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TITLE: Presentation 3 Ms F Mellington DATE: 24th February 2017 NUMBER OF SPEAKERS: 1 TRANSCRIPT STYLE: Intelligent Verbatim FILE DURATION: 30 minutes and 01 seconds TRANSCRIPTIONIST: Yvette

SPEAKERS

P: Presenter

1 http://www.transcriptioncity.co.uk 1. GP Eye Health Network: Lids, Ms F Mellington

P: When I was thinking about the talk I thought I wanted to try and give you a fresh perspective on things, rather than sort of keep going down the usual route of disease after disease after disease. I will be doing that a little bit but I thought I’d try and mix up the presentation a little bit. So, if we think of it like a downhill run. We’re going to be looking at all the different eyelid diseases, and hopefully will have a safe journey. I’m gonna try and point out some of the dangers along the way, so that we can avoid any pitfalls and negotiate safe and successful management to our patients. So, here we go.

These are the objectives of the talk. I’m gonna begin with an introduction talking about the key functions of the eyelids. Then I’m going to go over the anatomy of the lids, just to familiarise yourself with that. And then think about the manifestations of eyelid disease in these categories: Lids that can’t fully close, lids that won’t open, eyelid malposition, lid lumps and bumps, and lid swelling. There’s a little bit of overlap between these last two but we can go through those, and with a short summary at the end.

So to begin with, the key functions of the eyelids. Well the key function of the eyelid really is to protect the eye. They’re a physical barrier to protect the eyes from direct blows such as this, or from chemical splashes, or from foreign barriers for example. And key to the protection mechanism is the blink reflex. As with all reflexes it has an afferent sensory arm. In this case it’s the nasociliary branch of the first division of the trigeminal nerve which passes via interneurons in the [s.l. medela 00:01:33]. And then the afferent motor arm is via the temporal and zygomatic branches of the facial nerve to the orbicularis oculi muscle, which contracts to give us the blink. The stimulus is usually any irritant to the lids, the cornea or the conjunctiva. Another vital function of the lids is the production and the drainage of tears which are vital for the health of the eye and the comfort of the eye. There are three main layers to the tear film.

From inner to outer we have the mucus layer, which is supplied by the goblet cells of the conjunctiva, which of course lines the inner aspect of the lids as well as the surface of the eye. Then there’s the watery layer produced by the lacrimal gland primarily, but also by accessory lacrimal

2 http://www.transcriptioncity.co.uk glands of Krause and Wolfring which are located in the superior fornix under the inner side of the upper lid. And the outer layer is the oily layer, which is produced by the meibomian glands located near the eyelid margin as well the glands of Zeis which are associated with the lash follicles. Eyelids are also important for facial expression, so any aberrations of the eyelids, or any asymmetry between the two, has a great impact on cosmesis, and people are very bothered about it. So here is a section through the upper lid showing normal eyelid anatomy. And in simple terms the eyelid is a layered structure. So we’re gonna go from front to back, anterior to posterior, to go through it with you.

So just to point out before I do that, we think of the eyelid also in two main layers, or a front and a back. There is the anterior lamella which is outlined in red, and the posterior lamella, and the junction is the mucocutaneous junction, where the skin finishes and the conjunctiva starts. The first layer is the skin, then we have the orbicularis oculi muscle, followed by the septum. The septum runs from the orbital rim, downwards into the lid, and in the orbital rim it fuses with the periosteum. As it comes down into the lid it thickens to form the tarsal plate. The tarsal plate is imperative for the structure and stability of the lid. Behind this we have the conjunctiva, and as you can see, if I can use the pointer, as you can see here, these are the accessory lacrimal glands that I was talking about, that contribute towards the distribution of tears as well. There are two main elevators to the eyelid.

The first one is the levator palpebrae superioris which is supplied by the third cranial nerve, and the Müller's muscle, which is a smooth muscle, which is under sympathetic control. In the lower lid there are rudimentary equivalents of these, and they are divided into which are just known as the inferior retractors. And they are divided into a voluntary group and a sympathetic group.

Going onto the manifestations of eyelid disease. So first of all eyes that can’t fully close. So as you know, we call this lagophthalmos. There are many reasons why can have lagophthalmos. It can be the status after facial cosmetic surgery, as shown on the top left here. It can be following extensive reconstructive surgery, facial nerve palsy, here, we can see this gentleman has right sided facial nerve palsy and within the cases here of severe proptosis, lid retraction and severe chemosis. This is a case of severe thyroid eye disease. This is a case of lagophthalmos following facial cosmetic surgery, namely upper lid blepharoplasties, which were done elsewhere. As you can see it’s fairly obvious that too much skin has been taken, such that there is a vertical deficiency of

3 http://www.transcriptioncity.co.uk eyelid skin, preventing the eyes from closing. You can see it’s an unhappy patient, and rightly so, because the eyes will be uncomfortable, gritty, sore, watery. So the treatment for this in the first instance is lubricants.

