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Vascular Medicine 2009; 14: 371376

Ischemic steal syndrome following arm arteriovenous fistula for


hemodialysis
Payman Zamani Cardiovascular Division, Veterans Affairs Boston Healthcare System and Cardiovascular
Division, Brigham and Womens Hospital and Harvard Medical School, James Kaufman Renal Division,
Veterans Affairs Boston Healthcare System and Boston University School of Medicine and Scott Kinlay
Cardiovascular Division, Veterans Affairs Boston Healthcare System and Cardiovascular Division,
Brigham and Womens Hospital and Harvard Medical School

Abstract: Arteriovenous fistulae in the arm are commonly used for hemodialysis in
end-stage renal disease. Although physiological steal with reverse flow in the artery
distal to the fistula is common, hand ischemia or infarction are rare. The ischemic
steal syndrome (hand or forearm ischemia) is usually a result of arterial disease prox-
imal or distal to the fistula and/or poor collateral supply to the hand. The diagnosis is
primarily clinical; however, markedly reduced digital pressures and pulse volume
recordings support the diagnosis. Management requires imaging for focal stenoses
or disease in arteries proximal and distal to the fistula from the aorta to the hand. We
present a case caused by subclavian artery occlusion that was initially missed due to
focusing investigation only on the fistula. We describe the percutaneous treatments
and surgical revisions that attempt to restore flow to the hand without compromising
the fistula.

Keywords: angioplasty, fistula, gangrene, ischemia, peripheral vascular diseases,


subclavian artery, vascular fistula

Introduction Case
Arteriovenous fistulae in the arm are the preferred An 88-year-old man was admitted to the Boston VA
access for hemodialysis in end-stage renal disease. with a 5-month history of pain in the left arm and
The most common complication of fistula is throm- hand and a 2-month history of progressive dry gan-
bosis. More rarely, hand ischemia or digital infarc- grene on the tips of the second and third fingers of
tion occurs due to poor distal blood flow. All his left hand. He had end-stage renal disease due to
fistulae shunt blood away from the distal arm, and long-standing type II diabetes mellitus and hyper-
physiological steal (reversed flow in the artery distal tension, and received hemodialysis from a left
to the arteriovenous fistulae) can occur in 70% of upper arm brachial artery basilic vein transposition
radiocephalic fistulae and 90% of brachial artery fistula created 4 years earlier.
fistulae.1 However, symptoms of hand ischemia He had an extensive history of atherosclerotic
(pain, parasthesia, or gangrene) only occur in peripheral vascular disease including a right carotid
12% of radiocephalic fistulae and 510% of bra- endarterectomy 11 years earlier, a right below knee
chial artery fistulae.29 The following case highlights amputation 2 years earlier, and a left above knee
the approach and pitfalls to managing the ischemic amputation 6 months prior to his hand symptoms.
steal syndrome associated with a fistula in the arm. At the time of his last amputation he had subopti-
mal blood flow rates with the fistula during dialysis,
and he had a left fistulogram that showed a moder-
ate stenosis at the arterial-venous anastomosis of his
fistula, which was treated by cutting balloon angio-
plasty. In the following weeks he developed pain in
Corresponding author: Scott Kinlay, Director, Cardiac
Catheterization Laboratory and Vascular Medicine, his left hand and fingers during hemodialysis and
Cardiovascular Division, VA Boston Healthcare System, 1400 when moving his wheelchair. He developed a small
VFW Parkway, West Roxbury, MA 02132, USA. non-healing ulcer on his left index finger that pro-
E-mail: scott.kinlay@va.gov gressed to gangrene on three digits of the hand. His
Dr Robert Eberhardt was the Guest Editor for this manuscript. left arm and hand pain became continuous, and he

The Author(s), 2009. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav 10.1177/1358863X09102293


