Abstract
Vascular Ehlers-Danlos syndrome is associated with severe arterial fragility and life-threatening visceral complications. We report a 30-year-old woman with genetically confirmed vascular Ehlers-Danlos syndrome who presented with abdominal pain, nausea, and emesis. Persistent symptoms prompted serial computed tomography angiography (CTA), which revealed progressive celiac trunk and splenic artery dissections with multiple pseudoaneurysms. Given interval pseudoaneurysm growth and ongoing pain, she underwent joint endovascular repair by interventional radiology and vascular surgery via open left femoral exposure, selective catheterization, and combined Onyx and detachable coil embolization. Postprocedure CTA showed stable pseudoaneurysm exclusion with preserved distal perfusion. At 30-day follow-up, she remained asymptomatic with durable repair on CTA, demonstrating successful repair using combined Onyx-coil embolization. However, at 5 years, she had rupture of a more distal segment of the splenic artery, highlighting the need for continued surveillance even in the setting of repair.
Keywords: Celiac aneurysm, Coil embolization, Ehlers-Danlos, Onyx embolization
Vascular Ehlers-Danlos syndrome (VEDS) is an autosomal-dominant disorder caused by pathogenic variants in the COL3A1 gene, resulting in abnormal type III collagen and marked vascular fragility.1 Clinically, 61% of patients experience major arterial complications by age 40, including dissection or rupture.[1], [2], [3]
We describe a case of aneurysmal degeneration of multiple pseudoaneurysms of celiac trunk branches in a patient with VEDS managed with open femoral artery exposure and endovascular embolization with Onyx and coils. The patient consented to have her case published and presented and is participating in the VEDS Collaborative Natural History Study.4
Case presentation
A 30-year-old with VEDS (COL3A1 c.3417+1G>T, p.[Gly1122_Ang1139del]) was transferred for celiac and splenic artery pseudoaneurysms. Her history includes bilateral talipes equinovarus, spontaneous pneumothorax, two spontaneous sigmoid colon perforations, and bilateral internal carotid artery pseudoaneurysms. She smokes less than half a pack a day, and home medications included vitamin C, propranolol, losartan, atorvastatin, and sertraline. Family history is notable for a father and sister with VEDS who died from arterial ruptures.
The patient initially presented to an outside emergency department with emesis, diarrhea, lightheadedness, and subjective fevers. Computed tomography initially suggested hemorrhagic pancreatitis, and she was admitted, though a later overread suspected a ruptured aneurysm because of normal lipase levels. On hospital day 5, persistent abdominal pain prompted a computed tomography angiography (CTA) revealing complex celiac trunk dissection and pseudoaneurysm, a 1.1 cm aneurysm at the splenic-hepatic bifurcation, and proximal splenic artery dissection (Fig 1, A). Given her known VEDS, she was transferred to a tertiary care hospital for monitoring and blood pressure control. Her abdominal pain improved; follow-up CTA on hospital day 9 showed minimal change to the mesenteric lesions, she was discharged.
Fig 1.
A, Computed tomography angiography (CTA) imaging demonstrating two aneurysmal areas of the celiac trunk. B, Follow-up CTA demonstrating mesenteric aneurysm size progression to 2 cm.
Several days later, she returned to her local emergency department with recurrent abdominal pain, with CTA demonstrating mesenteric aneurysm size progression to 2 cm (Fig 1, B). She was admitted and then transferred to our intensive care unit. A repeat CTA the following day identified further mesenteric aneurysm size progression to 2.1 cm. Blood pressure management (goal systolic blood pressure <110 mm Hg) was initiated, and because of ongoing symptoms and aneurysm progression, surgical intervention was indicated.
Procedure
A joint approach was chosen with cosurgeons from vascular surgery (A.P., C.A.) and interventional radiology (J.K.) for catheter-based embolization of the pseudoaneurysms. Open surgical access of the left common femoral artery was performed because of concern for vessel fragility. The patient was positioned in a modified right lateral decubitus position with her left arm supported and her hips rotated flat. During the surgical time-out, contingency plans for rupture or bleeding were reviewed; these included celiac artery exclusion with an aortic stent graft (select devices and sheaths were available in the room), aortic occlusion balloon placement, and ability for retroperitoneal exposure for aortic control.
An oblique incision was made to expose the left common femoral artery, which was small and soft but otherwise normal appearing. The bifurcation was not dissected to minimize the risk of vessel injury. A pledgetted 5-0 Prolene purse-string suture was placed, and the artery was accessed with a micropuncture kit (Cook Medical) and a 5F sheath. With minor manipulation, this sheath caused an arterial tear, with bleeding controlled by upsizing to an 8F sheath. A long 5F VS 1 catheter (Cook Medical) was advanced into the aorta. A Benston wire was advanced over the bifurcation into the contralateral iliac artery, and the VS 1 catheter was formed over the iliac bifurcation to avoid excessive manipulation within the aorta.
