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January/February 2011 - SASSiT

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Volume 1 Issue 1 <strong>January</strong> – <strong>February</strong> <strong>2011</strong><br />

The official e-­‐Journal of the South African Society of Surgeons in Training<br />

In this Issue:<br />

Embolectomies,<br />

Fly- Fly fishing and<br />

Viticulture: What<br />

do they have in<br />

common?<br />

… Page 5<br />

Making sense of<br />

EVAR: What<br />

should should<br />

we be<br />

offering our<br />

patients?<br />

patients<br />

… Page 7<br />

7


Editor<br />

Bradley A David<br />

Editorial Committee<br />

Ferhana gool<br />

Abri Bezuidenhout<br />

Shreya Rayamaji<br />

Publishing, Design and<br />

Layout<br />

Bradley A David<br />

‘the Needleholder’ is the<br />

official online e-­‐Journal of<br />

the South African Society of<br />

Surgeons in Training <strong>SASSiT</strong><br />

Every effort has been made<br />

to ensure that all material is<br />

the original work of its<br />

authors and that where<br />

applicable outside material<br />

is appropriately referenced.<br />

Please feel free to email us<br />

with your concerns,<br />

comments, questions and<br />

ideas on how we can<br />

improve this publication:<br />

theneedleholder@gmail.com<br />

Volume 1 Issue 1 <strong>January</strong> – <strong>February</strong> <strong>2011</strong><br />

Contents<br />

Editorial<br />

Planning a career in vascular surgery. What the future holds?<br />

Dr Phillip Matley, President of VASSA …………………………………………….4<br />

Review<br />

A review of endovascular aortic repair (EVAR) in the elective<br />

management of abdominal aortic aneurysms.<br />

Gool F, Malherbe GF…………………………………………………………………………5<br />

Case Reports<br />

Dysphagia Lusoria: A case of an aberrant right subclavian artery and<br />

a bicarotid trunk<br />

Rogers AD, Nel M, Eloff EP, Naidoo NG……………………………………………13<br />

Case Report: Spontaneous Aortocaval Fistula<br />

Forgan T, Du Toit D…………………………………………………………….…………19<br />

Miscellaneous<br />

A biographical guide to the surgical tray:<br />

Michael E DeBakey…………………………………………………………………………11<br />

Thomas J. Forgarty………………………………………………………………………...18<br />

2


A career in vascular surgery<br />

in Southern Africa presents<br />

numerous interesting<br />

challenges for surgeons in<br />

training. Currently there<br />

are less than 40 registered<br />

specialist vascular surgeons<br />

in active practice in a<br />

country with a population of over 40 million,<br />

making fully trained specialist vascular<br />

surgeons a very precious resource. Out of<br />

sheer necessity, a great deal of vascular care<br />

and many vascular procedures are being<br />

performed by general surgeons but this is not<br />

ideal, given the increasing level of<br />

sophistication of vascular and endovascular<br />

surgery and the increasing demands and<br />

expectations of patients.<br />

Vascular disease is on the increase and this is<br />

contributed to by the fact that in developed<br />

counties, people are simply living to an older age.<br />

The scourge of diabetes has already been<br />

recognized as an international epidemic of<br />

worrying proportions. Tobacco use has not been<br />

brought under control and within our country,<br />

hypertension, hyperlipidaemia and aneurysmal<br />

disease are not only very common but tend to<br />

reflect strong genetic factors. Vascular trauma<br />

and unusual vasculopathies such as Takayasu<br />

Disease and HIV vasculopathy are particularly<br />

prevalent in our sub-­‐continent.<br />

Vascular surgery is one of the youngest surgical<br />

specialties. The endovascular revolution,<br />

particularly in the 1990s has almost totally<br />

changed the way that we work. In South Africa,<br />

unlike the United Kingdom and many other parts<br />

of the world, endovascular procedures have<br />

largely remained in the hands of vascular<br />

surgeons rather than radiologists and<br />

cardiologists. This has broadened the scope and<br />

interest level of vascular surgery enormously.<br />

Vascular surgeons provide primary vascular care<br />

and education, diagnosis and assessment as well<br />

as the whole range of medical, surgical and<br />

endovascular treatment. This is in contrast to<br />

several areas of surgical gastro-­‐enterology where<br />

PLANNING A<br />

CAREER IN<br />

VASCULAR<br />

SURGERY IN<br />

SOUTH AFRICA<br />

What does the<br />

future hold?<br />

Dr Phillip Matley<br />

President of VASSA<br />

Guest Editorial<br />

primary care and diagnosis is provided by<br />

gastroenterologists, and cardiac surgery where<br />

this is provided almost exclusively by<br />

cardiologists. The modern vascular surgeon has<br />

an intimate knowledge of vascular medicine and<br />

the medical management of the risk factors and<br />

the disease processes themselves. Furthermore,<br />

vascular surgeons have “hands-­‐on” involvement<br />

in diagnosis and imaging, particularly with the<br />

increasing use of colour-­‐flow duplex ultrasound.<br />

Vascular surgery works well in private practice in<br />

this country. Several private hospitals have<br />

established vascular centres of excellence<br />

including dedicated vascular wards, hybrid<br />

operating theatres (with ceiling-­‐mounted<br />

angiographic facilities) and multi-­‐disciplinary<br />

medical and para-­‐medical teams. Vascular<br />

disease is common in the group of patients who<br />

enjoy private medical cover and there appears to<br />

be an almost limitless number of patients with<br />

varicose veins requesting surgical treatment. The<br />

earning potential of vascular surgeons compares<br />

very well to other specialties in the general<br />

surgery arena.<br />

The endovascular revolution and the increasing<br />

sophistication of vascular care is challenging the<br />

conventional training and career pathways.<br />

3


Increasingly vascular surgeons will be trained in<br />

both radiological and surgical techniques and will<br />

acquire a broad knowledge and experience of<br />

vascular medicine in close association with<br />

cardiology. The historical connections with<br />

general surgery, gastro-­‐enterology and oncology<br />

will become ever more tenuous and it is likely<br />

that vascular trainees will have a very limited<br />

general surgery education before commencing a<br />

4-­‐5 year training programme of pure vascular<br />

surgery.<br />

Modern vascular practice calls for a wide range of<br />

both cognitive and technical skills including<br />

traditional open surgery as well as percutaneous<br />

catheter-­‐based techniques. There is currently<br />

very little role for laparoscopic vascular surgery<br />

but this may change as laparoscopic techniques<br />

develop further,<br />

The speciality is very demanding on the<br />

individual surgeon. The patients tend to be<br />

elderly and high-­‐risk with multiple co-­‐<br />

morbidities that require attention and control.<br />

Vascular emergencies are a daily phenomenon<br />

that will frequently interrupt weekends and<br />

nights and arranging appropriate cover for<br />

weekends and vacations can be a problem, given<br />

the scarcity of registered vascular surgeons.<br />

Group practices largely solve these issues but are<br />

uncommon among vascular surgeons in this<br />

country. With increasing sophistication may<br />

come increasing cost. The high-­‐tech specialties<br />

such as endovascular surgery consume a large<br />

portion of health care expenditure and may be<br />

increasingly unaffordable to patients and funders<br />

in the future.<br />

I practice in a group partnership that includes<br />

three specialist vascular surgeons supported by<br />

three vascular technologists, a full multi-­‐<br />

disciplinary team of para-­‐medical personnel and<br />

a great network of cardiologists, physicians,<br />

radiologists and anaesthetists. We practice in a<br />

private hospital that has a dedicated 22-­‐bed<br />

vascular ward and a hybrid theatre able to handle<br />

everything from a thoraco-­‐abdominal aneurysm<br />

to a carotid stent. We cover vascular<br />

emergencies on a one-­‐in-­‐three rota and allow<br />

ourselves 10 weeks of vacation a year. We do<br />

clinical research and audit and remain very<br />

involved in teaching and in the activities of our<br />

professional societies. It has been a great career<br />

and it keeps getting better.<br />

Surgery<br />

Calender <strong>2011</strong><br />

March UCT Update<br />

22-24<br />

March<br />

12-16<br />

April<br />

15-17<br />

April<br />

Written College exams<br />

European HPB congress/<br />

ASSA-SAGES meeting (Cape<br />

Town)<br />

ASiT congress (Sheffield, UK)<br />

16-18 May Oral College exams (Gauteng)<br />

15 June Closing date for exam entry<br />

21-26<br />

June<br />

28 Aug – 1<br />

Sept<br />

30 Aug-1<br />

Sept<br />

International Surgical Trainees<br />

Congress (Pretoria)<br />

International Surgery Week<br />

(Yokohama, Japan)<br />

Written College exams<br />

October Pretoria Controversies<br />

4


A review of endovascular aneurysm repair (EVAR)<br />

in the elective management of abdominal aortic<br />

aneurysms.<br />

Gool F, Malherbe GF<br />

University of Cape Town<br />

Aneurysms of the abdominal aorta (AAA) have long been recognized as a<br />

fatal disease process and significant advances have been made over the last<br />

