 All right. Good morning everybody. We have three presenters to get through. So we're going to go get started first up is Jim bell And why don't you see double? All right So Adam Jorgensen has already been kind enough to postpone his talk for a few weeks And I know Lloyd has some pretty neat stuff to talk about so I'll try to be quick here But hopefully some of you all got to see our patient this morning. She was kind enough to come in So thank you and as you can probably guess our patient does not have Diplopia So she's a 69 year old right-handed woman who came in with the chief complaint of not being able to look left with her left Eye this was found incidentally at a routine visit with her primary care physician in April of this year The patient was referred to a general Ophthalmologist who saw the patient a few months later in the interim She thought about it and she really couldn't recall a time that she ever did see double. She also After long thinking decided that maybe the only thing she could come up with was she had to turn her head to the left to see Oncoming traffic for the past few months, but that was about it The general ophthalmologist saw her and determined that she had a left six nerve palsy and thought it was new So the patient was referred to neuro ophthalmology She came to see us and we got a little more past ocular history at the age of four She was noted to have crossed eyes. This was noticed after she fell down some stairs She wasn't symptomatic from this So her parents decided to wait for strobizma surgery She decided to wait when she was an adult and she had it done at the age of 35 She never did have diplopia. She never patched an eye as a child She had cataract surgery in each eye in 2008 and she recently developed a paracentris catoma in her right eye That has been followed by a retina specialist and was determined to be a macular hole or pseudo hole So some other medical history she was born with a cleft lip She's always had as she puts it low lung capacity possibly kyphoscoleosis and she's anemic This morning actually I just learned that her father had crossed eyes, which was unknown to us before so her family history might be a little Contributory, but social history medications don't really contribute to the story So on examination she was 2040 in the right eye 2025 in the left eye no anasacorrhea or APD visual fields were normal Amzler grid was pretty much Agreeable with her story of her macular hole in the right eye and her tail was essentially normal as well Color vision was normal according to Ishaar color plates. She pretty much did not have Stereopsis she could not see the fly on that exam and Only pertinent finding on interior exam was that she had posterior chamber intraocular lenses We didn't dilate her because she was being followed by a retina specialist But all we saw in our undilated exam was fairly healthy-looking optic nerves So on to the meat of the story She was found to have a comatent 8 prism diopter right hypertropia Along with the following in her right eye Minus 3 abduction minus 2 adduction minus 1 infreduction and normal superduction The left eye showed minus 4 abduction normal adduction normal infreduction and minus 1 superduction In addition to all of this her right palpebral fissure Narrowed with left gaze and her left palpebral fissure narrowed with right gaze. So I was trying to take all this in I was pretty excited. I thought this patient has a left six. That means their left eye doesn't look left I got this but but then I saw all this So here's her strobusiness series and I'll just focus on two photographs since we got a lot of talk today Right here in right gaze you can tell that her right eye is not abducting well her left Eye it looks like it's coming short a little bit. She actually has good adduction with her left eye And this palpebral fissure is narrowed on right gaze and The left gaze photo her right palpebral fissure is narrowed Limited adduction with the right eye and limited abduction with left eye. So here's a video In case you didn't get to see her upstairs So here she's gonna look to the left and you can see the narrowing of that right palpebral fissure Which is pretty important narrowing of the left palpebral fissure on right gaze So she was kind enough to bring in some old photographs of herself and you can see here at a young age The cameras to her left and she's having some difficulty abducting with her left eye Again the cameras to the left still limited abduction with the left eye in a similar situation here when she's a little older She's more of an adult here and the cameras to her right and you can see limited abduction with the right eye in this photograph So just really quickly less than a week later We had another patient come in 29 year old woman exact same complaint couldn't look left with her left eye She was admitted for HSB meningitis in September discharged a couple days later and incidentally on follow-up with an outside Neurologist it was found that she couldn't abduct with her left eye. So she was referred to us This patient had known of this since she was a little girl. She'd never had strabismus surgery never worn a patch She had daily Horizontal binocular Diplopia that resolved within seconds and it never bothered her. So she never really did anything about it Her visual acuity was 2020 in