Endovascular abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome

Maseer A. Bade, MD,a Luis A. Queral, MD,b Dipankar Mukherjee, MD,a and Li Sheng Kong, MD,a Falls

Church, Va; and Baltimore, Md
Ehlers-Danlos syndrome is a connective tissue disorder caused by abnormal collagen synthesis. Vascular complications,
including aneurysm formation and spontaneous arterial perforations, are difficult to manage surgically and result in
significant operative mortality due to blood vessel fragility. We describe the first reported successful endovascular
abdominal aortic aneurysm repair in a patient with Ehlers-Danlos syndrome. We discuss the advantages endovascular
surgery offers over open surgery in these patients. We believe that endovascular repair of abdominal aortic aneurysms
preferentially over open repair merits consideration in patients with Ehlers-Danlos syndrome. ( J Vasc Surg 2007;46:
360-2.)
The Ehlers-Danlos syndrome (EDS) is a hereditary
connective tissue disorder caused by abnormal collagen
synthesis. The clinical features of this syndrome were first
described in 1682 by Job van Meekeren, who described a
case of a 23-year-old Spanish man who could stretch the
skin of his right pectoral area to his left ear.1 In 1901, Ehlers
described joint hypermobility, bruising tendency, and subcutaneous
hemorrhages. In 1908, Danlos described subcutaneous
nodules. The eponym Ehlers-Danlos was coined
by Weber in 1936.2 Vascular complications, including aneurysms
and spontaneous arterial perforations, are difficult
to manage surgically, due to blood vessel fragility. Furthermore,
open surgery is associated with significant operative
mortality. We report the first case, to our knowledge, of a
successful endovascular abdominal aortic aneurysm repair
in a patient with Ehlers-Danlos syndrome.
CASE REPORT
Our patient was a 67-year-old white female, who since age
three was noted to have easy bruisability after minor accidents,
hyperelasticity of the skin and joints, and spontaneous knee and hip
dislocations. At age 15, she was formally diagnosed with type I,
“Classic” Ehlers-Danlos syndrome (Fig 1). No skin biopsies were
performed since her physicians were concerned about wound
healing complications. In 1998, at age 61, a KUB obtained for
lower rib pain, revealed an enlarged, calcified aorta. Computed
tomography (CT) scan demonstrated a 4.0 centimeter infrarenal
abdominal aortic aneurysm. She had no known family history of
Ehlers-Danlos syndrome or aneurysms. On physical examination,
she had no evidence of peripheral aneurysms. Her laboratory
examination was unremarkable. In February 2002, she underwent
colectomy and end colostomy for a spontaneous colonic perforation,
complicated by wound dehiscence. When she underwent
colostomy reversal, wound healing complications protracted her
hospital course to 13 weeks.
A CT scan in October 2003, demonstrated aneurysm growth
to 5.2 cm (Fig 2). Due to aneurysm size, particularly in the context
of the uniform fatality of aortic hemorrhage and the predisposition
for arterial rupture in EDS patients, repair was indicated. We felt
that endovascular repair would be a safer option than open for
several reasons. First, due to arterial fragility characteristic in these
patients, we felt that the aorta would be injured with cross-clamp
application, and also, would tear with suture placement, as has
been previously described.3 Second, the patient had a “hostile”
abdomen and a history of multiple wound complications with prior
open operations. On February 26, 2004, we performed a successful
AneuRx endovascular abdominal aortic aneurysm repair, via
bilateral oblique groin incisions. On CT scan and intraoperative
angiogram, there were no significantly atypical features of the
aneurysm noted. A 24 mm x 14 mm x 13.5 cm main body device,
14 mm x 8.5 cm iliac leg, and a 14 mm x 5.5 cm iliac extender limb
were utilized. Completion angiogram revealed no endoleaks (Fig
3). The femoral arteriotomies were closed with 6-0 Prolene suture
in a running fashion, and the wounds were closed in two layers with
3-0 Vicryl suture. Atraumatic vessel loops were utilized for vascular
control. The arteries had a soft, thin consistency. A concerted
effort was made not to pull tightly on the suture line, but to loosely
coapt the arteriotomy edges.
Suture line oozing was managed with gelfoam-thrombin. Her
postoperative course was complicated by skin breakdown of the left
groin, which healed in 2 weeks with Silvadene application. The
patient failed to show for postoperative follow-up and CT scans,
From the Cardiac, Vascular, and Thoracic Surgery Associates, Falls Church,a
and The Mercy Vascular Center, Baltimore.b
Competition of interest: Maseer A. Bade, MD, presently serves a two year
appointment as a compensated consulting member of the Medtronic
Regional Scientific Advisory Board.
Reprint requests: Maseer A. Bade, MD, Cardiac, Vascular, and Thoracic
Surgery Associates, 2921 Telestar Court, Falls Church, VA 22033 (email:
This e-mail address is being protected from spam bots, you need JavaScript enabled to view it )
0741-5214/$32.00
Copyright © 2007 by The Society for Vascular Surgery.
doi:10.1016/j.jvs.2007.03.045
360