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Show EDITORIAL Managing Carotid- Cavernous Fistulas in Ehlers- Danlos Syndrome Type IV Phillip D. Purdy, MD In this issue of Journal ofNeuro- Ophthalmology ( pp 102- 106), Hideki Chuman, MD, PhD, Wayne T. Cornblath, MD, Jonathan D. Trobe, MD, and Stephen S. Gebarski, MD, describe two cases of Ehlers- Danlos IV ( EDS IV) that presented with direct ( spontaneous) carotid cavernous fistulas. In the first case, the clinical diagnosis of EDS IV was made between the time of the diagnostic arteriogram and the time of the treatment. In the second case, the diagnosis of EDS IV was confirmed by punch biopsy after the endovascular treatment. Both cases were treated by transvenous deposition of coils in the cavernous sinus. The authors' review of the literature regarding EDS IV is both timely and enlightening for those involved in the care of these patients. Given the frequency of presentation with carotid cavernous fistula, and the prominence of neuro- ophthalmologists in the early care and identification of this disease, familiarity with this clinical circumstance is certainly warranted in this community. The emphasis of the authors on the hazards and pitfalls in this disease is appropriate. By virtue of the genetics and vascular practices at our facility, we also have had occasion to observe several patients. One is tempted to say they should be followed by magnetic resonance angiography ( MRA), and their initial evaluation should include this technique. However, given the prevalence of intracerebral aneurysms in this disease, patients often have aneurysm clips that preclude routine MRA. Their need for long- term care and careful clinical follow- up is underscored by their propensity to develop new intracerebral aneurysms. Two of our cases with less fortunate outcomes than those presented here come to mind in emphasizing the hazards of this disease and the importance of delicacy in its management. In one patient, I had performed multiple arteriograms over several years because of the development of many intracranial aneurysms. She had also developed a carotid- cavernous fistula, which had been successfully treated. However, her fistula recurred. At the end of diagnostic catheter cerebral angiography, she developed an iliac artery tear where the catheter had dwelt. The extravasation was emergently treated with stent placement but the iliac artery began leaking at the ends of the stent. She was taken to the operating room emergently, where she began hemorrhaging uncontrollably from multiple venous sites and eventually died. In another patient, following diagnostic cerebral angiography, a splenic artery ruptured even though that vessel had never had a catheter or guidewire placed in it. Again the patient died. In spite of short- term endovascular triumphs, the long- term prognosis remains very guarded. I support the use of a transvenous approach with coil deposition as the safest among a group of unsafe available choices. Unfortunately, the transvenous route is not universally available, because some patients with direct carotid- cavernous fistulas have lost Director of Neuroradiology, Orien and Jack Woolf MD Distinguished Chair in Neurosurgery and Neuroangiography, University of Texas- Southwestern, Dallas, Texas, USA. Address correspondence to Phillip D. Purdy, Southwestern Medical Center 5323 Harry Hines Boulevard, Dallas, TX 75390- 8896, USA nJNeuro- QpMiqlmol,. Vol. 22, No. 2, 2002 „ , „ , . , . , . DOI: 10.109.7/ 01. WNO. P00001g220. § 3526.60 .. 73 , Copyright © Lip pincott Williams & Wilkins. Unauthorized reproduction of this articfe is prohibited JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 EDITORIAL their inferior petrosal sinuses. And although arterial fragility is well known in these patients, similar fragility affects the venous side ( witness our patient above who developed venous bleeding from multiple sites in the operating room). When a neuro- ophthalmologist first encounters a possible EDS IV patient, early consultation with an endo-vascular surgeon should be obtained with the aim of minimizing invasive procedures. MRA should be used to make a diagnosis of carotid cavernous fistula, in order that the catheter angiographic procedure may be reserved for the planned intervention. However, the coexistence of intracerebral aneurysms must be anticipated, and a full diagnostic angiographic procedure should be performed at the time of treatment, both for counseling and as a baseline for subsequent follow- up care. The role of genetic counseling of these patients and their families is appropriately highlighted in this paper. Developments in gene therapy represent the best hope for greater normality in the lives of those afflicted with this dreadful condition. _ 74 . . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. |
