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Request for ophthalmology and endocrine/rheum opinions re: Graves' orbitopathy

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  • Request for ophthalmology and endocrine/rheum opinions re: Graves' orbitopathy

    Clinical question: Does she need strabismus surgery for Graves' orbitopathy straightaway, or should steroids be given first?

    Someone close to me has h/o Graves' disease treated with radioiodine ablation in early 2017 with no subsequent problems on thyroid replacement therapy for 4 years.

    Also had sudden unilateral hearing loss around 2016. Rheum consult then thought she might have Cogan's syndrome.

    Then, perhaps unrelated, became very sick after 2nd Covid vaccine, (April 2021) and soon developed hearing changes in opposite ear. Treated with 6 day course of tapering steroids by ENT with resolution of those symptoms.

    However, she also developed diplopia around same time. Saw local ophthalmologist who diagnosed Graves' orbitopathy and referred to senior ophthalmologist at university for strabismus surgery. He is actually a pediatric specialist but apparently has special interest in strabismus surgery and operates on adults once/month. Senior eye dr. recommended strabismus surgery straightaway.

    I only know what I read in UpToDate, but UpToDate appears to recommend course of steroids as first step, and provides evidence that 12 week course of once weekly IV steroids more efficacious with fewer adverse effects than po steroids. It also suggests that if surgery still needed, outcomes are better if steroids given first. Further, the natural hx is variable and the problem apparently improves spontaneously in some.

    I mentioned all this to surgeon but he said he would not give steroids and would go straight to surgery.

    She will see endocrine 6/22 and rheum 6/23, all 3 are university faculty. Rheum consultant is senior, but endocrine consult is only 3 years out of fellowship. This should ordinarily be endocrine's call, but not sure if junior guy will have much experience with Graves' orbitopathy, or have conviction to contradict recommendation of senior ophthalmologist.

    Maybe surgery is best next step. I don't know, but hoping specialists here might want to weigh in. The senior ophthalmologist only operates on adults once/month, so tentative surg date set for late August, the first available opening.

    Thanks for any insights.
    Erstwhile Dance Theatre of Dayton performer cum bellhop. Carried (many) bags for a lovely and gracious 59 yo Cyd Charisse. (RIP) Hosted epic company parties after Friday night rehearsals.

  • #2
    Has anyone discussed Tepezza?

    Healthcare professional information is provided on TEPEZZA (teprotumumab-trbw), indicated for the treatment of Thyroid Eye Disease. See safety information.

    Comment


    • #3
      I’m just a lowly retina surgeon so taking care of thyroid eye disease (TED) is not my normal daily routine. But, I do recall that strabismus surgery for TED pts is NOT straightforward. From reading your posts, it sounds like the thyroid disease has been stable for 4 years. That’s good because that’s where I’d start first. Next, if she has TED, has she had imaging of her orbits performed to look at the muscle involvement (and make sure the optic nerve is ok)?
      Maybe strabismus surgery is indicated, but I’d seek a second opinion because these can be very difficult to treat, and usually require more than one surgery to get it right (if that can be done).

      Comment


      • #4
        There are also ophthalmologists who are trained in combined neuro-ophthalmologist/oculoplastics and these are the ones who, in my experience, deal with thyroid eye disease (TED) the most. They would not be the ones typically doing the strabismus surgery (pediatric ophtho usually does strabismus, even for adults), but because neuro/oculoplastics sees the most TED, they might have a better idea of whether the clinical picture makes sense for TED or not.

        With the complicated history it sounds like you want to make sure this is really TED, and that makes sense, because lots of things can cause diplopia. I’d personally want to make sure that other autoimmune conditions (like GPA) have been ruled out and MRI of the brain and orbits done and are consistent with TED.

        Also Tepezza is a very new drug and not many ophthalmologists have much experience with it (I don’t). But maybe it’s something to consider.
        Last edited by Dusn; 06-16-2021, 05:07 AM.

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        • #5
          Originally posted by eyecandy View Post
          Has anyone discussed Tepezza?
          The ophthalmologist recommended surgery with no mention of alternative or adjunctive therapies, but after I asked about the UpToDate recommendations, he then did say that he would recommend that the endocrinologist provide Tepezza. This wasn't part of his initial plan.

          Erstwhile Dance Theatre of Dayton performer cum bellhop. Carried (many) bags for a lovely and gracious 59 yo Cyd Charisse. (RIP) Hosted epic company parties after Friday night rehearsals.

