This Live Lecture helps you decide which patients presenting with blepharoptosis require imaging or systemic work up, and the options for fixing it surgically.
Lecturer: Dr. Robert Kersten
DR KERSTEN: This is Bob Kersten from UCSF. I do ophthalmic plastic and reconstructive surgery, and our Cybersight lecture this evening is going to be about the workup of the patient who presents with acquired ptosis. I’m not going to be talking about congenital ptosis, and although this was initially build as a lecture on both the workup and the management of ptosis, I do not plan to do that now. Okay. Here we are. So we’re gonna be talking today about a patient with acquired ptosis. And our goals today will be to demonstrate the various causes of ptosis and to distinguish those patients who need further workup from those patients who present with a garden variety mechanical ptosis with dehiscence of the levator or fatty infiltration of the levator caused by senescence, where we can take these patients directly to surgical repair, without the need for a more extensive systemic workup. And specifically, we’re gonna highlight those patients who have ptosis which may be associated with more serious — either neurologic or systemic problems. These are patients we don’t want to miss. Again, those, the majority of patients with acquired ptosis, it’s usually — the most common cause is a defect in the levator aponeurosis. This is a mechanical problem. The levator muscle itself is fine. The third nerve, the superior division of cranial nerve III going to the muscle, is fine. The problem is in the transmission of force from the muscle to the eyelid. The tendon of the muscle, the levator aponeurosis, is either stretched or thinned, or may actually pull loose and be dehiscent. These patients typically will have good levator excursion, because the muscle and the nerve are still working normally. They oftentimes will have a high eyelid crease, as we can see here. This is partially caused by descent of the eyelid and elevation of the aponeurosis attached to it, but in addition, there’s almost always a component of brow recruitment ipsilateral to the ptosis, which tends to pull the lid crease up as well. These patients should not have lag in downgaze. So these patients, as demonstrated here, will have good levator excursion, in this case showing about 15 millimeters of excursion from down to up. This is common in the aged population. We can also see it in younger patients, in whom it oftentimes may either be associated with rigid contact lens wear, where the act of removing the lens, by pulling down and out on the eyelid, day in and day out, for many years, causes the levator aponeurosis here to stretch out, it may occur after either any sort of periocular trauma or periocular surgery, and patients who do a lot of eyelid rubbing also may stretch out the levator and cause this junction or connection of the levator muscle to the tarsal plate — the aponeurosis here — to stretch, and allow the lid to descend, even though it’ll still have good elevation and descent. Here’s an example of a patient who has such thin eyelids, you can see very high, deep eyelid creases here. The more ptotic side — you can see she’s raising her eyebrow, just trying to get that eyelid above the pupil, because she still wants single binocular vision. When she closes her eye, you can actually see — you can see the blue irides shining through the lid. That’s because, when the levator aponeurosis has pulled all the way back up, the lid is thinner, and it’s almost translucent. Now, one other concept to be familiar with is that of Hering’s dependency. I think we’re all aware of Hering’s law as it applies to strabismus and to extraocular excursions. We know that yoke muscles should get equal and symmetrical innervation. This also applies to the levator muscle, and this may sometimes disguise a ptosis. Here’s an example of a patient. She presents with an obvious ptosis here in the left eye. You can actually see she’s a rigid contact lens wearer. She’s recruiting the brow on this side, to try and lift that eyelid up better, but when we actually lift the ptotic eyelid up to normal position, we then see the opposite lid dropping down. And this is because Hering’s dependency had resulted in increased innervation going to the levator on this side, symmetrical increased innervation to the contralateral eyelid. If this is her dominant eye, it’s more likely to be apparent than if it’s the non-dominant eye. But when we lift the eyelid up on the more ptotic side, all of a sudden she decreases the amount of nervous stimulation going to both sides, resulting in a contralateral drop. This is something we’d like to identify beforehand, and advise patients that surgery on one eyelid may cause further drop on the contralateral side. In this case, we elected to do bilateral surgery at the same time, to achieve this symmetrical result. Now, Hering’s dependency can also cause retraction of the contralateral eyelid. You can see here. She’s got a ptosis on this side. She’s trying to get that eyelid open. And in so doing, she overstimulates both levator muscles, causing lid retraction on this side. When we lift this eyelid, the ptotic eyelid, in order to allow her to reduce that excessive innervation, the contralateral lid retraction goes away. Now, it’s not always predictable whether or not Hering’s law will result in a persistent ptosis on the contralateral side, and therefore one may wish to operate on the more ptotic eyelid and then reevaluate. So, again, the large majority of ptosis is going to be involutional, a problem with the levator aponeurosis, and these patients really do not need further workup beyond a regular ophthalmologic examination, looking to make sure they have a good Hering’s — I misspoke. A good Bell’s reflex, so that we don’t worry too much about exposure. Making sure that their superior rectus is also working, and making sure that they have an adequate lacrimal lake. However, there are red flags in the presentation of patients with ptosis which indicate that these are gonna require