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Late Presentation Eye Disease

2 CPD in Australia | 1CD in New Zealand | 1 July 2018

By Esther Euripidou

Sometimes, a patient will present with an eye disease or subnormal vision at a stage when we have limited capacity to help them. Often when this is the case, there is an overlay of patient anxiety which may compromise optimal care and outcomes. The way in which you initiate a management plan and counsel the patient can help alleviate anxiety and encourage treatment compliance.

LEARNING OBJECTIVES

1. Understand when mind states, such as anxiety and depression, are overlayed on eye disease.
2. Develop examination strategies to work through mental barriers.
3. Develop communication skills to discuss treatment options with complex patients and successfully manage the referral process when necessary.
4. Know where to find further information to assist patients with late presentation eye disease.

As optometrists, we often encounter patients with diverse ethnicities, cultural beliefs, approaches to medicine, education and parenting. The reasons one patient may choose to visit an optometrist will differ from another and may not correlate with our own clinical expectations or understanding. Patients may choose to disbelieve or deny symptoms and they may struggle to understand a diagnosis. It’s understandable then that the reasons for late presentation can be many, from complete unawareness to insurmountable fear.

Some examples of late stage eye presentations I have seen are:

  • Cataracts at the hyper mature stage
  • Keratoconus
  • Retinal degeneration (e.g. maculopathy)
  • Glaucoma – hemifield or significant central loss
  • Pterygia with aggressive invasion of fibrovascular tissue into the cornea
  • Ptosis
  • Optic atrophy and brain disease
  • Embedded inert foreign body without patient sensitivity
  • High refractive error from compound issues.

BARRIERS TO PATIENT UNDERSTANDING

It is important to understand your patient’s perception of their vision loss and state of mind before you begin working on a management strategy. Are they anxious? Are they forming their views based on non-scientific information handed to them by family or picked up on the streets?

One would think that self preservation is a primal instinct, however the human mind is complex and sometimes a patient’s perception of their ocular condition will be ‘clouded’ by their state of mind. If a patient suffers from anxiety, they may resist the need to present at your clinic. Even after they have been diagnosed, they may be reluctant to comply with treatment, resulting in a cycle of further visual deterioration and increasing burden. Encouraging a positive frame of mind is important for vision preservation.

Traditionally held, non-scientific beliefs, passed down through generations can be major obstacles to making an appointment to see an optometrist. As an example, a pre-teenage myopic boy visited my clinic with his older sister. He was experiencing deteriorating vision, which he believed was caused by his private bedroom activity. With <6/60 acuity, his ability to learn at school had been compromised, however such were his feelings of guilt and fear that he hadn’t complained to his parents and had been reluctant to seek help until his life was significantly impacted. He was found to have quickly progressing myopia.

Other mental barriers to seeking optometric assistance that I have witnessed1 include:

“It runs in the family” – due to a family history of a particular ocular condition, the children are expected to ‘suffer’ with the same infliction, and for this reason the parent does not feel it warrants concern. For example, two highly myopic parents believed their child would most certainly be myopic and therefore, the child should manage for as long as possible without spectacles.

“I’m too old, I’m going to die soon” – I have seen many people who believe vision loss is a normal part of the ageing process and therefore, it should not cause alarm, anxiety, or necessitate intervention.

“It’s my own fault” – some people believe an ocular injury, through contact with an inappropriate substance, e.g. fresh lemon juice, baking soda, contaminated or expired eye drops, or an herbal teabag – is self-inflicted and therefore doesn’t warrant professional intervention.

“It can’t be helped” – the belief that keratoconus and advanced trichotillomania, caused by repetitive eye rubbing which results in corneal trauma and re-current inflammation of the eye lid/s just must be put up with because it is part and parcel of the condition.

“I’m on drugs, the doctor said I would get bad vision” – an acceptance of visual deterioration due to certain medications or recreational drugs.

“I was a smoker for 30 years, I deserve it” – the view that because the person engaged in an unhealthy activity, they deserve to be punished.

