermia, which had led to all-limb amputations and extraction of all dentition. Her anesthetic history included uncomplicated general anesthesia for non-obstetric surgeries. She underwent the prior cesarean delivery under spinal anesthesia due to maternal co-morbidities in 2011 when she still had lower extremities.
On physical exam, her left arm to distal forearm remained, suitable for peripheral IV placement. Her right arm was amputated from the shoulder level, and both lower extremities were amputated above the knee. Airway exam was MPI with adequate thyromental distance. Labs were all within normal limits.
Dural puncture epidural technique was employed for the gradual and careful achievement of the surgical block. She received a total of 15 mL of 2% lidocaine with 1:200,000 epinephrine over 10 minutes, titrated to T4 level bilaterally using ice packs as guides. She remained hemodynamically stable in the perioperative period, received no neuraxial opioids, and was discharged home uneventfully on postoperative day 3.
Discussion: Due to its rarity, there are few case reports of the successful use of neuraxial anesthesia in CIPA patients undergoing cesarean delivery. Our patient’s particular condition with altered limb anatomy increased concern for possible unpredictable response to neuraxial anesthesia. Furthermore, studies on CIPA patients’ autonomic nervous systems show varying degrees of catecholamine responsiveness. Dose adjustment might be necessary in these patients when neuraxial anesthesia is considered. Checking the successful level of block can also be challenging in CIPA patients. Some patients can experience the very extremes of temperature, such as ice, and this can aid in testing neuraxial anesthesia levels.
Since CIPA patients present various symptoms of differing severity, careful review of the physical exam, medical and surgical histories as well as individually customized preoperative planning is recommended.
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