50 years in the surgical management thereof. Treatment of an asymptomatic<br />

aortic aneurysm is indicated when the risk of rupture outweighs the risk of<br />

operation.<br />

AAA’s are now more frequently diagnosed, due<br />

mainly to the increase in life expectancy and also<br />

due to the development of better non-invasive<br />

imaging techniques such as Doppler ultrasound,<br />

Computer Tomography (CT) and Magnetic<br />

Resonance Angiography (MRA).<br />

Conventional open aneurysm surgery is<br />

associated with considerable operative mortality<br />

because the operation is extensive and the<br />

patients often have serious co-morbidities.<br />

In published series, the 30-day or in-hospital<br />

mortality has ranged from 3.8% to 8.2% in<br />

patients undergoing elective open repair of AAA’s.<br />

Intensive care and hospital admissions are<br />

extended with open repair. Severe postoperative<br />

complications are common and more importantly,<br />

the time taken for recovery to the preoperative<br />

state of wellbeing is usually prolonged.<br />

There are also subgroups of patients who are at<br />

increased risk during conventional repair because<br />

of their associated medical co-morbidities or<br />

anatomical variations:<br />

1. Patients with myocardial infarction, renal or<br />

liver insufficiency<br />

2. Inflammatory aortic aneurysms<br />

3. Horseshoe kidney, retro-aortic renal vein<br />

4. Hostile abdomen<br />

It was the combination of these factors that<br />

provided the impetus for the development of<br />

minimally invasive procedures as a treatment<br />

option. The advent of minimally invasive<br />

endovascular stent-graft techniques or the<br />

endovascular aneurysm repair (EVAR) as an<br />

alternative treatment has radically extended the<br />

management options for patients with aortic<br />

aneurysms.<br />

However, the advent of new technology to treat<br />

any surgical condition needs to be evaluated<br />

against conventional treatment<br />

before a surgeon can offer this<br />

choice to patients. The questions<br />

that needed to be answered are:<br />

1. What are the advantages of<br />

the novel approach over conventional<br />

treatment?<br />

2. What are the potential drawbacks of the novel<br />

approach?<br />

3. Does the long-term outcome of the novel<br />

approach match the tested outcomes of<br />

conventional management?<br />

4. What are the cost implications of the novel<br />

approach?<br />

The aim of this review will be to outline the<br />

developmental history and the application of<br />

EVAR technology in the elective management of<br />

AAA’s. Finally, this review will aim to assess how<br />

the evidence collected to date answers the<br />

questions posed above in relation to the use of<br />

EVAR.<br />

The history of EVAR<br />

The first animal studies on<br />

EVAR by stent-graft<br />

combinations date from the<br />

mid-1980s. Unpublished<br />

clinical experience in Russia<br />

dates from 1985 but it was<br />

Parodi and colleagues, from<br />

Buenos Aires, Argentina,<br />

who were the first to<br />

publish experience with a<br />

balloon-expandable stent<br />

attached to a polyester<br />

tubular prosthesis to treat<br />

AAAs in patients who were<br />

REVIEW<br />

5


unfit for open surgery. Their home-made stentgraft<br />

evolved from an endograft with one<br />

proximal stent for infrarenal fixation only to<br />

include a second stent for distal fixation to obtain<br />

effective exclusion of the aneurysm from the<br />

blood flow. These early patients had an aortoaortic<br />

tube graft. Early on the graft endograft was<br />

only used in aneurysms not involving the<br />

bifurcation but later an aorto-uni-iliac stent-graft<br />

was also used, resolving the problem of an<br />

inadequate distal aortic cuff for securing “fixation”<br />

and “seal.”<br />

Over the next decade, a succession of industrymanufactured<br />

endoprostheses was introduced into<br />

the market for treatment of a broad spectrum of<br />

patients with a AAA. A variety of endografts from<br />

different manufacturers are currently available.<br />

The ideal endograft should provide lifelong<br />

protection from rupture of the aneurysm without<br />

any risk of migration or displacement from the<br />

attachment sites. Components should be<br />

sufficiently robust and at the same time small<br />

enough to fit into a delivery system that can be<br />

negotiated easily through the access vessels.<br />

Application of EVAR in the elective<br />

management of AAA’s<br />

Although there are significant variations from<br />

brand to brand, each device consists of three key<br />

components:<br />

• A delivery system to allow placement<br />

• An attachment system that forms a blood tight<br />

seal between the graft and the abdominal aorta<br />

• Graft fabric, which acts as a conduit for blood<br />

flow that has been diverted from the diseased<br />

segment of aorta<br />

INITIAL RADIOGRAPHIC EVALUATION<br />

Although the vast majority of abdominal aortic<br />

aneurysms are infrarenal, less than 50 percent<br />

are amenable to endovascular repair due to<br />

anatomic considerations. Angiography and<br />

computed tomography (CT) are commonly<br />

obtained as initial radiographic studies to<br />

determine both the feasibility of an endograft and<br />

the appropriate size and configuration of the<br />

endograft.<br />

1. Angiography<br />

Since the inner lumen is imaged, but not the<br />

wall of the aorta, angiography cannot evaluate<br />

the true lumen diameter, extent of thrombus,<br />

plaque, and calcification.<br />

2. Computed tomography<br />

Measurements of diameters can be<br />

problematic problematic if the aorta is<br />

angulated and the longitudinal axis is not<br />

perpendicular to the imaging plane.<br />

3. CT angiography<br />

Angiography is more sensitive for measuring<br />

small, axially oriented vessels and vascular<br />

stenoses than CTA, combining angiography<br />

with spiral CT and 3D CTA provides the<br />

maximum amount of information<br />

ANATOMIC CONSIDERATIONS<br />

1. Proximal neck length<br />

The minimum length needed varies from<br />

device to device but may be as long as 15<br />

mm. Newer designs allow suprarenal fixation<br />

and require a shorter proximal neck length.<br />

Ideally, it should be a normal appearing<br />

segment of aorta, without abundant thrombus<br />

or heavy circumferential calcification.<br />

2. Distal neck length<br />

A tube endograft may be placed if a distal<br />

neck is of sufficient length and diameter as<br />

specified by the manufacturer. As an example,<br />

the Ancure device requires a distal neck at<br />

least 12 mm in length and no greater than 26<br />

mm in diameter<br />

3. Aneurysm diameter<br />

Undersizing the diameter of the endograft<br />

may lead to an inadequate seal and failure to<br />

exclude the aneurysm, while oversizing may<br />

lead to kinking of the device, causing a nidus<br />

for thrombus formation or a leak. In a report<br />

from a European registry of patients<br />

undergoing endografting (the EUROSTAR<br />

registry), postoperative complications,<br />

postoperative mortality, late rupture, and<br />

6


aneurysm-related death were all more<br />

frequent in patients with a preoperative<br />

aneurysm diameter ≥6.5 cm.<br />

4. Angulation<br />

An angle of 60º or more leads to difficulties in<br />

implantation, kinking, leakage, and the<br />

possibility of downward migration of the<br />

device.<br />

5. Iliac attachment site<br />

The common iliac artery is the preferred<br />

attachment site, although the external iliac<br />

artery may be used. When the attachment site<br />

is in the external iliac artery, the hypogastric<br />

artery will be covered by the endograft. If the<br />

hypogastric artery is patent, the potential<br />

exists to back fill the aneurysm via back flow<br />

through this vessel into the sac, resulting in<br />

ineffective exclusion of the aneurysm. This<br />

problem can be prevented by preprocedural<br />

embolization of the hypogastric artery.<br />

6. Femoral artery diameter<br />

To accommodate the delivery system of most<br />

devices, a minimal femoral artery diameter of<br />

8 mm is usually required.<br />

7. Accessory renal arteries<br />

Accessory renal arteries are present in up to<br />

30 percent of the population and commonly<br />

originate from the lumbar aorta. Exclusion of<br />

an accessory renal vessel by an endograft can<br />

result in partial renal infarction.<br />

8. Inferior mesenteric artery<br />

However, when the inferior mesenteric artery<br />

is patent and there is significant stenosis of<br />

the superior mesenteric artery, the inferior<br />

mesenteric artery may supply important<br />

collateral blood flow to the bowel. In such<br />

patients, covering a patent inferior mesenteric<br />

artery with an endograft may compromise<br />

blood flow to the bowel. Thus, endograft<br />

placement is contraindicated.<br />

PATIENT FOLLOW-UP<br />

The size of the abdominal aortic aneurysm<br />

following endovascular repair and endograft<br />

placement is followed with plain film of the<br />

abdomen, CT scan or Arterial Duplex sonography.<br />

• Follow-up protocols vary, but a typical<br />

schedule after uncomplicated endograft<br />

placement consists of abdominal plain films<br />