372 P Zamani et al.

had absent radial and ulnar pulses. He was evalu- min). The gangrene in his fingers remained stable
ated with an arch aortogram that showed a 45 mm and did not require surgical intervention.
occlusion of the left subclavian artery (Figure 1A).
His operative risk for a subclavian bypass was con-
sidered too high due to his co-morbidities, and it Pathophysiology of the ischemic steal
was initially thought an endovascular approach syndrome
would be unsuccessful due to the long occlusion.
After a second opinion, he was offered an endovas- Physiologic steal with reverse flow in the arm artery
cular approach using equipment commonly used to distal to the fistula is common after the creation of a
cross coronary artery occlusions. fistula because of the low vascular resistance of the
In the Cardiac Catheterization Laboratory, the fistula.7,10 This can be identified clinically by com-
left femoral artery was accessed and a 6-F LIMA paring the blood pressure distal to the fistula to a
guide was used to cannulate the left subclavian more proximal blood pressure or one in the contra-
stump. The artery was crossed using a 0.014 6g lateral forearm. For example, the systolic pressure
Asahi Miracle guidewire (Abbott Laboratories, index is a comparison of the systolic pressure in the
Abbott Park, IL, USA) (Figure 1B) and dilated with forearm below the fistula using a hand-held Doppler
1.5 9 mm and 2.5 15 mm 0.014 balloons. The device versus the contra-lateral arm.7 In most cases,
guide was exchanged for an 8-F LIMA guide and particularly with upper arm fistulae, the index is
the lesion stented with 9.0 mm balloon expandable below 0.8 in the 24 hours after creating the fistula.
stents dilated to 10 mm (Figure 1C). Angiography The index tends to increase in the months after crea-
from the left subclavian artery distally demon- tion of the fistula, most likely due to the development
strated a widely patent brachial artery, fistula, and of collateral arteries from the proximal inflow artery
a patent radial artery to the wrist. Immediately fol- that increases blood flow to the hand.7,10
lowing the procedure, the patient reported resolu- Although steal is common, symptoms of ischemia
tion of his pain and warmth in his left hand are rare and likely prevented by compensatory vaso-
(Figure 1D). He had hemodialysis from his fistula dilation of the distal arm arteries and subsequent
with improved dialysis flow rates (400500 ml/ increased collateral flow to the forearm and hand

Figure 1 (A) Arch aortogram showing proximal occlusion of the left subclavian artery. (B) Selective angiogram show-
ing the occluded subclavian stump with a 0.014" wire crossing the occlusion. (C) Successful stenting of the proximal
subclavian artery. (D) Left hand showing digital gangrene with hyperemia after stenting the subclavian artery.