the right eye 2125 in the left eye She refracted to 2020 and 2025, but she had some very prominent anti-symmetropia Normal color vision and stereopsis with her was actually fairly normal her Extracurricular movements were pretty much normal with her right eye and she showed minus four abduction with the left eye minus two adduction Normal supra in infreduction and narrowing of the left palpeperal fissure on right gaze. So With all that information So a little bit about Dwayne syndrome named after Alexander Dwayne who published his paper on this subject in 1905 and ophthalmology the title of his paper was congenital deficiency of abduction associated with impairment of adduction retraction movements contraction of the palpeperal fissure and oblique movements of the eye so you pretty much have the disease in the title of this paper which is Kind of convenient, but I'll talk a little bit more about it in his paper He actually credited multiple other authors who had previously worked on the subject So he wasn't the original author to describe this this problem But he sort of grouped previous findings together for everyone else to have an easier understanding of it in Europe They actually call it Stilling Turk Dwayne syndrome to spread the credit around a little more It happens in about one out of a thousand people a very small number have been shown to be familial both autosomal dominant and recessive patterns have been seen Monozygotic twin cases have been concordant and discordant a few of the cases have been linked to a specific gene DURS 2 on chromosome 20 This has been associated with decreased hearing too. So that's kind of an abnormal variant If you have sporadic Dwayne's it's more likely that you have other physical abnormalities And as you recall our first patient had a cleft lip at birth so that she fit that description as well It's kind of funny a little bit later. I saw another patient with a type 1 Dwayne's Which I'll show pictures of her in a few minutes, but she said that she was originally diagnosed by Dr. Hoffman who said, you know, you know what you have you have Dwayne's and he explained to her what it is and she said Okay, and she thought they were done and she told me then he looked in the eye and said now What are you missing and she said what are you talking about and he said all you people are missing something What are you missing? So a lot of these patients do have some other sort of abnormalities somewhere So There was a series of 835 Patients with Dwayne's syndrome who were looked at and over half of them had only involvement of the left eye Interestingly, no one really knows why that's the case a few patients had only involvement of the right eye and even less were bilateral So our first patients a little unusual in that regard over half the patients are women and no race seems to be affected more than other races Classically the teaching is that this occurs because of the lack of abducens nucleus and sixth nerve The lateral rectus develops anyway with innervation from an anomalous branch of the third cranial nerve The narrowing of the palpipereal fissure occurs because that third nerve causes contraction of the lateral and medial rectus at the same time And then the eyelids start to close Diplopia is not common with these patients They can acquire a favorite head position where they achieve an ocular vision So that might be why that second patient was able to have stereopsis on her exam So and I some atropia is fairly common in these patients, which we saw with the second patient And often there's no esotropia in primary gaze, which is important because with a sixth nerve palsy You would expect a large esotropia. That was another Sort of key finding in our patient. She did not have an esotropia. So that was sort of a red flag that maybe it wasn't a Sixth so in development of Most people the cranial nerves and extra ocular muscles develop in weeks four to eight of gestation Muscles come first then cranial nerve three then cranial nerve six and that'll become important in a minute There are three types of Dwayne's as Leah was Alluding to in our BCSC series. They give a little bit of help You can remember the type by the number of these in the description So type one is just limited abduction type two is limited adduction type three is limited abduction with adduction And all types have limited or narrowing of the palpable fissure with adduction So here's just a quick series of a patient who came in with the type one Dwayne's in the interest of time Just focus on this photograph here. You can see limited abduction with that left eye But it adducts quite well, but you see the narrowing of the palpable fissure on right gaze Here's sorry Quick video of that So there's that narrowing of the palpable fissure and then she does not have abduction with that She actually had normal down days So narrowing of the palpable fissure again and then limited abduction so a Couple of studies that kind of changed the way this may be thought about in 2005 There was a series of patients with Dwayne syndrome who were looked at with MRI And they had a control series of 60 patients who did not have Dwayne syndrome The purpose of this was to see if they could see a sixth nerve Using this modality and on all 60 of the patients they were able to see the sixth cranial nerve So it was obviously