Failing that, it would be to treat the cause, which is to put backs in the skin, with a skin graft to the upper lids. Here we can see it in more detail. This lady presented with a rapidly enlarging mass on the left upper lid. This is her in June 2015, this is her eight days later while she’s just awaiting biopsy on the day of surgery, rather. She had an upper lid melanoma. She subsequently went off to the plastic surgery team, for extensive excision and reconstruction. She then presented about 18 months later, so September last year, complaining of a very sore, gritty, watery eye, not really helped by ocular lubricants. If we look in more detail, she can’t fully close the eye, and this is because she’s missing most of the upper lid, so it’s not surprising really. So she underwent extensive reconstruction of the lid, and we replaced the anterior lamella with skin, and the posterior lamella, so the back layer with a free tarsal graft.

So that’s tarsal tissue from the fellow upper lid, and she’s now able to close her eyes. Lid retraction may be seen in thyroid eye disease, as in this lady, who has inactive thyroid eye disease and proptosis. This is her before her left upper lid lowering, where she has lagophthalmos, and this is her after left upper lid lowering, where she can now close her eyes, and it’s much more comfortable. So, every now and again I’m gonna put up a warning, so things to look out for. This is severe thyroid eye disease. It can be sight threatening and vision threatening. So lagophthalmos, any cause of lagophthalmos can potentially cause loss of sight, due to dryness, subsequent infection, corneal ulceration. This is a lady with thyroid eye disease with proptosis and lid retraction on the left, and this is her a year later, having had bilateral orbital decompression and upper lid lowering. Her eyes are much more comfortable now, and she’s a lot happier.

This is lagophthalmos in facial nerve palsy, where we can see the lagophthalmos here, and this is the staining with fluorescein, which is a lot of uptake showing a very dry eye. So the management largely depends on the cause, however the first port of call is ocular lubricants, followed by, if needed, punctal plugs, which are inserted into the inferior and superior puncta if necessary. We can close the lid with a temporary or permanent tarsorrhaphy here, this is a temporary one. Sometimes Botox can be used to lower the lid. As we know one of the

4 http://www.transcriptioncity.co.uk complications of Botox for cosmetic reasons is indeed inducing ptosis, or a droopy lid. So it can be used therapeutically, and applying an upper lid weight to weigh down the lid.

This is seen here in a lady with right facial nerve palsy, where she has upper lid retraction, because of the unopposed action of the eyelid retractors, because her orbicularis oculi muscle is weak. So she has unopposed action of her levator and her Müller's muscle causing lid retraction, as well as often a loose lower lid or ectropion. She’s had a gold weight inserted into the upper lid, here, giving her a very slight ptosis, but her blink is facilitated, so her eye’s more comfortable now. She no longer has any lagophthalmos. This is an example of a temporary tarsorrhaphy in a patient with facial nerve palsy, who had a secondary corneal ulceration.

Lids that won’t open. So this is a lady with blepharospasm, so this is bilateral involuntary blinking and closure of the eyes. It’s twice as common in females. And it’s usually a central blepharospasm is one of the main causes, but it can be secondary to any cause of ocular surface irritation, so trichiasis, blepharitis, a corneal foreign body. You might see people with a corneal foreign body presenting, and they just really won’t open their eyes, and that’s due to secondary blepharospasm. Treatment of this is really to treat any underlying cause, so if there’s a corneal foreign body, that needs to be removed. If they’ve got trichiasis, the lashes need to be epilated, or have electrolysis, etc. But for essential blepharospasm, botulinum toxin injections into the periocular area and into the facial area can have a 98% chance of success. It is however, as you know, temporary, so usually injections are needed every three months or so. The caution here is hemifacial spasm. So if the spasm is unilateral, you need to really be thinking about, this is usually caused by compressive lesion of the root of the seventh nerve, so scanning is needed here to exclude a tumour.

Inability to open the eyelid is also a feature of essential blepharospasm however it can also be seen in extrapyramidal conditions such as Parkinson’s disease. Here the treatment is botulinum injection specifically to the orbicularis muscle overlying the tarsal plate.

