September 2017

ADVANCING OPTOMETRY

OCT-A Gets to the root of the problem

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the ONLY 200° single-capture af image the ONLY 200° single-capture color image the ONLY 200° single-capture choroidal layer image September 2017

Editor JEFF MEGAHAN 02 11

National Communications OCT-Angiography Rank order of IOP Manager SANDRA SHAW Dr Linsday Howse and ­ medications Dr Julie Rodman Ruixi Wang and Clinical Editor Associate Professor MARK ROTH Dr Adrian Bruce BSc(Pharm) BAppSc(Optom) PGCertOcTher NEWENCO 06 FAAO OAM XEN Gel Stent 12 treatment system Clinical Quiz Cover design Dr Nathan Kerr Mark Roth Laura Gulbin 13 Optometry Australia 20 ABN 17 004 622 431 Dry eye in Sjögren’s Did surgery cause patients Suite 101, 70 York Street this patient’s NAION? Rachael Kwok and South Melbourne VIC 3205 Andy Mackner and Ph 03 9668 8500 Dr Adrian Bruce Fax 03 9663 7478 Dr Leonid Skorin Jr [email protected] 16 www.optometry.org.au Meningococcal 22 Copyright © 2017 Three steps for third nerve palsy: , palsy and Wesley Teoh Comments made in Pharma are of patient a general nature and intended for guidance only. Optometry Australia and Dr Brandon Powell and the individual contributors expressly 08 18 Dr Joseph Sowka disclaim all liability and responsibility Case report: XEN Gel Therapeutic NEWS of note to any person in respect of, and for the consequences of, anything done or Stent as an alternative to Mark Roth omitted to be done in reliance wholly or trabeculectomy partly on anything in this publication. Dr Nathan Kerr Pharma is distributed in Australia and New Zealand. All references to pharmaceutical preparations in Pharma are applicable within Australia. 2 SEPTEMBER 2017

OCT-Angiography Insight into macular vascular changes seen in pigmentosa using OCT-A

unremarkable, with no posterior Dr Lindsay Howse OD CASE REPORT 1 subcapsular . Both eyes exhibited similar findings on fundus evaluation. The optic nerves were Dr Julie Rodman An 18-year-old African American male pink and healthy, C/D ratio was 0.3, OD MS FAAO presented with a history of unknown with no waxy pallor. The macula was retinal dystrophy, presumed RP. Two intact, with no haemorrhaging, oedema Nova Southeastern University years previously, a prior doctor had or mottling. Retinal vasculature College of Optometry suspected RP based on mild clinical demonstrated slight vessel attenuation. Fort Lauderdale FL, USA signs but was unable to confirm In addition, there was mild bone without electroretinography. The spicule formation in the periphery and patient had no complaints of decreased mid-periphery. night vision or peripheral vision. After probing, the patient reported a slight SD-OCT of the macula appeared delay in visual recovery after looking healthy with no apparent structural at bright lights. The patient denied abnormalities. Vascularisation of a family history of RP. His systemic the retinal layers was visualised and (RP) is defined history was unremarkable and he was segmented using OCT-A. In addition, as a group of rare, hereditary retinal not taking any medications. objective quantification of vessel diseases that result in progressive density was evaluated for each eye loss of photoreceptors. Patients Best corrected visual acuities were using the AngioVue software. typically present with 6/6 OD, OS. , confrontations, and progressive loss of peripheral extraocular motility and colour vision Analysis of the angiograms revealed vision.1 For many years, the focus of were found to be normal. Intraocular decreased retinal vascular perfusion RP has been on understanding the pressure measured 11 mmHg OD, and capillary density in both the pathophysiology of the disease and OS. Anterior segment findings were superficial capillary plexus (SCP) identifying the various structural abnormalities that manifest throughout the disease progression, including arteriole attenuation, waxy pallor, bone spicule formation, posterior subcapsular cataracts and macular mottling. OD Optical coherence tomography angiography (OCT-A) is a new, innovative, non-invasive imaging modality that allows for advanced imaging and analysis of the macular vascular changes associated with RP that have not been previously reported. These findings have OS provided additional insight into the pathophysiology behind the disease, suggesting involvement at the level of the inner . We present three cases of RP at varying stages to highlight the changes in macular vascular density throughout the retinal and choroidal layers as the disease progresses.

Figure 1. OCT angiography and SD-OCT of the right and left eye of a patient with mild retinitis pigmentosa SEPTEMBER 2017 3

unremarkable and he was taking no medications. Best corrected visual acuities were 6/7.5- OD, OS. Pupils, confrontations, extraocular motility OD and colour vision were found to be normal. Intraocular pressure was measured to be 14 mmHg OD and 15 mmHg OS. Anterior segment findings were unremarkable, with no posterior subcapsular cataracts. Posterior segment findings of both eyes revealed mild pallor of the optic nerves with OS 0.3 round C/D ratio. The macula was intact, with no haemorrhaging, oedema or mottling. A mild was present in the macula right eye, greater than left eye. The retinal vessels exhibited mild vessel attenuation. The periphery was flat and intact with bone spicule retinal pigment epithelial clumping in all Figure 2. OCT angiography and SD-OCT of the right and left eye of a patient with moderate quadrants. retinitis pigmentosa Significant findings on the SD-OCT included epiretinal membrane and slight disruption of the photoreceptor integrity line in both eyes. OCT-A revealed an enlarged foveal avascular and deep capillary plexus (DCP) the inner retina in this patient with zone with abnormally low vascular with no apparent abnormality at the mild disease. density in the superficial and deep choriocapillaris. Careful evaluation of capillary plexus with more significant the vessel density maps revealed more involvement of the deep capillary significant loss at the level of the SCP CASE REPORT 2 plexus with no apparent abnormality than the DCP (Figure 1). seen at the level of the (Figure 2). With moderate RP, as seen with this Despite the relatively quiescent nature A 27-year-old Hispanic male presented patient, the DCP appears to be more of the fundus appearance and SD-OCT, with the chief complaint of diminution significantly involved than the SCP, OCT-A was able to detect and localise of vision in both eyes and a history although both complexes are severely irregularities in vascular flow within of RP. His systemic history was compromised.2

CASE REPORT 3

A 56-year-old Hispanic male presented with a chief complaint of blurry vision OD and decreased peripheral vision in both eyes. The patient had been diagnosed with RP 20 years prior in Cuba. His systemic history was unremarkable and he was taking no medications. The patient reported a family history of RP and diabetes mellitus type 2. OS Best corrected visual acuities were 6/18 OD and 6/15 OS. Extraocular motility was full; however, confrontation fields were significantly reduced in all quadrants in both eyes. Pupils were normal with no APD. The patient failed the HRR colour vision screening in both eyes.