before discharge and at one, six, and 12<br />

months, and then every year thereafter.<br />

Abdominal plain films are an economical and<br />

quick way to evaluate the integrity of the graft<br />

and the stability of graft appearance,<br />

alignment, and position.<br />

• CT scans, which are obtained on a similar<br />

schedule, are used to evaluate the diameter<br />

and volume of the aneurysm and to look for<br />

signs of endoleak or of endograft migration<br />

• A typical follow-up schedule in an<br />

uncomplicated patient post EVAR whose BMI is<br />

within normal limits, at our institution would<br />

be:<br />

• CT Abdomen at 4-6 weeks<br />

• Plain AXR at 4-6 weeks, 6 months and 12<br />

months; then annually<br />

• Arterial Duplex Sonography at 6 months, 12<br />

months then annually<br />

COMPLICATIONS<br />

Illustration of<br />

Endoleaks<br />

Type 1 – 4<br />

Complications that have been reported with<br />

endograft use include:<br />

• Vascular injury during deployment (sometimes<br />

leading to aneurysm rupture)<br />

• inadequate fixation or sealing of the graft to<br />

the vessel wall<br />

• stent frame fractures and separations<br />

• breakdown of the graft material<br />

7


Even after an aneurysm is successfully treated<br />

with an endograft, it remains a dynamic entity.<br />

The aneurysm will eventually thrombose and, by<br />

12 months, approximately 50 percent of<br />

aneurysm sacs have shrunk in diameter.<br />

1. Endoleaks<br />

2. Postimplantation syndrome<br />

Patients undergoing endovascular stent graft<br />

placement often experience an acute<br />

inflammatory syndrome characterized by<br />

fever, leukocytosis, elevation of serum Creactive<br />

protein (CRP) concentration, and<br />

perigraft air during the first week to 10 days<br />

after implantation<br />

3. Device migration<br />

Device migration is one of the major causes of<br />

secondary intervention after endovascular<br />

aneurysm repair. If untreated, potential<br />

complications include endoleak, aneurysm<br />

expansion, and rupture.<br />

CLINICAL OUTCOMES<br />

Short-term<br />

The short-term technical success rate for<br />

endovascular aneurysm repair ranges from 83 to<br />

over 95 percent. A 2007 systematic review<br />

identified four randomized trials of 1532 patients<br />

who were considered suitable candidates for<br />

either endovascular or open repair of nonruptured<br />

abdominal aortic aneurysms larger than 5.0 cm in<br />

diameter. 1 The 30 day all-cause mortality was<br />

significantly lower with endovascular repair (1.6<br />

versus 4.8 percent, relative risk 0.33, 95% CI<br />

0.17-0.64).<br />

The two principle contributing trials to this<br />

systematic review were:<br />

• The EVAR 1 trial included 1082 patients who<br />

were at least 60 years of age, with aneurysms<br />

at least 5.5 cm in diameter. At 30 days,<br />

mortality was significantly lower with<br />

endovascular than with open repair (1.6 versus<br />

4.6 percent, adjusted odds ratio 0.34, 95% CI<br />

0.15-0.74). Endovascular repair was also<br />

associated with a significantly shorter hospital<br />

stay (7 versus 12 days), although more<br />

secondary interventions (additional surgical<br />

procedures) were required with endovascular<br />

repair (9.8 versus 5.8 percent).<br />

• The DREAM trial evaluated 345 patients with<br />

aneurysms of at least 5 cm in diameter. There<br />

was an almost significant trend toward lower<br />

operative mortality with endografting than with<br />

surgery (1.2 versus 4.6 percent, risk ratio<br />

0.26, 95% CI 0.03-1.10). Moderate and severe<br />

systemic complications (cardiac, pulmonary,<br />

renal) were more frequent with open repair (26<br />

versus 12 percent), while moderate and severe<br />

local vascular or implant-related complications<br />

were more frequent with endovascular repair<br />

(16 versus 9 percent).<br />

The short-term survival advantage of<br />

endovascular repair appears to be much greater<br />

when endovascular repair is limited to patients at<br />

highest risk from open surgery. This was<br />

illustrated in a report of 454 consecutive patients<br />

who underwent elective repair (206 endovascular<br />

and 248 open surgery) of an abdominal aortic<br />

aneurysm. The overall 30-day mortality rates not<br />

significantly different for endografting and surgery<br />

(2.4 and 4.8 percent, respectively). However,<br />

among patients at highest surgical risk (American<br />

Society of Anesthesiologists class IV), the 30-day<br />

mortality rates were much lower with<br />

endovascular repair (4.7 versus 19.2 percent with<br />

open surgery).<br />

Long term<br />

The early survival benefit seen with endovascular<br />

repair compared to open repair described above is<br />

lost between one and four years, after which<br />

survival appears equivalent. This observation was<br />

seen in the 2007 systematic review discussed<br />

above, which included follow-up of 1473 patients<br />

from the DREAM (two years) and EVAR 1<br />

randomized trials (four years)<br />

Long term outcomes of patients who have<br />

undergone endovascular repair of abdominal<br />

aneurysms have been evaluated with and without<br />

comparison to patients who have undergone open<br />

repair.<br />

EVAR-1:<br />

In this large, randomized trial, endovascular<br />

repair of abdominal aortic aneurysm was<br />

associated with a significantly lower operative<br />

mortality than open surgical repair. However, no<br />

differences were seen in total mortality or<br />

aneurysm-related mortality in the long term.<br />

Endovascular repair was associated with<br />

increased rates of graft-related complications and<br />

re-interventions and was more costly.<br />

EVAR-2:<br />

In this randomized trial involving patients who<br />

were physically ineligible for open repair,<br />

endovascular repair of abdominal aortic aneurysm<br />

was associated with a significantly lower rate of<br />

aneurysm-related mortality than no repair.<br />

8


However, endovascular repair was not associated<br />

with a reduction in the rate of death from any<br />

cause. The rates of graft-related complications<br />

and reinterventions were higher with<br />

endovascular repair, and it was more costly.<br />

Conclusion<br />

1. What are the advantages of EVAR over<br />

conventional open repair?<br />

The results from EVAR1 and DREAM<br />

demonstrate that the short-term morbidity and<br />

mortality related to open repair is clearly<br />

improved in patients undergoing EVAR.<br />

However this benefit does not follow through in<br />

the long-term.<br />

In the select sub-group of patients with<br />

anatomical variants and/or a hostile abdomen<br />

EVAR is a very attractive option to avoid the<br />

unusually excessive risks of open surgery.<br />

2. What are the potential drawbacks of the<br />

novel approach?<br />

Higher long-term aneurysm specific morbidity<br />

and mortality is in large part due to the<br />

Endoleak phenomenon, as discussed earlier.<br />

Stent-technology depends on anatomic<br />

suitability and while EVAR maybe desirable in a<br />

patient it may not be applicable, because of<br />

unsuitable anatomy or problems with access.<br />

In view of uncertain long-term durability it<br />

should be used with caution in younger<br />

relatively low risk patients.<br />

The anticipated use of EVAR to treat patients<br />

who were not fit for open surgery has not been<br />

supported by the EVAR2 trial.<br />

3. Does the long-term outcome of the novel<br />

approach match the tested outcomes of<br />

conventional management?<br />

Long-term follow up in both the EVAR1 and<br />

DREAM trial demonstrate that morbidity and<br />

mortality are equivalent in both groups.<br />

However, the aneurysm specific morbidity and<br />

mortality was significantly higher in the EVAR<br />

arms of the studies. Initial speculation that this<br />

was related to the ‘developmental turnaround<br />

time’ of new technology, may not hold true as<br />

the American-based OVER trial has had very<br />

similar results compared to the EVAR1 and<br />

DREAM trials despite using the latest stent<br />

technology.<br />

4. What are the cost implications of the<br />

novel approach?<br />

EVAR is more costly than open repair not only<br />

because of the initial device costs but also<br />

because of the rigorous CT based follow-up<br />

used in most of the trials. However, in the<br />

EVAR and DREAM trials the long-term costs of<br />

all-cause morbidity associated with open repair<br />

such as wound complications, were not<br />

included in the cost analysis. Moreover, followup<br />

surveillance of the stent is moving away<br />

from CT towards a duplex based regime which<br />

is far more cost effective.<br />

In conclusion, the endovascular management of<br />

abdominal aortic aneurysms should not be viewed<br />

as a replacement for conventional open surgery.<br />

EVAR is a weapon in the armamentarium of the<br />

vascular surgeon, which if used with caution in<br />

the appropriate setting will form part of an<br />

effective elective management strategy for the<br />

treatment of AAA’s.<br />

REFERENCES<br />

1. Sajid MS, Desai M, Baker DM, Hamilton G. Endovascular Aortic Aneurysm<br />

Repair (EVAR) Has Significantly Lower Peri-operative Mortality in Comparison<br />

