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Huntington’s disease in the Parturient: Considerations for the Obstetric Anesthesiologist
Abstract Number: S4C-1
Abstract Type: Case Report/Case Series
Huntington’s Disease (HD) is a rare autosomal dominant disorder characterized by symptoms of dyskinesia, dystonia, psychomotor impairments, incoordination, cognitive decline, and behavioral difficulties. Creating safe anesthetic plans can be challenging as both general anesthesia and neuraxial may require alterations due to interactions with the disease. It has been postulated that patients with HD may have rapid cephalad spread after spinal bupivacaine administration that may result in high sensory levels due to altered CSF and brain proteins. 
This is a 34-year-old G3P2002 presenting at 39 weeks gestation with a PMH significant for Huntington’s disease (wheel-chair bound) and hyper-emesis gravidum requiring TPN via PICC line that presented for elective primary cesarean delivery due to weakness and inability to deliver vaginally. Preoperative evaluation was notable for facial and bilateral upper extremity choreic movements and upper and lower extremity muscle weakness. No cognitive decline was noted.
Anesthetic plan included a combined spinal-epidural technique with an initial intrathecal dose of 7.5mg of hyperbaric bupivacaine, 15mcg of fentanyl, and 150mcg of morphine (duramorph). Placement was successful despite frequent choreic movements of her upper body. Three minutes following the IT dose, a bilateral T3 sensory blockade was achieved. A phenylephrine infusion was employed immediately after the IT dose and the patient remained hemodynamically stable throughout the procedure and with adequate perioperative analgesia. Her postoperative course was unremarkable, and the patient was discharged home on post-operative day 2 in stable condition.
Huntington’s disease is rare in the parturient and may pose challenges for the obstetric anesthesiologist. General anesthesia may pose several risks including exacerbation of symptoms, aspiration of gastric contents and possible postoperative respiratory failure. Such risks limit the use of opioids, NMBD (i.e. succinylcholine) and anti-emetics. Regional anesthesia remains the preferred method of analgesia and anesthesia but may be technically difficult due to patient movement and the need for dose adjustments. Although the ideal spinal anesthetic dose remains poorly defined, our case lends support to an approach utilizing a combined spinal epidural catheter technique with a lower initial spinal dose to avoid rapid cephalad spread and possibly dangerous high anesthetic levels. 
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