Challenging Parathyroid Case Discussions with Expert Panel Sponsored by Endocrine Society & AAES

all right so let's get started good evening everyone we'd like to welcome everyone to the challenging parathyroid case discussion hosted by the american association of endocrine surgeons in collaboration with the endocrine society i'm julie mcgill from emory healthcare in atlanta and i'm one of tonight's moderators hi i'm tom connolly i'm the uh the second moderate who will be working uh with our excellent panel that we've assembled to discuss some really challenging uh parathyroid cases we're lucky to have such a good group of folks to help us work through these cases uh this discussion tonight will be recorded and will be available for you to review late after the event on the aaes youtube channel as a request we would prefer that you keep your devices on mute during the session we do encourage you to ask us questions and we would direct you to the chat section to send those questions and at the end of each of our presentations we will review those questions for you we are going to start by getting into our panelists first up is dr dolores shobak please tell us a little about yourself and your background dr shobak thank you so much julie i'm an endocrinologist at ucsf in the san francisco va uh in san francisco and delighted to be here tonight um parathyroid disorders are a special interest of mine and it's really great to be together with endocrine surgery colleagues because we work together so so very closely on the management of these patients so thank you for having me wonderful next up is dr shoni silverberg i thank you julie and thank you to the rest of my ex my co-panelists um i'm delighted to be here as well i'm a professor of medicine at columbia university college of physicians and surgeons uh where i am the clinical director of the metabolic bone diseases unit and medical director of the parathyroid center along with an esteemed member of the aaes dr james lee who is my surgical counterpart next up is dr corey sturgeon thanks julian tom it is quite honored to be on this distinguished panel uh i'm cord sturgeon i'm the chief of endocrine surgery here at northwestern university where i've been for about 18 years i did my training at ucsf where dolores is now and about 50 of my practice is parathyroid and our last panelist is dr lynn yep thanks so much julian tom i'm so honored to be part of this panel uh tonight um so thank you so much i am chief of endocrine and breast surgery here at university of pittsburgh um where i've been for about 12 years since i finished my fellowship i'm so excited to talk about the cases awesome all right well thank you everyone and uh with that let's get started with these cases awesome our first case is a 71 year old woman who presents with progressive bone density loss fatigue body aches and bone aches she has a past medical history of hypertension anxiety disorder gerd and osteoporosis her home medications include amlodipine doxa pin meloxicam pantoprazoli alendronate persuvastatin and cholecalciferol her initial labs show a calcium of 9.0 with a parathyroid hormone of 152 with a normal creatinine and vitamin d dr silverberg would you start us off and tell us what you make of these labs uh you're on i'm sorry i'm sorry i am now unmuted um to my way of thinking the diagnosis for this patient is very uncertain um the pth is high the serum calcium is is really low normal not even mid normal or towards the upper end of normal um so the first diagnosis that comes to mind uh in in my mind is not primary hyperparathyroidism but instead secondary hyperparathyroidism both because of the low normal calcium level and the fact that the parathyroid hormone level is significantly higher than we usually see in garden variety primary hyperpara it is of course possible that she has normal calcium primary hyperparathyroidism but one of the labs that is missing in the presentation here is an ionized calcium level which would be absolutely mandatory in order to make that diagnosis and um she also came with these urine labs which showed a 24-hour urine calcium of 135 milligrams and she had a urine volume of 1484 with a random creatinine of 81.5 dr shobak would you comment on her urine labs absolutely sure so what we the reason we do this is where we're looking for sort of low levels of calcium in the urine that might suggest a reason for the secondary hyperpara that i think this patient has maybe she is malabsorbing and or and or she has a very low calcium intake and then the urine uh calcium will be low in those circumstances now hers i consider sort of anything between about 150 to 250 as being within normal range so hers is a little bit on the low side it's not it's not marked hyper hypo calcium low urine calcium and obviously it's not hypercalceuria so she's got a a lowish urinary calcium and i'd be asking her you know about symptoms of malabsorption like frequent stools i'd be asking her about her calcium intake i'd be wondering whether someone told her you have a problem with calcium balance and maybe she's restricting her calcium intake so i'd be asking those questions one other thing that i i would like to have here is the full 24 hour urine creatinine and i did do a back of the envelope on this and it was about 1200 milligrams which is probably indicating a good collection otherwise you might say hey did this lady complete a full 24-hour urine but when we did the calculation for creatinine it was fine and so i think this is this does reflect an accurate uh 24-hour urine so i'd be i'd be wondering about her intake i'd be wondering about malabsorption as ashoni said um her 25 is fine so she's it's not vitamin d deficiency i'd be i i looked at the medalist she's not on any drugs that lower urinary calcium like thiazides for example or lithium those are things that we think about when we see a lower urinary calcium so those are