to Open Repair: A Systemic Review. Asian J Surg. 2008 Jul;31(3):119-23<br />

2. Malas MB, Freischlag JA. Interpretation of Results of OVER in the context of<br />

the EVAR Trial, DREAM, and the EUROSTAR Registry. Semin Vasc Surg. 2010<br />

Sep;23(3):165-9<br />

3. Albuquerque FC Jr, Tonnessen BH, Noll RE Jr, Cires G, Kim JK, Sternbergh WC<br />

3 rd . Paradigm shifts in the treatment of Abdominal Aortic Aneurysms: trends<br />

in 721 patients between 1996 and 2008. J Vasc Surg.2010 Jun;51(6):1348-<br />

52; discussion 1352-3.<br />

4. Deaton DH. Future Technologies to address the failed endoprosthesis. Semin<br />

Vasc Surg. 2009 Jun;22(2):111-8.<br />

5. Lederle, FA, Johnson, GR, Wilson, SE, et al. The aneurysm detection and<br />

management study screening program: validation cohort and final results.<br />

Aneurysm Detection and Management Veterans Affairs Cooperative Study<br />

Investigators. Arch Intern Med 2000; 160:1425.<br />

6. Szilagyi, DE, Smith, RF, DeRusso, FJ, et al. Contribution of abdominal aortic<br />

aneurysmectomy to prolongation of life. Ann Surg 1966; 164:678.<br />

7. Peppelenbosch, N, Buth, J, Harris, PL, et al. Diameter of abdominal aortic<br />

aneurysm and outcome of endovascular aneurysm repair: does size matter? A<br />

report from EUROSTAR. J Vasc Surg 2004; 39:288.<br />

8. Greenhalgh, RM, Brown, LC, Kwong, GP, et al. Comparison of endovascular<br />

aneurysm repair with open repair in patients with abdominal aortic aneurysm<br />

(EVAR trial 1), 30-day operative mortality results: randomised controlled trial.<br />

Lancet 2004; 364:843.<br />

9


Michael Ellis DeBakey was born as Michel Dabaghi (later Anglicized to DeBakey) in Louisiana to<br />

Lebanese immigrants. DeBakey graduated from Tulane University School of Medicine in New Orleans in<br />

1932. He completed his surgical fellowships at the University of Strasbourg, France, under<br />

Professor René Leriche, and at the University of Heidelberg, Germany, under Professor Martin<br />

Kirschner.<br />

‘DeBakey had a machine<br />

shop right outside the<br />

operating room. He<br />

regularly consulted with<br />

an engineer during and<br />

after procedures about<br />

the design of<br />

instruments’<br />

Michael Ellis DeBakey<br />

September 7, 1908 – July 11, 2008<br />

World-renowned Lebanese-American cardiac surgeon, innovator,<br />

scientist, medical educator, and international medical statesman.<br />

During a decorated five-decade medical career, it is without<br />

hyperbole that Dr. Michael E. DeBakey earned the right to be<br />

named as one of the greatest surgeons of the 20th century.<br />

While he was in medical school DeBakey conceived of the<br />

roller pump, the central idea of the heart-lung machine. The<br />

very notion that the heart can be temporarily replaced by a<br />

machine set the stage for a host of medical innovations—from<br />

bypass surgeries and heart transplants to artificial hearts—<br />

and a fundamental change in approach to heart treatment,<br />

heart health and the prevention of heart disease.<br />

DeBakey ushered in, and was often at the forefront of, medical innovations that have saved<br />

millions of lives. He performed over 60,000 operations himself. Chief among these techniques<br />

was arterial bypass and arterial reconstruction. He mastered ways of patching and grafting,<br />

bypassing and re-organizing arteries. He finally put down his scalpel in 1998 at age 90.<br />

Kenneth L. Mattox, chief of surgery at Baylor-affiliated Ben Taub Hospital in the Texas Medical<br />

Center, Houston, has no doubts that his mentor and long-time colleague did that many<br />

operations. “We were doing operations in four different rooms and he would move from room to<br />

room,” Mattox recalls. In the evening, “he sent out for food and we ate between cases.” At 11<br />

p.m., the entire team of residents, nurses, anesthesiologists, everyone, was exhausted, Mattox<br />

says. “I remember Dr. DeBakey sticking his head out a door and saying, ‘Doesn’t anybody else<br />

out there want an operation? We’re just getting warmed up.’”<br />

Mattox also remembers that DeBakey had a machine shop right outside the operating room. He<br />

regularly consulted with an engineer during and after procedures about the design of<br />

instruments. “He would look at the way an instrument fit in his hand, the way a clamp would<br />

spring or close,” Mattox says. “He would talk to the engineer, and a week later he would have a<br />

new instrument.”<br />

The Next time you ask your scrub<br />

sister for a DeBakey forceps<br />

remember the innovative spirit of<br />

its creator – Michael E. DeBakey.<br />

The DeBakey<br />

Forceps designed<br />

with atraumatic<br />

tips so as to<br />

minimize trauma<br />

to delicate tissues.<br />

11


Dysphagia Lusoria: A case of an aberrant right<br />

subclavian artery and a bicarotid trunk<br />

Rogers AD, Nel M, Eloff EP, Naidoo NG<br />

Vascular Surgery Unit, Division of General Surgery, Groote Schuur Hospital, Cape Town<br />

Abstract<br />

Dysphagia Lusoria is dysphagia secondary to an aberrant right subclavian artery<br />

that has a retro-oesophageal course. Adachi and Williams categorized aortic<br />

arch anomalies, showing that the right subclavian artery arising in this fashion<br />

(as the last branch of the descending aorta) is one of the more common.<br />

However, this very rarely co-exists with a bi-carotid trunk.<br />

We present such a case as is it manifested in a 36 year-old lady complaining of<br />

marked weight loss and dysphagia. The diagnosis remained elusive until a CT<br />

scan of the chest was performed; angiography further delineated the pathology.<br />

It is believed that the combination of the common carotid origins with the retrooesophageal<br />

course of the aberrant vessel more frequently accounts for<br />

symptoms in the absence of an aneurysm of the origin of the aberrant vessel.<br />

Several techniques to manage the aberrant vessel have been described in the literature, but we<br />