Going onto lid malpositions and specifically focussing on ptosis, ectropions and entropion. So with ptosis, or with any disease, we can think of it in terms of congenital versus acquired. This is a gentleman with congenital ptosis where there is dysgenesis of the levator muscle, and this is treated surgically, if needed, with brow suspension. This is

5 http://www.transcriptioncity.co.uk the gentleman before, so it’s unilateral. Before and after left upper lid brow suspension. So the lid movement is controlled by moving the brow. Sometimes you can see, although not really in this gentleman, some faint little scars just on the forehead. Most cases of ptosis that you’ll see will actually be involutional, so they’re age related due to the slackening of the attachment of the levator muscle to the tarsal plate. As in the case with this gentleman who underwent ptosis correction.

In involutional ptosis what you’ll often see is a very high skin crease, here and here, so it’s a lot higher than normal, and that’s due to the loosening of the attachment between the levator muscle and the skin, as well as the tarsal plate. So these are the causes of Ptosis, so congenital, aponeurotic or involutional, ageing. Sometimes this is due to contact lens wear as well, so in a younger patient, contact lens wear may be a cause of levator dehiscence. Neurogenic causes, myogenic, mechanical, traumatic, and we also to have to think about pseudoptosis, so when there’s ipsilateral enophthalmos for example. Caution. In every case of ptosis it’s imperative that you check the pupils and you check the eye movements. You also should look under the lid. This gentleman presented with a very slight ptosis, and if you look, it’s easy to miss, but if you look, he has a flattening of the eyelid margin. When you look under the lid, this is what we see. He had malignant melanoma, and subsequently underwent extensive excision of this, and I think he went on to have an exenteration of that.

What we need to exclude is these things: Horner’s syndrome, third nerve palsy, myasthenia gravis, a malignancy, and a chronic progressive external ophthalmoplegia or Kearns-Sayre syndrome, which I’ll talk about in a moment. This is a case of Horner’s syndrome. You can see the mild ptosis and you can see the anisocoria with the small pupil here. It’s due to an interruption of the sympathetic supply to the Müller muscle and other areas, anywhere along its route. It’s very important here that these patients have urgent scanning to exclude life-threatening causes. This is a case of third nerve palsy. This lady has a complete right third nerve palsy. She has a complete ptosis. She has a dilated pupil, compared to the other side, and whilst you can see that her abduction, which is lateral rectus muscle supplied by the sixth cranial nerve is working, in all other positions of gaze, she has reduced eye movement.

So this is a third nerve palsy. Myasthenia gravis occurs when there’s fatigability. That’s pretty rare, 1 in 10,000, but ptosis can be the presenting feature in about 70% of cases. This will require medical management. Myopathic ptosis. So I mentioned earlier chronic

6 http://www.transcriptioncity.co.uk progressive external ophthalmoplegia where there’s bilateral slowly progressive reduction of the eye movements with ptosis as seen above. When combined with a heart block this is Kearns-Sayre syndrome, sorry wrong button. As we can see here, the treatment is actually ptosis props. So this is the lady without her glasses, with her glasses, with the ptosis prop in situ. So she’s able to function very well, doesn’t need surgery. The problem with surgery is if you lift the upper lid, because her eyelids won’t roll up, there’s a risk that her corneas will dry out, and she gets exposure keratopathy. So in these cases I would actually encourage ocular lubricants and ptosis props. Maybe a family history, they don’t tend to have double vision, and she needs multi-disciplinary input.

Have a look at this video. [Video playing – Okay, so if you could move your jaw from side to side, that’s it, and then if you move your jaw forward and backward]. There we are, a bit loud, so I thought I’d skip over it. This is a miscellaneous ptosis, it’s Marcus Gunn jaw-winking ptosis. I don’t know whether you’ve ever heard of it? It’s congenital, it’s due to a miswiring, so on chewing when there’s stimulation of the ipsilateral pterygoid muscle, there’s elevation of the lid. So in a patient with ptosis it’s often masked because people often hold their chin in a certain position, particularly for photos, to elevate the lid, to mask it. Sometimes these people require surgery for cosmetic reasons, more than anything. It’s also quite useful to just double check jaw movement and then you’ve got your cause of your ptosis.

This is a lady who presented to eye casualty with ptosis. She’d had, the key thing here is you look how smooth her forehead is, so she’s been having some Botox injections, actually for headache management, and she presented with one of the complications which is ptosis. This is her after I put some Iopidine drops into her right eye. The effects of the ptosis from the Botox will resolve, because it’s a temporary thing, however they might have up to three months of the ptosis, so what we can do is give them Iopidine drops or Apraclonidine is the generic name, and this is a selective a2 agonist, acting on Müller's muscle. And because Müller's muscle is under sympathetic control, and it’s a smooth muscle, that can be stimulated, and is used as a temporary relief for this condition. Some people advocate using Phenylephrine drops, but I’d probably just use Apraclonidine really. In particular, just sit back, have a look at them, and compare the superior sulci.

