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///2013 Abstract Details
2013 Abstract Details2019-08-02T16:57:45-05:00

Management of a Parturient with Vascular Type Ehlers-Danlos

Abstract Number: S 45
Abstract Type: Case Report/Case Series

Brandon M Togioka B.S., M.D.1 ; Candice Morrissey M.D.2; Maggie Lesley M.D.3; James A Rothschild M.D.4; Gillian Isaac M.D., PhD5; Jamie Murphy M.D.6

Introduction:

Ehlers-Danlos Syndrome-Vascular Type (EDS-VT) has one of the highest mortality rates of any condition when associated with pregnancy (1). Patients with EDS-VT have a defect in type III collagen that causes tissue fragility and predisposes towards vascular and uterine rupture (1). We describe the management of a parturient with EDS-VT.

Case Description:

A 32-year-old (P 0-0-1-0) parturient with EDS-VT presented for induction of labor at 40 2/7 weeks. She was diagnosed (along with her mother who had uneventfully vaginally delivered two full term infants) with EDS-VT by genetic testing showing the COL3A1 mutation. She exhibited scoliosis, thin skin, visible veins, upper extremity hyperextensibility and reported easy bruising. Full body CTA revealed no evidence of aneurysm or dissection in any major vessels. Neuraxial anesthesia was not offered due to concern for epidural hematoma formation.

A healthy infant was delivered in the OR resulting in significant tears to the vagina and cervix. Blood loss was slow, albeit unremitting. After 1.2 liters of blood loss with a HR in the 150s the patient cried “an elephant is on my chest…I can’t breath!” Esmolol was given with resolution of symptoms. Bilateral arterial lines were placed to rule out dissection and GETA pursued. After a short stint in the ICU the patient was taken back to the OR for continued bleeding and stabilized after an estimated 5 liters of blood loss treated with 14 packed red blood cells.

Six days post-discharge the patient presented neurologically intact with a headache. CTA showed a new right vertebral artery dissection. Due in part to concerns over anticoagulation a plan for watchful waiting was made. The patient’s headache resolved. Follow-up pan-body CT was planned, but never completed.

Discussion:

Literature supports cesarean delivery of patients with EDS-VT at 32 weeks. Scheduled delivery is favored because it avoids the hemodynamic changes associated with labor, creates more easily approximated tissue planes, and allows for better mobilization of resources (2). A 32-week delivery is recommended to optimize fetal lung maturity while ensuring that cesarean section occur before most EDS patients go into spontaneous labor (32-35 weeks) (2). In this case, vaginal delivery was allowed because of the mother’s history of successful vaginal deliveries. A family history of uncomplicated delivery should probably not be seen as reassuring when determining a delivery plan.

References:

1. Hammond R, Oligbo N. Ehlers Danlos Syndrome Type IV and pregnancy. Arch Gynecol Obstet. 2012; 285: 51-4.

2. Lurie S, Manor M, Hagay ZJ. The threat of type IV Ehlers-Danlos syndrome on maternal well-being during pregnancy: early delivery may make the difference. J Obstet Gynaecol. 1998; 18:245-8.

SOAP 2013