James H. Black, III, MD, FACS
Johns Hopkins School of Medicine
The EJ Wylie Traveling Fellowship was conducted between July 2012 and July 2013. The purpose of the fellowship was to meet European colleagues who had a clinical and scientific interest in the management of patients who have connective tissue disorders (CTD) with vascular manifestations. Via such introductions, novel research and clinical paradigms could be designed and considered. Furthermore, given the relative rarity of CTD, sharing our collective experiences would allow us to gain the insight to challenge some of the conventional wisdom in the clinical realm.
The management of CTD has progressed remarkably and my EJ Wylie Traveling Fellowship demonstrated the significant progress that USA and European groups have made through diligent research. Indeed, the prior conventions that genetic disorders imply a constitutional weakness of arterial structures in CTD cast a long shadow over the field until recently. There is growing evidence that elastin and collagen fiber maintenance is a life-long process with many potential mechanisms for improvement. In this spirit, I sought some of the best opportunities for professional interchange.
The Fellowship aided my travel to Ghent, Belgium. Often called the “Venice of Northern Europe”, this picturesque city is traversed with canals among its medieval castles, period buildings, and vaulted cathedrals. The city was the host of the First International Symposium of Ehlers-Danlos Syndrome. In this venue, groups from around the world convened to consider the diagnosis, treatment, and knowledge deficiencies of patients with EDS. Indeed, particularly for patients with Vascular Ehlers-Danlos, the agreement among the Symposium was one of clinical desperation for a viable medical option or prophlyaxis.
The Fellowship motivated important interchange to help center the discussion about vascular surgery and it potential to favorably impact not just VEDS, but other CTD as well. I was able to lend my expertise to the discussion of endovascular strategies to address vascular catastrophes as well as introduce the concept of safe elective surgery over the reactionary stance to delay until faced with life threatening ruptures. This contradicts the conventional wisdom in VEDS. As a vascular surgeon, the concepts I have taken in “hypotensive hemostasis” in ruptured aneurysms, can have translation to reducing hemodynamic shear stresses in elective surgery of CTD patients where tissue fragility is a factor in safe surgery.
While I had originally considered a collaborative approach to tissue banking with European colleagues, we have appreciated imaging of vascular structures and surveillance strategies represent a “low hanging fruit” to better identify CTD patients at imminent risk. I have realized via the Fellowship travel, the imaging we as vascular and endovascular surgeons have mastered has the potential for demonstrating untoward anatomy and aneurysm progression in a very deliberate fashion. In a group of physicians I met through the Fellowship, we agreed accuracy of such imaging can be pivotal in decision-making, particularly in the pre-teen and teenage patients where the conventional adult thresholds may be too conservative.
The Fellowship also demonstrated the benefits of centralized systems to address complex genetic diseases. Certainly, the smaller size of EU countries makes the logistical hurdles less cumbersome, but in France, all VEDS patients are referred to Georges Pompidou European Hospital. This allows rapid translational research and clinical trials for effective medical therapies. As a consequence, their team shared my clinical impressions of the lesser numbers of men appreciated with the disorder as people become aware of their VEDS disorder in their 20s and 30s. Whether gonadal hormones play a role in early demise of young men, or female hormones are protective, may have important biological implications - not just for syndromic aneurysms but also degenerative disease.
In summary, the EJ Wylie Traveling Fellowship provided among the best professional experiences of my academic career. Testimony to its success is my growing network of like-minded doctors who manage CTD and the list of questions raised in my mind about how I can improve the diagnosis and treatment of the CTD patient, who at times are among the most challenging and desperate patients we encounter. Without a doubt, both of these benefits of the Fellowship will echo decades into my future career.
Posted March 2014