“I thought it would go away, so I just waited, until it didn’t” – a lack of awareness or refusal to acknowledge the likely deterioration of particular eye diseases, such as retinal detachment/macula off, can be prevented by taking action.

“I make Jai wear glasses all the time, especially when he is using an iPad, so the eyes can remedy” – a presbyopic parent who does not comprehend strategies for myopia control with distance only (low minus
power) spectacles.

In forming your opinion of your patient’s perception and state of mind, take careful note of their comments during your consultation, particularly if they are completely inconsistent with your clinical findings. These can be used in summating evidence as part of the referral process. Additionally, individual personality traits and beliefs should be observed. Note in particular; stress factors/the presence of another significant individual and their concerns/communication limitations/regressing behaviours.

Taking down the name and contact details of the patient’s ‘significant other’ person can be helpful if you experience difficulty in communication or in the referral process. Privacy laws need to be respected when managing patients. It is important to clearly understand the patient’s relationship of the significant other person and to take an evidence based approach, with documentation to support your evaluation of a patient with impaired capacity (whether temporary or fluctuating).

Denial Behaviour

Denial is the first stage of the coping cycle in disease or illness.2 Behaviours observed in denial are:

  • Non-acceptance/disbelief
  • Minimisation of signs or symptoms
  • Dissociation/escape/distraction from oneself
  • Signs of internal conflict/distress.

There can also be elements of:

  • Blame-shifting
  • Justifying a previous choice based on perception
  • Regressive behaviour
  • Avoiding a sense of guilt or responsibility for a situation.

Consider these statements in retrospect to the above:

“I’m only 52, I’m too young to have cataract”

“But I can drive perfectly well in the day time and have not had any accidents”

“I’m too busy with my work to get time off to have cataract surgery. I’ll do it when I retire in five years”.

“I’ll do my prostate this year and my macula injection next year”

“When I was young, I fell over and it was most likely that when I hit my head I caused some damage to my vision”

“The computer made my vision deteriorate”

“I didn’t wear sunglasses until I got a skin cancer on the side of my nose”

“I can’t have cataracts, I’ve been using Bright Eyes cataract drops that prevent them from forming”.

“You mean I’m going blind, I don’t want to know if I’m going blind.”

“You didn’t hear properly, I just want R-E-A-D-I-N-G glasses”.

“The last optometrist called it mild and said I wouldn’t need surgery just yet”. (Perhaps misinterpreting “just yet”).

When patients in the denial phase are unwilling to consider, or even accept referral, there will be negative implications for both patient and practitioner. As a practitioner, it is important to be aware of that legally, you are obliged to follow up and ensure every reasonable attempt is made to address the patient’s vision loss, with telephone communications, SMS alerts and copies of mailings clearly documented on patient files.

Some patients may suffer from an associated denial condition called anosognosia.3,4 This means they have a genuine inability to recognise that a problem exists. I have experienced two presentations of patients with this complete lack of self awareness. Both required urgent surgery which made their management challenging.

EXAMPLES OF LATE PRESENTATIONS

Spectrum Disorders

Patients at the far end of the abnormal behaviours spectrum can also present with challenging behaviours. In one clinical case, I experienced a patient whose obsessive compulsive disorder manifested as ocular mysophobia. The patient took extreme measures to protect her eyes following cataract surgery, with rituals that included removing her lampshade hat only once she had moved from the waiting room into the consulting room, wearing large wrap sunglasses (even indoors), packing her eye protection items very systematically and even avoiding eye contact. While in my clinic, she refused to put her chin on the chin rest of the microscope as she was fearful of a bacterial infection occurring during a slit lamp eye examination. Despite explaining that during the procedure there would be no physical eye contact, using paper chin rest liners and cleaning down the equipment thoroughly with alcohol wipes, she remained unable to approach the slit lamp for examination.

