Okay, our last abstract before the beverage break, and please come back after the beverage break because we have a real treat with Hunt Bajer giving us a talk on service and neurosurgery. It is a talk by Dr. Andrew Wilkinson on aneurysms and autosomal-dominant polycystic kidney disease, high rates of de novo formation, and screening failures. There you go. So left is forward, right is back, and you can also scroll back and forth. Thank you. All right, thank you. It's an honor to be able to present results of our study of cerebral aneurysms and polycystic kidney disease. I have no disclosures. By way of background, autosomal-dominant polycystic kidney disease is the most common potentially fatal monogenic disease, with prevalence estimated at 1 in 400 to 1 in 1,000, which corresponds to about half a million patients in the U.S. The increased incidence of aneurysms in this population has been well described, but, of course, you only find the aneurysms that you look for, and, accordingly, there's a wide range in the reported prevalence of aneurysms in these patients, a series range from 4 to 33 percent. Probably the most comprehensive study prospectively screened over 350 consecutive patients with polycystic kidney disease, finding 12 percent harbored intracranial aneurysms, including 23 percent in those over the age of 60. The majority were small, and all in that particular study were in the anterior circulation. So the goal of our study was to examine both neurologic as well as renal outcomes of patients treated by either modality, open surgery or endovascular, as well as those patients that were observed. And in doing so, we met with some of the nephrologists at our center and came across a discrepancy in recommendations for screening in this population among the two communities. The recommendations we were familiar with were the stroke recommendations, which I've listed on the left. These are known to neurosurgeons and neurologists that recommend offering noninvasive screening, that is, CTA or MRA, to polycystic kidney patients, really to all polycystic kidney patients, but particularly those with risk factors. For the nephrologists, on the other hand, their consensus guidelines call for selective screening only, based on the presence of other risk factors. Interestingly, they cite high-risk professions, such as airline pilots. This is in their consensus document, as well as patient anxiety. And so when we're talking about half a million patients in the U.S., this is a significant number who are either screened or not screened. On the side, not part of this study, but when we started to look at this through a database, perhaps unsurprisingly, given that nephrologists manage the majority of these patients, their recommendations are the ones that are followed. And so really only about 20 percent of these patients are screened for aneurysms. But for the current study, we performed a retrospective review at our institution of all patients with both polycystic kidney disease, as well as aneurysms, which were both those who were treated, as well as those who were observed. There were 45 patients who harbored 71 aneurysms. This is consistent with other studies, which have documented a high incidence of patients having multiple aneurysms. Eleven patients presented with rupture. And this was one way in which we broke it down. So the highest number of aneurysms were treated electively after screening. The next highest number, 22, were managed with observation. And then 12 aneurysms were treated in the ruptured setting. This included 12 aneurysms treated in 11 patients. There was one patient in whom we could not definitively identify the aneurysm that had ruptured. And so two aneurysms were coiled. Among treated aneurysms in the ruptured subset, just over half were treated microsurgically. And among those treated electively, over 80 percent were treated with microsurgery. And this is a much higher rate than the nonpolycystic kidney patients of open treatment. Factors favoring open treatment included the relatively young age of these patients, their often borderline renal function, and a desire to avoid contrast if necessary. And as I mentioned, oftentimes they harbored multiple aneurysms, which facilitated the treating of multiple aneurysms through a single craniotomy. Briefly to review, those who presented with subarachnoid hemorrhage, among these patients, the average size was 7 millimeters. Again, younger patients, so the average age was 47. This is consistent with other studies, which show they present with rupture about 10 years earlier than other patients. And I'll point out, half were on dialysis at the time of rupture. And then the last two lines, almost two-thirds had known polycystic kidney disease at the time of their rupture. And two of them had actually had prior imaging. And so these were de novo aneurysms that had formed in the interval. Just over half, as I mentioned, were treated microsurgically. Only two had acute kidney injury, and two had poor outcomes at six-month follow-up. These were both patients who presented with high-grade hemorrhage. So I'll review what we classified as the screening failures, and these included both failure to screen as well as patients who were screened, but which we considered failure. So seven of the 11 ruptures, as I mentioned, were candidates for screening, in that their