2019 RANZCO Congress Celebrating 50 Years of Eye Care

This year’s annual Royal Australian and New Zealand College of Ophthalmology’s (RANZCO) congress was a special one for many reasons. Firstly, it marked 50 years since the College’s inception. RANZCO was formed in 1969 taking over from the Ophthalmological Society of Australia and the congress served as a wonderful platform to pay tribute to extraordinary voluntary contributions from many ophthalmologists in establishing our college as one of the peak bodies representing eye care across the Asia Pacific region.

Without question, the post-operative recovery period following a routine cataract operation is just as important as the intraocular technique

Dr Ravi Singh

Secondly, RANZCO launched our Reconciliation Action Plan (the RANZCO RAP) after wide consultation with Fellows, the Aboriginal and Torres Strait Islander communities and Reconciliation Australia. The aim of the RANZCO RAP is to strive for “equal health outcomes and equitable opportunities for Aboriginal Torres Strait Islander Peoples…(which can be) achieved through the principles of mutual respect and self-determination”. The RAP was launched by Nova Peris who gave a moving account of her own experiences growing up as an Indigenous Australian and those of her mother and grandmother. RANZCO CEO David Andrews said “the launch of the Plan may be the end of the planning process but more importantly it is just the start of action”.

Thirdly, the congress was an auspicious occasion as the College Medal was awarded for the first time to a female ophthalmologist – Dr Anne Brooks. Dr Brooks is a Melbourne based ophthalmologist and well deserving recipient of the College Medal for outstanding service to the College. She has been involved in teaching registrars in the three special clinics at the Royal Victorian Eye and Ear Hospital, was a founding member of the Australian and New Zealand Glaucoma Society, and has over 100 publications in medical literature. As someone who was the beneficiary of Dr Brooks’ tireless teaching during my registrar training, I am delighted for Dr Brooks on this achievement.

Finally, the congress was combined with the AAPOS (American Academy for Paediatric Ophthalmology and Strabismus) and APSPOS (Asia-Pacific Strabismus and Paediatric Ophthalmology Society) annual meetings – the International Convention Centre in Sydney was awash for a week with ophthalmologists from all around the globe! The following provides a summary of just some of the meeting highlights.

NEURO-OPHTHALMOLOGY

The Neuro-ophthalmic Emergencies Symposium, chaired by Associate Professors Celia Chen and Clare Fraser, was a fantastic update on how to systematically approach the patient with disc swelling. Associate Professor Helen Danesh-Meyer gave a great talk on Unilateral disc swelling: the good, the bad and the down-right evil, beginning by emphasising that the optic nerve has only two ways it can express itself. It can turn pale (either by cupping or atrophying) or it can swell. With that basic principle in mind, she then highlighted the different types of unilateral optic nerve swelling. The only type of “good” optic nerve swelling is that associated with optic nerve head drusen – and we luckily work in an era where ancillary testing can help us distinguish this. Autofluorescence (drusen are hyperautofluorescent), B scan (showing the calcification) and enhanced depth optical coherence tomography (OCT) all help make this diagnosis. When working out the cause of “bad” types of unilateral disc swelling, she advised to work out if there are any other abnormalities in addition to the disc swelling – so called “swelling plus”. Such abnormalities could be on history (for example, clinical symptoms of giant cell or recent rashes, joint symptoms or fevers), other ocular findings (uveitis, retinal haemorrhages or cotton wool spots) and finally, neurological features (such as cranial nerve abnormalities). These “plus” features direct our ancillary testing. For example, if there is disc swelling plus uveitis, we are likely to want to send off bloods to rule out causes such as sarcoidosis, TB or syphilis. Conversely, disc swelling plus neurological signs leads to neuro-imaging.

In the absence of “plus” signs, age is very important in determining the cause of unilateral disc swelling. If over the age of 50, the differential remains between non-arteritic anterior ischaemic optic neuropathy (NA-AION) and arteritic anterior ischaemic optic neuropathy (AAION). The management and outcome for patients are entirely different depending on distinguishing between the two conditions. A/Prof Danesh-Meyer touched upon the FDA recommendations to patients regarding PDE5 inhibitors (such as Viagra) in the context of NA-AION. If patients have had previous NA-AION, we are obliged to discuss the potential increase of recurrence with taking PDE5 inhibitors. In patients who have not had an episode but have a “disc at risk” plus diabetes or hypertension, informed consent is required for the risk of NA-AION.