a more exhaustive evaluation before proceeding to surgery. And these red flags are ptosis associated with anisocoria, and that can be either with miosis or mydriasis, ptosis associated with diplopia or extraocular muscle dysfunction, if there’s significant variability in the ptosis from day to day or throughout the day, if there’s globe displacement or proptosis, indicating an underlying orbital problem, any ptosis that has an unexplained sudden or recent onset should be further evaluated, acquired ptosis with poor levator function — remember that average acquired ptosis, due to disinsertion of the levator aponeurosis, should have good levator function. So if someone has acquired poor levator function, that requires additional inquiry. And obviously we need to always be aware of the possibility of associated ocular inflammation, which can cause a ptosis, due to the swelling associated in the soft tissues. So ptosis and anisocoria. This can, again, either be associated with miosis or mydriasis. In this example, this is a patient who presented with ptosis, and he also has anisocoria. We can see here a smaller pupil on this side, a larger pupil on this side, and this had developed immediately upon awakening from a cervical spine operation. And in this case, it’s damage to the cervical ganglion here, the stellate ganglion in the neck, that causes an interruption of the sympathetic nerve supply to Horner’s muscle — rather, to Mueller’s muscle — here in the upper eyelid, as well as to inadequate — as well as interruption of the supply to the pupillary dilators. Another patient with ptosis and anisocoria, slightly more miotic on this side than this side, it’s a little bit harder to see, because of his dark irides, but this was after a knife wound. Again, with injury to that cervical ganglion. Now, one of the potential ophthalmic emergencies that we may encounter is demonstrated by this patient here. This is a 57-year-old woman, and she developed new onset ptosis and complained of right ocular pain, which followed immediately after a severe coughing episode. And we instilled topical cocaine drops to diagnose her Horner’s syndrome, and you can see on this side her pupil dilated well. There was no dilatation of the pupil on this side, which indicates to us that this indeed is a Horner’s syndrome, and because of her coughing spell, and her ocular pain, we ordered an urgent CT angiogram of the neck, and it showed that she did have a carotid dissection going on, which was interfering with the sympathetic fibers, which run along the carotid. So something that we don’t want to miss. Any new sudden onset Horner’s, especially if associated with head or neck pain, we need to rule out a possible carotid dissection. Another patient, another potential ophthalmic emergency, this patient has ptosis associated with mydriasis. The pupil is slightly larger on this side than it is on this side. You can also see in addition to his ptosis he also has a little bit of an exotropia. We can see that the globe is drifting out here. And that’s because this is an evolving third nerve palsy, where we have unopposed — the medial rectus is paretic here, the levator muscle is paretic, and the pupil is involved here. The pupillary sphincter muscle is not working well. And the lateral rectus is unopposed, causing a bit of an exotropia. These patients need to be urgently worked up for a posterior communicating artery aneurysm. And that is demonstrated on this angiogram here. The definitive study now, probably, if it’s available to you, would be a CT angiogram. We can generally avoid doing a direct puncture angiogram, to make this diagnosis with currently imaging modalities. Another type of ptosis that requires additional workup is anybody who presents with a variable ptosis. In this case also of unexplained onset. This gentleman came in with a several-month history of ptosis, and he said it was worse on the left than the right, but it varied quite a bit. Tended to be worse toward the end of the day. And you can see these pictures were both taken within about 10 minutes of each other, when he was here in the office. This was his initial presentation with obvious ptosis, which on this side is completely occluding the pupil, even though you can see he’s trying really hard to recruit the frontalis to get that eyelid open. We had him rest with his eyes closed for five minutes, had him open them again, and now he has still a ptosis, but less obvious. Here’s another young boy who had a similar problem. This is him when he first presented. Obviously bilateral severe ptosis. Recruiting the frontalis to try and get his eyes open. We had him rest his eyes closed for five minutes’ time, and he’s now able to open reasonably normally, no longer having to recruit the frontalis. Again, both of these things indicating underlying ocular myasthenia gravis. This is also elicited by having the patient maintain sustained upgaze. This is a patient who actually previously had ptosis surgery on the left eye with a frontalis sling, although a diagnosis had not been made for his poor function ptosis, which was acquired. Here he is at the start of the evaluation. I’m having him look up. And here he is, about a minute later, as he tries to maintain upgaze. You can see that levator muscle on this side becomes fatigued, and it starts to close. Now, one test that may be helpful to us is to do an ice test, where we take an ice bag and have the patient hold it over the affected eyelid for two minutes’ time. And here’s an example of the patient before. She holds the ice bag for two minutes, and then after you can see there’s been an improvement in that ptosis, and this is something that’s almost as reliable as the Tensilon test, and does not require Tensilon and having atropine on hand and starting an IV and all the other things that go along with Tensilon. Now, myasthenia will present 75% of the time with ocular findings, and that’s because the extraocular muscles are the muscles that — some of the muscles we use the most in our body. So they’re some of the ones that are most affected early on with this disorder. Of those patients who present with ocular findings, only about 60% will develop systemic findings, and that’s usually within two years’ time. Now, a more recent study, which was published two years ago, found a slightly lower instance of progression to systemic disease. 