Figure 3. OCT angiography and SD-OCT of the right and left eye of a patient with advanced retinitis pigmentosa Continued page 4 4 SEPTEMBER 2017

Normal Early RP Moderate RP Advanced RP

SCP

DCP

Choriocapillaris

Figure 4. OCT angiography showing the macular capillary density of the superficial capillary plexus (SCP), deep capillary plexus (DCP) and choriocapillaris of various stages of retinitis pigmentosa (RP) compared to those of a normal patient

mildly affected due to preservation of RP is characterised by classic OCT-Angiography the photoreceptor integrity line and symptomatology and clinical ellipsoid zone at the fovea. OCT-A findings including nyctalopia, visual From page 3 showed decreased vascular density field constriction, bone spicule in the SCP and DCP with the left eye pigmentation and abnormal full-field affected more than the right eye. Mild electroretinograms. RP affects the changes were observed at the level photoreceptor-RPE cell metabolic Intraocular pressure was measured of the choriocapillaris, but most of complex and begins with a loss of the to be 14 mmHg OD, OS. Anterior the change was observed in the SCP rod photoreceptors as evidenced by the segment findings revealed mild and DCP with the DCP more severely initial involvement of the equatorial meibomian gland disease, , impacted than the SCP (Figure 3). The and peripheral retina. trace amount of pigment on the OCT-A density maps confirmed that corneal endothelium, and PC IOL the severity of disease correlated with Over time, the cone photoreceptors with 1+ diffuse PCO in both eyes. reduction in vascular density within become involved, resulting Significant posterior segment findings the inner retinal SCP and DCP, with in progressive visual loss. of both eyes included: waxy pallor reduction in DCP directly related to Histopathological studies have of both optic nerves with 0.2 C/D visual function. identified shortening of the ratio, retinal vascular attenuation, photoreceptor outer segments as pigmentary changes and RPE mottling the earliest identifiable finding in Discussion of the macula, and bone spicule RPE RP. 2 Typical clinical features of RP clumping in the periphery in all Retinitis pigmentosa is the term used include peripheral retinal bone- quadrants. to designate a group of inherited spicule deposition, optic nerve pallor retinal disorders characterised by and retinal vessel attenuation. Loss SD-OCT showed mild macular oedema progressive loss of photoreceptors of normal retinal vasculature is with cystic pockets of fluid in both and retinal dysfunction resulting believed to be due to photoreceptor eyes, but visual acuity was only in deterioration of central vision.1 degeneration.3 SEPTEMBER 2017 5

