2019 FSA Posters

P011: ANESTHETIC MANAGEMENT IN A PATIENT WITH HUNTINGTON’S DISEASE
Richard Tennant, DO, David McEnerney, DO, Giuseppe Giuratrabocchetta, MD; University of Florida College of Medicine- Jacksonville

Introduction: Huntington’s Disease (HD) is a rare neurodegenerative disease involving autosomal dominant inherited CAG trinucleotide repeat expansion on chromosome 4.  It typically manifests in patients aged 30-40 years of age and is ultimately fatal. It is characterized by progressive chorea, dementia, and psychiatric symptoms. Treatment is primarily focused on symptom management as there are currently no treatments that modify the disease process.1

Case: A 63 year-old female with PMH of HD, seizures, dementia, anxiety, depression, hyperlipidemia, and adult failure to thrive presented for open reduction internal fixation (ORIF) of a right proximal humerus fracture. Anesthesia was induced with fentanyl, lidocaine, propofol, and rocuronium. The patient was intubated with a Miller 2 blade with a grade 1 view. Anesthesia was maintained with total intravenous anesthesia using propofol and intermittent boluses of fentanyl. She received dexamethasone, IV acetaminophen, and ketorolac. Neuromuscular blockade was reversed with sugammadex and extubation was without issue. She tolerated the procedure well without complications. Five days later, she presented for revision of ORIF and hardware removal of right proximal humerus after presenting to the emergency department with dizziness, weakness, and a hemoglobin of 5.9. Anesthesia was induced with propofol, fentanyl, and rocuronium. She was intubated with a Mac 3 blade with a grade 2 view. The anesthetic was maintained with sevoflurane. She received ondansetron. Neuromuscular blockade was reversed with 3 mg neostigmine and 0.4mg of glycopyrrolate. She was again extubated without issues. She tolerated this procedure well without complications.

Discussion: Since HD is very rare, we must rely on case reports to assess general anesthetic safety in these patients. There have been multiple case reports regarding patients with HD undergoing anesthesia. Many of these patients take psychotropic medications and anesthesiologists should be aware of potential medication interactions. There have been cases that have reported prolonged apnea, generalized tonic spasms, postoperative fever, prolonged recovery, increased sensitivity to benzodiazepines and barbiturates, and postoperative shivering. There have also been reports of genetic variations and decreased levels of plasma cholinesterase that may prolong the duration of succinylcholine.2,3 However, a few cases, like the one we present, have gone smoothly without complications, despite varying anesthetic regimens. There are likely many variables to how well the anesthetic is tolerated. Further studies should be performed to help elucidate the best anesthetic choices for these patients.

References:

1. “Huntington’s Disease: a clinical review” P. McColgan et al. European Journal of Neurology 2018 25:24-34

2. “Anesthetic Management of Patients with Huntington Disease” Jonathon Kivela M.D. et al. Anesthesia and Analgesia February 2010, Volume 110, Issue 2, pp515-523

3. “Anesthetic management of a patient with Huntington’s Chorea- A case report” Kang et al. Korean Journal of Anesthesiology