20% progressed to systemic disease. And also we used to say that if they haven’t developed systemic myasthenia within two years’ time, they won’t. But in their series, they found about 30% of patients who did progress actually progressed after two years’ time. So we know now that this is something that we have to be on guard for, and we can’t relax our concern about systemic myasthenia after two years’ time, which we used to believe. The ice test is oftentimes helpful to us. This is a patient who did not respond to an ice test. And he had sudden onset of very poor function ptosis. And I see something like this — he actually had figured out how to do a frontalis sling. He took a Band-Aid, attached it to his eyelid, and taped it to his forehead, to try to make this frontalis action more effective for him. When I see somebody with sudden onset of marked ptosis, then one of the key things in my differential would be ocular myasthenia. If they have severe ptosis, sometimes the ice test will not be positive. We do always order acetylcholine receptor antibodies in these patients. These will be present in only about 50% to 60% of patients with pure ocular myasthenia, whereas 90% of patients with systemic myasthenia will have measurable anti-acetylcholine receptor antibodies. Here’s another example of a patient that we want to keep in mind. This is a patient who actually presented with a diagnosis of Graves ophthalmopathy. He had proptosis, he had marked limitation of elevation here on this side, he also had a lower lid entropion. Proptosis, as we can see here on imaging — he has a markedly enlarged inferior rectus muscle. However, his thyroid function studies were all within normal limits. Even though we thought this was most likely a euthyroid Graves, given the isolated enlargement of the muscle there, and his limited elevation with significant restriction, we went ahead and did a biopsy of this, which was consistent with Graves ophthalmopathy. What I should note here, however, is that in addition to his limited elevation, he also had other evidence of limited excursions. Here in downgaze you can see that not only does he have limited elevation, as we saw in this view here — he also has limited depression, which we really wouldn’t expect from Graves ophthalmopathy. He also had limited adduction here and limited abduction. So he showed sort of global extraocular muscle dysfunction on the left side. And this should alert us to the association of Graves’ disease and myasthenia gravis. And in fact, this patient, followed nine months later, had developed marked ptosis on the contralateral side, lagophthalmos because of weakness, and did indeed have positive acetylcholine receptor antibodies. We need to remember that about 1% of patients with Graves ophthalmopathy will also have a coexisting myasthenia. Another patient here, just showing — this is a patient with Graves ophthalmopathy. Has bilateral proptosis, some left lower lid retraction, and here he is with sustained upgaze, and you can see, even though he’s trying to keep his eyes open, as I try and hold him in upgaze, his lids gradually descend on both sides. And this also is a patient — in his case, his acetylcholine receptor antibodies were negative, but I’m quite confident that this represents another case of Graves ophthalmopathy associated with myasthenia gravis. Now, I should also mention that patients whose ptosis gets worse at the end of the day is not a definitive diagnosis of myasthenia, and in fact, most ptosis gets worse toward the end of the day. So I find it more helpful if there’s a history of variation throughout the day, or especially if there’s a history of ptosis that alternates from side to side. But here’s an example of the sort of patient who would also get evidence of a diurnal fatigue toward the end of the day. This patient just has a levator disinsertional ptosis, elevated lid crease here. He’s raising his eyebrow in an attempt to clear the visual axis. But as the day goes by, that frontalis gets a little bit weaker, and we can simulate what happens by blocking the frontalis, and you can see his ptosis gets worse. And he did indeed complain that ptosis was worse toward the end of the day. This was not because of myasthenia, but rather because of fatigue of the frontalis muscle. Now, one other thing to keep in mind that can give a history of variable ptosis is evidenced by this patient here. This is a patient who had a history of previous right Bell’s palsy, and he now has aberrant reinnervation. An aberrant reinnervation after Bell’s palsy is quite common. A majority of patients will show some evidence of synkinesis between — usually between the buccal branches and the zygomatic branches, as you can see here. Now, this is a patient — here he is without smiling. When we ask him to puff out his cheeks, the cheek on this side, the buccal branch of VII, has normal function, but on this side, he can’t puff out the cheek as well. But in fact, he gets an exacerbation of ptosis on this side. This is not due to a third nerve issue. This is due to aberrant innervation of the zygomatic branch of cranial nerve VII, which causes the orbicularis here to spasm when attempting to activate the buccal branch, and should not be confused with myasthenia. So we talked about ptosis and anisocoria, we talked about ptosis and variability of ptosis. Ptosis and diplopia is another red flag that indicates a need for evaluation. Here is a patient who presented with left upper eyelid ptosis, but in testing her extraocular functions, you can see the globe on this side does not elevate as well on this side. And that’s because you see the Hirschberg reflex is displaced inferiorly here, and it’s more centered on the pupil. That tells us that this eye does not look up as well as this eye, and she did