all things i i think about in a case like this okay um she also came with this bone density report and um doctor showback back to you if you don't mind sure sure interpret our bone density yeah i mean so she's over the age of 50 and there we use the t scores to tell us whether she meets bone density criteria for osteoporosis and and you've made it easy for me because really at every site she's below negative 2.5 so she has across the board at the spine at the both sides in the hip she's got osteoporosis so she has uh you know significant bone demineralization if i'm working up a parathyroid patient i'm also often adding a distal one-third radius to uh forearm measurements to the bone density because that can be helpful here it would probably also show low numbers but in some cases that might be the only site where it's where it's low okay and this patient when she was referred in already had all of these imaging studies completed when she came to see you dr sturgeon what do you make of this well so uh first of all just to reset we have a patient that our experts are both not certain have hyperparathyroidism and um you know with the calcium 9.0 and elevated pth and osteoporosis i i see how people might jump to that conclusion but i agree with them that this is this is something i'm not certain about and one thing i would point out is this actually happens quite a bit that patients get these imaging studies uh in the process of a workup and it's not quite even clear if they have hyperpara and a big point i would like to drive home to the audience is that imaging studies like this system eb scan 4d ct ultrasound are not for the purposes of making the diagnosis or ruling out the diagnosis or ruling in the diagnosis they are only for the purpose of planning a surgery so i would say dr mcgill i wouldn't order these tests unless i knew the patient had hyperpara and we were planning an operation and if the patient really really has hyperparent i'm not dissuaded by negative tests like this but i think um the best answer your question is that these negative findings do not change my thoughts about the diagnosis okay and then um dr silverberg you said that you were thinking leaning towards secondary causes can you go over some of the other secondary causes and then um talk a little more about the importance of the ionized calcium sure um so the most common secondary cause that that we see doesn't apply in this in this particular patient because we already know that her vitamin d levels are adequate but in the world the most common explanation uh for a high pth and a normal calcium level is at least in the states is vitamin vitamin d deficiency um and we um we've gotten that message out pretty well um so people do treat vitamin d deficiency uh when they see it um to see what happens whether the pth comes down or conversely whether the calcium goes up so one of the possibilities is that you have a patient with a garden variety hypercalcemic primary hyperparathyroidism who has vitamin d deficiency which has lowered the calcium level into the normal range and then when they're de-replete the calcium becomes elevated again what people don't often think of quite as readily as they do a vitamin d deficiency is what dr shobak mentioned earlier which is calcium insufficiency um that is to say somebody said oh my gosh you have a calcium issue you better not have a bite of ice cream or of pizza because your calcium is going to go up so um there are people who are putting themselves on very low calcium intake and the pth can be persistently high then we get into the realm of disease more more commonly recognized disease states ckd chronic kidney disease some people are surprised to know that even in individuals with gf bars in the 40s there is a component that not everybody has an elevated pth but some people do and certainly anywhere in stage three to five you can see um secondary hyperparathyroidism malabsorption is another big one whether it's celiac disease bowel resection or more commonly um as a more modern uh iatrogenic issue people who've had gastric bypass for obesity liver disease and then finally just to mention medications um that was alluded to earlier bisphosphonates or dinoximab the oral bisphosphonate that she is on would be unlikely to cause the pth to go up this much um but uh iv bisphosphonates can particularly in people who are not on enough calcium and denasimab almost always raises pth um uh although probably not to 152. oh i'm sorry i you also mentioned about the ionized calcium so that actually is not does not come under the rubric of secondary hyperparathyroidism but there are people who have normal total calcium levels and elevated ionized calcium there is no indication or no reason in the hypercalcemic hyperparathyroid patient to go to the expense and difficulty of measuring and ionized calcium on the other hand when the calcium is normal and we're wondering if this is normal calcium primary hyperpara that diagnosis can be made in individuals whose ionized calcium is level is elevated um uh although their total calcium is not wonderful thanks for clarifying that doctor yep with this case where would you go from here any final recommendations yeah so i think this was a really great case because i see a fair number of patients like this just like dr silverberg said most of the times their vitamin ds are nicely replete but they just haven't really um had enough calcium so in this patient i would definitely recommend that she take um calcium uh about a gram a day given her osteoporosis and her age and and everything else about her um and then recheck labs uh about two to three months and see how things look and go from there wonderful um we have a few questions from the chat room tom do you want to read through those sure so one of the questions that i noticed was out there is specific to ppis and calcium absorption and how that may play a challenge as