Abnormalities within the retinal focal electroretinogram is negatively pivotal role in staging and categorising vasculature as well as changes affected by apoptosis of the ganglion the disease. in ocular blood flow have been cells.9 implicated in the pathogenesis of OCT-A provides excellent insight into RP. In fact, angiography The resolution provided by OCT-A has the microvascular changes occurring and indocyanine green angiography provided outstanding visualisation of throughout the retina and choroid and have illustrated the abnormal retinal the SCP and DCP vascular networks allows for additional insight into the and choroidal vasculature in RP within the inner retina and has been pathophysiology behind the disease. It noting increased dye transit time, able to identify varying features appears that alterations in the vascular narrowed vessel lumen and reduced that cannot be distinguished by density and pattern of flow seen on dye concentration.4 In addition, classical fluorescein angiography. The angiogram correlate with disease electoretinogram shows increased superficial vascular plexus is located severity. OCT-A may exhibit signs of arteriovenous transit time and reduced in the ganglion cell layer and in the disease earlier and should be utilised blood flow velocity.5 nerve fibre layer. The deep vascular to provide timely management of plexi are located in the inner nuclear affected patients. Since the advent of OCT, it has become layers and external plexiform layer. the standard for assessing anatomical abnormalities via high resolution OCT-A illustrates different 1. Rezaei KA, Zhang Q, Chen C, Chao J, et tomographic images. OCT is able to morphological features of the retinal al. Retinitis pigmentosa. Lancet 2006; accurately assess the photoreceptor blood supply for the two plexi.10 The 368: 1795-1809. segments and thicknesses of retinal vascular supply of the superficial 2. Liu G, Liu H, Du Q, Wang F. Optical coherence tomographic analysis of layers in RP patients and has shown plexus is characterised by multiple retina in retinitis pigmentosa patients. a decrease in foveal thickness, linear structures having a centripetal Ophthalmic Res 2016; 56: 111-122. interruption of the IS/OS junction, distribution converging towards the 3. Zhang Q, Lee CS, Chao J, Chen CL, 5 et al. Wide-field optical coherence and reduction in choroidal thickness. fovea. Secondary vessels arise from tomography based microangiography for Correlations between the retinal the main vessels forming a web-like retinal imaging. Nature 2016; 6: 22017. structure and visual acuity have been pattern. The calibre of the vessels is 4. Merin S, Auerbach E. Retinitis pigmentosa. Surv Ophthalmol 1976; identified in affected patients. uniform throughout the scan. The 122: 502-508. deep plexus comprises an interwoven 5. Dhoot DS, Huo S, Yuan A, et al. Evaluation of choroidal thickness in Historically, RP has been characterised pattern of vessels that surround the retinitis pigmentosa using enhanced as a disease of the photoreceptors foveal avascular zone in numerous depth imaging optical coherence with loss of the outer retinal cells. horizontal and radial interconnections. tomography. Br J Ophthalmol 97: 66-69. 6. Stone JL, Barlow WE, Humayun MS, de However, new research is identifying The calibre of the vessels is also Juan E, et al. Morphometric analysis of disorganisation and damage to the uniform throughout the scan.11,12 macular photoreceptors and ganglion inner retina including death of the cells in with retinitis pigmentosa. Arch Ophthalmol 1992; 110: 1634-1649. retinal ganglion cells. Outer retinal OCT-A in patients with RP 7. Jones BW, Pfeiffer RL, Ferrell WD, Watt involvement can occur as a result demonstrates alterations in the normal CB, el al. Retinal remodeling in human of chronic apoptosis of the ganglion vasculature within the superficial retinitis pigmentosa. Exp Eye Res 2016; 150: 149-165. cells and disorganisation of the inner and deep retinal layers as well as 8. Saha S, Greferath U, Vessey KA, retina.6-8 throughout the choroid. The SCP Grayden DB, el al. Changes in ganglion and DCP vessel densities are reduced cells during retinal degeneration. 9 Neuroscience 2016; 329: 1-11. OCT-A is a recently developed, non- in patients with RP. It has been 9. Toto L, Borrelli E, Mastropasqua R, invasive, contrast-free technique for postulated that this reduction in blood Senatore A, et al. Macular features imaging the retinal and choroidal flow occurs early in the disease process in retinitis pigmentosa: correlations among ganglion cell complex thickness, microvasculature. The technique and leads to eventual ischaemia, capillary density, and macular function. uses the movement of red blood cells retinal vessel damage and cell death Invest Ophthalmol Vis Sci 2016; 57: in the retinal vasculature to create involving both the inner and outer 6360-6366. 10. Spaide RF, Klancnik JM, Cooney MJ. an image of retinal blood flow. This retinal space. Furthermore, vessel Retinal vascular layers imaged by allows for segmentation and evaluation density abnormalities at the level of fluorescein angiography and optical coherence tomography angiography. of all retinal layers and capillary the DCP appear to be directly related to JAMA Ophthalmol 2015; 133: 45-50. networks. Analysis of OCT-A images macular function and visual potential. 11. Lumbroso B, Huang D, Jia Y, et al. in RP patients has confirmed the Clinical Guide to Angio-OCT Non Invasive, Dye-less OCT Angiography. 1st aforementioned involvement of the Analysis of the three patients ed. New Delhi, India: Jaypee Brothers inner retinal layers in the pathogenesis presented in this article suggests that Medical Publisher: 2015. of RP. severity of disease correlates with 12. Savastano MC, Lumbroso B, Rispoli M, In Vivo Characterization of degree of reduction in vascular flow retinal vascularization morphology It has been documented that as seen on the segmented angiograms using optical coherence tomography photoreceptor damage leads to changes as well as with diminution in the angiography. Retina 2016; 35: 2196- 2203. in the inner retinal tissue including vessel density map (Figure 4). Because the retinal ganglion cells. Despite OCT-A is able to identify and quantify varying hypotheses regarding the loss of normal retinal vasculature morphological changes to the inner in RP patients, it should be used to retina, a direct correlation has been monitor disease progression in affected found between ganglion cell thickness patients. Vascular changes may and macular function in RP patients. precede functional changes as seen on Furthermore, it appears that the multi- visual field testing and may thus play a 6 SEPTEMBER 2017

XEN Gel Stent glaucoma treatment system

Treatment system from the limbus in the superonasal Dr Nathan Kerr quadrant. There is no conjunctival MBChB MD FRANZCO The XEN Glaucoma Treatment System incision or suturing required, leading consists of the XEN Gel Stent and to a safer procedure and faster post- the XEN Injector. The XEN Gel Stent operative recovery. Royal Victorian Eye and Ear Hospital is a soft, flexible, hydrophilic tube Centre for Eye Research Australia composed of porcine-derived collagen Like other filtration surgery, anti- Associates, Melbourne that has been shown to have good metabolites are administered prior to biocompatibility.1 insertion to prevent subconjunctival scarring. While the procedure looks Cross-linked with glutaraldehyde simple, it is technically demanding to prevent degradation, the XEN and must be well-executed to creates a permanent outflow pathway achieve optimum results and between the anterior chamber and prevent complications. However, THE XEN Glaucoma Treatment System the subconjunctival space. The XEN once mastered, the XEN represents is a new option for patients with is 6 mm long and has an inner lumen the safest, fastest and least-invasive refractory glaucoma. This minimally- diameter of 45 µm. These dimensions method of creating a new outflow invasive device drains aqueous into have been selected based on the pathway to the subconjunctival space. the subconjunctival space, a procedure Poisseuille equation (which predicts long-considered the gold standard for flow based on tube length, internal Indications glaucoma surgery. diameter and fluid viscosity) to prevent hypotony. The XEN Glaucoma Treatment System Previously, this could be achieved is indicated for the management only with invasive operations such of refractory glaucoma, including Surgical technique as trabeculectomy or insertion of a primary open-angle glaucoma, glaucoma drainage device, which The XEN is performed under local pseudoexfoliative glaucoma and carry not-insignificant risks. The anaesthetic in a 15-minute operation. pigmentary glaucoma, which is XEN aims to provide a safer and Typically performed as a stand-alone unresponsive to maximum tolerated more predictable way of achieving procedure, the XEN can also be medical therapy. The XEN can be subconjunctival filtration. In doing combined with cataract surgery for performed as a primary procedure2 so, the XEN expands the role of patients with both refractory glaucoma or in eyes where previous surgical minimally-invasive glaucoma and visually-significant cataract. The treatments, such as other MIGS surgery (MIGS) across the spectrum implant is inserted using a preloaded devices or trabeculectomy, have failed. of glaucoma and has the potential injector via an ab interno approach to revolutionise the way refractory through a 1.2 mm clear corneal As the XEN is minimally-invasive glaucoma is treated. incision, exiting the 3 mm and has a good safety profile, it