in fact note diplopia. But she did not — she had not volunteered this to me. She was not actually aware that she had double vision until I took her through her extraocular excursions, and then when we forced her to look up, she then volunteered — oh, yes. I do see, in fact, two fingers now. This is an indication for evaluation. And in this case, we did imaging of the orbit. And she had what proved to be metastatic breast carcinoma, which was involving the levator and superior rectus muscle, causing paresis and underaction of both of these structures. Causing the ptosis, as well as an inability to elevate the globe. This is a patient with three things that are of concern to us. He’s a young man in his mid-teens with new onset ptosis. Well, that really is something that we wouldn’t really expect to see. He also complained of diplopia in upgaze, and you can see here he has marked limitation of elevation in here. And then he had developed a spontaneous conjunctival hemorrhage, the day before he came in for evaluation. Sadly, imaging on CT and then subsequent MRI showed a rhabdomyosarcoma, a parameningeal rhabdomyosarcoma, originating in the ethmoid sinus, with intracranial extension. This is a very different rhabdomyosarcoma than the type that we typically see in the orbit. Those occur in younger patients, are usually limited to the orbit, and have a very good prognosis if treated with biopsy, radiation, and chemotherapy. This patient, sadly, went on to succumb from the disease, once he had intracranial invasion. Now, this is an example of a patient who has ptosis and limited extraocular excursions, but she does not have complaints of diplopia. Obviously when we have disconjugate gaze, which develops after someone is visually mature, we would expect them to have double vision. Sometimes they can learn to ignore it. But this patient represents a unique subset of ptosis and limited extraocular excursions. You can see her in primary here. Here she is now in right gaze. Here she is in left gaze. We can see that she does not have good excursion. And when we look at her face, she actually has minimal facial expression. What is referred to as a “masked facies”, and this is quite suggestive of chronic progressive external ophthalmoplegia. This is something that is a mitochondrial myopathy, which affects the extraocular muscles, the levator, and the remainder of the extraocular muscles, as well as the muscles of the face. But because — for reasons we’re not really completely clear on — these patients generally do not complain of diplopia, even though we would think they should have diplopia, because this is acquired. This is a defect in the mitochondrial DNA, and it results in deletions in mitochondrial DNA, which results in poor energy production by the cells. Which is most manifest by those muscles in the body which are most dependent on oxidative metabolism. The extraocular muscles being among those. The facial muscles. And we can see involvement of the heart, with cardiac conduction defects, and also pigmentary retinopathy, in what’s known as Kearns-Sayre syndrome, or ophthalmoplegia-plus. Now, there are two other muscular dystrophies we would mention just in passing. Oculopharyngeal dystrophy, and myotonic dystrophy. The chronic progressive external ophthalmoplegia is usually transmitted by the mother to both her male and female offspring, because the mitochondria that we get at birth all arise from our maternal mitochondria. But in oculopharyngeal dystrophy, as well as myotonic dystrophy, this is an autosomal dominant mutation, and in both cases is associated with a systemic muscular dystrophy as well. Oculopharyngeal dystrophy is something which here in North America we typically think of in patients of French Canadian ancestry, although there are pockets of this disorder around the world. Patients may either be homozygous or heterozygous, and if they’re homozygous, they have worse — or earlier onset, and worse dysfunction. These patients have difficulty swallowing, because of involvement of the oropharyngeal muscles, and this can lead to life-threatening aspiration, and so if we have a patient where we suspect this, they need to be seen by an ear, nose, and throat doctor, and if in fact they have defective swallowing, they normally will undergo a cricopharyngotomy to prevent the risk of aspiration. This over time becomes a systemic muscular dystrophy. This is a patient who’s had it for a prolonged period of time. They get wasting of their facial muscles. They get temporalis wasting as well, as we can see on both sides here. And oftentimes have limb dysfunction as well. Now, myotonic dystrophy actually is a little bit atypical amongst the other muscular dystrophies, in that the face and neck, hands, forearms, and feet tend to be preferentially involved. And these patients have a difficulty where they have a hard time releasing their grasp. If you shake their hand, they will hold onto your hand, and then they will very slowly be able to relax the muscle. This comes in two types, type I and type II. Those are two different autosomal dominant mutations, and type II may be associated with diabetes and testicular failure as well. These patients oftentimes have fairly early severe ptosis and ophthalmoplegia, and they will oftentimes have typical frontal balding here, and may also have polychromatic cataracts visible on slit lamp examination. And again, they have difficulty relaxing their grip, so when you grasp and shake their hands, they have a hard time releasing the grip. Another example of ptosis associated with double vision — in this case, also some globe displacement — this is a 19-year-old woman who presented with a fairly subtle right upper lid ptosis. But again, you can see she doesn’t elevate the right eye quite as well as the left eye. She was not aware of this, but oftentimes patients will not know that they have problems with elevation. And only when we took her through and demonstrated her extraocular excursions did she note there was a little limited elevation on this side. Again, when I see a combination of limited