favoured open ligation and transposition to the right carotid artery.<br />

Case Report<br />

A 36-year-old women presented to the Gastro-<br />

Intestinal Unit of Groote Schuur Hospital with a<br />

three year history of dysphagia. The dysphagia<br />

was initially only for solid food and intermittent,<br />

but more recently she describes a constant ‘stuck<br />

in the throat’ foreign body sensation. She also<br />

admitted to significant (almost 30kg) weight loss.<br />

She was very anxious, and eager to determine<br />

the source of the ailment and was concerned<br />

about the possibility of malignancy.<br />

Physical examination revealed only evidence of<br />

recent weight loss and routine laboratory data<br />

were within normal limits.<br />

There were no abnormalities on chest Xray or<br />

ECG.<br />

Barium Swallow demonstrated an indentation at<br />

the level of the third thoracic vertebra. A small<br />

diverticulum was also reported to be present, just<br />

above the aortic arch.<br />

No pathology was demonstrated during<br />

oesophagoscopy, oesophageal manometry,<br />

larygoscopy and pharyngoscopy.<br />

A course of Antacids and H2 receptor blockers<br />

was initiated for two months, with little benefit.<br />

Psychotherapy was considered and<br />

antidepressants prescribed.<br />

At this time a CT scan of the chest and abdomen<br />

demonstrated an aberrant right subclavian artery,<br />

originating from the descending aorta.<br />

The patient was referred to the Vascular Surgery<br />

Unit for further investigation and possible surgical<br />

intervention. Digital Subtraction Angiogram<br />

confirmed the diagnosis. The ARSA originated<br />

distal to the origin of the left subclavian artery<br />

and coursed through the posterior mediastinum<br />

behind the oesophagus. In addition, the<br />

angiogram also demonstrated a common origin of<br />

the common carotid arteries as a second branch<br />

of the arch.<br />

The patient underwent a right supraclavicular<br />

incision and surgical ligation of the ARSA with<br />

right subclavian–carotid transposition using an<br />

end-to-side anastomosis.<br />

The patient had an uneventful postoperative<br />

course and remains symptom free after follow-up<br />

of twelve months.<br />

Literature Review<br />

Figure 1 – Barium Swallow<br />

The characteristic diagonal<br />

impression at the level of<br />

the third and fourth<br />

vertebrae<br />

The first case of a symptomatic aberrant right<br />

subclavian artery (ARSA) was described in the<br />

medical literature by Hanuld in 1735. 1<br />

The term ‘Dysphagia Lusoria’, however, was<br />

coined by Bayford in 1794 to describe dysphagia<br />

secondary to a retro-oesophageal (aberrant) right<br />

subclavian artery (ARSA). He described a lady<br />

who died of oesophageal obstruction and<br />

resultant emaciation. The condition, in his words<br />

‘may be called lusoria, from Lusus Naturae (Latin<br />

for ‘freak of nature’) that gives rise to it’. 2<br />

13


Figures 2 and 3 – Angiogram – The aberrant right subclavian<br />

artery and its course demonstrated on AP and Right anterior<br />

oblique views. Note also the common origin of the carotid arteries.<br />

Burckhard Komerell is credited with the first<br />

radiological description, in 1936, as the condition<br />

had only been diagnosed at postmortem prior to<br />

that time. Komerell’s name survives as the<br />

eponym for the diverticulum sometimes present<br />

at the origin of this attenuated vessel. He stated ‘<br />

. . . the pulsating mass behind the oesophagus<br />

does not consist of the right subclavian itself,<br />

because the calibre of this vessel is much smaller.<br />

Much more likely this mass consists of an aortic<br />

diverticulum, from which the right subclavian<br />

artery originates.’ 3<br />

The incidence of ARSA varies between 0.4 to<br />

1.8% of the population, and is probably the<br />

commonest significant aortic arch anomaly. 4<br />

The Adachi Williams Classification<br />

In about 80% of individuals, three branches arise<br />

from the aortic arch: the brachiocephalic trunk,<br />

the left subclavian artery and the left common<br />

carotid artery. This Adachi described as Type A. 5<br />

11% of individuals have an Adachi type B pattern,<br />

which consists of a common trunk for the left<br />

common carotid and the brachiocephalic artery<br />

and therefore has only two aortic arch branches. 5<br />

The next most common type, Adachi C, has a<br />

vertebral artery originating proximal to the left<br />

subclavian artery as a 4 th branch of the arch. 5<br />

The origin of the retroesophageal right subclavian<br />

artery as the last branch occurs in between 0.4<br />

and 2% of individuals. 4,5,6<br />

The Adachi and Williams Classification recognizes<br />

four basic morphologies within this group: Types<br />

G, CG, H and N (see figure 7.) 4<br />

This case report demonstrates features of Adachi<br />

H, where the right subclavian artery is anomalous<br />

(as in type G), but where the right and left<br />

common carotids arise from a common stem or<br />

bicarotid trunk.<br />

Embryology<br />

Figure 4 – 3 D Reconstruction –<br />

Right posterior oblique view.<br />

The right subclavian artery develops during the<br />

6 th to 8 th week of gestation. The proximal part<br />

originates from the right 4 th aortic arch artery,<br />

and the distal part from the right dorsal and right<br />

seventh intersegmental arteries. 3,7<br />

In these cases, the right 4 th aortic arch artery<br />

and/or the right dorsal aorta involute cranial to<br />

the seventh intersegmental artery – the<br />

connection between the aortic sac and the right<br />

subclavian artery disappears. The right<br />

subclavian artery develops from the right 7 th<br />

intersegmental artery and the distal segment of<br />

the right dorsal aorta. Differential growth shifts<br />

Figure 7 – Retro-oesophageal Subclavian Anomalies. Note<br />

the rare Type H, with the bicarotid trunk.<br />

the origin cranially and lies close to the origin of<br />

the left subclavian artery. It originates dorsally<br />

and therefore has a retroesophageal course. 3,7<br />

The aberrant right subclavian artery stems from<br />

the dorsal margin of the aortic arch, between the<br />

top of the arch and where it lies against the<br />

vertebral column. The proximal part of the artery<br />

14


has a wider diameter than the distal part and the<br />

artery passes through the mediastium in a retrooesophageal<br />

position. The artery may arise from<br />

a diverticulum at the proximal descending aorta,<br />

referred to as Kommerell’s diverticulum. 3,7<br />

Presentation<br />

There are several descriptions of childhood<br />

dyspnoea resulting from an ARSA, and cases of<br />

pneumonia have been ascribed to the condition<br />

on the basis of aspiration and dysphagia. Relative<br />

tracheal laxity may account for this presentation<br />

8, 9<br />

in childhood.<br />

Two thirds of individuals are believed (on the<br />

basis of autopsy studies and retrospective<br />

studies) to remain completely asymptomatic<br />

despite the anomaly. There are a few proposed<br />

mechanisms as to why certain individuals become<br />

symptomatic:<br />

1. Increased oesophageal rigidity with ageing<br />

2. Aneurysm formation<br />

3. Aortic elongation with ageing<br />

4. The presence of a bicarotid trunk. 7,8<br />

In a study by Klinkhamer, published in 1966 and<br />

reviewing all articles from 1763, it is stated that<br />

the aberrant right subclavian artery was found to<br />

be associated with a bicarotid truncus (common<br />

origin of the right and left carotid arteries) in 85<br />

of 295 cases (29%) In 60% of cases there was a<br />

normal origin of the two carotids, and in 10%<br />

they were observed to be closer to one another<br />

than normal. 8<br />

It is believed that the combination of the common<br />

carotid origins with the retro-oesophageal course<br />

of the ARSA more frequently accounts for<br />

symptoms in the absence of an aneurysm of the<br />

origin of the aberrant vessel. In fact, several<br />

authors have questioned the relationship between<br />

the aberrant vessel (in isolation) and the<br />

dysphagia. 8<br />

Klinkhamer, for instance, felt that the aberrant<br />

artery itself was not an adequate explanation for<br />

the dysphagia because some patients have had<br />

very large ARSA’s, without dysphagia or<br />

respiratory symptoms. He maintained that<br />

symptoms are usually only present when the left<br />

and right carotids arise together or close to one<br />

another and therefore prevent the trachea and<br />

oesophagus from being bent forward where the<br />

ARSA crosses. 8<br />

In 80% of individuals, the Brachiocephalic (BCA)<br />

and Left Common Carotid Arteries (LCCA)<br />

originate from the arch 4cm apart. Because the<br />

arch lies obliquely and curves from front and the<br />

right backward and to the left, the BCA is more<br />

ventral than the LCCA origin. This allows the<br />

ARSA from being bent forward (note diagram). If<br />

they arise commonly, the two carotids form a ‘V’<br />

that prevents forward or flexion movements of<br />

the trachea and oesophagus. These structures<br />

are therefore compressed by the SCA posteriorly<br />

and by the CC origins antero-laterally. 6-10<br />

Investigation 7-11<br />

Barium swallow may demonstrate the<br />

characteristic diagonal impression at the level of<br />

the third and fourth thoracic vertebrae.<br />

A pulsating mass may be visualized at endoscopy.<br />

Digital Subtraction Angiogram, CT with contrast,<br />

or MRI may confirm the diagnosis and enable one<br />

to visualise the arch anatomy.<br />

Motility studies are frequently performed during<br />

the diagnostic phase of investigation. A high<br />

pressure zone in the region of the vessel has been<br />

described. Manometry cannot be used to<br />

diagnose the condition nor has it been of any<br />

assistance in distinguishing which patients may<br />

benefit from surgery.<br />

Therapy<br />

Several reports have described improvement with<br />

conservative therapy. These patients usually had<br />

inconclusive findings on manometry. In light of<br />

the relative infrequency of symptoms in patients<br />

with isolated ARSA, some authors have therefore<br />

advocated trials of therapies like prokinetics and<br />

antireflux medications.<br />

The majority of symptomatic patients have<br />

benefited from surgical intervention. Janssen<br />

