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Anesthetic Implications of Undiagnosed Ehlers Danlos Type IV in a Parturient with Gestational Hypertension
Abstract Number: S-70
Abstract Type: Case Report/Case Series
Objective: To report a case of a patient who presented for induction of labor for an in-vitro fertilization pregnancy complicated by gestational hypertension, multiple abdominal surgeries with adhesions, and undiagnosed Ehlers-Danlos Type IV.
Case Summary: A 33 year-old G2P0 white female at 38 + 5/7 weeks gestational age presented to the labor unit for induction of labor secondary to gestational hypertension. She had never been seen in this facility prior to arrival to the labor unit. A labor epidural was placed at the patient’s first request for analgesia. Subsequently, the patient was brought to the operating room for an emergency cesarean section due to fetal bradycardia. The patient’s intraoperative course was complicated by the need for resection of necrotic bowel and severe hemorrhage requiring thirty-three units packed red blood cells, twenty-three units fresh frozen plasma, seven units platelets, three units cryoprecipitate, one dose of activated factor VII (approximately 100mcg/kg), and uterine artery embolization. Post operatively, CT angiography of her chest and neck was performed to rule out pulmonary embolism and to evaluate a new neck mass. This study revealed multiple large internal carotid and subclavian artery aneurysms in addition to aortic and hepatic artery aneurysms. Genetic testing was performed given the patient's complicated history and post-operative findings and revealed previously undiagnosed Ehlers-Danlos Type IV.
Discussion: Type IV Ehlers-Danlos (EDS IV), unlike other forms of EDS, appears to be associated with a high incidence of pregnancy complications. These complications include rupture of bowel, aorta, vena cava, and uterus; vaginal laceration; and post-partum uterine hemorrhage. EDS IV complications, rare in childhood, affect 25% of patients before the age of 20, and 80% by the age of 40. The incidence of EDS has been estimated to be 1 in 5,000 and 1 in 20,000 individuals. EDS IV accounts for just 10% of all cases. It carries a high risk of maternal morbidity and mortality, estimated to be as high as 25%, predominantly due to spontaneous arterial rupture.
Conclusion: A diagnosis of EDS IV should be considered in young people with unexplained bowel or arterial rupture. In-vitro fertilization should be undertaken with caution given the increased risks for pregnancy-induced hypertension. Pregnant women with EDS IV should be considered at high risk for bleeding and other complications and should be followed at tertiary care centers with immediate access to multidisciplinary specialty services.
Wiggins DA, Main E. "Outcomes of pregnancies achieved by donor egg in vitro fertilization--a comparison with standard in vitro fertilization pregnancies". Am J Obstet Gynecol. 2005 Jun;192(6):2002-6.
D. J. Taylor, I. Wilcox, and J. K. Russell, “Ehlers-Danlos syndrome during pregnancy: a case report and review of the literature,” Obstetrical and Gynecological Survey, vol. 36, no. 6, pp. 277–281, 1981.