elevation and ptosis, I want to think about a possible orbital cause. On imaging, she actually had a very large dermoid cyst here, of the orbital roof, which was compressing the levator superior rectus complex. It had probably been present since birth, but very slowly enlarging, and only become symptomatic in her second decade. This patient’s an example, again, of an orbital cause of ptosis associated with diplopia. She had fairly sudden onset of a right upper eyelid ptosis, and complaints of vague orbital pain. Her past history was positive for having had a malignant melanoma of the trunk, which had been removed several years previously. She does have good upgaze here, and was not really complaining of diplopia in upgaze, but on downgaze, you can see she has -1 limitation here. Looking at a Hirschberg reflex on this side, compared to this side, now. She did not actually have anisocoria, but we had dilated to look in the back of the fundus on this side, which is why she has a mydriatic pupil. But this combination of ptosis and limited depression in this case led to orbital MRI, which showed a mass here in the orbital roof. Which proved to be metastatic melanoma. So, again, ptosis and limited extraocular excursions, when they’re acquired, make us think about a possible orbital cause. If the ptosis is bilateral with limited ocular excursions, then one of the muscular dystrophies, such as chronic progressive external ophthalmoplegia, or oculopharyngeal dystrophy or myotonic dystrophy, is higher in our differential. Another example of a patient who had unexplained recent onset. She was a young girl, only 16, but she said over the last two months, she’d had persistent drooping of this left upper eyelid. She has good levator function here, and she showed no evidence of ocular inflammation, but she was a rigid contact lens wearer. And when we elevated the eyelid, we could actually see that she had obvious inflammation going on in the superior fornix. Doubly inverting it, we actually found that she had a lost contact lens, which had traveled up into the superior fornix, caused an inflammatory reaction, and caused onset of ptosis. In this case, it’s a mechanical ptosis, just caused by inflammation and swelling and the gravitational effect of that. And here she was, one week later, after the lens had been removed, and already we can see her ptosis is starting to improve. Now, here’s another example of a patient who has a fairly new onset ptosis. And again, if I have a new onset ptosis, that came on fairly suddenly, I need to explain this. Interestingly, she had ptosis which we can see here — it’s fairly subtle, but it’s subtle because she’s recruiting her frontalis on this side, compared to this side, because she wants to get that eye open and maintain her single binocular vision. She had no evidence of proptosis. And she had good levator function. And she had no lag in downgaze. So essentially a normal examination here. We did an ice test, which was negative, and I decided we had to explain this somehow. It had only been there for three months’ time. So we got orbital imaging, and this actually showed what proved to be a lymphoma involving the superior rectus levator complex, and leading to the ptosis. Okay. This is an interesting case that we see here in North America and Europe probably much more than we would in some parts of the developing world. But this was an interesting case. It was referred by her primary care physician to me, to, quote, “rule out” an aneurysm. She was 28 years old, and she’d awoken Sunday morning with a significant right upper lid ptosis. She mentioned that she had been partying hard on Saturday night. She denied any pain, diplopia, or diurnal variation. She stated she was in excellent health and denied any systemic problems, and no systemic medications. The tipoff as to what was going on in this patient was seeing when we looked at her eyebrows, however. You can see this is a typical thing that we would expect in a new onset ptosis. This patient is raising his right eyebrow, because he’s recruiting the frontalis in an attempt to clear the visual axis. This patient could not recruit her frontalis, and in fact, she has a medial brow ptosis. And this led us to question her, as to whether she’d had a Botox injection, and in fact, she had. She said on Thursday, she’d gotten Botox, but she’d actually forgotten that by Sunday. And, of course, the onset of the effect of Botox and its muscle paralysis is usually three to five days after injection. So she was injected on Thursday, up here in the glabellar region, and Sunday morning, she woke up, and now she has a ptosis. And this is a Botox-induced ptosis. Now, in that differential of sudden onset ptosis, we want to think about a third nerve palsy. But there we would normally expect to see pupillary abnormalities, as well as limited extraocular excursions. Horner syndrome we talked about. If it’s sudden onset with a small pupil on that side, we then want to image that patient to rule out the possibility of a dissecting carotid aneurysm. Myasthenia is certainly high in my differential in any sudden onset ptosis, but we also need to think about the possibility that the patient had Botox injections. And because the ptosis can be delayed, sometimes they will not volunteer that history unless you ask for it. Another patient similarly, with Botox-induced ptosis. And again, you can see she’s not really recruiting her frontalis on this side, because that muscle’s been paralyzed. But we can see we have a little more nasal-brow ptosis here than on the opposite side. That tells us that they might have injected a little bit closer down toward the brow, and allowed some of this to migrate into the levator muscle. This can sometimes be managed with topical iopidine drops. Again, this is not a sympathetic problem. The sympathetic muscle, Mueller’s muscle, is working just fine here. But if you stimulate Mueller’s muscle, it will help to overcome the paresis of the levator muscle, which is, of course, a cholinergic phenomenon, which is blocked by the botulinum toxin. Another