This gentleman has quite a hollow sulcus, and it’s quite full here, on the side of his ptosis. He has, has it happens, bilateral orbital lymphoma,

7 http://www.transcriptioncity.co.uk which turned out to be rather aggressive, and was treated with radiotherapy. Again, looking at the difference in superior sulci, this lady has quite a full sulcus, normal [unclear 00:18:42] position, hollow sulcus ptosis. If you look from above, which is a nice way to check for proptosis or indeed enophthalmus, this lady is enophthalmic, so she has pseudoptosis.

Ectropion and entropion. Ectropion is out turning of the lid. Entropion is in turning of the lid. There are multiple causes, congenital, age related, scarring and facial nerve palsy, for example, causing paralytic ectropion. You get a watery eye, irritated eye, red eye. Treated with lubricants and surgically. This here is an example of a lesion on a lower lid. It’s a basal cell carcinoma, causing subtle ectropion. And in a bit more detail here, so you can see the basal cell carcinoma. You need to feel with your finger, and if you feel a thickening, think about whether or not there’s a tumour here. This lady had cicatricial ectropion due to eczema. She underwent left lower lid tightening, followed by a skin graft to the lower lid, taken from the supraclavicular fossa, which gives a good colour match.

Looking at entropion now. Entropion, in turning of the lid, tends to affect the lower lid more than the upper lid, because the lower lid is less stable because it has a shorter tarsus. It’s important to treat, because it can be vision-threatening, because it can cause secondary infection and corneal ulceration. Usually due to age-related laxity of the lower lids, but it maybe secondary to scarring of the inner aspect of the eyelid. It may cause secondary blepharospasm. It’s treated in the first instance with ocular lubricants. The temporary measure may be Botox and lower lid tightening. Just looking in a moment towards lumps and bumps. It’s important to look under the lid. This gentleman had a squamous conjunctival papilloma.

Briefly moving onto lid lumps and bumps. A stye, this is an abscess of the lash follicle and it’s associated and of Zeis or Moll, seen towards the bottom of the picture here. Can discharge through the skin. Treatment, hot compresses, oral antibiotics such as co-amoxiclav or flucloxacillin. Here a number of benign lid lesions and abscesses. This is a cyst of Moll, it’s translucent. A cyst of Zeis, opaque. These are benign lesions, they don’t need to be removed. Removal is thought to be cosmetic, and is not paid for currently by the CCG, as I understand it. Similarly, xanthelasma is deemed to be a cosmetic legion these days, and one has to check the cholesterol level, as you know, from people with xanthelasma. Somebody with unilateral non-resolving conjunctivitis,

8 http://www.transcriptioncity.co.uk look at the eyelid margin, see if they’ve got molluscum contagiosum, a double-stranded DNA virus of the pox group. That needs to be removed.

This is a large sebaceous cyst, arising from the eyebrow hairs, and this is a greasy stuck on lesion seborrheic keratosis, again it’s a benign lesion. It can be removed for cosmetic reasons, but that’s the main indication really. An acute chalazion is an abscess of the meibomiam oil glands within the tarsal plate. Hot compresses, oral antibiotics, so co- amoxiclav five day course will help. If not resolving, incision and curettage. You can get more of a chronic lymphogranulomatis inflammation of the meibomiam glands, due to chronic blockage of meibomiam gland, which is also a chalazion, but not an acute one. If it bursts through the tarsal conjunctiva you can get a granuloma, seen here. If persistent, incision and curettage.

Other lumps and bumps, racing through now. Papillomas, benign warty lesions. Again, if they are impeding in the vision there’s an indication for removal, with a shave biopsy, and these are epidermal inclusion cysts. This is a pyogenic granuloma. It’s a highly vascular tissue, and it’s usually, as a benign lesion, it’s usually due to trauma. Often people, I’ve seen a couple in young men, if they’ve been picking, so consistently stirring up this abnormal inflammatory response. It ended up being quite a huge legion and was excised successfully. Malignant legions, keratoacanthoma, usually rapidly grows over a period of 2-4 weeks, followed by involution. It often has a keratin filled crater. It’s now thought to be on the spectrum of squamous cell carcinomas, so they need to be excised. The most common eyelid malignancy is a basal cell carcinoma, more common in people who have had excessive sun exposure and smokers. Lots of different types, and slightly more common in women, but not necessarily.

