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Neuraxial anesthesia for cesarean delivery in a parturient with vascular Ehlers-Danlos syndrome and Goodpasture syndrome
Abstract Number: T-46
Abstract Type: Case Report/Case Series
Patients with vascular Ehlers-Danlos syndrome (EDS) are high risk during pregnancy, labor and delivery, with mortality rates around 11.5%.(1) Considerations include fragile tissues, excessive bleeding, spontaneous pneumothoraces, valvular prolapse, and dissections or rupture of major vessels. They are high risk for premature labor, uterine prolapse and rupture and severe postpartum hemorrhage. Labor and vaginal delivery have risk for uterine rupture, hemodynamic volatility and vascular strain. Instrumental delivery and cesarean delivery have added risk. Neuraxial anesthesia may be unreliable, especially with dural ectasia, and may increase the risk for epidural hematoma. Neuraxial anesthesia may be preferable for hemodynamic control and to reduce the risk of pneumothorax with positive pressure ventilation. Goodpasture syndrome is an autoimmune disorder leading to glomerulonephritis with or without diffuse pulmonary hemorrhage.
The patient was a 32 year-old G1P0 diagnosed with vascular EDS by genetic testing. She had a past history of multiple spontaneous pneumothoraces and a C1-C2 neck fusion for instability. She had no known large vascular or cardiac manifestations, or issues with tissue healing. Her Goodpasture syndrome was diagnosed during an episode of hemoptysis but with minimal renal involvement. This was treated and considered to be in remission. Recent testing indicated moderate to severe bullous restrictive disease. Serial echos and cardiac MRIs done during pregnancy showed normal cardiac structures, function and vessel diameters. MRI showed dural ectasia in the lumbar region.
Elective cesarean delivery in cardiac OR with primed cardiac bypass pump at 35 wks was chosen to reduce risks associated with spontaneous labor. Despite the dural ectasia, epidural anesthesia was decided on to facilitate hemodynamic stability, to avoid positive pressure ventilation, to facilitate early maternal newborn bonding and to vocalize any abnormal symptoms. Large bore IVs and a left radial arterial line were placed. An epidural catheter was placed at the L3/4 interspace without complication. Epidural lidocaine 2% with epinephrine and bicarbonate was slowly titrated to a surgical block. Epidural fentanyl and morphine were given. Surgeons performed an uncomplicated low transverse C-section. Blood loss was 500cc. After delivery, the patient was admitted to the cardiac care ICU for 24 hrs of observation. The neuraxial block resolved in a normal fashion with no neurological concerns. She was discharged home without any complications 72 hrs postpartum and continued an uneventful course.
Conclusion: This case illustrates safe and uneventful delivery for a parturient medically complicated by vascular EDS with history of Goodpasture syndrome using epidural anesthesia for elective cesarean delivery at 35 wks GA in a controlled, multi-disciplinary setting.
1. NEJM. 2000; 342:673-680