Figure 1. The XEN injector is passed Figure 2. Magnified view showing advance- Figure 3. Magnified view showing rotation through a clear corneal incision, across the ment of the injector into the subconjunctival of the bevel in the subconjunctival space anterior chamber, and advanced through the space sclera into the subconjunctival space SEPTEMBER 2017 7

can be considered earlier in the Evidence of subconjunctival filtration with the disease process than trabeculectomy. safety and fast recovery of minimally- Additionally, because the XEN The efficacy and safety of the XEN invasive glaucoma surgery. While drains to the subconjunctival space has been evaluated in the APEX there will always be a place for it has greater IOP-lowering efficacy study, a prospective, multi-centre, trabeculectomy in selected patients, than other MIGS procedures and is 24-month study of 215 patients in particularly those with very advanced therefore also suitable for cases with 14 countries with mild-to-moderate disease, for the majority of patients significant IOP elevation and more primary open angle glaucoma and no with refractory open-angle glaucoma advanced disease. history of intraocular surgery. Patients the XEN provides an attractive underwent either the XEN procedure alternative. The XEN is contraindicated in active alone or in combination with cataract neovascular glaucoma or if there is surgery. intraocular silicone oil, vitreous in the anterior chamber, or an anterior In a preliminary report, there was 1. Shute TS, Dietrich UM, Baker JFM, et al. chamber intraocular . The an average reduction in IOP of 7.6 ± Biocompatibility of a novel microfistula procedure should not be performed 4.8 mmHg to a mean of 13.8 mmHg implant in nonprimate mammals for the if there is significant conjunctival at 12 months.3 This was associated surgical treatment of glaucoma. Invest Ophthalmol Vis Sci 2016; 57: 8: 3594- scarring or an existing glaucoma with a reduction in IOP-lowering 3600. doi:10.1167/iovs.16-19453. drainage device in the target medications from 2.6 ± 1.1 at baseline 2. Kerr NM, Wang J, Barton K. Minimally quadrant. The safety and effectiveness invasive glaucoma surgery as primary to 0.6 ± 0.8 at 12 months. There stand-alone surgery for glaucoma. Clin of the XEN has not been established were no cases of bleb leak and only Exper Ophthalmol 2016. doi:10.1111/ in congenital or infantile glaucoma. one patient (0.5 per cent) developed ceo.12888. 3. Barton KB, Chelvin S, Vera V. XEN45 symptomatic hypotony requiring Implantation for Primary Open-Angle injection of viscoelastic. There was Post-operative management Glaucoma: One-Year Results of a no difference in efficacy between the Multicenter Study. European Glaucoma Society Congress 2016. The XEN creates a bleb that XEN as a stand-alone procedure or 4. Galal A, Bilgic A, Eltanamly R, is typically low, diffuse and in combination with cataract surgery. Osman A. XEN glaucoma implant comfortable. Post-operative recovery The 24-month results are due to be with mitomycin C 1-year follow- up: result and complications. J is much faster than trabeculectomy published this year. Other smaller Ophthalmol 2017; 2017: 5457246. with little blurring of vision, minimal studies have shown similar results.4–6 doi:10.1155/2017/5457246. restriction on physical activity 5. Sheybani A, Dick HB, Ahmed IIK. Early clinical results of a novel ab interno and less need for intensive topical The success rates of XEN compare gel stent for the surgical treatment steroids. Fewer post-operative favourably with gold-standard of open-angle glaucoma. J Glaucoma visits are required compared to trabeculectomy. In a multi-centre, 2016; 25: 7: e691-6. doi:10.1097/ IJG.0000000000000352. trabeculectomy and there is no need retrospective interventional cohort 6. Sheybani A, Lenzhofer M, Hohensinn for suture removal or lysis. While study, there was no detectable M, Reitsamer H, Ahmed IIK. complications such as malposition, difference in failure rate between Phacoemulsification combined with a new ab interno gel stent to treat open- occlusion or rarely, erosion can occur, stand-alone XEN insertion or angle glaucoma: Pilot study. J Cataract these can typically be prevented trabeculectomy with mitomycin C. 7 Refract Surg 2015; 41: 9: 1905-1909. with good surgical technique. As the doi:10.1016/j.jcrs.2015.01.019. 7. Schlenker MB, Gulamhusein H, Conrad- XEN requires bleb management and Future of glaucoma surgery Hengerer I, et al. Efficacy, safety, and occasionally needling, the procedure risk factors for failure of standalone ab interno gelatin microstent implantation is best performed by glaucoma The XEN represents a major advance versus standalone trabeculectomy. surgeons who are experienced in bleb in the surgical management of 2017. doi:10.1016/j. management. glaucoma. It combines the efficacy ophtha.2017.05.004.

Figure 4. Deployment of the XEN Gel Stent Figure 5. The XEN Gel Stent in situ draining Figure 6. cross section with XEN Gel into the subconjunctival space aqueous from the anterior chamber to the Stent in place. subconjunctival space All images: © 2017 Allergan. Used with permission 8 SEPTEMBER 2017

CASE REPORT XEN Gel Stent as an alternative to trabeculectomy

Dr Nathan Kerr MBChB MD FRANZCO

Royal Victorian Eye and Ear Hospital Centre for Eye Research Australia

Eye Surgery Associates, Melbourne

A 57-YEAR-OLD Caucasian woman with open-angle glaucoma refractory to medical and laser treatment was referred to me by her optometrist for a second opinion and to discuss alternatives to trabeculectomy.