An aortogram confirmed the aneurysms (Fig 2, A). The celiac trunk was engaged, and an 0.014 microwire and microcatheter (2.5F Cantata catheter; Cook Medical) were advanced into the distal splenic artery aneurysm. Due to concern for an aneurysmal dissection with autofenestration of the splenic artery and fragility of the remaining arterial wall, a total of 4.5 mL of Onyx 34 (ethylene vinyl alcohol copolymer) was injected distally while carefully withdrawing the catheter. This filled the splenic artery aneurysm and the proximal portions of the left gastric, common hepatic, and right phrenic arteries. The microcatheter was then withdrawn and redirected into the celiac artery false lumen and pseudoaneurysm, and this was embolized with multiple detachable coils (Concerto PGLA Helical; Medtronic) ranging from 14- to 10-mm diameters and all 30 cm in length to prevent Onyx reflux into the aorta (Fig 2, B and C).
Fig 2.
A, Left anterior oblique aortogram demonstrating celiac and superior mesenteric arteries, including the two pseudoaneurysms arising from the celiac and splenic arteries. B and C, Celiac artery aneurysm after Onyx embolization of the splenic artery aneurysm and coil embolization of the celiac artery aneurysm, in anteroposterior (AP) (B) and left anterior oblique (LAO) (C) projections. D, Completion aortogram demonstrating successful embolization of both aneurysms, with persistent flow through the superior mesenteric artery and the left phrenic artery.
Completion arteriogram demonstrated a patent celiac artery origin and filling of the left phrenic artery without opacification of either aneurysm (Fig 2, D). The VS 1 catheter was then brought into the right iliac artery to allow over-the-wire straightening prior to removal. A limited left iliac arteriogram showed no dissection or rupture of the iliac arteries. Surgical control of the left common femoral artery was obtained with nontraumatic clamps, and the sheaths were removed. The arteriotomy was repaired with the pledgetted purse-string suture and an additional pledgetted suture with hemostatic closure.
Follow-up
The patient's postoperative course was unremarkable. She was observed in the intensive care unit postoperatively with a systolic blood pressure goal of <110 and then transferred to the ward. She noted improved abdominal pain, tolerated diet and activity, and her left femoral access site had no complications. CTA prior to discharge confirmed successful embolization, and she was discharged on postoperative day 4.
At follow-up on postoperative day 39, she remained asymptomatic. Imaging demonstrated stable postembolization changes, and although there was initial concern for blush at the femoral access site, the hyperdensity was found to be surgical pledgets after duplex showed no arterial abnormality. She remained on an angiotensin-receptor blocker, beta-blocker, vitamin C, and statin with strong smoking cessation recommendations. At 5 years, she has had a ruptured splenic artery distal to the originally treated segment because of reconstitution by dorsal pancreatic and pancreatic magna collaterals and underwent splenectomy and distal pancreatectomy. She has been stable since.
Discussion
Mesenteric arteriopathy affects ∼30% of VEDS patients, most commonly involving the celiac (10.5%), splenic (7.9%), and hepatic (5.8%) arteries.1,5 Asymptomatic dissections and stable small aneurysms can often safely be managed with close surveillance. When intervention is needed, endovascular treatment has been shown to be well tolerated in carefully selected patients.[5], [6], [7]
Prior series support both open and endovascular management of mesenteric pathology in VEDS. Brooke et al6 reported 12 elective interventions, including three embolizations via open access. A French study documented embolization for ruptured mesenteric vessels using glue and coils.7 The Mayo Clinic’s 30-year review noted 30 interventions in 15 VEDS patients, including both open and endovascular repairs.2 Shalhub et al5,8 reported 34 repairs of splenic artery pathology and a separate series of 12 interventions involving splenic, hepatic, or celiac branches.
Combined coil and Onyx embolization has been effective for visceral aneurysms.9 Balloon-assisted Onyx embolization with and without coils has also been described in VEDS; however, balloon use requires caution given the risk of dissection or rupture in fragile arteries.10,11 Additional handling considerations in the setting of fragile vessels include avoiding vessel loops and stiff catheters, minimizing sheath exchanges by selecting the largest anticipated sheath at the outset, and favoring open femoral exposure with directly visualized pledgeted repair and bovine pericardial reinforcement for closure.
In this case, Onyx was injected first to limit manipulation of fragile arteries. Proximally, coils were used to prevent extravasation into the aorta. Though not combined in a single space, coils and Onyx were used synergistically to successfully occlude both pseudoaneurysms. Despite durable exclusion of the treated lesions, continued longitudinal surveillance remains essential in patients with VEDS because new or progressive arterial pathology may develop elsewhere, as demonstrated in this case.
Conclusions
VEDS remains an infrequently encountered condition for most physicians. This case highlights a quaternary institution's interdisciplinary approach to successfully treat rapidly evolving celiac and splenic artery aneurysms utilizing both Onyx and coil embolization.
Funding
None.
Disclosures
None.
Footnotes
The editors and reviewers of this article have no relevant financial relationships to disclose per the Journal policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest.
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