patient with sudden onset of ptosis, and again, any sudden onset of ptosis needs to be explained. In this case, her ptosis had come on after a bout of emesis, and on imaging, she had a spontaneous subperiosteal hemorrhage here. Now, one other cause of ptosis which may be overlooked is evidenced by this patient here. This is a 72-year-old gentleman who had ptosis, ocular irritation, and discharge. And this had been going on for several years’ time. A couple of key findings to look at here. He’s got gross eversion of the lid. He’s got this mucoid discharge in the fornix on this side, and he also has lash ptosis. His eyelashes are pointing down toward the floor. And when I elevate his upper eyelid, it spontaneously everts, revealing this mucoid discharge and severe ocular irritation, and this is, quote, “floppy eyelid syndrome”. This is almost always associated with obstructive sleep apnea, and it can be thought of as an abnormality of sleeping position. These patients sleep face down, because when they try to sleep supine, they get airway obstruction. So they automatically roll over onto their side or onto their face. It’s usually associated with very restless sleep. So they tend to rub their eyelids against the bedclothes, leading to lash ptosis, marked elongation of the eyelids, and discharge and a papillary injection. The first intervention in these patients is always to send them for a sleep study, to diagnose their obstructive sleep apnea. And to get this treated usually with continuous positive airway pressure. If you try to surgically correct this without addressing the underlying abnormal sleep position, it almost always will recur. So in this case, we put the patient on a sleep mask to try to protect his eyelids, got the sleep study, and once he was on CPAP, we then proceeded to do repair of all four lids with a bilateral tightening of the lateral canthus on both sides. Now, you always want to think about floppy eyelid syndrome in patients with acquired ptosis, and it’s true — most of them will sort of announce themselves. They have this big bullfrog neck. Ptosis, which we can see here, and complaints of ocular irritation, especially upon awakening in the morning. This body habitus will be present in approximately 75% of patients with obstructive sleep apnea, but 25% of patients will have a more normal — a thinner body habitus, and still can have sleep apnea and floppy eyelid syndrome. Lash ptosis is highly suggestive. Easily distractable eyelids, as we mentioned. And although we typically see this in men, we can on occasion see it in women as well. This was a woman who presented with really not even ptosis — but she just had chronic ocular irritation upon awakening in the morning. And when we applied traction above the eyelid, you can see she spontaneously everts the right upper eyelid here, exposing the underlying fornix. That is abnormal. Nobody, when you put superior traction, should spontaneously evert their eyelid. If they do, they have marked elongation. I had to argue with her internist, because I said I wanted a workup for sleep apnea. That I suspected she had sleep apnea, because I thought she had floppy eyelid syndrome. And he said — oh, you know, sleep apnea only occurs in men. And she doesn’t have a bull neck or the other characteristic findings. But I pressed him on this. And I said please just trust me here. I think we need to check this out. And sure enough, she had an apnea hypopnea index of 111 episodes of hypopnea per hour, and oxygen desaturation down to 86%. So she is somebody who definitely has obstructive sleep apnea, and this was suspected based on her floppy eyelids. This patient — just a reminder to us to always think about the possibility that we can see ocular irritation resulting in ptosis. This is a patient who had an adenoviral conjunctivitis. This was epidemic keratoconjunctivitis. And she had a positive lymph node, which was palpable right over here, and she had obvious ptosis here. You can see she’s recruiting the frontalis, trying to get that eyelid back open. And this was caused just by edema of the eyelids, causing a mechanical effect. We also need to think about ptosis and inflammation in a patient like this. You can see she’s got a right upper eyelid ptosis. It’s relative, in that she’s trying as hard as she can to get her eyes open. She’s causing retraction on the opposite side. But when we look closely, we see this umbilicated lesion here in the upper eyelid. And this is a molluscum contagiosum. And as we know, those mollusca will shed viral particles into the conjunctival cul-de-sac, and cause a papillary conjunctivitis, and weighting down of the eyelid, caused by the swollen papillae. Treatment in this case is to excise the molluscum. And this patient here, with persistent — a several-month history of left upper eyelid ptosis — she was actually referred to consider ptosis repair, but when we lift her left upper eyelid up, we can see that she has superior limbic keratoconjunctivitis. Again, this is a patient with recent onset — new onset ptosis. Fairly sudden. And with ocular inflammation. We also need to think about the possibility of mechanical ptosis. This is a patient who presented with a several-week history of ptosis. He’s pretty obvious, because you can see this fullness of the eyelid here. Again, he’s recruiting the frontalis to try and get that eyelid open. We evert the eyelid, and here we can see he’s actually got a large pyogenic granuloma erupting from a chalazion in the upper eyelid. That’s an example of pseudoptosis. Another example — this is a patient who was referred in to evaluate ptosis, but as we can see, he’s actually got dermatochalasis here. And after doing just a blepharoplasty, we can see that he has no true ptosis. He just had skin draped over the lid margin, contacting the lashes. So patients with ptosis — again, the large majority of these patients are going to be caused by simple mechanical distraction of the levator aponeurosis. Their examination is characterized by good levator function and elevated eyelid crease. And usually a fairly gradual