The patient had a history of primary open-angle glaucoma diagnosed seven years previously and had presenting intraocular pressures in the mid 20s. Her central corneal thickness was reduced at 533 microns in her right eye and 540 microns in her left eye. There was a family history of glaucoma affecting her mother who was diagnosed in her late 60s. Figure 1. OCT shows thinning of the retinal nerve fibre layer. Her mother was treated with topical medications and reportedly suffered field loss but not blindness.

The patient’s general health was good slow recovery and time-off required measured by Goldmann applanation and she was not regularly taking any from her work as a gardener, and was tonometry were 16 mmHg in her medications. interested in seeking an alternative. right eye and 18 mmHg in her left Her friend in the United States eye. There was no pseudoexfoliation Despite treatment with bilateral had undergone the XEN Gel Stent or cataract in either eye. Dilated selective laser trabeculoplasty (SLT) procedure with good results and the fundoscopy revealed cup-to-disc by her original ophthalmologist and patient was interested to know if she ratios of 0.9 with thinning of the good adherence with three topical was a candidate for the procedure. neuroretinal rim both superiorly and ocular hypotensive medications, her inferiorly in each eye. There was a intraocular pressures remained in the resolving Drance haemorrhage in her Examination high teens. Her ophthalmologist noted left eye. a Drance haemorrhage in her left eye On examination her best-corrected and advised that trabeculectomy was visual acuity was 6/6 in each eye. Her Optical coherence tomography (OCT) required. was white and healthy. showed marked thinning of the retinal Both anterior chambers were deep nerve fibre layer in each eye (Figure Having read the information sheet, the and quiet and her angles were open 1) and automated perimetry showed patient was worried about the risks, on gonioscopy. Intraocular pressures early field loss with slow progression SEPTEMBER 2017 9

Surgical treatment of refractory glaucoma without conjunctival incisions or suturing

in each eye on guided progression invasive operation and faster post- injected acetylcholine to constrict the analysis (Figures 2A and 2B). operative recovery. For many patients pupil and protect her crystalline lens. it can delay or prevent the need for Then I filled the anterior chamber We discussed the patient’s diagnosis invasive surgery. The patient was with viscoelastic before checking and our shared goal of preventing carefully counselled on the risks and and hydrating the XEN before vision loss from glaucoma. Given the benefits and elected to proceed with implantation. patient’s age, presenting pressures, the XEN Gel Stent. reduced corneal thickness, family The injector was passed across the history of glaucoma, and development anterior chamber and through the Surgical course of a Drance haemorrhage at 18 mmHg, sclera into the subconjunctival space we discussed the need to achieve an In the operating room under a at exactly my markings. I rotated the IOP in the low double digits which peribulbar block, I marked the bevel of the needle toward 12 o’clock can realistically be achieved only with planned exit point for the XEN Gel to ensure a superior bleb. I then subconjunctival filtration surgery. Stent. To control subconjunctival deployed the stent carefully into the fibrosis, I injected mitomycin C subconjunctival space before checking While trabeculectomy is the current posteriorly and swept it away from the for correct length and position using a accepted gold standard, we discussed limbus to prevent the development gonioprism. the option of a XEN Gel Stent as an of an avascular bleb. I made a 1.5 alternative. The XEN has a favourable mm clear corneal incision and a safety profile and offers a much less single side port to stabilise the eye. I Continued page 10

Figure 2A. Humphrey visual field guided progression analysis, Figure 2B. Humphrey visual field guided progression analysis, right eye left eye 10 SEPTEMBER 2017

XEN case report From page 9

Lastly, I removed the viscoelastic with bimanual irrigation and aspiration, checked for the development of a bleb, and hydrated the wounds before administering intracameral antibiotic to prevent infection. At the end of the procedure I placed a pad and shield over the eye and prescribed topical steroids every two hours and antibiotics four times a day.

The patient was instructed to Figure 3A. Slitlamp photography of the XEN Gel Stent in position discontinue glaucoma medications in the operated eye, to avoid heavy lifting or straining, keep the eye dry, and wear a shield at night for the first week.

An appointment was scheduled for the following day and the patient was given my mobile number to call in case of emergency.

Outcome At the day 1 post-operative visit, the patient’s visual acuity was 6/9 in the operated eye and her intraocular pressure was 7 mmHg without sequelae. Her anterior chamber was deep and there were no macular folds or choroidal effusions. The XEN was Figure 3B. Low diffuse bleb associated with the XEN Gel Stent well-positioned and there was a low diffuse bleb and no leak (Figures 3A and 3B). required, which is appealing from a As with all subconjunctival At one week post-operatively, her patient perspective. procedures that form a bleb, there visual acuity had returned to 6/6 is a small risk of bleb-related and her intraocular pressure was 10 While numerically low pressures complications. However, compared mmHg. Antibiotics were discontinued are common, hypotony is rare.2 to trabeculectomy, the morphology at one week and steroids tapered Accordingly, its favourable safety of the XEN bleb is different, tending over the next three months. At three profile enables the XEN Gel Stent to to be low and diffuse. Like any stent, months the patient is off all eye-drops be offered at an earlier stage of disease there is a small risk of occlusion or and her visual acuity is 6/6 and IOP and in many patients it can avert or exposure; however, this is rare and 9 mmHg. The fellow eye has been delay the need for invasive surgery.3 can generally be averted with proper scheduled for surgery. If additional pressure reduction is technique and placement. required, medical therapy can be restarted and the procedure does 1. Schlenker MB, Gulamhusein H, Conrad- Discussion Hengerer I, et al. Efficacy, safety, and not preclude further operations risk factors for failure of standalone ab The XEN Gel Stent represents such as trabeculectomy or glaucoma interno gelatin microstent implantation a major advancement in the drainage device insertion from being versus standalone trabeculectomy. Ophthalmology 2017. doi: 10.1016/j. treatment of refractory glaucoma. It performed. ophtha.2017.05.004. provides intraocular pressure levels 2. Sheybani A, Dick HB, Ahmed IIK. Early approaching those of trabeculectomy, Patients should be monitored closely clinical results of a novel ab interno gel stent for the surgical treatment of open- in a fast, minimally-invasive post-operatively. Optimal outcomes angle glaucoma. J Glaucoma 2016; 25: operation with a good safety profile require bleb management including e691–696. and rapid post-operative recovery.1 needling and therefore the procedure 3. Kerr NM, Wang J, Barton K. Minimally invasive glaucoma surgery as primary Unlikely trabeculectomy there are is best performed by surgeons stand-alone surgery for glaucoma. Clin no conjunctival incisions or suturing experienced in bleb management. Exp Ophthalmol 2017; 45: 393-400. SEPTEMBER 2017 11