Finally, she turned to “evil unilateral disc swelling” and focused much of her talk on recent advances in our understanding of optic neuritis. Traditionally we have understood this to be unilateral visual loss in patients under the age of 50, associated with pain on eye movement, reduced colour vision and a swollen optic nerve. The Optic Neuritis Treatment Trial (ONTT) showed there was no advantage in giving IV steroids to improve vision in these patients – however those treated with steroids had a quicker visual rehabilitation than those treated with placebo. Follow up studies have shown the risk of multiple sclerosis in the optic neuritis group to be 50% at 15 years but this is modified depending on the presence or absence of white matter lesions on MRI. While this information is not new, A/Prof Danesh- Meyer referred to the “new kids on the block” as two conditions – neuromyelitis optica (NMO) and anti-MOG syndrome.

Without question, the post-operative recovery period following a routine cataract operation is just as important as the intraocular technique

She acknowledged that “both may on first glance be almost indistinguishable from ‘typical’ optic neuritis unless differences are specifically looked for”. The reason it is important to distinguish them is the visual prognosis is overall poor and treatment with steroids must be initiated promptly to optimise outcomes. NMO is thought to be an auto-immune condition with circulating antibodies to Aquaporin-4 which is located in blood vessels and on astrocyte foot processes. Patients present with optic neuritis, transverse myelitis and area postrema syndrome (for example, intractable hiccoughs or nausea and vomiting). After initial therapy with intravenous corticosteroids, these patients require long term immunosuppression to control their disease.

Anti-MOG syndrome, on the other hand, presents with bilateral optic neuritis in 50% of patients, often with profound disc oedema and visual loss. This condition is very sensitive to steroids but often recurs once the steroids are weaned.

RETINA

We were very fortunate to have Dr Emily Chew from the National Eye Institute in the USA to give several talks over the course of the congress. Dr Chew is a world leader in retina research and her work includes the AREDS1 and AREDS2 studies, the DRCR.net studies and the MacTel study. We were delighted to hear her give the 2019 Retina Update Lecture on Macula Telangiectasia Type 2: and update of the Mac Tel Research Group: a 15 year journey from an exam to global connections. Macula telangiectasia type 2 is a rare condition affecting men and women equally with a prevalence of approximately 0.1%. Patients present early in the disease with mild blurring of vision and metamorphosia. Clinically, they have pigmentary disturbance at the macula and temporal macula telangiectatic vessels. OCT and fundus autofluorescence (FAF) are crucial in making the diagnosis, with intra-retinal cavities and ellipsoid zone disruption allowing it to be distinguished from more typical age-related macular degeneration (AMD) changes. The MacTel project is an international consortium of ophthalmologists and scientists that aims to find a treatment for the disease. In part, the success of the project is thanks to generous support from the Lowy Foundation. The Centre for Eye Research Australia (CERA) and the Save Sight Institute are two of the bodies involved in the current treatment trial of Renexus (NT-501) which is a ciliary neurotrophic factor (CNTF) that aims to slow photoreceptor cell death. The rationale for using CNTF was highlighted by Dr Chew in many animal models, including one in which a mouse with ‘MacTel-like’ disease was given a neurotrophic growth factor which resulted in a functional and anatomical rescue for the retina. The Renexus implant is inserted into the vitreous via a small incision at the pars plana and then anchored to the sclera with a fixation suture. It uses encapsulated cell technology (ECT) to slowly extrude the CNTF over many years. Dr Chew presented the results of the Phase 2 study, in which there were a number of Australian participants. The study had the primary endpoint of change from baseline in the ellipsoid zone loss as measured by SD-OCT 24 months after insertion of the implant. Secondary endpoints included change in retinal sensitivity as measured by microperimetry, change in best corrected visual acuity (BCVA) and change in reading speed. Sixty-seven patients (99 study eyes) were randomised – 48 received the CNTF implant and 51 received sham. There was a statistically significant difference between the CNTF arm and the sham arm in terms of the change in ellipsoid zone (EZ) loss with the actively treated eyes showing less loss. In addition, the change in the EZ loss was highly correlated with change in retinal sensitivity – those patients with better EZ on OCT also had better retinal sensitivity. The conclusion from the Phase 2 study was that the NT501 delivering CNTF has a beneficial effect by reducing the progressive loss of photoreceptors compared to sham treated eyes. We are now involved in the Phase 3 studies which once again is an international effort which has thus far enrolled 224 patients, including many from Australia.