The most important thing is they’re not all nodular, and that’s where the confusion can come in. They can be cystic, they can be pigmented. They can be superficial, and like here, well a red plaque. Treatment is largely surgical excision, a topical imiquimod can be used. Vismodegib is a fairly new treatment for those people with metastatic disease, or recurrent disease, or those too unwell for surgery. This gentleman had three lesions, which were all excised. If you can see here, this is ever such a small lesion, but if somebody’s got one lesion, they’ve got a very high chance, because they’ve got sun exposed damaged skin, they’re very likely to have other lesions elsewhere. So also look in the hairline, that’s another area often missed. So if you can have a think where the

9 http://www.transcriptioncity.co.uk lesions are, you may well be wrong, because it’s here. Okay, so this is more [unclear 00:25:11] BCC, so it’s [unclear 00:25:14], difficult to spot, sometimes can be thought of as misdiagnosis chronic inflammation of the eyelid margin.

Here it’s quite difficult to point out, but he’s actually got a loss of lashes and there is a slight change in the lid architecture and it’s thickened skin along there with a mild ectropion. If you look at what he went onto have. If you look at the extent of the excision that he had, you can see how [unclear 00:25:36] it is, and it’s often larger than clinically apparent. This is him after a reconstructive surgery. Squamous cell carcinomas, they can also occur in Asian people, and people of colour, it’s not all just Caucasian patients. This gentleman had right lateral canthal squamous cell carcinoma proven on punch biopsy. This is the extent of the excision. It’s fully excised, and this is him perioperatively, just at the end of his operation following reconstruction. So, which one’s the melanoma? I’ll give you the answer, it’s this one. This one is a pigmented BCC. It’s very easy to get into the mind-set that all BCCs look the same and their nodular. They’re not, and they can be cystic, they can pigmented.

If there’s a change, or if there’s change in mid-architecture, it’s growing, it’s bleeding, any pigmented legion along these lines, one needs to biopsy it. This is a lady who complained of a little bit of pigmentation on the right upper lid, seen a little bit here, it’s very faint. On eversion of the lid you can see she’s got a malignant melanoma. Malignant melanoma’s pretty rare around the eyelid, less than 1% but they can have various types. It’s important to look under the lid if you can. Other important lesions are Kaposi sarcoma, which we are not seeing so often now, but more often seen in patients with AIDS, as you know. Treatment’s radiotherapy, the disease is not curative, and Merkel cell carcinoma is a rapidly growing lesion, usually in elderly women, it’s a purple nodule. Again, anything rapidly growing, needs excision.

Onto the last few slides. Beware of the first chalazion in anybody over 40, okay. That’s the take home message really. One of the differential diagnoses is actually sebaceous gland carcinoma, a very aggressive, 10% overall mortality rate, and 67% five year mortality rate if metastasis. Beware the chalazion again in people over 40, this lady, she’s in her 70’s, was treated for several weeks with a left upper lid chalazion. On biopsy, she had a lymphoma. She’s done very well with treatment actually, she’s had some rituximab chemotherapy, and it’s resolved. When you evert the lid, it’s not always a chalazion or a granuloma,

10 http://www.transcriptioncity.co.uk especially in an elderly gentleman. You have to really think about malignancies, rather than going for the diagnosis of chalazion. Again this is a lymphoma.

Brief run through for eyelid swellings. Acute dacrocystitis due to nasolacrimal duct obstruction. Treat with antibiotics and they need DCR operation. Preseptal cellulitis is inflammation, infection in front of the orbital septum. The key things here are that the eye is white. You can move your eye, there is no visual compromise. There is no pain on eye movement. Compared to orbital cellulitis, where you have all of those features. Proptosis, pain on eye movement, red eye, reduced vision, may have pupil abnormalities, so relative afferent pupillary defect. You need to gently, if you can, prize the eyelids open, and have a look. They need to be admitted for IV antibiotics.

So in summary, we’ve talked about eyes that won’t fully close, eyes that won’t fully open, eyelid malpositions and eyelid lumps and bumps. As you can see, on the whole, a lot of things that you see are benign. Mostly they will be, but you need to be aware of the warning signs. So a few key pointers, with ptosis, check the pupils and eye movements. If they’re abnormal they need urgent referral. For chalazia, watch out for the first chalazion in anybody over 40. For lid lesions, this is very subtle, can easily be missed, but look out for a loss of lashes, and a change in the lid architecture. So hopefully, we’ve gone down a downhill route of looking at all the possible lid diseases, avoided some pitfalls, and hopefully our patients will be managed successfully and safely. Thank you. [Clapping].

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