Rank order of IOP medications

Mean reduction in IOP Ruixi Wang (mmHg) at 3 months BVisSC MOptom Rank order Medication class Preparations (95 per cent CI) 1. Bimatoprost 0.03% Prostaglandin Bimatoprost 0.03% 5.77 (5.04 – 6.50) analogues Latanoprost 0.005% 4.85 (4.24 – 5.46) 2. Latanoprost Dr Adrian Bruce Travoprost 0.004% 4.83 (4.12 – 5.54) BSc(Optom) PhD PGCertOcTher 3. Travoprost Bimatoprost 0.01% 4.74 (1.91 – 3.19) 4. Bimatoprost 0.01% Tafluprost 0.0015% 4.37 (2.94 – 5.83) Unoprostone 0.15% 1.91 (1.51 – 2.67) 5. Levobunolol Australian College of Optometry 6. Tafluprost Beta-blocker Levobunolol 0.25% 4.51 (3.85 – 5.24) 7. Timolol Timolol 0.25%, 0.5%, 0.1% 3.70 (3.16 – 4.24) 8. Brimonidine Carteolol 1% 3.44 (2.42 – 4.46) Levobetaxolol 0.5% 2.56 (1.52 – 3.62) 9. Carteolol Betaxolol 0.25%, 0.5% 2.24 (1.59 – 2.88) 10. Levobetaxolol 11. Apraclonidine Alpha 2 agonist Brimonidine 0.2% 3.59 (2.89 – 4.29) Apraclonidine 0.5% 2.52 (0.94 – 4.11) 12. Dorzolamide A SYSTEMATIC review published 13. Brinzolamide by Li et al1 in the journal of the Carbonic anhydrase Dorzolamide 2% 2.49 (1.85 – 3.13) 14. Betaxolol American Academy of Ophthalmology inhibitors Brinzolamide 1% 2.42 (1.62 – 3.23) included 20,275 participants from 114 15. Unoprostone randomised controlled trials (RCT). The aim of the RCTs was to determine the Table 1. Ranking Table 2. Mean reduction (including 95 per cent confidence inter- effectiveness of single topical medical from most-effective to val) in IOP in mmHg for all IOP-lowering medical treatments included treatment using four medication classes least-effective in the study (excluding miotics) when compared with placebo or another single active topical medical agent. apraclonidine and its performance was conjunctival hyperaemia, periorbital better than carteolol, betaxolol and skin pigmentation, hypertrichosis, To be included in this systematic levobetaxolol, which are beta-blockers. pigmentation and heterochromia. review, at least 60 per cent or more of Unoprostone and betaxolol were the the participants in each trial needed least effective in lowering IOP. In addition, there have been reports of to be diagnosed with primary open patients who developed anterior angle glaucoma (POAG) or ocular The study ranks the relative efficacy of after receiving latanoprost. Although hypertension (OHT). Studies were IOP-lowering medications and therefore the risk is small, it should be used with excluded if combination treatment or can be used to guide clinical decision- caution in patients with co-existing surgical intervention was utilised; there making for medical treatment of POAG. intraocular inflammation including were fewer than 10 participants in each However, it should not be considered uveitis due to the risk of developing group and/or fewer than 28 days of a solitary reference for choice of cystoid macular oedema (reversible), follow-up after randomisation. medication that should be used for and in patients with a history of treatment of POAG. herpetic infection due to the increased All medical treatments were more risk of reactivation of herpetic eye effective than placebo in lowering IOP. The following factors must be disease. At three months, the mean reductions considered when prescribing medical in IOP, ranked from the most to the therapies, including mode of action, Beta blockers least-effective topical agent, are listed adverse effects, contraindications, the in Table 1. patient’s systemic health conditions, Beta-blocker is the next best IOP existing risk factors, extent of damage lowering medication in terms As shown in Table 2, prostaglandin present and current rate of progression, of efficacy. When compared to analogues were more effective in predicted life expectancy, level of prostaglandin analogues, it has a lowering IOP when compared to other compliance and financial burden of short onset of action (30 minutes) and drug classes. There was no statistically treatment. is therefore beneficial for rapid IOP significant difference in efficacy reduction such as in acute angle closure between the different prostaglandin attack. However, beta-blocker has more Prostaglandin analogue analogues, including bimatoprost, systemic contraindications and higher travoprost, latanoprost or tafluprost. Prostaglandin analogue is usually risk of adverse effects when compared selected as the first line medical to prostaglandin analogues. Levobunolol was more effective when treatment of POAG due to its superior compared to other beta-blockers. efficacy when compared to other Non-selective beta-blocker acts on The mean difference in IOP between medication classes, convenient beta-1 and beta-2 receptors while timolol 0.5% or timolol < 0.5% was once-daily dosing and hence better cardioselective beta-blocker acts on small and not statistically significant. tolerability and compliance and beta-1 receptors. Blockage of beta-2 Interestingly, brimonidine was minimal systemic contraindications. more effective at lowering IOP than The topical adverse effects include Continued page 12 12 SEPTEMBER 2017