Conversion disorder is a diagnostic category applied to patients who present with neurological symptoms such as blindness without an organic cause3 (previously known as hysterical blindness). A psychological trigger, such as stress is often the cause. I experienced a referral from Red Cross, of a female patient in her 20s, who presented with a constant state of blur. Repeated measurements of visual acuity, accommodation and visual fields were inconsistent. Her concentration to tasks was poor, and on her first visit, her only disclosure as a possible cause was a lack of sleep. However, on her second visit, the patient disclosed her personal stress issue: she was denied access to her three young children through a court order.

Where anxiety is suspected on a presentation the patient declines a referral to a GP, it may be wise to guide the patient to services such as the Black Dog Institute website, which has links for anxiety self testing and information on how to seek help (www.blackdoginstitute.org.au).

Complete Blindness in One Eye

Complete blindness in one eye, without awareness, is most commonly seen in elderly patients, particularly among those with declining mental function, which limits self analysis. Even simple occlusion for measuring monocular acuity can become quite confusing for these patients and produce confounding results. Hearing impairment, with a need to lip read, can exacerbate the situation, causing the patient to resist occlusion.

Gross time hesitation and even annoyance to occlusion is characteristic among people with low vision. At the start of the test, demonstrate how an occluder works. This is best done with full parallel posture to the patient and by demonstrating how the occluder sits over the eye on your own face (as the examiner). Elasticised occluders are not recommended at early stages of the eye examination as the patient needs to
establish trust.

Stagnation and confusion is best avoided with simple instructions and by nominating appropriate targets in the first instance. A simple ‘one or two’ choice works well with appropriate and clever target selection. Additionally, the patient may ask for a validation score for each letter read, simply because they want to know if they are correct on each target.

In sensitive patients, trial frames, prism bars and our traditional tools (e.g. retinoscope) can be an invasion of personal space. Care must be taken so as not to upset the patient – a simple trigger can set up further barriers to good communication.

USEFUL CLINICAL STRATEGIES

Accuracy of Measurement

The initial presenting visual acuity test can be set up as a test for more than simple acuity. Alternate occlusion gives clues to eye muscle imbalance with any reported target movement. The actual time to focus gives insight on how sharp or slow muscles are working. Compensating head movements and other strategies to reach a target acuity will also offer valuable clues. Taking control of the occluder is pertinent to establishing a true score – patients who self-occlude can manipulate the test score by controlling the test time.

Visual acuity can be scored using two modes:

  1. Atonic visual acuity – rest/relaxed mode (three second maximum time allowance and the patient is asked to keep their focussing ‘relaxed’)
 

2. Best visual acuity (separately squinting/ forced muscle mode with time allowance).

I have observed a five line difference in VA line of letters and up to two dioptres by taking these eye postural differences into account.

Shifting eye position with alternate eye occlusion also helps to analyse extra ocular muscle imbalance at distance. Always instruct the patient to keep the eye that is under the occluder open and relaxed and maintain adequate distance (occluder to eye socket) so as not to make contact.

Significant Visual Field Loss-Detection on initial visual acuity measurement

Vertical line presentations from projector images are excellent targets for preliminary analysis of later stage glaucoma, maculopathy and other central visual axis pathology. Using for example, a projector image of the five letter 6/12 acuity line as a solid bar of light with five capital letters as a test target, provides excellent opportunity for analysis. Observing immediate reactions and comments regarding the test eye precisely after the immediate occlusion of the other eye gives a baseline on relative eye score.

Maculopathy will produce much head bobbing, and vertical adjustment posture to help find targets. A larger area of disturbance, manifest over longer time, will produce an eccentric fixation preference. Head movement compensation – immediate directionality shift – can give clues to the corresponding area of the eye affected. It can be abrupt to the point where head constantly shifts up and down for clarity on targets. This is static perimetry in its basic form, investigating the horizontal raphe. Clever selection of target presentation is required for suspected eye conditions.

CATARACT CASE STUDY

While working in an affluent Sydney suburb, a family of patients decided to bring their grandmother to see me. They wanted to know whether there was anything I could do to assist in her vision.