          Comment


          • #6
            Originally posted by Eye3md View Post
            I’m just a lowly retina surgeon so taking care of thyroid eye disease (TED) is not my normal daily routine. But, I do recall that strabismus surgery for TED pts is NOT straightforward. From reading your posts, it sounds like the thyroid disease has been stable for 4 years. That’s good because that’s where I’d start first. Next, if she has TED, has she had imaging of her orbits performed to look at the muscle involvement (and make sure the optic nerve is ok)?
            Maybe strabismus surgery is indicated, but I’d seek a second opinion because these can be very difficult to treat, and usually require more than one surgery to get it right (if that can be done).
            It was stable. After the second PFE Covid vaccination (causal or coincidental, I don't know), she became very ill and then developed left ear symptoms similar to those prior to her sudden hearing loss on the right (better following steroids this time), simultaneously with diplopia.

            UpToDate suggests that surgical results are better when the disease has been stable for significant period of time, and reports evidence that surgical results are better following course of steroids.

            She had imaging of the optic nerve at the eye appt, and MRI has been scheduled.

            I asked surgeon about risks and he said 1/10,000 loss of vision, and 20% need for repeat surgery.
            Erstwhile Dance Theatre of Dayton performer cum bellhop. Carried (many) bags for a lovely and gracious 59 yo Cyd Charisse. (RIP) Hosted epic company parties after Friday night rehearsals.

            Comment


            • #7
              Originally posted by Dusn View Post
              There are also ophthalmologists who are trained in combined neuro-ophthalmologist/oculoplastics and these are the ones who, in my experience, deal with thyroid eye disease (TED) the most. They would not be the ones typically doing the strabismus surgery (pediatric ophtho usually does strabismus, even for adults), but because neuro/oculoplastics sees the most TED, they might have a better idea of whether the clinical picture makes sense for TED or not.

              With the complicated history it sounds like you want to make sure this is really TED, and that makes sense, because lots of things can cause diplopia. I’d personally want to make sure that other autoimmune conditions (like GPA) have been ruled out and MRI of the brain and orbits done and are consistent with TED.

              Also Tepezza is a very new drug and not many ophthalmologists have much experience with it (I don’t). But maybe it’s something to consider.
              Our consultant was certain that this was TED (haven't seen that acronym before :-)). She does have endocrine and rheum appts next week. Will see if rheum thinks this might be something else. The ophthalmologist said this definitely wouldn't be Cogan's (even if she had Cogan's) because that would cause anterior uveitis, which she did not have.

              MRI is scheduled, not yet performed.
              Erstwhile Dance Theatre of Dayton performer cum bellhop. Carried (many) bags for a lovely and gracious 59 yo Cyd Charisse. (RIP) Hosted epic company parties after Friday night rehearsals.

              Comment


              • #8
                Thank you all for taking the time to write. I greatly appreciate the insights.
                Erstwhile Dance Theatre of Dayton performer cum bellhop. Carried (many) bags for a lovely and gracious 59 yo Cyd Charisse. (RIP) Hosted epic company parties after Friday night rehearsals.

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                • #9
                  Interesting timeline. Likely TED, but can’t be sure until a repeat MRI. My question is how do we know the ophthalmic disease is quiet? This was a sudden change after vaccine. Definitely don’t want strabismus surgery on muscles that aren’t quiet. That’s a guarantee the surgery will fail.

                  What gives me pause is why this doc was so quick to recommend surgery? Without an recent updated MRI - that makes me uncomfortable. No EOM surgery until TED is quiet. If TED is active then try Tepezza first- for sure.

                  Also, like Dusn I’m not an Oculoplastics or Strab specialist but I do manage some mild TED pts. If the optic nerve isn’t threatened then there’s no reason to rush into anything. Also, outside of MRI how do we know the strab is stable without multiple prism measurements?

                  Comment


                  • #10
                    Originally posted by eyecandy View Post
                    Interesting timeline. Likely TED, but can’t be sure until a repeat MRI. My question is how do we know the ophthalmic disease is quiet? This was a sudden change after vaccine. Definitely don’t want strabismus surgery on muscles that aren’t quiet. That’s a guarantee the surgery will fail.

                    What gives me pause is why this doc was so quick to recommend surgery? Without an recent updated MRI - that makes me uncomfortable. No EOM surgery until TED is quiet. If TED is active then try Tepezza first- for sure.

                    Also, like Dusn I’m not an Oculoplastics or Strab specialist but I do manage some mild TED pts. If the optic nerve isn’t threatened then there’s no reason to rush into anything. Also, outside of MRI how do we know the strab is stable without multiple prism measurements?
                    I asked the consultant if the surgery was time-sensitive and he said, "No." His first available opening was late August, so we should know more by then.