Clinical Quiz

Associate Professor Mark Roth

BSc(Pharm) BAppSc(Optom) PGCertOcTher NEWENCO FAAO OAM

A 48-YEAR-OLD, Caucasian woman presents with a long-standing, large, right inferiotemporal, oval, conjunctival lesion. The lesion is 6 mm in diameter and 3 mm thick, with associated dilated blood vessels. There is a yellow-white corneal line adjacent to lesion and one prominent follicle and hair in the centre of the mass. The patient is asymptomatic. Conjunctival lesion Image: Associate Professor Mark Roth

What is your diagnosis? Answer page 24

head, the rate of retinal ganglion cell target IOP. They may be considered as IOP medications atrophy and degree of neuroretinal rim first-line treatment when the extent degeneration cannot be calculated and of existing glaucomatous damage is From page 12 quantified easily. profound. Fixed combination therapy is more cost-effective and offers the receptor is problematic in patients The effect of the medication on the convenience of once or twice-daily with asthma and chronic obstructive patient’s quality of life is even more dosage which maximises compliance pulmonary disease. Selective beta- difficult to quantify and requires and adherence. Fewer drops also mean blockers may also cause bradycardia, long-term follow-up. In contrast to reduced exposure of the ocular surface hypotension and hypoglycaemia. IOP, visual field is a more meaningful to preservatives and less risk of toxicity. outcome measure to quantify the effectiveness of treatment because it It has been published that fixed Alpha 2s and CAIs correlates with the patient’s quality combination of latanoprost 0.005% Alpha-2 agonists are contraindicated of life such as driving. However, only and timolol 0.5% is statistically in patients taking monoamine oxidase 20 per cent of randomised controlled more effective in lowering IOP inhibitor. Carbonic anhydrase inhibitors trials included in the systemic review when compared to monotherapy.2 (CAI) are contraindicated in patients reported visual field as an outcome However, there is no clear evidence with sulfonamide allergies and patients measure. demonstrating the superiority of fixed with corneal graft and endothelial combination agents. Therefore, it will dystrophies as they may exacerbate The extrapolation of visual field result be interesting to see where combination corneal decompensation. Both CAIs is also difficult due to the heterogeneity agents fit in the rank order of topical and alpha-2 agonist require two to three associated with different reporting IOP-lowering medications for the times daily dosing, which reduces methods and relatively short follow- treatment of POAG. The other fact compliance. up time of three months. It will be to keep in mind is that unlike single interesting to see studies which have active agents, the dosing regimen and visual field results as an outcome when treatment times of fixed combination Limitations evaluating the effectiveness of topical agents cannot be optimised. IOP is the primary and most common IOP-lowering medication. outcome to quantify the effectiveness 1. Li T, Lindsley K, Rouse B, et al. of topical medical therapies in the It is worth noting that this study Comparative effectiveness of first-line medications for primary open-angle literature. IOP is the only known does not consider the role of fixed/ glaucoma. Ophthalmology 2016; 123: 1: modifiable risk factor for treatment of unfixed combination agents in the 129-140. glaucoma and IOP reduction correlates treatment of POAG. Combination 2. Polo V. Treatment of glaucoma with the fixed combination of latanoprost 0.005% with preservation of visual field. The agents are generally used when single and timolol 0.5%. European Ophthalmic extent of damage to the optic nerve agents are inadequate for reaching the Review 2009; 3: 2: 33-36. SEPTEMBER 2017 13

A sight for sore eyes Clinical guidelines for managing dry eye in Sjögren’s patients

evaluate the severity of symptoms: • How often do your eyes feel dryness, discomfort or irritation? • Would you say it is often or constantly? • When you have eye dryness, discomfort or irritation, does this impact your activities, for example, do you stop or reduce your time doing them? • Do you think you have dry eye?

A patient reporting ‘yes’ to any of these questions warrants further investigation.

Diagnostic tests The researchers note that in each patient, the clinician must determine whether the dry eye is due to AS PART of the Sjögren’s Syndrome aqueous production deficiency, Rachael Kwok Foundation’s initiative to develop excess evaporation, or combined BVisSci MOptom clinical guidelines for practitioners, mechanisms. Because the sequence of a panel of eye-care professionals and evaluation can influence the results of Dr Adrian Bruce consultants was formed to determine the subsequent tests, they recommend consensus guidelines for managing BSc(Optom) PhD PGCertOcTher performing the tests from least dry eye associated with Sjögren’s invasive to more invasive. syndrome. Foulks et al published Australian College of Optometry their recommendations in the April Direct observation without any 2015 issue of The Ocular Surface. manipulation of the , tear film, These clinical guidelines are based or ocular surface should be performed primarily on the 2007 report of the first. Direct observation includes using International Workshop on Dry Eye the specular reflection to observe the (DEWS), as well as evidence from key tear meniscus (is it scanty?) and the peer-reviewed publications. The level tear lipid layer (is it coloured?) These of evidence determined the strength of observations alone help to show if the recommendation. an aqueous deficiency or meibomian dysfunction or both are present. Sjögren’s syndrome The phenol red thread test is then Sjögren’s is an autoimmune disease, performed, prior to instilling dye primarily affecting the exocrine and taking care not to induce reflex glands of mucous membranes, tearing. The Schirmer 1 test is tricky resulting in dry eye and dry mouth, to interpret except in severe dry eye, often with additional muscoskeletal as it can induce reflex tearing and disturbance and damage to other body instilling anaesthesia may change the systems. tear volume. The phenol red thread test may be an alternative. Dry eye is one of the most quality- of-life and activity limiting problems Instillation of topical fluorescein in Sjögren’s syndrome. Symptoms should be followed by measurement include discomfort and fluctuation of vision. The researchers recommend the use of four specific questions to Continued page 14 14 SEPTEMBER 2017

Sjögren’s patients From page 13

of the tear film break-up time (TFBUT). Corneal staining should be recorded 1.5–3 minutes after fluorescein instillation. Conjunctival staining with fluorescein can be assessed using the yellow Wratten filter, or by instilling lissamine green and making observations after three minutes.