The slightly milky pupils greeting me suggested cataract. The woman walked assisted by family, and having never experienced a vision test, could not understand the concept of a letter chart. The family was proud of their grandmother – despite being blind, she was still able to complete her ironing tasks to perfection and had been more than capable, over many years, of managing the whole family’s laundry. She was a very tactile person.

Direct ophthalmoscopy showed a dense central shadow; nuclear sclerosis, identical right and left. Vision was less than count fingers at 30cm.

Despite the woman having hyper mature cataracts, surgery remained an option.

Figure 1. Capsulorhexus being performed in a hyper mature cataract with tryptan blue stained anterior capsule. Courtesy Dr. Amar Argarwal5

Hyper mature cataracts carry greater complication risk due to increased time required for phacoemulsification, which increases strain to the open eye.5 However, the need for surgery is gauged more on what is happening with the other eye. Benefits and risks can weigh more heavily if the other eye is non-functional or low vision. Is there a risk of complete blindness? Glare sensitivity and poor contrast caused by cataract can be debilitating. Decreased perception, particularly with driving and perceived risk of accident/fall, are predictors of a need for intervention.

I discussed options for cataract surgery with the grandmother and her family, and its benefits should she become able to see.

“But can’t you see, she is happy?” was the response. I could not deny that the woman was genuinely happy and as proud as a grandmother can be. However, the new diagnosis gave a label for her sight impediment, and after prolonged discussion, the woman’s main carer/daughter agreed to discuss the prospects of cataract surgery with her mother so that the referral process could be initiated.

Despite numerous phone calls, I was unable to contact the family again. Ultimately, if a patient is unable to dictate their own pathway, the carers are ultimately responsible. This does make me concerned about the potential for elder neglect.

SECONDARY GLAUCOMA RISK IN HYERMATURE CATARACT

Patients who are resistant to conventional therapy and decline referral for cataract management on more than one occasion and with one or more practitioner need to be warned about the risk of secondary glaucoma:

1. Phacolytic glaucoma, which presents with extreme pain and in a spontaneous nature6

2. Phacomorphic glaucoma, which results in angle closure from lens swelling (causing pain and haze but less acute than phacolytic glaucoma).6

Dense cataracts are difficult to manage and should be referred to an ophthalmologist for further counselling and detailed observations. Cataract surgeons are more suitably qualified to make the call of “it’s too late” but this is rarely the case.

REFRACTIVE CHANGES

New Myopia

A gauge of high myopia can be established from the best visual acuity score on a small reading card. I use a near card (resembling a mobile phone in size) with coarse targets in the first instance and use the inverse  formula relationship for dioptric conversion; best fixation distance 33cm: three dioptres of myopia; 25cm: four dioptres; 20cm: five dioptres; and 10cm: 10 dioptres. Comparisons between eyes are useful to ascertain the similar or varying degrees of myopia. Trial frames can give some people anxiety and limit tolerance time for accurate retinoscopy. Giving these patients breaks from the refractor head can avoid them feeling ‘closed in’, during the refraction.

New Hypermetropia

Diabetic control, in the form of metformin, has been documented to cause a hypermetropic shift of up to four dioptres. In the first four weeks of high dose treatment, refractive shifts are common and can play havoc with the mindset of the newly diagnosed diabetic.7

EXTERNAL EYE CONDITIONS

Eyebrows, Eyelid Skin and Ptosis Observing skin folding around the brow line – particularly the brow frontalis and corrugator supercilli muscle – will help you understand compensatory muscle assistance.8 The furrows in the brow line will be deeper or more prominent in the dominant eye. The depth of the furrows can give some insight into the duration of forced muscle compensation, such as in glare combat with cataract. Smaller vertical aperture size can also indicate the more active eye.

Figure 2. U shape weld to metal frame as ptosis crutch. Photo courtesy of Framecare, Australia10

In late stage upper lid ptosis, horizontal furrows across the forehead can occur as part of the compensatory response. The head may tilt backwards, and a reclined posture will help for visual tasks. Due to this being a longstanding adaptation, the patient may not be aware of their ‘chin up’ posture.

