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An Extraordinary Catastrophe: Fibromuscular Dysplasia Causing a Spontaneous Common Iliac Artery Aneurysm Dissection with Rupture Leading to Massive Hemorrhage and Death in a Parturient
Abstract Number: T 69
Abstract Type: Case Report/Case Series
A healthy 32 yo G2P1 WF was admitted for spontaneous labor at 35 weeks gestation. She had an uncomplicated delivery, postoperative course and was scheduled for discharge two days later. She acutely developed right hip pain and collapsed. She was pale, diaphoretic, incontinent, but spontaneously ventilating and had a pulse, although hypotensive. She was resuscitated with IV fluids and vasopressors. Labs were unremarkable. She remained hypotensive despite resuscitative efforts. Arterial line was placed, labs were redrawn, revealing a hematocrit of 12. Following transfusion of four units PRBCs we proceeded to the operating room for exploratory laparotomy. She had three large bore IVs, and a rapid sequence intubation was performed with Etomidate and Succinylcholine, and she was hemodynamically stable throughout this process. Upon incision, her MAP fell to 35 mmHg. She was placed on the massive transfusion protocol, a norepinephrine infusion was started and central access was obtained. No source of bleeding was identified, but a large right sided retroperitoneal hematoma was noted extending from the pelvis to the diaphragm. Hemostasis was not obtained, cardiac arrest ensued and patient expired. Post-mortem studies revealed a 5cm right common iliac artery aneurysm, with a 9cm dissection with extension into both external and internal iliac arteries that ruptured, secondary to fibromuscular dysplasia and cystic medial necrosis. Family history was positive for SCD in her father and brother.
Spontaneous iliac aneurysm dissection with rupture in pregnancy is an extremely rare and unreported event. Over half of the non-traumatic aortic dissections that occur in women under 40 are in the context of pregnancy. Dissections that occur in parturients most commonly involve either the coronary arteries, the thoracic or abdominal aorta, and often these patients have underlying connective tissue disorders or a bicuspid aortic valve (1). There are few isolated cases of large vessel dissections. Risk factors include hypertension and age independent of pregnancy (2). There are numerous reviews of aortic aneurysms and dissections in the setting of connective tissue diseases and pregnancy, with varying recommendations for management of these. During late pregnancy, it is believed that hormonal changes lead to a loss of structural integrity and a loosening of the ground substance which further enhances risk for dissection (3). A reasonable degree of suspicion, early diagnosis and intervention is crucial for survival.
1. West J Emerg Med. 2011 November; 12(4): 571–574.
2. Int J Legal Med. 2012 Jun 28.
3. Ann Thorac Surg. 2013 Feb;95(2):701-3.