concluded that in the absence of another cause of<br />

the symptoms and after a trial of medical<br />

management, surgery should be considered. 7 In<br />

1994, Kieffer reported on 19 patients who<br />

underwent surgery, of whom 16 had complete<br />

resolution of their symptoms. 12<br />

Gross first reported surgical management of this<br />

condition in 1946. He described dividing and<br />

ligating the ARSA via a left thoracotomy, in a 4<br />

month old infant. 13 Lichter was the first to<br />

describe surgery on an adult with this condition in<br />

1963. It is not until the last thirty years that<br />

surgery has become the standard therapy for this<br />

condition, and several authors have advocated<br />

various approaches. 14 Pome, in a review of the<br />

literature published in 1987, found only twenty<br />

reported surgically treated patients. 15<br />

The optimal exposure of the aberrant artery origin<br />

is undoubtedly achieved via a left thoracotomy.<br />

This is particularly important when the origin of<br />

the ARSA is dilated. 7,14<br />

Simple ligation and division has been noted to be<br />

inadequate therapy in a significant number of<br />

patients due to the development of subclavian<br />

15


steal syndromes. The onset of this syndrome<br />

may be immediately postoperatively or late<br />

(described up to seven years later). In addition,<br />

cases of gangrene of the right arm have been<br />

described following simple ligation of the aberrant<br />

vessel. 14<br />

Smith and Pifarre have described reimplanting the<br />

right SCA with a graft onto the ascending arch via<br />

a left thoracotomy. This is a technically<br />

challenging exercise and involves passing the<br />

Right SCA and the graft from the posterior<br />

mediastinum to the anterior, as well as deep<br />

anastomoses. 16,17<br />

The anastomosis of the RSCA to the ascending<br />

arch is much easier to perform via a right<br />

posterolateral thoracotomy, as described by<br />

authors such as Bailey. 18<br />

Pifarre identified a problem with the right<br />

thoracotomy approach in one case report. If the<br />

artery is not divided close to its origin, thrombosis<br />

of the stump may lead to the persistence of<br />

dysphagia. 17<br />

Schumacker described performing an end to side<br />

anastomosis of the R SCA with the right carotid<br />

artery via a median sternotomy. This has been<br />

the method described by at least three further<br />

surgeons. However, this anterior approach also<br />

provides suboptimal control during the dissection<br />

and division of the vessel. The possibility of<br />

transient cerebral ischaemia and the<br />

consequences of damage to an atherosclerotic<br />

carotid artery exist with this approach. Mok<br />

therefore recommended that the divided aberrant<br />

right SCA should be anastomosed to the aortic<br />

arch, with or without an interposition graft. 10<br />

Orvald and Kunlin advocated a cervical approach,<br />

but Kunlin described significant haemorrhage in a<br />

patient when attempting to perform the<br />

procedure via a cervical incision and had to resort<br />

to a median sternotomy. We have already<br />

highlighted the potential complication of residual<br />

dysphagia in cases where a long stump is left. 15<br />

Syders reported a combined approach using both<br />

cervical and left carotid approaches to reimplant<br />

the right SCA onto the right carotid. This, while<br />

considerably safer, involves having to reposition<br />

the patient for the second incision. 15<br />

Lemire described a transternal approach for the<br />

division and reimplanting of the Right SCA to the<br />

ascending aorta. The incidence of pain and<br />

pulmonary complications are probably higher, but<br />

this approach has cosmetic advantages. 19<br />

Pome recommended that a right thoractomy may<br />

be employed for patients without significant<br />

ectasia of the origin of the Right SCA; patients<br />

with ectasia should undergo a left thoracotomy<br />

and cervical incision. 15<br />

The importance of dividing the stump proximally<br />

is again highlighted in the paper by Pome. He<br />

advocated using the aorta rather than the carotid<br />

to avoid a possible subclavian steal syndrome. 15<br />

Janssen reports six cases of dysphagia lusoria<br />

diagnosed and managed between 1992 and 1997.<br />

Three patients responded to either medical<br />

management (antacids etc), or dietary<br />

modification. One patient underwent a right<br />

carotid-subclavian end to side bypass via a right<br />

supraclavicular approach. A persistent RSCA<br />

stump may account for his occasional residual<br />

dysphagia for solid food. Two patients underwent<br />

a two incision approach (ie thoracotomy and<br />

cervical incision). 7<br />

Kieffer et al have the largest single-centre series<br />

of patients who have received therapy for<br />

symptomatic or aneurismal aberrant subclavian<br />

arteries. They divided their patients into four<br />

distinct subgroups:<br />

1. dysphagia lusoria without aneurysm<br />

2. symptomatic occlusive disease of the artery<br />

3. aneurysmal disease of the artery itself<br />

4. aneurysmal disease of the thoracic aorta or<br />

origin of the aberrant artery<br />

It would appear sensible to manage all patients<br />

with aneurismal disease with stentgrafting in light<br />

of the high rupture rate (22.6%) and consequent<br />

mortality rate (100%) independent of the<br />

diameter of the aneurysm. The perioperative rate<br />

of patients undergoing surgical repair of these<br />

aneurysms was 26.9%. 12<br />

There are few reports in the literature of<br />

endovascular or hybrid approaches to this<br />

pathology. Although long term results are still<br />

pending, initial results are promising. Shennib et<br />

al have described minimally invasive, hybrid<br />

endovascular approaches . A right supraclavicular<br />

approach was used to perform a right carotidsubclavian<br />

bypass prior to division of the right<br />

SCA. They then deployed an occluder in an<br />

antegrade fashion into the proximal end of the<br />

right SCA in order to maintain good control of the<br />

deployment and to avoid embolization of the<br />

occluder into the aortic arch from a retrograde<br />

approach. A right femoral artery access was used<br />

via a 9F sheath. 20<br />

Endoluminal grafts have also been used with<br />

some success in the presence of aneurysm of the<br />

ARSA origin. In these cases it may be necessary<br />

to consider performing a further anastomosis<br />

between the left carotid and subclavian artery if<br />

overstenting was intended.<br />

Kopp et al have reported a series of six patients<br />

managed by a variety of techniques during a<br />

seventeen year period. One of the patients had<br />

16


a covered wall stent with an occluded proximal<br />

stent graft lumen inserted via a transbrachial<br />

approach. The right SCA was then anastomosed to<br />

the right common carotid artery. 21<br />

References<br />

1. Williams GD, Aff HM, Schmeckebier M, Edmonds HW, Grand EG. Variations in the<br />

arrangement of the branches arising from the aortic arch in the American whites and<br />

negroes. Anat Rec 1932; 54: 247-251<br />

2. Bayford D. An account of a singular case of obstructed degluitition. Memoirs Med<br />

Soc London 1794; 2: 275-86<br />

3. Kommerell B. Verlagerung des Osophagus durch eine abnorm verlaufende Arteria<br />

subclavia dextra (Arteria lusoria). Fortschr Geb Roentgenstrahlen 1936; 54: 590-595<br />

4. Rahman HA, Sakurai A, Dong K, Setsu T, Umetani T, Yamadori T. The<br />

Retroesophageal Subclavian Artery – A case report and Review. Acta Anat Nippon<br />

1993; 68: 281-287<br />

5. Adachi B. Das Arteriensystem der Japener. Bd 1 1928: 22-42 Kenkyu-sha<br />

Publishing Co, Tokyo<br />

6. Saito T, Tamatsukuri Y et al. Three Cases of Retroeophageal Right Subclavian<br />

Artery. J Nippon Med Sch 2005; 72 (6): 375 – 382<br />

7. Janssen M, Baggen MGA, Veen HF, Smout AJPM, Bekkers JA, Jonkman JGJ,<br />

Ouwendijk RJTh. Dysphagia Lusoria: clinical aspects, manaometric findings, diagnosis,<br />

and therapy. Am J Gastroent 2000; 95: 1411-1416<br />

8. Klinkhamer AC. Aberrant right subclavian artery. Clinical and roentgenologic<br />

aspects. Am J Roentgenol Radium Ther Nucl Med 1966; 97: 438-446.<br />

9. Gross RE, Neuhauser EBD. Compression of trachea by anomalous innominate<br />

artery: operation for its relief. Am J Dis Child 1948; 75: 570-574<br />

10. Mok CK, Cheung KL, Kong SM, Ong GB. Translocating the right subclavian artery<br />

in dysphagia lusoria. Br J Surg 1979; 66: 113-116<br />

11. Harada H, Ito T, Yamamoto N, Abe T. Surgical Treatment of an Aneurysm of the<br />

Aberrant Right Subclavian Artery Involving an Aortic Arch Aneurysm and Coronary<br />

Artery Disease. Am Thorac Cardiovasc Surg 2001; 7 (12): 109-112<br />

12. Kieffer E, Bahnini A, Koskas F. Aberrent subclavian artery: Surgical treatment in<br />

thirty-three adult patients. J Vas Surg 1994; 19: 100-111<br />

13. Gross RE. Surgical treatment of dysphagia lusoria. Ann Surg 1946; 124: 532-534<br />

14. Lichter I. The treatment of dysphagia lusoria in the adult. Br J Surg 1963; 50:<br />

793-796<br />

15. Pome G, Vitali E, Mantovani A, Panzeri E. Surgical treatment of the aberrant<br />

retroesophageal right subclavian artery in adults (dysphagia lusoria). J Cardiovasc<br />

Surg 1987; 28: 403 – 411<br />

16. Smith JM, Reul GJ, Wurash DC, Cooley DA. Retro-oesophageal subclavian arteries:<br />

surgical management of symptomatic children. Card Vasc Disease Texas Heart Inst<br />

1979; 6: 333-334<br />

17. Pifarre R, Dieter, RA, Niedballa RG. Definitive surgical treatment of the aberrant<br />

retro-oesophageal right subclavian artery in the adult. J Thorac Cardiovasc Surg 1971;<br />

61: 154-159.<br />

18. Bailey CP, Hirose T, Alba J. Re-establishment of the continuity of the anomalous<br />

right subclavian artery after operation for dysphagia lusoria. Angiology 1965; 16: 509-<br />

513<br />

19. Lemire GG, Rabbat AG, Trudel J. Dysphagia lusoria: current surgical approach. J<br />

CArdiovasc Surg 1978; 19: 311-313<br />

20. Shennib H, Diethrich EB. Novel approaches for the treatment of the aberrant right<br />

subclavian artery and its aneurysms. J Vasc Surg 2008; 47: 1066-1070.<br />

21. Kopp R, Wizgall I, Kreuzer E, Maimarakis G, Weidenhagen R, Kuhni A, Conrad C,<br />

Jauch KW, Lauterjung L. Surgical and Endovascular Treatment of Symptomatic<br />

Aberrant Right Subclavian Artery (Arteria Lusoria). Vasc 2007;15 (2): 84-91<br />