we're making our diagnosis i don't know if uh one of our endocrinologists would like to weigh in on that um that question for us please i'll give a comment there i think this level of pth is pretty high for just a simple um proton pump inhibitor therapy 150 is pretty high and many times it'll affect both calcium absorption and magnesium absorption and there you often don't see the pth go very high so i think it's a bit high a pth for proton pump but it's a good thing to think about because it may certainly affect calcium absorption and that might be why the urine calcium's on the low side and it might lead you as dr yip recommended to give her um to liberalize her calcium intake it might lead you to give her calcium citrate instead of the carbonate because she won't have an acid stomach to absorb the calcium carbonate with very good um one of the questions i think we'll get to in a bit about genetic testing um i i think one question that has not been posed out there but i think we all ask as surgeons is is there a scenario that you would recommend a patient with true normal calcium primary hyperpair you've done your whole workup that you would actually recommend a surgery for and maybe we could get an endocrinologist and a surgeon to weigh in if we have just a last minute or two here before we have to switch over so um i'll hop in for the endocrinologist's point of view um i i am loathe to send people to surgery um who have normal normal normal calcium like um first of all i often can't convince myself that there isn't a component of secondary hyperparathyroidism and in almost all cases um the the only manifestation uh and organ manifestation that counts is low bone density and there are um great treatments for osteoporosis that work in patients who have hyperpara so i often treat um that treat them having said that um there are we know that there are people with primary hyper normals at calcium primary hyperpara who have adenomas and when they're removed the serum calcium goes down within the normal range um the data are sparse in terms of the natural history some studies suggest that there may be an improvement in bone density afterwards they can't by definition have marked hypercalceria because that would be an exclusion criteria because that's a cause of secondary hyperbaric and i will say that in the um international guidelines that are coming up there are at the decision ultimately among these 91 panelists from around the world was that there are no guidelines for surgery in normal calcium primary hyperpara well there's our answer as surgeons yeah thank you very much i think that puts the nail in that coffin julie i think it's time to move on to the next case perfect all right okay so case number two um so we have a 66 year old man who presented in the office uh after a recent parathyroid surgery and he continues to have the same symptoms he was dealing with before his surgery with severe weakness excuse me severe fatigue and weakness um as far as his past medical history there was none of significance and he was not on any medications at the time of presentation um so when we um with this kind of patient um dr surgeon we went um went back and did some research as we would want to do a surgeons but can you kind of walk us through your steps um lucky enough to have these kind of information to find the old labs you got some good lab good folks working in your office sure uh thanks tom so essentially a patient comes in who's had an operation for primer hyperpara but they have the same calcium that they did before surgery and the same symptoms and and unfortunately we do see this quite a bit and it requires it requires a fair amount of work to uh to do it right and i would say bottom line is you got to start from scratch the what you want to do is obtain as much information as you can you get the operative report from that operation you get the path report from that operation you try to identify notes and you look at all the work up that was done beforehand first and foremost i'd say you want to make sure that the patient really has hyperpara you know go back and look and see how secure that diagnosis was before that operation because you'd be surprised sometimes that's the issue right there and then um afterwards i actually find it really helpful to draw a picture of the operation you read the opera note you look at the path report you ask yourself what was and what wasn't found what was and what wasn't taken out and you know oftentimes they'll be like biopsies and stuff like that and you can you can essentially figure out what the patient um has left in their body and what's really been taken out based on the pathologist so that's kind of where i start and then the next thing i do is i kind of redo everything you know so i recheck their labs in cases like this where there's a failure you want to try to understand why or is it a failure because they had a very very clear diagnosis in clear imaging they just couldn't find it or did they find all the parathyroid glands and they were normal looking or whatever it was and i'll redo things like 24-hour urine calcium and of course if we're contemplating re-operating on patients i would love in a perfect world i'd love to have two concorded imaging studies before going back in for a redo that's it in a nutshell thank you so we did our research uh and found an old open path and if you'll give us our next slide please ma'am um actually let's go back we did see on the previous imaging that was non-localizing we skipped over that real quick but the last imaging was non-localizing we did get the uh on the next slide we have uh the op and path reports and so as we look through this uh we can see that there uh through the report that surgeon did identify four glands by the report they were told to be of relatively normal size but the left superior seem to be the largest gland that gland was removed and we saw