a good test to go looking for a sixth nerve Not a single one of the patients with type one Dwayne's that they looked at had a sixth nerve Both of the patients they looked at with type two Which is the most rare form of Dwayne's had a sixth nerve and two out of the five Patients with a type three had a sixth nerve So the importance here isn't I think as much the patients who didn't have a sixth nerve because that was already thought it was that some Of these patients actually do have a sixth nerve So that became pretty important information of it another similar study a few years later Looked at abducens nerve abnormalities which included the absence of a sixth nerve And every patient with type one and type three Had some sort of an abnormality of the sixth nerve But only some of them with type two had an abnormality that could be seen on MRI So at least a couple of these patients with type two Dwayne's As far as people could tell with imaging had a perfectly normal looking sixth nerve So what does all that mean since initially we thought that this happened because of an absence of a sixth nerve Well another way to look at it is more of a continuum than three completely distinct and separate types And I think it's easiest to start with a type three so in a type three You have a certain number of fibers that are meant to go to that medial rectus This is sort of a new a new way of looking at it a new theory I don't think there have been any studies to completely confirm this but You have a certain number of fibers that should be devoted to that medial rectus from the third nerve And if you divert enough of them to the lateral rectus, you're going to end up with more limited adduction At the same time that lateral rectus is going to contract in a more strong fashion because it's got more fibers going to it Which is going to even more contradict the adduction action of that medial rectus Leading delimited adduction and at the same time these patients either have an abnormal or a completely absent sixth nerve So they're going to have limited abduction and that's how you end up with a type three if you take some of those Diverted fibers and give them back to the medial rectus. You end up with stronger adduction Probably to the point that you don't really notice it on exam So then now the patients are able to add up just fine, but they still have that abnormal six so they can't have so That would be a type one If you take a patient more like the type three and divert most of their fibers That should have gone to the medial rectus and send them to the lateral rectus again You end up with limited adduction, but if they have a normal sixth nerve, they're going to have normal abduction Which leads you to a type two with just limited adduction and normal abduction So the interesting thing there is that the common link doesn't seem to be the sixth nerve It seems to be a third cranial nerve, which is a little different than the classical way of looking at this disease And it's kind of hard to do large studies looking at this because a type two Dwayne's is sort of important in that regard And it's quite rare To be seen in clinics. So anyway, I thought that was very interesting and I apologize My brain feels like spaghetti junction and Louisville, which is Why I have that photograph up there In the end even though some things have changed one thing hasn't and that's the treatment Suggestions for these patients in 1905. Dr. Dwayne said in general an operation is not required and is to be avoided with possible And for the most part that still holds true today Strabismus surgeries don't improve motility. They just improve the direction of gaze and Today that's that's still for the most part the case Strabismus surgery does have a part for these patients if they're Gaze where they achieve binocular vision is really abnormal That can be really uncomfortable and Can look abnormal to it can make them self-conscious of the way they're walking around So at times Strabismus surgery can be helpful to help them achieve stereopsis in a more normal gaze And the patient that I mentioned with type 1 Dwayne's she actually had to have neck surgery because she was walking around like this all Day for years, so that can be helpful. It's just important to explain to them that if you go with that route It's not going to improve their motility. It just might help them hold their head in a more normal position So I'd like to give thanks to our photographers Who stayed late on a couple days to help me out with these patients and of course to our patient who came in that was very helpful, so thank you very much and Take any questions now the up shoot down shoot. Oh with the Dwayne's So that wasn't really mentioned in the new theory that I was reading about and that's that's interesting that you bring that up So I think that's still sort of in the dark as far as I could tell. I don't know if anyone else has read something different Like I said, there's not there's not a huge amount of new information just because of how rare the type 2 is and That's pretty important because those patients are so different from the other two types So I don't have a great answer to that It was really neat to see the old photographs for our patient She she was very prompt with them. We saw her in the morning and we had the pictures by noon So thank you