Vascular Medicine 2009; 14: 371376


Ischemic steal syndrome 373

to maintain adequate perfusion.10 Hand ischemia (ratio of the fistula forearm to contralateral forearm
may occur during hemodialysis because dialysis pressure). Digital pulse volume recordings in most
tends to lower venous return, reducing cardiac out- asymptomatic patients with healthy fistula show a
put and lowering the perfusion pressure in the fistula phasic waveform that is augmented by occluding
outflow artery and collaterals that supply the the venous limb of the fistula.10 However, in the
hand.10 ischemic fistula syndrome, digital systolic pressures
Pathological steal with continuous ischemic are usually less than 50 mmHg and the resting digital
symptoms can occur because of proximal inflow dis- pulse volume recordings are flat but return to a pul-
ease, reduced collateral flow to the hand, or distal satile waveform after occlusion of the venous limb.10
outflow obstruction.11,12 These all disturb the nor- A systolic pressure index of less than 0.57 is more
mal compensatory mechanisms (peripheral vasodi- commonly found in patients with ischemic steal syn-
lation and increased collateral flow) to preserve per- drome. In one study, abnormal nerve conduction
fusion to the distal arm. Any vascular disease that studies were more common with progressively
affects the proximal or distal arteries (e.g. athero- lower systolic pressure indexes supporting ischemia
sclerosis, vasculitis, Buergers disease) can reduce as a cause of some neuropathic symptoms.7 In
flow to cause symptoms. Proximal inflow disease another study, a digital to brachial artery pressure
can occur in 2030% of patients with ischemia of less than 0.6 had the best test characteristics for the
the hand,2,8 as illustrated by our case of subclavian ischemic steal syndrome.5 However, in most
artery occlusion. reports, there is considerable overlap in these physi-
Although end-stage renal disease is associated ological tests between symptomatic and asymptom-
with many atherosclerosis risk factors, the risk fac- atic patients,5,7 thus these tests need to be inter-
tors for ischemic steal syndrome include diabetes preted in the appropriate clinical setting.
mellitus, peripheral artery disease, age greater than
60 years, women, upper arm versus lower arm fistu- Neuropathies in the differential diagnosis
lae, multiple operations in the same limb, and the Severe neuropathic syndromes occurring shortly
use of PTFE grafts.3,4,8,13,14 These factors presum- after fistula creation require urgent attention. Ische-
ably relate to increased or more diffuse arterioscle- mic monomelic neuropathy is a rare cause of
rosis of the arteries in the forearm and hand, and sensory-motor impairment of the forearm without
poor development of collaterals. tissue necrosis. The initial case reports consisted of
sudden and permanent sensory and motor
impairment involving multiple nerve groups of a
Assessment distal limb and were associated with nerve conduc-
tion studies showing axonal loss and reduced con-
Diagnosis of the ischemic steal syndrome duction velocities.1517 Cases occurring immediately
Since physiological steal is common, ischemic steal after fistula creation were thought to be due to a
syndrome is primarily a clinical diagnosis. Signs transient reduction in blood flow that caused ische-
include pallor, diminished or absent peripheral pulses mia of the vasa nervorum, but was not prolonged
in the arm or hand, sensory or motor neuropathy, enough to cause tissue loss.7,15,1719 It is most com-
and distal infarction or ulceration. The occurrence monly associated with diabetes, atherosclerotic vas-
of ischemic steal has been divided into early (less cular disease, and upper arm fistulae. Immediate
than 30 days after fistula creation) or late (30 or revascularization or ligation of the fistula is required
more days) presentations.7,14 Severe presentations for this acute presentation, but may not prevent per-
of critical limb ischemia with motor dysfunction manent nerve damage.15,17
immediately after fistula creation usually need urgent Involvement of all of the distal nerves of the fore-
revision, revascularization, or ligation. Milder symp- arm (radial, ulnar and median nerves) with
toms may resolve spontaneously, likely due to the decreased nerve conduction studies and axonal loss
development of collaterals to the hand, whereas distinguish ischemic monomelic neuropathy from
later presentations are usually progressive.7,10 focal nerve injuries such as hematoma, abscess, or
Several non-invasive physiological techniques venous aneurysm that tend to affect only one
can assess pressure and flow distal to the fistula to nerve.19,20 Carpal tunnel syndrome tends to be
quantify the extent of steal. These techniques may more common in the fistula arm than the contralat-
identify those at risk of developing the syndrome or eral arm, but is distinguished by an abnormal nerve
provide supportive evidence for the diagnosis in the conduction study affecting primarily the median
presence of clinical findings. nerve.21
Physiological measures of distal limb perfusion A more indolent and less severe form of ischemic
include digital plethysmography and pulse volume monomelic neuropathy may occur, and one group
recordings, the digital pressure index (ratio of digital suggests that the diagnosis may be more likely with
to brachial pressure), and the systolic pressure index a progressive decline in serial nerve conduction