it did see a drop in the pth during surgery from 66 to 25 at the five minute mark on our path report it did concur with what the surgeon had seen biopsies were performed of all four glands uh with the largest being the left superior removed and a weight as you can see so doctor yep based on the information we have here both on the how we dictate our operative notes how we uh interpret these path reports can you weigh in and give us some thoughts on on uh how to proceed yeah so i it's reassuring to see that they biopsied the other glands so i think certainly as dr sturgeon mentioned um even though the surgeon describes seeing them of course you've all been wrong before even you know sort of the most expert surgeons so it is nice and reassuring to see that they were biopsy then were confirmed parathyroid tissue um and looking at the report just really making sure that the description uh matched sort of what you expect the inferior superior glands to be just to again confirm mentally that these are these are truly um parathyroid glands and in the correct anatomic position so i think given that there were three normal glands one abnormal and large gland i would worry about sort of two possibilities so number one is again reassessing the diagnosis um just really making sure that this is truly primary hyperparathyroidism as opposed to something else like fhh which you would expect to see mildly enlarged glands but uh non-uh cure post-operatively and then um again i guess if you were convinced that it was primary hypopara that potentially um this patient had a supernumerary gland somewhere that was missed during the initial operation unfortunately in a reoperative setting i would not blindly go after supernumerary gland that would require definitely positive imaging thank you so we uh did some repeat labs um as everyone mentioned to to try to figure out the next steps and uh you can see those labs here um we see that uh still complaining of the same uh same symptoms um and on the next slide i believe we see that we did get a 24 urine for calcium and so dr shobak maybe you could help us walk through this uh these labs and maybe the 24 year for calcium again absolutely so when i look at this i'm looking at the volume that's collected that looks substantial and then i'm also looking at the urine creatinine and that looks like it's a uh it's an adequate collection for men i usually look for 15 to 25 milligrams per kilogram as defining what's an adequate collection for men we don't have his weight but that's what i'd be uh that's what i'd be looking for to make sure that it's adequate because the urinary calcium here is low now what numbers do i use there well kind of as a rule of thumb before i start doing any arithmetic if it's less than 100 i'm starting to worry either the calcium intake is very restricted or the patient has hypocalceuria and this is a low urinary calcium anything under 100 then what's been shown from multiple series and different countries even is that that calcium to creatinine clearance ratio it's the it's that clearance ratio that you need to calculate and you get a calcium on the blood and on the urine and a creatinine on the blood in the urine at the same time when you do your 24 hour or even your spot urine if that clearance ratio is less than 0.01 i'm it's hypocalceria for sure but i'm also worried about fhh there there's plenty of overlap however and that's where you can get into trouble anything between .01 to .02 is definitely fair game for primary hyperpara and anything over uh .02 is likely to be primary hyperpara so you it's a there's a big gray zone uh this patient lands below 0.01 so i'm thinking fhh but i'm still wanting to be wanting to be sure and there i'd be thinking in today's world i need to do a genetic test to figure out if this patient is truly fhh because this patient has failed the first parathyroid ectomy by an able surgeon and so i'd be looking for a genetic panel and a genetics referral where i could get the three forms of fhh looked for in the genetic analysis and that would be the calcium receptor the g alpha 11 and the adapter protein so there's three forms of fhh we need to deal with now and and so we'd have to consider that in this patient thank you so our next slide um [Music] did show that we did have uh genetic testing performed and did document a mutation casr gene dr silver maybe you could weigh in on the the role of genetic testing when to order it and how to interpret some of those results uh give us some direction there so the question always comes up we have a patient who um has labs that biochemically look like primary hyperparathyroidism and has a very low um urinary uh calcium to creatinine uh clearance ratio do we have to go any further can we just make this um diagnosis based on on the clinical presentation and the answer um in in today's world is yes um as dr shobak said um there are up to 20 of patients with primary hyperparathyroidism who can have a calcium to creatinine clearance ratio less than 0.1 um and uh and many of our patients are as dr schobeck suggested in that in-between area and by the way conversely there are patients who have fhh who um who have calcium to creatinine clearance ratios above 0.02 uh about 10 of of fhh patients so you can't make it just um based on that on the urine um it's uh and that's particularly because uh in patients who may have low vitamin d or ckd um the urinary calcium can be low the testing is now pretty much universally available um but it is important to note that it is not universal it should not be universally done so there should be an index of suspension before sending somebody for genetic testing and one of the questions that um uh always comes up is this is this something that's covered by insurance um i know that some of this is state by state but in patients in whom there is a high degree of clinical suspicion we have never had any trouble getting