Cosmetic Botox injection can also induce partial paralysis of the assisting muscle groups causing old spectacles to suddenly feel uncomfortable and/or a sense of vision loss. Patients may decline admitting to Botox treatment or may only disclose this essential history when probed later in the analysis. History is best revisited with highly specific questions. Asking these questions during testing will also allow patients time to relax their eyes between vision tasks.

Another skin condition which can be analysed in an optometrist’s work up is the degree of slackness in the eye lid skin. Accordion style folding, and the appearance of excess skin should be analysed as part of floppy eye lid syndrome. This condition can be associated with keratoconus, diabetes, mental retardation and obstructive sleep apnoea,9 which may have previously been undiagnosed.

In sleep apnoea disorder, recurrent subconjunctival haemorrhages can be caused by a pressure balance problem from the tightness of the sleep mask seal. Internet chat rooms relating to sleep apnoea commonly discuss how to avoid these ‘blood clots’ in the eye. They are not true blood clots but rather micro-bleeds under the conjunctival layer that produce a characteristic flame shape. Referral to an ophthalmologist is recommended if there is recurrence at the same site.

Upper lid ptosis may occur in myasthenia gravis, as a result of failing surgeries or facial deformity, and as part of the general aging process. A ptosis crutch can be fitted to traditional spectacles and can dramatically improve quality of life. The ptosis crutch, listed as ‘Spectacle repairsptosis bar’ on the Framecare website, can be fitted through Framecare in Brisbane.10

Blink rate is a very common prompt for an eye test. After excluding dry eye, blink rate needs to be assessed as part of a psychogenic myoclonus when all other eye and vision related causes have been eliminated. Blink rate can return to normal when there is distraction and/or non-attention to the presentation. The blink rate may be more of a concern for the carer/parent rather than the patient. This must be clearly differentiated from spasmodic tic or twitch associated with hemifacial spasm.

Pingueculae and Pterygia

Late stage and recurring pterygia are difficult to treat. Successful surgery usually requires an autograft and long healing time. Outdoor workers are most at risk, particularly farmers, tradespersons and even outdoor café workers. Recurrence after treatment is quite common. The Australian Pterygium Centre has pioneered autograft surgery and has extensive pictures, information and resources on their website.10

Scarring of Eye Tissue with Tattoo Procedure

When eyelid tattooing goes wrong, the complications can be significant. I had a patient present to me with eyelid tattooing of the whole inner lid margin. The person, who had decided to get the tattoo on a whim while on holiday, had been injured through the tattoo process, causing posterior blepharitis. The tattoo line was very specific to the inner lid margin and had caused complete scarring of the lower eyelid margin. The lower eyelids were markedly thickened.

Figure 3. A case of pterygium. Photo courtesy of Professor Lawrence Hurst, pioneer of P.E.R.F.E.C.T. for pterygium surgery.

The patient had experienced significant dry eye symptoms (at age 35), and scar tissue further exacerbated the condition by disrupting the meibomian gland function.

OPTOMETRIST AS COUNSELLOR

Any discussions with patients need to be at a level the patient can grasp key information and with detail the patient can understand. Textbook terminology may not be comprehensible, particularly by patients with late presentation eye disease. Keep language as simple as possible and direct your conversation to the patient rather than the support person or interpreter. Encourage and allow adequate time for questions and offer additional follow up resources. Printed resource material can be mailed if there is inadequate time during the consultation.

A communication pathway via carer/relative needs to be established if there are decisions to be made or you anticipate further engagement with the patient. You may need time to work out what type of referral is suitable or even manageable before concluding on a plan of action.

Patients often need time to process information about their condition and take steps to change. Comanaged care, with a general practitioner, will be essential for challenging cases and for patients with a depressive illness.

Low Vision Referral

The opportunity to generate patient interest in vision aids and mobility training may be within a narrow window of time. This makes your decisiveness critical. Up-to-date knowledge on what is available to help low vision patients should be conveyed as a pathway rather than a choice to make. Constant review of low vision resource providers is important so that you understand what is available and how to go about recommending them.