Vascular Medicine 2009; 14: 371376


374 P Zamani et al.

studies over several months associated with a to reverse flow in the more distal radial artery can be
reduced systolic pressure index (< 0.5).7 In this set- treated by percutaneous radial artery embolization
12
ting, revascularization may improve the neurologi- or ligation (distal radial artery ligation, DRAL),
18,23 providing there is antegrade flow in the ulnar
cal symptoms.7
artery and an adequate palmar arch.
Tests to localize the cause of steal
Non-invasive and invasive testing has an important
role in determining any potentially reversible causes Methods to modify the fistula
of the ischemic steal syndrome. Imaging with ultra-
sound or arteriography (conventional, computed Surgical revision of the fistula improves distal blood
tomography, or magnetic resonance techniques) flow by redirecting flow from the fistula to the hand
can assess proximal inflow and outflow disease in or by improving collateral flow to the hand. Tech-
addition to fistula flow rates.10 niques that reduce the diameter of fistula and/or
Duplex ultrasound can assess whether there is any lengthen the fistula increase resistance and decrease
fistula inflow or outflow disease. Functional fistulae flow in the fistula according to Poiseuilles law.2,13
usually have flow rates of greater than 600 ml/min,10 The DRIL procedure (see below) increases distal
and fistulae with flow rates of < 250 ml/min are flow by creating a low resistance collateral circuit
more likely to thrombose. Since flow rates are exag- to the distal hand.10
gerated and often non-laminar in the inflow artery,
manual occlusion of the venous limb of the fistula is Fistula banding
usually required to see the typical post-stenotic Fistula banding or plication aims to increase the
blunted or monophasic flow velocity, suggesting a resistance of the fistula to divert flow down the
proximal arterial stenosis.18 Increased flow veloci- native artery (Figure 2). However, this reduces
ties or absent flow in the distal arteries of the fore- flow in the fistula and threatens its survival. As a
arm suggest intrinsic disease distal to the fistula as a result, many reports of banding show high rates of
cause of ischemic steal syndrome. fistula thrombosis.4,8 Intraoperative pressure or
Marked reverse flow in the radial artery distal to flow monitoring may improve the success of the
the fistula may cause ischemia. Demonstration of a procedure while preserving fistula flow. In one
patent ulnar artery and palmar arch is required prior series, distal artery flow was monitored by digital
to considering embolization or ligation of the radial plethysmography to achieve a digital pressure of
artery distal to the fistula to prevent hand steal. greater than 50 mmHg and a digital to brachial
pressure index greater than 0.69. This approach
relieved steal symptoms in all 16 patients, but only
Indications for revascularization 10 (63%) had satisfactory graft function for more
than 6 months.9 Another approach is to monitor
The indications for revascularization are: the flow reduction in the venous limb of the fistula
to prevent severe flow reductions that would pro-
1) any proximal inflow disease with poor fistula mote fistula thrombosis.24 In a series of 78 patients
flow with ischemic steal syndrome and high fistula flow,
2) rest pain, muscle weakness, or necrosis in the banding was tailored to reduce fistula flow to
hand5,7,8,18,19 400 ml/min in autogenous grafts, and 600 ml/min in
3) ischemic monomelic neuropathy7,18,19 prosthetic grafts. Ischemic symptoms were relieved in
86% of patients with 91% of autogenous fistulae
remaining patent at 12 months.24 Graft survival
Treatment of disease proximal or distal to the with banding was less successful with prosthetic
fistula grafts (only 58% patent at 1 year) and the authors
suggested that higher flow rates (> 750 ml/min) in
Proximal artery disease can be treated by percutane- the graft were required to prevent thrombosis.24
ous methods, as in our case, or by surgical revascu- More recently, the minimally invasive limited
larization. Percutaneous methods for proximal dis- ligation endoluminal-assisted revision (MILLER)
ease offer less morbidity, and as many patients with technique describes a modified method of
ischemic steal syndrome have co-existing athero- banding.25 In this procedure, the fistula is exposed
sclerotic vascular disease, probably less mortality and a 45 mm balloon is introduced into the fistula
than surgery.22 and inflated. A non-resorbable suture is tied around
Flow-limiting disease distal to the fistula can be the inflated balloon and vein to achieve a defined
treated by angioplasty,11 or by surgical bypass that reduction in balloon diameter. In the original report
may be part of a surgical revision of the fistula. of 16 patients, all had improvement in symptoms,
Severe hand steal from a radiocephalic fistula due two required further revision of their fistulae and

Vascular Medicine 2009; 14: 371376


Ischemic steal syndrome 375

Figure 2 Diagrams illustrating several methods to reduce distal artery steal by the arterial venous fistula. (DRIL, distal
revascularization, interval ligation; RUDI, revision using distal inflow; PAI, proximal arterial inflow graft.)

