• Title: Compelling intermediate-term data on the operative treatment of iliac endofibrosis
  • Open Access: Ja
  • Language: English
  • Year: 2023
  • DOI/URL: https://doi.org/10.1016/j.jvs.2023.04.008
  • Publication Date: 01-08-2023
  • Journal: Elsevier - Journal of Vascular Surgery
  • Authors:

    David Andrew Rigberg

  • Pubmed ID: 37481278
The paper by van Hooff et al. presents longer term follow-up on a large number of patients with iliac endofibrosis who have undergone operative repair with endarterectomy and patching (excluding those treated only for a kink, which they have previously published). The results after a median of 11 years are excellent, with overall operative satisfaction at 91.7% and 94.5% continued symptom reduction. This outcome is even more encouraging than the numbers suggest, because these patients are involved in very high levels of athletic performance. Although prior reports have described some intermediate-term results, these new data are compelling.
In addition to patient satisfaction, the authors provide evidence of the longer term durability of autogenous saphenous vein patches for these reconstructions. This is of great importance for patches under tremendous hemodynamic forces. Even longer periods of follow-up are required before there are definitive data on the best material for patches in this setting.
The authors performed an extensive operation, with retroperitoneal and femoral incisions, dividing and exteriorizing the external iliac artery through the groin, endarterectomy with a patch, and re-anastomosing the repaired vessel (with shortening if needed). Although useful for disease extending beyond the inguinal ligament, most cases can be done without a groin incision or dividing the external iliac artery, unless vessel shortening is required. This technique seems overly complex with the potential for complications. The key to successful treatment would seem to be identifying the particular variant presenting correctly—redundancy, kinking, fibrosis, or a combination of these—and to ensure that the issues are addressed. One should also be mindful of associated thromboembolic complications and arterial dissections.
This excellent report adds to a growing body of literature on diagnostic techniques, treatment strategies, and outcome data for iliac endofibrosis and provides strong evidence for the effectiveness, safety, and longer term outcomes of interventions for this disease. It is important to consider the young ages of these patients, and certainly longer term follow-up will be needed, because many decades of durability are required of these repairs.
The opinions or views expressed in this commentary are those of the authors and do not necessarily reflect the opinions or recommendations of the Journal of Vascular Surgery or the Society for Vascular Surgery.

  • Title: The biology of external iliac artery endofibosis: What can we learn from this disease to help us treat other fibroproliferative arteriopathies?
  • Open Access: Ja
  • Language: English
  • Year: 2019
  • DOI/URL: https://doi.org/10.1016/j.jvs.2018.06.188
  • Publication Date: 01-04-2019
  • Journal: Elsevier - Journal of Vascular Surgery
  • Authors:

    Upchurch, G.