getting it covered i suspect that if you had a patient with kidney stones and you're in calcium of 380 i would hope that the insurance company or somebody would would flag it and not not necessarily do testing certainly at least for fhh they might do it for other hyperparathyroid syndromes i think that one of the things that's sort of interesting here dr shobak mentioned about the three forms of fhh i thought one form was enough to give me a headache but it turns out that there are three different ones with three different genetic profiles fh2 almost is very very very rare but fh3 is seen in about 20 or 25 percent of patients and they actually do have more symptomatic disease certainly this patient's clinical complaints are not consistent with what most of us think about as the clinical syndrome of fhh which is a very benign clinical presentation with generally no complaints um and this person has lots of body aches um i will just mention that um that the fact that that this person has an abnormality in the calcium sensing receptor and we are thinking that maybe it's an fhhh3 is a little bit different from what we might expect but i think that it does make the point that there is a fair amount of overlap and also that even in patients um with garden variety old-fashioned fhh1 that in a certain percentage the calcium sensing receptor testing is not abnormal it's not necessarily because the calcium sensing receptor is perfect but they only test for known abnormalities so there are many abnormalities that they may not test for awesome we have a few um questions one of the questions fits in with what we're talking about and said is there any utility in doing the calcium creatinine clearance ratio in a patient who has a normal 24-hour calcium urine i would say i would say yes it really does depend on the circumstances you're dealing with if for example the urinary calcium is quote within the normal range but the patients failed a parathyroid exploration by a good surgeon and just this kind of circumstance where you feel comfortable that there's parathyroid pathology hyperplasia it's for gland i i think you have to realize that there's a fairly wide gray zone um for a minority of patients and and we you know you just can't get around that so i think it is worthwhile uh even if it's normal and i've even had i hate to i hate to admit it i've even had patients in kindreds where there's definitely fhh1 and the pro band had kidney stones and her father had kidney stones so all bets are sometimes off unfortunately and what about someone also asked what if you came back with the same labs but your genetic testing was negative i can i can i can comment on that i think i agree with what uh dr silverberg said which is that sometimes the calcium sensing receptor levels of gene expression may not be um immediate the pro the problem may not necessarily be mediated by a mutation in the coding sequence of the receptor that gets picked up it may be something like that if the physiology and the biochemistry fits with fhh um it it could be it could still be that or it could be primary hyperpara where the patient just has a low urinary calcium we definitely see that so you have to make a clinical judgment in those cases and those are tough ones i agree with the questioner all right awesome let's move on to case number three um this is a 51 year old woman who presented with fatigue weakness body aches memory issues and polyuria her initial lab showed a calcium of 10.9 with a parathyroid hormone level of 35. her past medical history is significant for hypertension obesity with a bmi of 46 and type 2 diabetes her home medications include a combination blood pressure medicine amlodipine hydrochlorothiazide homostartin as well as carvedilol clonidine metformin and denudia here are her follow-up labs and dr silverberg if you can help us work through these that would be great showing the calcium of 11.2 with the repeat parathyroid level of 48. um so just to make a couple of clinical points um the main point that i want to make uh and if people take home no other message from things that i've said tonight let it be this one um there is no such thing as normal hormonal hyperparathyroidism that's not a disease so anybody who has uh hypercalcemia and in this particular patient it's not even close right so this patient has a serum calcium of 11.2 and an ionized calcium that is very healthfully elevated um any normal uh parathyroid hormone level is not normal because anyone who has hypercalcemia that is not pth mediated should have a suppressed pth level so that's that is the the first take home message this is routine garden variety hypercalcemic hyperparathyroidism um that that's number one then the the the other sort of clinical points to make um given what we've seen so far i think someone is not on mute i could i can mute myself but it's that would be a problem okay thank you so much so um the other um sort of lesser points to make uh first of all is uh the fact that this woman is 51 years old um so we we see a a a a large proportion of patients who are diagnosed within five to ten years of menopause okay listen to me thank you um we see a large proportion of patients being diagnosed with primary hyperparathyroidism within five to ten years um of menopause and the way i always think of it which i is actually quite simplistic um is that the estrogen it was was keeping the calcium in the skeleton and when estrogen is is gone in the post-menopausal state the calcium um uh is able to come out more readily of the skeleton and the patients become hypercalcemic so that's that's the first thing to point to make about her age the second point to make about age and primary hyperparathyroidism is that the extent of elevation of ph that we expect varies with age so the upper limit of the normal a much better time than we are i think anyway um so uh gino segre from mass general um used to uh