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Anesthetic management of an obstetric patient with Ehler-Danlos Syndrome type III associated with postural orthostatic tachycardia syndrome
Abstract Number: RF6BI-384
Abstract Type: Case Report Case Series
INTRODUCTION: Ehlers-Danlos syndrome is a hereditary disease affecting connective tissue that causes joint hypermobility, skin elasticity, and bruising. There are eleven types of Ehler-Danlos syndrome. Classical types, I and II, are most often related with increased tissue distensibility and fragility. Type III, familial hypermobility, is associated with musculoskeletal and autonomic symptoms, but rarely causes life-threatening complications. Type III is often associated with dysautonomia in the form of postural orthostatic tachycardia syndrome (POTS). POTS causes orthostatic intolerance in patients due to abnormal baroreceptor activity. This can pose a serious threat to patients during labor and delivery and requires careful monitoring of hemodynamics.
CASE: Our patient is a 32-year-old G2P0010 with past medical history of Type III Ehlers-Danlos, who presented for induction of labor at 39 weeks. The patient endorsed a history of POTs, gastroparesis, obstructive sleep apnea, and generalized dystonias. She was well managed for POTS prior to pregnancy. Cardiology closely followed the patient and had started her on fludrocortisone and metoprolol secondary to fluctuating heart rate and blood pressure. After discussion with multidisciplinary team, patient was planned to receive early epidural for postoperative pain management. This decision was made as POTS patients are often noted to have tachycardia with increased pain and stress. Early epidural placement reduced the risk of tachycardia secondary to pain or stress. A labor epidural was successfully placed on first attempt without hemodynamic changes. Epidural block was established with 5 mL of 0.125% bupivacaine and maintained with a 5cc autobolus, delivered every 30 minutes, of 2 mcg/mL-0.125% fentanyl-bupivacaine epidural solution. The patient tolerated progression of labor well with adequate anesthesia. She delivered vaginally and was discharged home two days later.
DISCUSSION: Ehler-Danlos syndrome is often associated with bleeding tendency in patients (2). These concerns are more pronounced with vascular types. In patients with vascular types of Ehler-Danlos syndrome, cesarean section is often recommended to reduce the risk of vessel rupture or dissection (2). However, in patients with hypermobility Ehler-Danlos syndrome, vaginal delivery is often recommended with the assistance of instruments to reduce the risk of Valsalva (2). There are several concerns for patients with POTS- pain and stress can increase tachycardia (2). Additionally epidural anesthesia may cause vasodilation and resultant hypotension. There is some evidence to support the use of fluid preloading and left lateral recumbent position for blood pressure control. Phenylephrine may also allow for reduced risk of hypotension associated with epidural anesthesia.
1. Volkov et al. Obstet Gynecol Surv 2007;62: 51–7
2. Jones et. al. International Journal of Obstetric Anesthesia 2009;17:365–369