///2017 Abstract Details
2017 Abstract Details2019-08-02T15:54:53-05:00

Management of a parturient with autoimmune autonomic ganglionopathy

Abstract Number: SAT-65
Abstract Type: Case Report/Case Series

Sharon Abramovitz M.D.1 ; Jennifer Landon M.D.2; Jaime Aaronson M.D.3

Intro: Autoimmune autonomic ganglionopathy (AAG) is a disorder mediated by antibodies to nicotinic acetylcholine receptors in autonomic ganglion. Patients with AAG present with a variety of symptoms, including orthostatic hypotension, gastrointestinal dysmotility, and bladder dysfunction.1 We report the management of a patient with AAG who presented for 2 Cesarean sections (CS) within 2 years.

Case: The patient is a 37 year-old G1P0 with AAG, migraines, and childhood seizures who presented at 39 weeks for CS. She was diagnosed with AAG at age 21, when she presented with orthostatic hypotension and gastroparesis, which both improved with IVIG and plasmapheresis. Current symptoms included urinary retention requiring catheterization, nausea, and constipation. In the OR, an epidural was placed to avoid abrupt sympathectomy. A phenylephrine infusion was initiated, followed by a test dose of 3 mL 2% lidocaine with epinephrine 1:200,000; then 20 mL lidocaine was given and a bilateral T7 level was achieved. She required an additional 5 mL lidocaine and fentanyl 100 mcg, resulting in a T4 level. The neonate was delivered 9 minutes after incision, when the patient reported sharp abdominal pain. She required nitrous oxide via mask and IV morphine, fentanyl, midazolam, and ketamine for relief. On POD 1 and 3, she received IVIG as planned. Her hospital stay was otherwise uneventful. Two years later, the patient again presented for CS. In the OR, a phenylephrine infusion was initiated and CSE was performed using 1.6 mL hyperbaric bupivacaine 0.75%, fentanyl 20 mcg and morphine 200 mcg. An epidural catheter threaded easily, and a bilateral T4 level was achieved. Sixteen minutes after incision, the patient complained of sharp abdominal pain, which persisted despite 10 mL 2% lidocaine with epinephrine via the epidural catheter, as well as epidural fentanyl, IV morphine, midazolam, and ketamine. Patient controlled epidural analgesia was ineffective for post-op analgesia. A hydromorphone IV PCA was started on POD 1.

Discussion: AAG is a rare disorder with sparse literature regarding anesthetic management. Dysautonomias pose significant anesthetic risks, especially when cardiovascular lability is present. There is no evidence to support either general or regional anesthesia in non-pregnant patients. Although there is a risk of marked hypotension with spinal anesthesia, studies involving non-pregnant patients with Shy-Drager syndrome indicate it may be modulated with adequate pre-operative volume replacement and vasopressor support.2 Resistance to local anesthetics in patients with autonomic dysfunction due to Shy-Drager syndrome has also been reported, but the mechanism is poorly understood. In our case, the patient remained hemodynamically stable, but required additional analgesics under spinal and epidural anesthesia. Both neuraxial techniques were safe, but less effective at the usual doses.

Vernino et al Auton Neurosci 2009

Niquille et al Anesth Analg 1998

SOAP 2017