17


Embolectomies, Fly-fishing &<br />

Viticulture:<br />

What do they have in common?<br />

The answer to the question above is simple when one<br />

explores the biographical history of Thomas J. Fogarty,<br />

who as a physician and professor, inventor and<br />

entrepreneur, has saved tens of millions of lives most<br />

notably, by pioneering the tools and methods of less<br />

invasive vascular surgery.<br />

Fogarty was born and raised in Cincinnati, Ohio. His precocious mechanical ability and business<br />

instincts began as a child, designing and building soapbox derby racers and model airplanes, the latter<br />

of which he sold to children from his neighborhood. Soon, he upgraded to a motor scooter; and when<br />

he became frustrated with its gears, he built (and sold) a centrifugal clutch that is still used today in<br />

some simple motors.<br />

Fogarty's interest in medicine began in early high school when he took a job as an equipment cleaner<br />

and later scrub technician at Cincinnati's Good Samaritan Hospital. In the latter position, Fogarty was<br />

frequently able to observe operations and the problems surgeons encountered. By the time he<br />

graduated from high school, Fogarty knew that his calling was to make surgery simpler, cheaper, faster<br />

and safer through technology.<br />

Before he graduated in 1960 from the University of Cincinnati Medical School, Fogarty had designed his<br />

most significant invention. The Fogarty Balloon Embolectomy Catheter, like many revolutionary<br />

inventions is simple in concept. Fogarty built the prototype in his attic, attaching the fingertip of a latex<br />

surgical glove to a catheter using fly-tying techniques familiar to him from boyhood fishing<br />

expeditions.<br />

In 1969 Fogarty patented his device, which is now being used in over 300,000 procedures every year,<br />

all over the world. The catheter was a great improvement on previous embolectomy methods. First, it<br />

does not cut off blood flow, increasing the risk of the patient's losing a limb; and second, the entire<br />

procedure can be performed in one hour through a single small incision, instead of using many larger<br />

incisions and forceps, with the patient under general anesthesia for hours.<br />

Fogarty's balloon catheter procedure was the first successful example of minimally invasive vascular<br />

surgery. Since its introduction, Fogarty and others have developed numerous spin-off applications: for<br />

example, the first balloon angioplasty, performed with a Fogarty catheter in 1965, has led to over<br />

650,000 such operations per year.<br />

He also provides venture capital to other medical device inventors devoted to solving "real-life clinical<br />

problems." In the 1990s, Fogarty became a Professor of Surgery at Stanford University Medical Center.<br />

As if this were not enough, Fogarty has also founded an award-winning vineyard: Thomas Fogarty<br />

Winery, outside of Palo Alto, California.<br />

To date, Thomas Fogarty has personally earned 63 patents, with many others pending. He has<br />

authored or co-authored over 150 professional articles, and is a member of 29 professional societies.<br />

He has won a series of prestigious awards, including the San Francisco Patent and Trademark<br />