  • Pubmed ID: 30819592

Arterial endofibrosis is a vascular disease that typically affects the external iliac artery in young patients who undergo extreme exercise, mainly bicyclists.1 The diagnosis is indirectly suspected when these extreme athletes undergo ankle-brachial indices (ABIs) before and after extreme exercise. Whereas the diagnosis can often be suggested by exercise ABIs and computed tomography angiography, the diagnosis is classically best defined by angiography. Dr Cherry and his group were one of the first to recognize this lesion when they described seven patients who underwent replacement or repair of the external iliac artery. Although originally skeptical as to whether this was indeed a “true” vasculopathy (ie, see median arcuate ligament syndrome and functional popliteal entrapment), with >7 years as Dr Cherry's partner and his being kind enough to share the films with us, I am completely convinced that this disease is real and often debilitating in these extreme athletes. One has to be willing to work hard to make the diagnosis in these typically young, ultrahealthy patients in whom standard walking tests are not adequate to drop the ABI. In our peripheral vascular laboratory, we actually purchased a stationary bicycle to assist in making this diagnosis.
In the paper by Posthuma and colleagues from The Netherlands, the authors have married our understanding of the impact of hemodynamic forces with the changes in the biology of the arterial wall. In this study of 19 mainly male bicyclists, the authors clearly document a disease whereby a hyperactive intima and media generate changes in the arterial wall that lead to lumen narrowing. The authors also note in this series that 14 of the 19 patients had disease primarily on the left, with no patient presenting with bilateral disease. This is in contrast to a recent series by Politano and Cherry, who suggested that 4 of 27 repairs were bilateral.
In this series, the authors performed endarterectomies only, no bypasses, on 19 patients for external iliac artery endofibrosis. The histopathologic features of these endarterectomized samples were then compared with cadaveric external iliac artery samples, which the authors state in their limitations paragraph are likely not the right controls (not age-matched normal). Regardless, we are able to get a nice snapshot of the impact of the hemodynamic process whereby the external iliac artery is pinned down in the pelvis by the internal iliac artery. A study also suggested that the relatively fixed inguinal ligament plays a role in this disease process as well.2 These arteries also often lengthen and become kinked. The extreme shear in a relatively fixed position leads to the activation and proliferation of myofibroblasts. This process looks similar to both neointimal hyperplasia and fibromuscular dysplasia. Fibromuscular dysplasia, which often occurs in ptotic kidneys that are “pinned” in the pelvis, probably represents a fibroproliferative arteriopathy. During the course of this proliferative process, extracellular matrix proteins like elastin, collagen, and vimentin are increased significantly, leading to arterial stenosis. In addition, thrombotic factors (factor X and tissue factor) and protein activated receptors (1 and 4) are also increased.
Perhaps vascular surgeons who are asked to see these patients might be unwilling to operate on this disease process, instead suggesting retirement from competitive cycling. Please recognize that many of these ultracompetitive athletes make a living performing extreme exercise. As a surgeon scientist, I applaud these authors for trying to understand the pathobiology of this disease process. I also suggest that we need more studies like the present one to help us not only biologically define the disease but, importantly, urge us to take the next natural step and start using our knowledge about this disease and its proliferative nature to develop an effective medical therapy. Sounds like a great KO8 project for an aspiring surgeon scientist.
The opinions or views expressed in this commentary are those of the author and do not necessarily reflect the opinions or recommendations of the Journal of Vascular Surgery or the Society for Vascular Surgery.

L'endofibrose de l'artère iliaque externe, survenant chez des cyclistes de moyen ou haut niveau, bien que de connaissance récente (Walder et coll., 1984, 1, 2) est maintenant une entité bien connue (Chevalier et coll., 3). Elle se manifeste cliniquement par une gêne, voire une douleur prédominant au muscle quadriceps, suivie d'une impression de paralysie de tout le membre inférieur, parfois de gonflement, mais ne survenant que lors d'un effort submaximal et régressant rapidement lorsque cet effort devient moins important.
Toutefois, d'autres étiologies peuvent présenter une symptomatologie pratiquement identique ainsi qu'en té- moigne l'observation suivante:

  • Title: Iliac Artery Endofibrosis
  • Open Access: Ja
  • Language: English
  • Year: 2016
  • DOI/URL: https://doi.org/10.1016/j.ejvs.2016.04.006
  • Publication Date: 01-07-2017
  • Journal: Elsevier - European Journal of Vascular and Endovascular Surgery
  • Authors:

    R.J. Hinchliffe

  • Pubmed ID: 27161328
The investigation and management of peripheral arterial diseases in elderly patients, frequently with multiple co-morbidities, are familiar to all vascular surgeons. These patients are at high risk of limb loss and premature death and intervention is easy to justify. In fact, vascular techniques have advanced rapidly to meet the challenges in this population and it is now possible to offer medical therapies and interventions to preserve life and limb even in the most elderly and frail patients.
Vascular disease in younger patients, especially those who are elite athletes, represents a different challenge. Healthcare professionals including physical therapists and doctors in primary care and sports medicine rarely manage conditions such as iliac artery endofibrosis. Most vascular surgeons infrequently manage the condition. Patients presenting to a vascular surgeon with suspected iliac artery endofibrosis often do so against a backdrop of delay or failure to suspect or diagnose vascular disease. Consequently, patients frequently present late. They are often frustrated by their symptoms and impaired athletic performance and have been investigated extensively by an inter-disciplinary group of healthcare professionals usually focusing on musculoskeletal pathology. They may have seen neurologists and even psychologists.
Most athletes experience minor injuries from which they make a rapid and full recovery. Therefore, the expectations of these young people and those associated with their welfare are high. Invariably, they expect a rapid and perfect solution with early return to competitive sport. For many it is unfathomable that they may have a career-threatening disease.
The indications and outcomes of vascular interventions in younger patients are concerning because they remain largely unchartered territory. The effectiveness of vascular surgery in preventing amputation and death is difficult to dispute in older patients with advanced disease. However, its effectiveness in restoring perfect health and improving athletic performance remains to be established. The demands placed on a vascular surgical reconstruction in an athlete far outweigh any that might be considered necessary or normal for most patients within the realms of standard clinical practice. And the durability of any surgical repair will, perhaps be required for half a century or more. These factors undoubtedly explain why many vascular surgeons feel a certain reluctance to offer surgery to the youngest patients with mild or moderate (in many instances only haemodynamically significant under the most severe exercise load) vascular disease unless there is clear evidence that the underlying disease process is likely to be life or limb threatening.
Published data on the outcomes of surgical intervention in the short term are sparse and almost non-existent beyond 5 years. There have even been reports of deaths and major (limb-threatening) complications following surgery. And yet there appear to be an increasing number of patients being offered surgery. For patients there are clearly a number of complex motives not least financial ones for professional athletes. But given the requirements and demands placed on vascular reconstructions it is hard to understand why any sensible vascular surgeon would recommend vascular reconstruction for a young otherwise perfectly healthy person with iliac artery endofibrosis.
There are some reasons why iliac artery endofibrosis appear to be increasing. Cycling in particular is a booming sport with increasing participation. High training loads and extreme aerodynamic cycling positions may be contributory. There is an increasing awareness of the problem especially among the professional peloton. The detection rate may also be increasing in those presenting to vascular surgeons following attempts that have been made to standardise (non-invasive) diagnostic algorithms to detect the disease in specialist units.
The true incidence especially in elite athletes may just be a lot higher than previously recognised with haemodynamic evidence of endofibrosis in some 10–20% of elite athletes. And there is some suggestion that surgeons have become more liberal in their indications for surgery. This may be consequent to observations of progression of endofibrotic disease in those who continue athletic activity and a smattering of reports of acute limb ischaemia due to dissection and occlusion of external iliac arteries. Preliminary outcome data from specialist units appear encouraging with few serious complications and most athletes appearing to resume competitive sport.
There are difficulties for surgeons and patients alike in that there appear to be no viable alternatives to relieve symptoms other than exercise cessation or surgery. Conservative therapies such as the use of biomechanics and physiotherapy appear to be largely ineffective, and interventions that may prove useful for peripheral artery disease due to atherosclerosis such as supervised exercise therapy and angioplasty/stenting are either ineffective or contra-indicated.
For many, exercise cessation is the most reasonable management strategy. Persuading elite athletes and more surprisingly “weekend warriors” that this is the most sensible decision should not be underestimated and may be extremely difficult. Others may wish to continue to exercise if their symptoms are stable.
Until recently, efforts to understand iliac artery endofibrosis have been limited to a handful of specialist centres that have reported on their own diagnostic algorithms and surgical outcomes. The INSITE Group have performed a consensus study on the current diagnosis and management of iliac endofibrosis. This is a useful first step to draw together some of the disparate opinions on the diagnosis, natural history, and management of the condition. However, much remains to be done if we are going to standardise the overall management of the condition and therefore improve patient outcomes and prevent unnecessary patient harm. Key issues include delineating the natural history of the disease and identifying those at highest risk of developing the condition and of developing the condition and especially its complications.
A disease registry for patients with iliac endofibrosis will prove invaluable to patients and surgeons alike. It is no longer acceptable to operate on patients and fail to record their outcomes for external scrutiny and the promotion of clinical research. The use of these sorts of registers has greatly improved our understanding of a number of other rare diseases. And in some cases it has also been possible to perform randomised trials.
For elite cyclists and triathletes, iliac artery endofibrosis could be considered to amount to an occupational disease. Athletes and their governing bodies should consider the wider impact of the disease on their sport and members. Both have an important role to play in promoting and contributing to research in the field. Only with the support of athletes, the medical teams that manage them, and governing bodies will it be possible to perform the necessary clinical research to improve care.
Perhaps it is time for vascular surgeons to look to the example set in sports cardiology. Sudden cardiac deaths are shocking in young athletes. These experiences have galvanised the sporting community to work with cardiologists to generate the necessary research to identify those at highest risk of sudden death and develop methods to mitigate the risk. Few would dispute the progress that has been made in sports cardiology including the delivery of national cardiac screening programmes. These advances could be replicated in peripheral arterial diseases.

  • Title: Intermittent leg ischaemia during competition cycling
  • Open Access: Nee
  • Language: English
  • Year: 1990
  • DOI/URL: https://doi.org/10.1016/0140-6736(90)92236-b
  • Publication Date: 22-09-1990
  • Journal: The Lancet
  • Authors:

    François Mosimann and Jean Walder

  • Pubmed ID: 1975912