say that the upper limit of normal for pth at the age of 45 should be 45 um and so if someone has a pth that is significantly higher and it is quite young the 51 year old is is not quite quite there um that that may be inappropriate for their age um and uh i think that that was oh the uh the urine um this is a pretty marked hypercalceuria um it looks like uh it's a pretty complete collection um both from a volume point of view and a creatinine point of view and so i would look at this person and think just on the basis of lab values alone that this patient makes meets the criteria for a referral to an endocrine surgeon for treatment of her disease perfect thank you um this was her bone density study that came with her dr shobek would you pick through this with us sure so you can see the uh that she's had the spine and the sights in the hip measured and there she's barely osteopenic only at the spine is she osteopenic that's uh a t score between uh negative 1 and negative 2.4 and at the other side she's actually maintained her bone density but at the forearm i think you can appreciate that she's two standard deviations below a young normal uh and that's what the t-score means and so this the forearm is a site of almost pure cortical bone and parathyroid hormone does demineralize cortical bone even more readily than it does trabecular bone like the spine but i think this is a clear-cut situation where that particular site is very helpful and you know that she's had pth excess even though the levels aren't terribly high for her parathyroid hormone she's had pth excess chronically and it's affected her cortical bone wonderful um at this point we started to get localization studies we started with an ultrasound which did not show an adenoma and at the institution of this patient she they had a um 4d parathyroid ct protocol and it did show something above the isthmus and no other potential sites were seen no ectopic sites were seen um dr yep what do you think about and here this is the picture of what it shows dr yep what do you think about the imaging studies and then also what would your operative plan be if you're going to take her to the or yeah so first of all i just want to reiterate what dr sturgeon had mentioned earlier that imaging results don't necessarily make or break the diagnosis so we've already decided that this patient has um primary hypothyroidism associated with osteopenia um and she has reasons to undergo surgery based on her 24-hour urine calcium and also her serum calcium too so i think you know this patient is going to surgery and regardless of whether or not that imaging study is positive or negative um i would schedule her and get her set up for surgery um the this location for a parathyroid lesion is a little bit unusual um something above the isthmus is is highly odd uh i think i would be a little bit skeptical about that result and basically just treat her as having negative imaging from the beginning i think the one thing we always do our parathyroid surgeries with intraoperative pth so i think in this situation she'll probably um start at a pretty low level which always begs the question of where are you going to end to ensure that you've cured this patient um and and i think uh for this patient with a fairly low pth to start off with likely in this negative imaging likely she's going to need a four gland exploration so i would plan and set her up for that uh and prepare for that from the very beginning and so dr sturgeon going along with what dr yip was saying would you do you still think there's value in using the interoperative pth and do you have a target that you would be aiming for or just kind of using it as a guide what do you think about this patient yeah so i think that we see this all the time where patients have preoperative pth values that you've reviewed and you know figuring out whether or not they have hyperpara and rarely do those values exactly line up with what we see in the or you know so if i if i had this patient i wouldn't just automatically assume that iopth would be worthless but i'm with lynn on this where i would assume this is a non-localized patient and i would not go forward thinking i'm going to do something focused or unilateral and use pth i would do a four gland expiration on this patient i do have the ability of fortunate to be able to measure pth and to measure it fairly quickly and so we do measure it for all these cases and um i think i've been surprised more often than not that the intraoperative findings are a little bit more classic in patients and and you can kind of tell that you've had a significant drop in pth uh either from the the pre-incision the pre-excision value being considerably higher than what you've seen in the past the other thing i would mention is that i agree also dr yip this is a real funny place for a parathyroid and um one of the values of uh well one of the things that i would do is i would probably do my own ultrasound just to see what that is you know i know that an ultrasound was done and they said quote-unquote non-localizing i think it'd be very helpful for the surgeon to know what that looks like before they start the operation maybe dealing with some other pathology for example that you need to know about maybe a thyroid cancer or something else and hopefully that answers your question tom you want to look at the questions you're on mute thank you so um i think one of the questions that seems out there is what do we determine what's a low pth an inappropriate pth that's actually considered low so you have a high calcium where's the cutoff is it 20 is it 10 is it 5 where would we um call that mr silverberg so i mean the absolute cutoff is the lower limit of the assay normal which depends on the assay and on your laboratory so a lot of people it's 15 or below i think that a value of 20 or 18 um would be considered would be considered low except as i said in a