While declining cognitive function may render sophisticated low vision aids useless, patients who can grasp technology should be encouraged to use it. iPad and notebook style computers are ideal for demonstrating the ‘stretch magnification mode’ function for reading small print.

Figure 4. EZSee wireless large print keyboard. Photo courtesy of Vision Australia12

Vision Australia is well equipped to assist people with low vision, however to get on board with the referral, your patient will need to have a clear understanding of why you wish to make the referral, the types of services Vision Australia can offer and the potential benefits for the patient.

I have had patients decline the service as I was unable to convince them of their worthiness to receive the service or of the benefits they would gain from even low budget aids and technologies. Sadly, a negative mind set cannot interpret the real value of low vision aids, even when tailored to their needs.

A declined Vision Australia referral is a disappointing result, especially when you have invested time in establishing trust. It can also be confronting to find a patient has lost the will to move forward with their disability and there are compounding psychosomatic manifestations of depressive illness.

Communication to the General Practitioner

Keeping your patient’s GP informed about any ocular conditions is important. Your report to the GP can include critical vision measurements and possibly, some feedback on patient alertness and understanding, their capacity to function independently with daily living tasks and associated risk assessment on falls based on any visual field deficit.

CONCLUSION

This article is intended to encourage optometrists to reflect on their practice in terms of goals and strategies for atypical case presentations. On occasion, we must take a few steps back and rethink our approach to examination. It is vital that we really understand the patient’s mind frame before working out a tangible solution to their visual dilemmas. Late presentation eye disease can be mentally challenging and confrontational. As optometrists we hope these people will seek our professional advice before consulting chatrooms as they can be given erroneous information.

Human Services, Australia was contacted for ‘inverse statistics’13 – that is the percentage of people who have not had their eyes tested in a ten-year period, five-year period, male versus female who have not had a billed eye test, remoteness/rural versus urban dwellers and any other statistic that encompasses non-presentation for an eye test. Their response was, “The Department of Human Services administer and pay Medicare benefits, we do not capture information on when a test has not been claimed”. The volume of undiagnosed pathology is unknown, and optometrists rely on screening programs to reach patients with compromised vision. Prompting existing patients with reminder messages is important in maintaining communication and care.

          Esther Euripidou achieved her Bachelor of Optometry Honours in 1987. A clinical optometrist with 30 years in private practice at various eastern Sydney locations, she is particularly interested in developing strategies to deal with anxious patients. 

 

References
1. Case studies -patients presented to independent optometry practice- inner Sydney
2. Denial. (n.d.) Wikipedia definition retrieved 15/02/2018, from https://en.wikipedia.org/wiki/denial
3. Lexicon of Psychiatry, Neurology and the neurosciences by Frank J. Ayd, Jr Lippincott, Whilliams and Wilkins, Second Edition. - Page 657
4. Anosognosia, The most devastating symptom of mental illness. www.mentalmeds.org/articles/anognosia.html
5. www.ophthalmologymanagement.com/issues/2016/asurgical-challenge-hypermature cataracts
6. Canadian Medical Association Journal. Glaucoma due to Hypermature Cataracts. Marvin. L. Kwitko. M.D. p569
7. Arch Ophthalmology 2011. Jan 129 (1) 56-62. Refraction in adults with diabetes. Barbara Klein, Kristine
Lee and Ronald Klein
8.Superior Procerus muscle. Muscles of the Face-mimetical muscles- Medical Art Library.  https://www.medicalartlibrary.com/face-muscles
9. Kanski.J & Bowling.B. Clinical Ophthalmology – A Systematic Approach. Seventh Edition. page 50.
10. Ptosis crutch. https://framecareaustralia.com.au/frame-repairs/ptosis-bars
11. www.univmed.org/wp-content/uploads/2011/02/nurbuanto.pdf-pterygia
12. https://shop.visionaustralia.org EZsee wireless large print keyboard.
13. www.humanservices.gov.au (Webstats) (Primary Health Network) (MBS online)