polycystic kidney disease was known prior to their rupture. Only two had had previous screening, which were three and five years prior to their rupture. And so five had had no screening in accordance with the nephrology guidelines, but not in accordance with the stroke guidelines. I'll point out one. This was an unfortunate but interesting case. This was a patient who had been screened. This is a contrasted MRA, which was performed just as screening. Of course, hindsight is 20-20, and it's easy to pick out on this single slice, which was read as normal. You can see an area of abnormality there, anterior to the ACOM complex. Again, reported as normal. Unfortunately, the patient presented five years later with his rupture. And now you can see much more clearly on the angiogram the ruptured ACOM aneurysm. Fortunately, this was treated with coiling, and the patient did well. Moving on to the patients that were treated electively. So 37 aneurysms in 25 patients. The average size of the treated aneurysm was 5 millimeters. That's smaller than our non-polycystic elective treatment average. Again, young patients, the average age was 47. They're generally healthier, so only 20% were on dialysis at the time of treatment. And they had high rates of both familial as well as personal history of subarachnoid hemorrhage, that is, so-called ISUA type 2 patients that had bled from a separate aneurysm. Just over 80% were treated electively. There were no cases of kidney injury. And importantly, all were discharged home with good outcomes. Among patients that were observed, these were small aneurysms, generally in older, sicker patients. And none of the aneurysms that were observed were seen to rupture later. Lastly, rates of de novo development. In 316 years of clinical follow-up, there were eight de novo aneurysms noted, including three which were treated. The incidence of de novo development, and this was only a minimum because not all of them had radiographic follow-ups, so we assume that those who didn't have radiographic follow-up did not have aneurysms. The last follow-up was 2.5% per year. To put this into perspective, that's an order of magnitude higher than the de novo development rate among patients with familial aneurysm syndromes. So to conclude, elective treatment of these patients can be performed safely with either open or endovascular techniques. The rate of de novo aneurysm development was high, warranting repeat screening in those with known aneurysms. Anesthesia screening is currently performed, is done only selectively, and the majority of patients presenting with subarachnoid hemorrhage had previously known polycystic kidney disease. I'd like to thank my neurosurgical mentors, Dr. Pandy, Dr. Thompson, Dr. Coining from nephrology, and thank the committee for the opportunity to present. We have some time for questions, and I'm going to bring this back towards a socioeconomic perspective for a second and ask you, in Michigan are you having trouble getting these patients' screening MRs or CTAs paid for? It's a great question, and to be honest, I'll have to ask the nephrologist. In general, what I heard from them is no, at least for an initial screening. Dr. Wolfe, can you step up to the microphone, Stacy? Very good talk. How often are you suggesting screening for these patients since they seem to be having de novo aneurysms more frequently, and then that goes along also with the cost? Sure. Yeah, so great question. So the population that we encounter is, you know, those who have a known aneurysm, you know, if it's either treated or non-treated at one year, and then the next one's dependent on other risk factors, but at least every two years for the first five years. Dr. Blum. Thank you for that question. So just historically, you know, some of these conditions, familial conditions, have dysplastic vessels, which has made surgical treatments sometimes less favorable, plus you may have the issue of multiple craniotomies depending on the instance. So it was interesting that 80% were treated microsurgically. Was there some other consideration that went in for that? So we did see a trend toward, you know, keep in mind this was over 15 years, so we did see a trend towards increasing the vascular treatment towards the later period of the study. And then, you know, younger age, so desire for durability of treatment, as well as their borderline kidney function I think were factors that pushed us. But it is a higher microsurgical rate than our non-polycystic kidney cohort. So in the two patients who were screened but inadvertently missed three and five years before, how much growth, if any, occurred in the interval between this missed screening and the rupture? Great question. So one of the screenings we weren't able to pull, you know, it's a 15-year-old MRA, which we were not able to pull and look at, so we know the report was negative. In the one I showed you, the aneurysm looks about the same size, which is, you know, there are previous papers I think out of MGH showing, you know, not a lot of aneurysms have been observed and then shown to rupture, but, you know, they had a similar size at the time they were observed and then ruptured. Okay. Thank you very much. Thank you. Congratulations.

cerebral aneurysms

autosomal-dominant polycystic kidney disease

screening recommendations

retrospective study

treatment options