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///2009 Abstract Details
2009 Abstract Details2019-08-03T15:55:31-05:00

Anesthetic Management of Parturient with Ehlers-Danlos Syndrome and History of Harrington Instrumentation for Severe Scoliosis

Abstract Number: 115
Abstract Type: Case Report/Case Series

Michelle Hoangquocgia DO1 ; Arthur Calimaran MD2

Introduction: The Ehlers-Danlos Syndrome (EDS) is a group of inherited connective tissue disorders. Although the severity varies among the subgroups (I to X), the main features are hyperextensibility of the skin, increased joint mobility, and connective tissue fragility. Parturients who have undergone extensive spinal surgery to correct severe scoliosis pose a particular challenge to anesthesiologists attempting neuraxial anesthesia. We described the anesthetic management of a parturient with both EDS and history of Harrington Rods placement for severe scoliosis.

Case Report: A 28 year-old, 68 inch, 90 kg, G1P0, at 36 3/7 weeks gestation presents to the Labor & Delivery suite for trial of labor and vaginal delivery. She was diagnosed with EDS type II with no signs or symptoms of abnormal coagulopathy. She has no associated cardiac anomalies. At 13 years old, she had Harrington instrumentation from the mid-thoracic spine to L4. On exam of her spine, there were no reliable surface landmarks due to the surgery. Laboratory data were all within normal limits. Patient did not want labor epidural. After approximately 12 hours in labor, cesarean section was called by her obstetrician for failure to progress. Patient opted to have spinal anesthesia because she wanted to be awake during the surgery. At second attempt, spinal was successfully placed around the lower lumbo-sacral area. A T4 sensory level was achieved following intrathecal dose of 0.75% hyperbaric bupivacaine 12 mg , morphine 200 mcg, and fentanyl 20mcg . Vital signs were stable throughout. She delivered a healthy newborn, and her postoperative course was uneventful.

Discussion: Our patient presented a unique scenario, having both EDS and history of extensive spinal surgery for severe scoliosis. Anesthetic considerations of patients with EDS include a possible higher risk of epidural hematoma due to increased fragility of the epidural veins, despite a normal coagulation profile. Moreover, performance of neuraxial anesthesia in patients with history of extensive spinal instrumentation is technically demanding and may be complicated with unpredictable sensory block. Our anesthetic management was based on her desire to be awake during surgery, on her EDS previously diagnosed as subgroup II with no associated abnormal bruising or bleeding, and on several successful spinal anesthesia that have been reported in patients with previous extensive spinal surgery.

References: 1)Beighton P: McKusicks Heritable Disorders of Connective Tissue, 5th ed 2) Crosby E, et al. Can J Anaesth 1989;36

SOAP 2009