young person where um we've seen primary hyperparathyroidism with with real elevations in calcium and pths in the occasionally in the high teens and certainly in the low 20s very good and then the other question i'll ask um uh dr schoberg um there's a question about how do you determine an adequate creatinine collection if you could define that for this sure um i use the i use the normal ranges of 15 milligram 15 to 25 milligrams per kilogram body weight if somebody is extremely obese and there's a lot more fat than muscle mass then you might need to modify it a little bit or conversely if they're very muscle bound then you probably are looking for 25 milligrams per kilogram but that's the range i use figuring that women typically have less muscle mass so i accept 15 milligrams per kilogram as kind of an average amount of creatinine that they should be excreting and then i that helps me determine that the collection almost regardless of the volume is adequate and then one last question on this case people ask if the assessed media is negative what percentage of the time does the ct end up helping you and giving you a localization i do this for one of us me and lynn yeah i mean i don't um you know again coming back to the original point which is that imaging is helpful but it's certainly not the make or break so i don't usually get multiple different types of these preparation for these surgeries i'll get an ultrasound to assess the thyroid and make sure there's no thyroid pathology and then to also look for the paras and then and get access to me be and that's pretty much the the extent of the type of imaging that i'll get pre-op but if those are both negative and the diagnosis is secure i'm not going to get another study for localization purposes so i don't i don't know the answer to your question unfortunately so uh yeah i think it's entirely dependent on who does the first study there are some institutions with a lower um you know accuracy of system maybe they're never called positive even when we might look at them and think they're positive actually so in our we have a large referral from us from community hospitals and if there's a negative system maybe and i repeat it um it'll turn positive about 60 of the time that's a rough and unpublished number but frankly i kind of do what land does i ultrasound them myself and if i find the the obvious you know sonographically obvious parathyroid item i don't put them through multiple repeat imaging studies uh so i think that um you know it is entirely dependent on the facility and how frequently they do cesta media and all that sort of stuff i i kind of reserve the 40 ct for those people who i feel like really need to be localized and have uh negative conventional imaging such as system eb and ultrasound great thank you all right tom case four sounds great so if you'll get us the next slide please sorry that's okay okay so uh this is a 34 year old uh uh lady who presented with uh 14 weeks pregnancy and an elevated serum calcium level uh as you can see she didn't have any significant past medical history and um no family history that was significant at her young age presentation and uh she's only on a prenatal island um so based on um based on that uh the the next slide please so here's some additional labs um we can see that her calcium was 10.6 her pth was 116. kidney function is normal normal thyroid function and we see that she has a low vitamin d level at 16. so dr silverberg maybe you could give us some thoughts on how to proceed at this point so the firs the first thing to um remember uh is that normally uh in pregnancy serum calcium total calcium goes down so um patients uh serum albumins uh go down in pregnancy and actually it's a pretty early finding uh and so that normally uh the serum calcium follows suit um so when the calcium is frankly elevated it's really frankly elevated so there's really no question about what's going on so this lady has clear primary hyper parathyroidism in pregnancy not of pregnancy it's not a disease caused by her pregnancy she just happens to have it at the same time um uh one thing that has changed in terms of uh our management um is that that we now follow most patients who are pregnant and have primary hyperparathyroidism successfully and uneventfully through their pregnancy and that um may well be uh associated with the fact that the disease as it presents at least in in the us um is milder than it used to be and so uh in the old days when the diagnosis of primary hyperparathyroidism was made in pregnancy the serum calcium was often very very elevated and and the disease was more severe now if you have patients who have calciums in the mid tens they generally do fine and the fetus also generally does fine so so surgery is not the default option um for patients who have primary hypoparathyroidism in pregnancy on the other hand there are um cases where surgery is the appropriate uh response and that would be anybody who had a threshold calcium that was more than one milligram per deciliter above the upper limit of normal so in the lab of this patient it would be 11.2 or greater of concern in this particular patient in evaluating her labs and in terms of her management is the fact that she is very vitamin d deficient not just sorta she's very vitamin d deficient and as i mentioned before this relatively profound vitamin d deficiency may be lower ring for serum calcium to 10.6 rather than it being at 10.6 so we would want to know what happens when her vitamin d is a little higher before a decision is made to operate or not to operate thank you next slide please so we did uh go ahead and repeat the repeat the vitamin d and repeated some labs next and so here are the results we have now so dr shobak could you help us uh with maybe thoughts on vitamin d replacement um and and uh how we can proceed at this point sure so maybe even comment on how this calcium affects uh the