GLAUCOMA

The final day of the congress brought the Plenary Symposium on normal tension glaucoma (NTG) – an update on current management and future directions. The symposium highlighted that NTG rather than being the “rare cousin” of primary open angle glaucoma (POAG), is actually far more common than once thought. Additionally, there are several risk factors for NTG such as migraine, nocturnal hypotension and obstructive sleep apnoea that should be teased out of the patient’s history when considering the diagnosis. NTG poses difficulties in terms of deciding who needs treatment and when and what the target pressure needs to be, given the already low measurements. In making the decision to treat, we always need to weigh up the risk and consequence of progression with the potential effects of the treatment. Dr Mitchell Lawler gave a great summary of the contribution of blood pressure to the risk of NTG progression. Studies have shown that reduced nocturnal blood pressure, and the duration of decreased mean arterial pressure over night, play an important role in NTG progression. The difficulty remains that many patients who have NTG are also on systemic anti-hypertensive treatment. When addressing this issue, Dr Lawler encouraged us to determine if they are on this for primary prevention (stopping a potential cardiovascular event) or secondary prevention (to prevent another event). Secondary prevention remains an unequivocal reason to continue antihypertensives. In some cases, and with careful discussion with a patient’s GP or cardiologist, it may be appropriate to stop these medications if the patient is on them for primary reasons and if the ophthalmologist can demonstrate significant progression despite active treatment. He also discussed that topical beta-blockers may not be the drug of choice, or even a second line in NTG patients, given they can contribute to lower diastolic blood pressure over night and overall reduction in blood pressure throughout the day. Finally, he turned to the surgical management of these patients which he described as “walking the IOP tightrope”. With already low IOPs to start with, surgical management must lower the IOP sufficiently to stop progression without resulting in hypotony – this can be extraordinarily difficult if the patient is worsening with an IOP of 10mmHg. Surgical management of NTG follows that of POAG. Minimally invasive glaucoma surgery (MIGS) has been used in NTG and he presented some data of a small group of patients who had a mean IOP reduction of 2.0mmHg following cataract surgery plus iStent inject – there was, however, no control arm. He also showed a very good consecutive case series from Moorfields where NTG patients had an average IOP at four years of 10.2mmHg following trabeculectomy and a hypotony rate of only 2.3%. This still remains a difficult subtype of glaucoma to manage given the low pressures that patients present with.

Dr Chandra Bala

OCULAR ONCOLOGY

Imaging within ophthalmology is advancing at such a pace that it often reveals surprises in the retina not readily seen on examination. Certainly, many otherwise unseen choroidal nevi are revealed during routine wide field imaging and the question often remains, “what is the malignant potential?” On the Australian and New Zealand Society of Retinal Specialists (ANZRS) specialty day, we had a fantastic update on ocular tumours from one of our younger fellows fresh from her fellowship with the Shields in the USA – world renowned ocular oncology experts. The incident of all uveal melanomas in Australia is 9.8 cases/million/year – or approximately 200-220 new cases diagnosed each year. It is useful to know what the suspicious features of choroidal lesions are so we know who to refer on for a second opinion. These features, which suggest a second opinion is required, include:

1) thickness of greater than 2.0mm on ultrasound,

2) presence of subretinal fluid,

3) new symptoms (such as blurred vision or floaters),

4) orange pigment (often seen best on FAF), and

5) features on imaging such as hollowness or a diameter greater than 5mm.

In 2019, multimodal imaging is crucial and predicts the risk of transformation – the five year risk of malignant transformation depends on the number of risk factors present. If none of the listed factors above are present, the five year risk of malignant transformation is 1%, conversely if five are present the risk is 55%.

RANZCO President Associate Professor Heather Mack speaking at the graduation and awards ceremony

Once a choroidal nevus has been diagnosed, the management is expectant. We do no treat nevi but it is important to have good baseline imaging – colour fundus photography, ultrasound and EDIOCT are all important. Depending on the location, a review every six to 12 months is appropriate. A study published by the Shields in 2001, looked at 278 patients with choroidal nevi and at least seven years of follow up and could demonstrate increasing size in 31% without clinical signs of malignant transformation. Therefore, we can expect growth in about a third of nevi without it necessarily being a melanoma.

CONCLUSION

RANZCO 2019 celebrated many facets of eye care within the Asia Pacific – too numerable to summarise here. We are fortunate to have such a talented group of eye care professionals delivering care to patients in our part of the world. The congress provided us all with a brilliant update while giving us the opportunity to catch up with colleagues, meet with industry and celebrate our amazing profession. Bring on Brisbane 2020!

Dr Amy Cohn is a Melbourne based ophthalmologist with special interest in medical retina and cataract surgery. She is on the RANZCO Scientific Committee as well as being a Visiting Medical Officer at RVEEH and Southern Health and a Senior Research Fellow at the Centre for Eye Research Australia. Dr Cohn sees patients privately in East Melbourne, Footscray, Glen Waverley and Armadale.