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Ehlers-Danlos IV in pregnancy- leads to uterus in ventral hernia and necrotic small bowel.
Abstract Number: F-10
Abstract Type: Case Report/Case Series
Introduction: Ehlers–Danlos syndrome type IV-vascular type results from a mutation in the COL3A1 gene. Patients are prone to spontaneous rupture and dissection of arteries, bowel and uterus. Complications occur in 25% of individuals by age 25, 80% by age 40. Life threatening uterine rupture complicates > 6% of pregnancies (1). Since patients only synthesize type I collagen; their skin, intestine and blood vessels are fragile (2). Due to the high risk associated with labor and vaginal delivery, early term cesarean delivery is advised (3).
Preoperative assessment: A 31 year-old female at 31+6 weeks gestation complicated by E-D IV. She had a history of popliteal aneurysm ligation; right carotid artery aneurysm and left carotid occlusion (50%). She presented with a ventral hernia containing her gravid uterus and bowel, resulting in painful necrosis of abdominal wall. She had been taking increasingly high doses of narcotics. After six days of conservative management with IV hydromorphone PCA and betamethasone for fetal lung maturation, signs and symptoms consistent with small bowel obstruction developed and an ex-lap and caesarean delivery were indicated.
Intraoperative course: GA with thoracic epidural for post-op pain management was performed without complications. Delivery via classical incision was uneventful. Bowel revealed diffuse areas of distention and necrosis thought to have potential for improvement so no immediate resection was performed. Significant loss of domain resulted in inability to close fascia. . She was left intubated in the ICU until after the second procedure on POD #1.
Postoperative course: POD1, abdominal wash-out; POD2, mid-jejunum resection, mesh placed for hernia defect; POD3, IR for drain placement for anastomotic leak; POD4, TEP removed. Plan for PICC line for long term TPN and definitive repair.
Conclusions: Careful consideration of the patient’s coexisting diseases, anxiety, high likelihood of a long procedure and an open abdomen with high risk of aspiration were balanced. The benefit of minimizing stress of catecholamine surge on arterial walls outweighed the risk of GA. TEP was provided for post-op analgesia, due to her probable long postop recovery and previous pain relief issues.
1) Pepin, M et al. N Engl J Med 2000; 342:673-680.
2) Pope, FM et al. Proc Natl Acad Sci USA 1975;72:1314–1316.
3) Erez, Y et al. Fetal Diagn Ther 2008;23:7-9.