baby at this point okay sure so um she had her vitamin d repeated what i would probably do with the level of 16 would be to give her 50 000 units of d3 once a week perhaps for four weeks and start her on a very low dose replacement i'd do this very gently because she's already hypercalcemic i'd probably check her calcium labs two weeks after i started that just to make sure i didn't really um raise her calcium uh dramatically so i'd very gently replete her and it looks like she was repleted and her calcium went up substantially so um and her pth really didn't change all that much so so the diagnosis is still clear-cut but she's more hypercalcemic than she was before and i assume we have a vitamin d level of at least something in the range of 30 nanograms per mil so she's right at the beginning of her second trimester and this would be a time i'd be notifying my endocrine surgery colleagues about this patient and and probably considering given that level of um calcium uh uh considering localizing her and and taking and recommending uh surgery what what this might do to the baby it's it's not severe or dramatic hypercalcemia but what it might do is suppress her own her baby's parathyroid so that when the if we left this alone and so that when the baby uh was delivered the um neonatologist taking care of that baby would need to watch that baby carefully for hypocalcemia so sort of delayed parathyroid function in the baby that's what i'd be concerned about thank you dr shobak next slide please so um now we uh have a patient who's been referred to surgery um and we have to talk about imaging options so dr sturgeon maybe you could give us some direction there please sir yeah sure i mean i i want to just echo something dr shobek just said this communication between the services is so critical uh uh that we're aware of the patient we want to operate in the early first or the early second trimester and they want to achieve a relatively normal vitamin d and everything so i really appreciated that comment i think that really is essential we're all on the same page we're moving to the same goal at the same pace um you know obviously with hyperpara we're always talking about imaging trying to figure out where things are but in this pregnant patient i would probably just do ultrasound and that's all i would do i don't want to inject her with a radiopharmaceutical nor do i want to give her a contrast load nor a um you know a ct scan i know we can shield the abdomen and all it says we certainly do ct scans for appendicitis all the time but you know frankly i think ultrasound is going to give me the information that i need uh it's non-invasive it's painless and i would i would probably no matter what it shows do it again myself uh so that i've got the surgical plan uh pretty clear very good so here's our ultrasound report uh and if we can see the next slide and here is seen on the ultrasound so um dr yep how would you proceed at this point you've got your ultrasound you've got your diagnosis and your endocrinologist telling it's time to to do a surgery so i think the only other thing that i would add in her evaluation is that since she's a young patient i would really want to make sure that her family history didn't include anything that would concern me about men1 um or any kind of inherited endocrinopathy because that would certainly change the extent of surgery and certainly the management of her disease intraoperatively so that would be the only other thing that i would add to her pre-operative evaluation but it looks on this ultrasound that she has a hypochoic nodule um on the left side um it's labeled as left superior um it looks like it's almost lateral to the to the um lobe and that initial image so um i'm not 100 sure but um i i think certainly i would start on the left side and plan to use intraoperative pth to help guide the extent of surgery is that her third yeah it looks like yeah thank you um next slide so um i guess back to the timing of surgery um doctor maybe you could comment uh as far as the second trimester and um the is there a perfect time and then maybe some post-operative thoughts if it's multi-layering disease and and and maybe how that affects her her management post-op yeah so the second trimester of course as we sort of discussed it is the ideal time to operate i usually do have these pregnant patients see their high-risk ob just to make sure that everyone's on the same page there is a risk of pregnancy loss and so i think it's very it needs to be very clear to everybody involved um during this time we would usually check fetal heart tones pre and postoperatively uh but again there's not uh unfortunately i usually counsel the patient if there's not much we can do if there is an issue with with the baby during the early second trimester but that is the safest time um so we coordinate it with uh high risk ob we make sure the patient is aware of implications postoperatively i think that's one of the benefits of using intraoperative pth is that especially in the case of four gland hyperplasia if we end up having to do a three and a half gland resection that final pth can sometimes be prognostic in terms of how likely is it that this patient's gonna be hypocalcemic post-op but i would definitely aggressively manage her calcium put her on replacement calcium post-operatively make sure her vitamin d levels were adequately replete as we already did to really reduce the risk of hypocalcemia after surgery so thank you wonderful we are approaching the top of the hour and we'd like to thank everybody for joining us today a special thank you to our panelists for their time and energy and expertise and we'd like to thank the endocrine society for working with the american association of endocrine surgeons to collaborate on this project

Channel: AAES

Views: 11,834

Keywords:

Id: JmuhATeWgCE

Length: 56min 29sec (3389 seconds)

Published: Fri Feb 25 2022