history of onset. We can see it in younger patients with rigid contact lens wear, or after any sort of periocular trauma or periocular surgery. Those patients who require further thought before rushing into surgery are anybody with ptosis and diplopia. Anybody with truly variable ptosis — especially if it varies significantly throughout the course of the day, or from day to day — and I found it quite helpful now that many patients will have a cell phone with a camera on it. I ask them to take some pictures. I wanna see a picture of when you first wake up in the morning. I want to see one later in the day, and I want you to take a picture — if you ever look in the mirror and notice it seems more droopy, please take a picture for me. And if they’ll bring in several photos throughout the day, this diurnal variation may be much more apparent to you. Ptosis and anisocoria — obviously it could either be a small pupil, in which case we worry about Horner’s syndrome, and again, always remember a new onset Horner’s syndrome, especially if associated with neck trauma or headache, neck pain or headache, you need to rule out dissecting carotid aneurysm. Ptosis and anisocoria with a dilated pupil on the ptotic side — also usually associated with some evidence of extraocular muscle dysfunction, usually with some exotropia, because the third nerve is weak, but the sixth nerve is still functioning normally, resulting in exotropia — need to think about an expanding aneurysm. Sudden onset of a complete third nerve ptosis which spares the pupil is actually very reassuring to us. This is almost always caused by a hypertensive/vascular third nerve palsy, and will usually spontaneously resolve. A partial third nerve palsy or an evolving third nerve palsy is something that worries me far greater. And in these patients, they need to have CT angio, if it’s available, to rule out an expanding intracranial aneurysm. Ptosis which has sudden onset or unexplained onset should be evaluated especially for associated orbital pathology, or, again, for the possibility of myasthenia gravis. Globe displacement or proptosis, of course, goes in concert with an orbital process that could also cause ptosis. Always remember to look for evidence of inflammation, such as a patient with molluscum, or with superior limbic keratoconjunctivitis. And remember, in acquired poor levator function, this is either going to be myasthenia, or we need to think about one of the various muscular dystrophies we talked about. Chronic progressive external ophthalmoplegia, or oculopharyngeal dystrophy, or myotonic dystrophy. Now, even keeping all that in mind, I wanted to show you one example where, even if you try to get everything right, you can still get fooled. This was a patient who presented with a fairly mild right upper lid ptosis, five-year history, hadn’t really been changing. She denied diurnal variation. She had normal extraocular excursions. And I thought this was just a garden variety levator disinsertion. And so I did not do additional workup. I did not image her. I just took her for surgical repair. And when I got her into surgery, what I found is that Mueller’s muscle had been completely replaced by what proved to be a low grade MALT lymphoma. So even though you try and remember to evaluate for orbital problems or systemic problems, every once in a while we can still get fooled. So that is what I wish to cover in our time here. I think we have a little time to try and go over any questions. Okay. So what are the surgical options for ptosis? I think that we will have a full lecture to devote to this. But basically the surgical management of ptosis depends on the amount of levator function. If there is good function of the levator, good excursion from up to downgaze, I will do a neosynephrine test, where I instil one drop of 2.5% neosynephrine, and if that results in a good elevation of the lid, I always prefer a conjunctival Mullerectomy repair, as I think it’s easy to do, it’s more predictable, and gives better height and contour. So that’s what I would do for someone with good function ptosis who responds to neosynephrine. If somebody has a good function ptosis but fails to respond to neosynephrine, then I would do some sort of a levator aponeurotic advancement. Usually a lid crease. And shorten the levator and reattach it to the tarsal plate. If a patient has a decreased levator function ptosis, and if it’s decreased below, you know, 6 to 8 millimeters, then I will usually do a frontalis sling, where I attach the eyelid to the frontalis muscle. I’m just taking advantage of that natural tendency we have to try to maintain binocular vision by raising our frontalis when we develop a droopy eyelid. And I pointed that out in multiple patients as we were going through, because it’s something you sort of need to train yourself to look for. Now, a frontalis sling works very, very well in patients who recruit their frontalis. It does not work well if somebody does not recruit their frontalis. Most often that would be a congenital ptosis with amblyopia. If a patient has amblyopia, they have no desire for single binocular vision. They usually will not recruit their frontalis. And then the sling is not working very well. In that circumstance, I would usually do a supramaximal levator resection, to go in and remove — resect up to 20 or 30 millimeters of levator muscle, and leave the eyelid up at the superior limbus on the table. There is greater risk of exposure and lagophthalmos, but that’s about your only option, if they don’t recruit the frontalis. Next question is: What do you do for a patient diagnosed with ocular myasthenia? Once that diagnosis has been established, normally our first attempt is to try systemic therapy. But ocular myasthenia is fairly refractory to medical management. Mestinon is usually the drug of choice, which is given first, which is an acetylcholinesterase antagonist, and that just allows whatever acetylcholine is there to stick around longer. If they don’t tolerate Mestinon, you can also give fairly low doses of prednisone, and that may be