                    Is Tepezza to the go-to drug now among ophthalmologists? UpToDate recommends steroids first, with Tepezza a salvage drug for non-responders. They note trials showing its effectiveness but remark that it hasn't been compared head-to-head with steroids:

                    "Moderate-to-severe or progressive symptoms

                    Glucocorticoids – For patients with moderate-to-severe orbitopathy, we suggest initial treatment with glucocorticoids [20]. These can be given either orally or IV, depending on the severity of the disease. For the patient with moderate symptoms (inflamed eyes and increasing diplopia or proptosis [≥3 mm above upper limit of normal for race], mild corneal irritation), a trial of oral (prednisone, 30 mg/day for four weeks) or IV (methylprednisolone, 500 mg once weekly for weeks 1 to 6, then 250 mg once weekly for weeks 7 to 12 with cumulative dose 4.5 to 5 g over 12 weeks) glucocorticoid therapy should be initiated. If the initial oral dose is ineffective, higher doses may be required and a switch to the IV route should be made. For more severe or progressive cases, initial IV therapy is appropriate [21,22].


                    The European Thyroid Association recommends initial treatment with IV glucocorticoids (methylprednisolone, dose as above) for moderate-to-severe, active orbitopathy, citing several studies that suggest it is more efficacious and associated with fewer side effects than oral therapy [5] (see 'Glucocorticoids' below). Similar trials have not been performed in the United States, where initiation with oral glucocorticoids remains the most common first-line treatment.


                    Contraindications, intolerance, or lack of response to glucocorticoids – If high-dose glucocorticoid therapy is contraindicated, cannot be tolerated (eg, steroid psychosis, poorly controlled diabetes), or is ineffective, options include other medical therapies, external orbital radiation, or orbital decompression surgery. (See 'Medical therapies' below and 'External orbital radiation' below and 'Orbital decompression surgery' below.)


                    The choice of therapy should be individualized based upon shared decision-making, regional expertise, availability of therapies, and cost. There are few direct comparison trials to guide the selection of secondary therapies. If there is no initial response to the first few doses of glucocorticoidsand a decision is made to proceed with alternative medical therapy, we suggest teprotumumab (an insulin-like growth factor 1 [IGF-1] receptor antibody). Randomized trial data show striking efficacy compared with placebo [23-25] (see 'Teprotumumab' below). Trial data also show efficacy for mycophenolate mofetil compared with IV glucocorticoids [26], and tocilizumab (an interleukin-6 antibody) compared with placebo [27]. (See 'Medical therapies' below.)

                    ***

                    Teprotumumab — Teprotumumab (an insulin-like growth factor 1 [IGF-1] receptor inhibitor) was approved for the treatment of Graves' orbitopathy by the US Food and Drug Administration (FDA) in 2020, based on the findings from two 24-week trials comparing teprotumumab with placebo in 171 patients with active, moderate-to-severe orbitopathy [23-25]. In each trial, a greater proportion of patients in the teprotumumab group had a reduction in clinical activity score and degree of proptosis (69 versus 20 percent with placebo and 78 versus 7 percent with placebo, respectively). The durability of efficacy requires confirmation with long-term follow-up studies. Eye symptoms in the patients in the trial had to have begun within nine months of trial entry, and it is unclear whether the drug would be as effective in patients whose disease was of longer duration. In addition, there was no comparison with the effectiveness of glucocorticoids, the standard therapy for patients with moderate-to-severe orbitopathy. Cost may also play a role in clinical decision-making, and the price of this drug has yet to be decided."
                    Erstwhile Dance Theatre of Dayton performer cum bellhop. Carried (many) bags for a lovely and gracious 59 yo Cyd Charisse. (RIP) Hosted epic company parties after Friday night rehearsals.

                    Comment


                    • #11
                      Tepezza is a great newer option, but it can often be very difficult to get insurance converage because of high cost. There is no rush to do strabismus surgery. As others have said above, the need for stability is critical and doing prisms (even easier with fresnel prism) is a simple way to help confirm diagnosis and confirm stability. Usually steroids in an acute flare up is a primary part of treatment. The other critical surgery would be an orbital decompression if there is nerve compression (often easier to do with skilled rhinologist vs oculoplastic specialist).

                      Comment


                      • #12
                        All this talk about Tepezza which is a very new and expensive medication... It is very unclear to me if there is active thyroid eye disease (maybe I missed it somwhere), for which Tepezza is actually indicated according to my oculoplastics colleagues.