all were patent at a mean of 3 months of mally to a larger artery, relative flow into the native
follow-up.25 artery and collaterals is increased without compromis-
ing fistula flow volumes.29 In one case series of 30
Distal revascularization, interval ligation (DRIL) patients, 84% were free of ischemic symptoms and
In this procedure, the artery distal to the fistula is 87% of the fistulae were patent at 12 months.29
ligated to prevent reversal of flow in the distal
artery, and a bypass graft is placed from the brachial
artery well above the fistula to the antecubital or Ligation of the fistula
forearm artery distal to the ligation (DRIL)
(Figure 2).26 The DRIL procedure effectively acts When other treatment options fail or extensive vas-
as a low-resistance collateral artery to the distal cular calcification prevents revascularization or fis-
arm10 and improves symptoms in over 90% of tula modification, ligation of the fistula can resolve
patients, while preserving the fistula in 73100% the ischemic symptoms, but with the need to create
of cases.2,14,18,27,28 The potential disadvantages of another vascular access.
DRIL are that the distal arm is dependent on a
graft for blood supply because the native artery is
Conclusions
ligated, and that distal anastomoses are technically
more difficult in patients with diffuse disease in the Ischemic steal syndrome causing symptoms after arm
distal forearm arteries. fistula creation are rare but challenging to manage.
In our case example, decreased flow in the fistula was
Revision using distal inflow (RUDI) initially thought to be due to a fistula stenosis.
The RUDI is designed to treat ischemic steal syn- Although angioplasty improved fistula flow, it exac-
drome with a brachial artery fistula. In contrast to erbated steal to cause hand ischemia and ultimately
ligating the native artery, RUDI ligates the fistula at digital infarction. This prompted investigation of
its origin and creates a bypass to the fistula from one vascular causes of insufficiency beyond the fistula.
of the more distal forearm arteries (Figure 2).13 The Although the diagnosis of ischemic steal syn-
other forearm artery (not used as the graft source) drome is principally clinical, abnormal digital
must be patent to prevent recurrence of a steal syn- pressures or pulse volume recordings support the
drome. RUDI tends to reduce flow in the fistula and diagnosis by quantifying the severity of vascular
long-term patency and flow rates in large series have steal. The diagnosis can be distinguished from neu-
not been reported. ropathic pain by nerve conduction studies. Abnor-
mal nerve conduction studies in all three distal
Proximal arterial inflow (PAI)/proximal nerves of the forearm are more characteristic of
arteriovenous anastomosis (PAVA) ischemic monomelic neuropathy.
The PAI or PAVA procedure moves the origin of the The cause of the ischemic steal syndrome should
fistula more proximally in the arm.29 The fistula is be directed at localizing disease from the aorta to
ligated at its origin and a PTFE graft is run from the the hand. Duplex ultrasound and arteriography
more proximal brachial or axillary artery to the fistula should aim to interrogate the arteries proximal
(Figure 2). By moving the graft feeder more proxi- and distal to the fistula in addition to fistula flow.

Vascular Medicine 2009; 14: 371376


376 P Zamani et al.

Our case illustrates the pitfalls of focusing only on 13 Minion DJ, Moore E, Endean E. Revision using distal
the fistula as a cause of reduced fistula flow, as a inflow: a novel approach to dialysis-associated steal syn-
more severe proximal lesion was not considered in drome. Ann Vasc Surg 2005; 19: 625628.
14 Yu SH, Cook PR, Canty TG, McGinn RF, Taft PM, Hye
the initial workup. In our case, subsequent recogni-
RJ. Hemodialysis-related steal syndrome: predictive factors
tion of the subclavian artery occlusion led to and response to treatment with the distal revascularization-
successful angioplasty and stenting borrowing tech- interval ligation procedure. Ann Vasc Surg 2008; 22: 210
niques familiar to those employed in crossing coro- 214.
nary artery occlusions. In the absence of proximal 15 Redfern AB, Zimmerman NB. Neurologic and ischemic
or distal disease, several surgical techniques to mod- complications of upper extremity vascular access for dialy-
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cessfully treat the ischemic steal syndrome while 16 Riggs JE, Moss AH, Labosky DA, Liput JH, Morgan JJ,
preserving the fistula. Gutmann L. Upper extremity ischemic monomelic neurop-
athy: a complication of vascular access procedures in ure-
mic diabetic patients. Neurology 1989; 39: 997998.
17 Wilbourn AJ, Furlan AJ, Hulley W, Ruschhaupt W. Ische-
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