effective, but it can be difficult to keep patients on prednisone for many, many years, which these patients usually need. Okay. So the next question is: One-year-old child after neck and head trauma with ptosis. Would you image to exclude carotid dissection? If there is no miosis and there’s nothing else to suggest… I think if there’s neck trauma, this patient probably should be imaged for their neck. Not just because of the ptosis. If there’s no anisocoria, then more likely the ptosis is caused from the blunt trauma to the head, rather than trauma to the neck, resulting in a traumatic carotid dissection. But that patient, either way, I think, needs to have their neck imaged, to exclude a C spine injury or something like that. Maximum CM in children? I’m not sure what CM is referring to. Is that something that… Um… Okay. I’m not sure what happened to that question. I’m sorry. I’m not sure what CM referred to. And now I’m… Okay. The next question is: How do you treat ptosis in children at an early stage? That will depend on whether it’s felt to be amblyogenic or not. And patients who develop amblyopia from congenital ptosis — usually it’s not from occlusion of the visual axis. It’s more likely to be caused by induced astigmatism on that side. So the droopy eyelid sort of presses on the cornea, and causes a with-the-rule astigmatism, and then that can result in a refractive amblyopia. If I think they have refractive amblyopia or are at risk for it, then I will either do a levator resection, or a frontalis sling, depending on their levator function. If they do not, then we typically can get better results if we wait until they’re a little bit older, ’til they’re 4 or 5 years of age, and we can better judge the amount of levator excursion, et cetera. Okay. How may I reduce use of traction suture to shorter days? Well, if you’re talking about a traction suture in ptosis, like a Frost suture, I don’t use them at all. So I know that historically, when we would do either frontalis sling or levator resection, some surgeons advocate putting in a Frost suture to the lower lid and taping it to the forehead. I just stress to the parents to lubricate the surface of the eye every couple hours for the first few days. And I’ve been doing this procedure for 30 years, without using a Frost suture, and I don’t think that you need to use it. Patient with ocular myasthenia we already discussed. Okay. A female child, nine years of age, with intermittent ptosis over 2 to 3 years’ time, which resolved after puberty? Any comments? I’m happy for her. I’m not sure… You know, that would certainly make me be concerned about ocular myasthenia. But I’ve actually seen one patient who had a third nerve caused by an aneurysm, which had waxing and waning ptosis, as the aneurysm was sort of stretched and swelled a little bit, and then kind of contracted down a little bit, and it took about six years before that… The underlying aneurysm was diagnosed. Fortunately it was diagnosed before it had ruptured. But… Probably myasthenia, if it’s intermittent and then resolved. When to correct ptosis due to third nerve palsy? That is… You know, what you need to do with these patients is correct their strabismus first. If you lift their eyelid up without aligning their globe, they’re just gonna have severe diplopia. And they will not try to use the frontalis sling. But generally we use a frontalis sling, and we do that after a patient has had corrective strabismus surgery. Try to get them to single binocular vision, at least in primary. Next question: How do I prevent frontalis sling extrusion on the forehead? Well, I think that would kind of depend on what material was used. I typically will do a lid crease incision and suture the sling directly to the tarsal plate. And then I do my three stab incisions, medially, centrally, and temporally, right at the eyebrow. I don’t do an incision up in the forehead itself. I put all three incisions right along the superior brow. You don’t really need to do that center one any higher than that. And I bury the knots deeply, and then do a two-layered closure of the skin over that. CM — conjunctival Mullerectomy. Okay. I think we’ve already talked about conjunctival Muellerectomies. I’m trying to remember what the question was. Would I do that in a minor ptosis? Yes, I definitely would do it if they responded to neosynephrine. If they even have a modest response to neosynephrine, they’re not fully corrected, I’ll do a Mullerectomy. But then I’ll do more of a Mullerectomy. I might take 10 millimeters or 12 millimeters or even 14 millimeters, on occasion. Okay. If you have strabismus and ptosis on a patient, which one would be managed first? We always manage the strabismus first. And that’s because, number one, in order to get the eyelid to… You need to have single binocular vision to get a sling to work, for instance. And sometimes repositioning the eyeball is necessary before you can judge where to put the eyelid. So if you have somebody who’s got an esotropia and a ptosis, I don’t really know exactly where to put the eyelid, until the esotropia has been corrected. And then I can judge the distance between the pupil and the globe. Why do some children develop squint despite early ptosis surgery and glasses correction? You know, I’m not certain. I’m really not a pediatric ophthalmologist. I do pediatric oculoplastic surgery. And I’m not sure that I’ve ever seen a patient who developed a squint after ptosis repair and glasses correction. But it sounds like somebody might have had a high AC/A ratio, and maybe it wasn’t fully corrected with glasses. I’m not sure exactly what might have been going on with that one. So I don’t know if I can answer that. But I think it’s right at 10:00 now. And I think we’ve gotten through our questions. Anything else that we want to try and cover before I sign off? Okay. Well, listen. I appreciate everyone’s attention. I hope that was helpful. And we will schedule a session to speak at greater length, and demonstrate the various surgical options to correct ptosis. All right. Take care, and we’ll see you soon.