Association's Inventor of the Year (1980).<br />

18


Case Report: Spontaneous<br />

Aortocaval Fistula<br />

Forgan T, Du Toit D<br />

Stellenbosch University; Tygerberg Hospital, Dept<br />

of Vascular Surgery<br />

Abstract<br />

A 58 year old man presented with malena stools<br />

and a pulsatile abdominal mass (which turned out<br />

to be an aortocaval fistula (ACF.) At surgery, a<br />

Introduction<br />

A rare complication of infrarenal abdominal aortic<br />

aneurysms is ACF, which occurs in up to 4% of<br />

ruptured or symptomatic aneurysms. 1,2 Due to<br />

the rarity of ACF and the elusive clinical<br />

presentation, they are easily overlooked.<br />

Presentation ranges from the typical signs of<br />

lower back pain, palpable abdominal aortic<br />

aneurysm, machinery abdominal murmur and<br />

high-output cardiac failure to cardiac and renal<br />

failure with few other obvious signs or symptoms.<br />

16,17 Preoperative diagnosis assists in patient<br />

preparation and the selection of the most<br />

appropriate therapeutic modality.<br />

Case Report<br />

A 58 year old male was referred to our institution<br />

from a district hospital with a history of having<br />

passed malena stools, shortness of breath and<br />

hypotension. He was known to have an abdominal<br />

aortic aneurysm (AAA) of 5 cm. He had been<br />

treated for congestive cardiac failure for the<br />

previous year and was also known to have renal<br />

dysfunction. He was also an ex-smoker.<br />

On arrival he was hypotensive with a mean<br />

arterial pressure of 55mmHg and had a<br />

tachycardia of 120. He had a Glasgow Coma<br />

Score of 15 and was tachypnoeic with a<br />

respiratory rate of 30. He was complaining of<br />

central abdominal pain.<br />

On further examination he was an overweight<br />

man with a mildly tender abdomen and a pulsatile<br />

central abdominal mass. It was difficult to<br />

delineate if the mass extended above the costal<br />

margin. No thrill was palpable and no murmur<br />

was heard on auscultation of his abdomen. On<br />

rectal examination he had malena stool. The<br />

patient had a raised JVP, bibasal crepitations and<br />

bipedal oedema.<br />

A blood gas was done which showed a pH of 7,3,<br />

lactate of 5, base excess of -4 and an hb of 8.<br />

Further blood tests revealed that he had mild<br />

Figure 1, Contrasted CT Abdomen showing early<br />

filling of the IVC with contrast.<br />

Figure 2, Contrasted CT Abdomen revealing an<br />

Aortocaval fistula arising in the distal aorta<br />

renal dysfunction.<br />

At this stage the working diagnosis was that of an<br />

aorta-enteric fistula.<br />

In light of the above information a central venous<br />

catheter was inserted, careful fluid resuscitation<br />

was started and the patient was taken for an<br />

emergency CT scan of his abdomen. On returning<br />

to the resuscitation area after his scan he acutely<br />

dropped his blood pressure, decompensated and<br />

was intubated.<br />

The results of the CT scan revealed a 7.7 cm<br />

infrarenal AAA. The IVC and common iliac veins<br />

were distended and early reflux of contrast was<br />

seen (Fig. 1). A communication between the distal<br />

aorta and the IVC was also demonstrated. (Fig. 2)<br />

After further resuscitation the patient stabilized<br />

and he was taken to theatre, where a midline<br />

laparotomy was done. On opening the abdomen<br />

about a liter of ascitic fluid was noted, in<br />

19


conjunction with a congested liver. In light of his<br />

initial complaint of malena a gastrotomy was<br />

performed, revealing no blood in the stomach and<br />

a small pre-pyloric ulcer. The ulcer had a black<br />

spot in its base, which was over sewn.<br />

At this stage the patient had been fully<br />

resuscitated and was stable. It was thus decided<br />

to continue with the surgery and address his<br />

aortocaval fistula.<br />

A standard transabdominal retroperitoneal<br />

exposure was performed, with medial visceral<br />

rotation and mobilization of the duodenum away<br />

from the aorta. Once the aorta was suitably<br />

exposed, proximal and distal control was achieved<br />

(proximally to the infrarenal aorta and to bilateral<br />

common iliac arteries distally), IVC control was<br />

obtained with digital pressure and the aneurysm<br />

sac was opened. After haematoma evacuation<br />

from the aneurysm sac the fistula was identified<br />

and closed with transaortic gore-tex suture. Once<br />

the fistula was closed lumbar arterial bleeders<br />

were suture ligated. A standard Dacron graft was<br />

used to repair the aneurysm.<br />

Post operatively the patient did well. After initial<br />

worsening in renal function (possibly due to<br />

hypotension and contrast nephropathy) there was<br />

normalization within two weeks. His CCF also<br />

recovered dramatically, and on discharge from<br />

hospital all cardiac failure medication had been<br />

stopped.<br />

At three month follow up the patient was doing<br />

well with normal renal functions and no signs of<br />

cardiac dysfunction.<br />

Discussion<br />

The most common cause of aortocaval fistula is a<br />

degenerative aneurysm that has eroded into the<br />

adjacent IVC. It may also result from penetrating<br />

abdominal trauma and iatrogenic trauma at<br />

lumbar disc surgery. Rare causes include mycotic<br />

aneurysm, syphilis and connective tissue<br />

disorders such as Ehlers-Danlos syndrome and<br />

Marfan’s syndrome. The overwhelming majority of<br />

patients affected are males in their seventh and<br />

eighth decades of life.<br />

The original description of an aortocaval fistula<br />

(ACF) is attributed to James Syme in 1831. 3 In<br />

later years, Javid, 4 Eisman and Hughes 5 and<br />

DeBakey et al 6 all reported successful repairs.<br />

Prior to the production of prosthetic grafts for<br />

vascular replacement, only desperate measures<br />

such as quadruple ligation and packing were<br />

available. 7 Matas, who described the technique of<br />

endoaneurysmorraphy for traumatic fistulas,<br />

significantly influenced the treatment of ACF. 8<br />

Because the aortocaval fistula is rare and has an<br />

elusive clinical presentation, it can easily be<br />

overlooked. Clinical presentation is commonly<br />

acute but long-standing complaints such as<br />

cardiac and renal failure are also reported. Half<br />

the patients present with high-output,<br />

hyperdynamic circulation with a widened pulse<br />

pressure and a relatively low diastolic pressure. 13<br />

The triad of low back pain, a palpable abdominal<br />

aortic aneurysm and a machinery abdominal<br />

murmur/ continuous bruit is diagnostic 16 and may<br />

be associated with high output cardiac failure and<br />

regional venous hypertension. 14,15<br />

In ACF, fluctuations in haemodynamic status are<br />

as a result of the arterio-venous (AV) fistula<br />

diverting blood flow from the high resistance<br />

arterial circuit to the low resistance and high<br />

capacitance venous circuit. There is a resultant<br />

decrease in total peripheral resistance and a<br />

subsequent increase in venous pressure,<br />

resistance and volume leading to acute pulmonary<br />

oedema and CCF (decompensated cardiac failure<br />

due to increased venous return occurs in 35% of<br />

patients). 16 There is therefore a resultant increase<br />

in heart rate, stroke volume and cardiac output. If<br />

the fistula persists, the myocardium<br />

hypertrophies, and can dilate and lead to<br />

irreversible, hyperdynamic cardiac failure. It is<br />

important to note that cardiac failure secondary<br />

to ACF is refractory to medical treatment. 13 In our<br />

case the patient had been managed for<br />

progressively worsening cardiac failure for a year<br />

before his surgery. Post fistula repair his failure<br />

resolved, indicating that he had had a small<br />

fistula that had been slowly enlarging during that<br />

time.<br />

The pathophysiology of the renal dysfunction in<br />

ACF is unclear. It may not simply be the result of<br />

decreased renal blood flow caused by the heart<br />

failure, but may also arise from increased central<br />

venous pressure decreasing the renal perfusion<br />

pressure, or redistribution of renal blood flow. 17<br />

Haematuria and acute renal failure may occur as<br />

a result of a renal infarction due to renal arterial<br />

problems, or due to renal congestion from a<br />

perforation of an AAA into the renal vein. 18<br />

A contrast-enhanced CT scan has become the<br />

standard method for making a definitive preoperative<br />

diagnosis in haemodynamically stable<br />

patients. The findings on CTA include early<br />

detection of contrast medium in a dilated IVC,<br />

which is isodense with the adjacent aorta, an<br />

associated AAA; the loss of the normal anatomic<br />

space between the IVC and the aorta; and rarely,<br />

the fistula itself may be visualized. 18 The<br />

diagnosis may also be made with magnetic<br />

resonance imaging (MRI), duplex Doppler<br />

ultrasound or digital subtraction angiography. 10<br />

20


Perioperative mortality is high, ranging from 20 to<br />

40%. This is due to the typical patient having<br />

multiple comorbidities. 9 Successful treatment<br />

depends on management of perioperative<br />

haemodynamics, control of bleeding from the<br />

fistula and prevention of deep vein thrombosis<br />

and pulmonary embolism.<br />

The treatment options available have evolved as<br />

technology has advanced. Therapeutic options<br />

include standard operative tube graft repair,<br />

endovascular repair or a hybrid approach.<br />

Operative repair entails a transabdominal<br />

approach with transaortic suture of the fistula and<br />

tube graft placement. Endovascular repair<br />

involves the correct patient selection (this<br />

includes overall patient condition and anatomical<br />

considerations), followed by the endovascular<br />

placement of a bifurcated endovascular graft. 19<br />

Endovascular repair is complicated by theoretical<br />

concerns about the persistent communication<br />

between the aortic sac and the IVC. The presence<br />

of this communication could facilitate the<br />

development of a high-flow type II endoleak<br />

which, if allowed to mature, may lead to<br />

persistent increased cardiac output and sac<br />

pressurization with a resultant increase in the<br />

diameter of the aneurysm sac. Case reports have<br />

revealed that, even if present, the endoleak is<br />

often not significant and usually self limiting. 19 If<br />

the patient’s anatomy is unsuitable for<br />

endovascular repair, a hybrid approach may be<br />

considered. In hybrid procedures, the IVC is<br />

stented, thereby closing the fistula; followed by<br />

open aneurysm repair. By stenting the fistula<br />

prior to open repair, the patient’s haemodynamics<br />

are stabilized, and pulmonary embolism and the<br />

high risk of massive haemorrhage from the fistula<br />

during aneurysm repair are prevented. 20<br />

Conclusion<br />

Aortocaval fistula is a rare complication of aortic<br />

aneurysm. The presentation varies widely;<br />

ranging from acute decompensation to insidious<br />

worsening of apparently unrelated medical<br />

problems, such as cardiac and renal failure.<br />

Preoperative identification of the fistula and<br />

selection of the most appropriate therapeutic<br />

modality for the patient are vital to a successful<br />

outcome.<br />

References<br />

1. Baker WH, Sharzer LA, Ehrenhaft JL. Aortocaval fistula as a complication of abdominal aortic aneurysms. Surgery<br />

1972; 72 :933 –8<br />

2. Syme J. Case of spontaneous varicose aneurysm. Med Surg J 1831;36 :pg104-5.<br />

3. Javid H, Dye WS, Grove JW, et al. Resection of ruptured aneurysms of abdominal aorta. Ann Surg 1955;142:621 -<br />

3.<br />

4. Eisman B, Hughes RH. Repair of an abdominal aortic vena cava fistula caused by rupture of an arteriosclerotic<br />

aneurysm. Surgery 1956;39:498-04.<br />

5. Debakey ME, Cooley DA, Morris GC, et al. Arterio-venous fistula involving the abdominal aorta. Report of four cases<br />

with successful repair. Ann Surg 1958;147:646-58.<br />

6. Lehman EP. Spontaneous arteriovenous fistula between the abdominal aorta and the inferior vena cava. Ann Surg<br />

1938;108: 694-700.<br />

7. Matas R. Surgery of the Vascular system. In: Keen WW, editor. Surgery: its principles and practice. Philadelphia: WB<br />

Saunders; 1909.p. 2.<br />

8. Gourdin FW, Salam AA, Smith RB III, Perdue GD. Aortovenous fistulas due to ruptured infrarenal aortic aneurysms.<br />

South Med J 1982; 75 :913 –6<br />

9. R. Schmidt, C. Bruns, M. Walter and H. Erasmi, Aorto-caval fistula—an<br />

uncommon complication of infrarenal aortic aneurysms, Thorac Cardiovasc<br />

Surg 42 (1994) (4), pp. 208–211.<br />

10. Gaa J, Bohm C, Richter A, et al. Aortocaval fistula complicating abdominal aortic aneurysm: diagnosis with<br />

gadolinium-enhanced three-dimensional MR angiography. Eur Radiol 1999; 9: 1438-1440<br />

11. Aorto-caval fistula from acute rupture of an abdominal aortic aneurysm treated with a hybrid approach Matthias<br />

Siepe, Sabrina Koeppe, Wulf Euringer and Christian Schlensak Journal of Vascular Surgery 49(6) June 2009 pg<br />

1574-1576<br />

12. Farid A, Sulivan TM. Aortocaval fistula in ruptured inflammatory abdominal aortic aneurysm. A report of two cases<br />

and literature review. J Cardiovasc Surg. 1996; 37:561-565<br />

13. Rajmohan B. Spontaneous aortocaval fistula. J Post Grad Med. 2002; 48:203-205<br />

14. Abbadi AC, Deldime P, Van Espen D. The spontaneous aorto caval fistula: a complication of the abdominal aortic<br />

aneurysm. Case report and review of the literature. J Cardiovasc Surg. 1998; 39:433-436<br />

15. Sier JC, Meijer S. Aorto-caval fistula as a complication of ruptured abdominal aortic aneurysm. Neth J Surg 1980;<br />

32:154-156<br />

16. Cortis BS, Jablokow VR, Shah AN, Cortis PF. Spontaneous rupture of an abdominal aortic aneurysm into the inferior<br />

vena cava: a case report and review of literature. Mt Sinai J Med (NY) 1972;39:566-72.<br />

17. Albalate M, Octavio JG, Llobregat R, et al. Acute renal failure due to aortocaval<br />

fistula. Nephrol Dial Transplant 1998; 13: 1268-1270.<br />

18. Abrams Angiography- Vascular and interventional radiology (IV ed) vol 1. Pg<br />

962<br />

19. M. Vetrhus, R. McWilliams, C.K. Tan, J. Brennan, G. Gilling-Smith and P.L.<br />

Harris Endovascular Repair of Abdominal Aortic Aneurysms with Aortocaval<br />

Fistula, European Journal of Vascular and Endovascular Surgery Volume 30,<br />

Issue 6, December 2005, Pages 640-643<br />

20. Siepe M, Koeppe S, Euringer W, Schlensak C. Aorto-caval fistula from acute<br />

rupture of an abdominal aortic aneurysm treated with a hybrid approach J<br />

Vasc Surg. 2009 Jun;49(6):1574-6.<br />

"Everyday Challenges in<br />

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