                        I doubt the pediatric ophthalmologist would be recommending strabismus surgery in the context of active inflammatory disease in which case deciding between tepezza, oral steroids, IV steroids vs surgery is a moot point. Endocrinology and Rheumatology are not going to manage these eye findings as they have very little idea of the pathophysiology, exam, prognosis, and treatments.

                        It is also extremely common for pediatric ophthalmologists to handle adult strabismus as they are the de facto strabismus experts.

                        The only other thing I would consider is an Oculoplastics consult as they typically assess for and handle active TED. From what I gather there has only been a comprehensive ophthalmologist and a strabismus surgeon in the picture.
                        Last edited by TheDangerZone; 06-16-2021, 06:11 PM.

                        Comment


                        • #13
                          Originally posted by I-doc View Post
                          Tepezza is a great newer option, but it can often be very difficult to get insurance converage because of high cost. There is no rush to do strabismus surgery. As others have said above, the need for stability is critical and doing prisms (even easier with fresnel prism) is a simple way to help confirm diagnosis and confirm stability. Usually steroids in an acute flare up is a primary part of treatment. The other critical surgery would be an orbital decompression if there is nerve compression (often easier to do with skilled rhinologist vs oculoplastic specialist).
                          The staff did do a test with prisms. During the interview, the consultant did say that prisms could be used to correct vision (at least that was my understanding), but in this patient's case it wouldn't be practical because the prisms would be thicker than Coke bottles.

                          Of course, the ophthalmologist has only seen her once, so stability is not confirmed. At one point he declared (to patient) emphatically, "You've had this for a long time." Something about the exam or history convinced him that the symptoms were longstanding although she only noticed/reported them after second vaccine in late April. He was sure the problem was more chronic than that.
                          Erstwhile Dance Theatre of Dayton performer cum bellhop. Carried (many) bags for a lovely and gracious 59 yo Cyd Charisse. (RIP) Hosted epic company parties after Friday night rehearsals.

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                          • #14
                            Originally posted by TheDangerZone View Post
                            All this talk about Tepezza which is a very new and expensive medication... It is very unclear to me if there is active thyroid eye disease (maybe I missed it somwhere), for which Tepezza is actually indicated according to my oculoplastics colleagues.

                            I doubt the pediatric ophthalmologist would be recommending strabismus surgery in the context of active inflammatory disease in which case deciding between tepezza, oral steroids, IV steroids vs surgery is a moot point. Endocrinology and Rheumatology are not going to manage these eye findings as they have very little idea of the pathophysiology, exam, prognosis, and treatments.

                            It is also extremely common for pediatric ophthalmologists to handle adult strabismus as they are the de facto strabismus experts.

                            The only other thing I would consider is an Oculoplastics consult as they typically assess for and handle active TED. From what I gather there has only been a comprehensive ophthalmologist and a strabismus surgeon in the picture.
                            I'm not sure how the ophthalmologist could have formed an opinion about active vs quiescent inflammatory disease. My impression (perhaps incorrect) was that he saw diplopia due to Graves' orbitopathy and reflexively recommended strabismus surgery. (That may be appropriate. I just want to be sure.)

                            I've seen this syndrome in many interventional cardiologists in years gone by: See coronary stenosis at angiography ---> perform coronary intervention (previously PTCA, then stent) without further reflection. We now know that was not the best care, but that reflexive behavior was common in the past.
                            Erstwhile Dance Theatre of Dayton performer cum bellhop. Carried (many) bags for a lovely and gracious 59 yo Cyd Charisse. (RIP) Hosted epic company parties after Friday night rehearsals.

                            Comment


                            • #15
                              Originally posted by TheDangerZone View Post

                              The only other thing I would consider is an Oculoplastics consult as they typically assess for and handle active TED. From what I gather there has only been a comprehensive ophthalmologist and a strabismus surgeon in the picture.
                              I searched for Oculoplastics specialists and found three on faculty. As you indicated, thyroid eye disease was listed among conditions they treat, where it was not mentioned in the bios of other faculty members who were not Oculoplastics specialists.

                              What does such a specialist offer, in particular? That is, if strabismus surgery is the surgical treatment for quiet TED with diplopia that did not resolve with steroids/medications, and if the pediatric specialist is typically the strabismus surgery expert, what does the Oculoplastics consultant provide that the pediatric consultant would not? Do they manage steroids/Tepezza, etc.? Do they perform a different type of surgery that might be needed for TED? What else?
                              Erstwhile Dance Theatre of Dayton performer cum bellhop. Carried (many) bags for a lovely and gracious 59 yo Cyd Charisse. (RIP) Hosted epic company parties after Friday night rehearsals.

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