MANAGEMENT APPROACHES

Communication The researchers emphasise the importance of communication between the practitioner and patient regarding the nature of the disease, aggravating factors and goals of treatment. Given the multiple Figure 1. 45-year old female with Sjögren’s syndrome. Inferior corneal lissamine green contributing causes of dry eye, the staining punctate and coalesced pattern. Image: Associate Professor Mark Roth patient should consider dry eye therapy as part of their overall health management.

Mild, episodic dry eye can be managed with modification of the patient’s environment and activities of daily living. These include: avoiding a longer residence time but are more Systemic therapy systemic medications that can reduce likely to blur vision, although newer tear secretion, maintaining agents containing hyaluronic acid Traditional treatments for posterior margin hygiene, avoiding very dry may disrupt this constraint. and meibomian gland or windy environments and limiting dysfunction include hot compresses certain tasks associated with reduced and lid hygiene. In more recalcitrant Therapeutics blink rate. When these preventive cases, the researchers recommend measures are inadequate, there is a Moderate-to-severe ocular surface the use of topical antibiotics or low logical sequence of therapies that can disease often requires the use of anti- dose oral tetracyclines to decrease be used. inflammatory therapies. Studies have bacterial colonisation of the lid shown topical corticosteroids are margins. Omega 3 essential fatty acids effective in decreasing clinical signs supplements are also considered to Lubricants and symptoms. However, the possible have anti-inflammatory effects. Tear volume replacement with ocular complications associated with long- lubricants is the first line of therapy for term use such as cataracts, intraocular The evidence of their use in treating dry eye in Sjögren’s disease. Among pressure spikes and infection, limit dry eye is growing, and at 1000- the wide variety of formulations their use to short-term or pulse 1500 mg per day for three months, using carboxymethylcellulose therapy. The researchers recommend it is considered a low-risk dietary and hydroxymethylcellulose, the a two- to four-week course for cases supplement.1 Foulks et al2 recommend researchers concluded that none is inadequately controlled with other that patients taking more than 3000 clearly superior. therapies. mg per day omega 3 supplements should do so only under a physician’s Given the toxicity of preservatives to Topical cyclosporine (Restasis or care. the ocular surface, preservative-free compounded) has been shown to unit dose vials are recommended increase tear production, and improve Contact lenses when tear supplements are used in patients more than four to six times a day. with chronic aqueous-deficient dry Large-diameter rigid, gas-permeable Ophthalmic ointments may be used eye. No significant side-effects have lenses are recommended to control before bedtime to provide relief of been reported, and long-term safety severe ocular surface damage, when dry eye symptoms and enable sleep. of topical cyclosporine has been other treatments are insufficient. Typically, thicker preparations have confirmed in the literature. Therapeutic scleral contact lenses SEPTEMBER 2017 15

in Sjögren’s patients serve to protect the ocular surface from the FDA approves first treatment for GCA eyelids and environment, relieve discomfort and improve quality THE US FOOD and Drug tocilizumab along with standardised of vision. Retention of a fluid Administration (FDA) has approved prednisone regimens achieved reservoir over the and lack subcutaneous tocilizumab for the sustained remission compared to of corneal touch are key elements treatment of giant cell arteritis (GCA) patients receiving placebo with to the therapeutic effect of scleral in adults. The drug is marketed as standardised prednisone regimens. lenses. Actemra by health-care company Hoffmann-La Roche. The standard of care for patients with GCA is steroid therapy; however, Further options The decision follows the results of prolonged courses of steroids often Once the inflammatory component a double-blind, placebo-controlled are required, leading to substantial of dry eye is controlled, occlusion study which involved 251 patients steroid-associated complications. of the lacrimal puncta using with GCA. Researchers looked for plugs can be considered. This sustained remission from the disease The FDA granted the application for can be performed on a temporary from week 12 to week 52. Actemra a ‘Breakthrough Therapy’ or permanent basis. Those designation and a ‘Priority Review’. unresponsive to intensive lubricant It was found that a greater proportion and anti-inflammatory therapy may of patients receiving subcutaneous www.fda.gov require use of topical autologous serum. This is typically dosed four times daily, and can be used in conjunction with other therapies. Manage lid-disease before trabeculectomy The most severe cases of dry eye may consider partial closure of TO AVOID bleb-related infection blepharitis. No increased risk was the interpalpebral fissure, using (BRI), lid disease should be managed identified with the antimetabolite used botulinum toxin injections or prior to trabeculectomy surgery, during trabeculectomy surgery or the surgical options, to reduce surface a study has found. Researchers type of conjunctival reflection adopted exposure. conducted a clinical study of BRI for surgery. Neither age nor dry eye following trabeculectomy surgery. was identified as a risk factor. Eye-care practitioners frequently The study involved documenting encounter patients with dry historical data review from patients’ An increased risk of BRI was identified eye symptoms. Although most records, self-reported questionnaires in eyes with chronic blepharitis. patients complaining of dry eye specific to ocular surface symptoms To minimise the risk of infection do not have Sjögren’s, it should be and a repeated detailed clinical following trabeculectomy surgery, considered as a possible aetiology, examination of the lid, ocular surface it may be advisable to manage lid particularly when it is associated and tear film. disease in patients prior to performing with inflammation, difficulty in trabeculectomy surgery, or offer an management, dry mouth, arthritis Twenty-eight cases and 31 controls alternative treatment such as a shunt. or other systemic evidence of were assessed. Researchers found inflammation or autoimmune that the overwhelming risk factor Br J Ophthalmol 2016; Oct 18 (Epub disease. The researchers strongly for development of BRI was chronic ahead of print) encourage clinicians to refer patients with suspected Sjögren’s disease to a rheumatologist for systemic disease diagnosis and management. New study of infectious conjunctivitis

THE FIRST patient has been enrolled conjunctivitis and two for bacterial 1. Deinema LA, Vingrys AJ, Wong CY, in a phase 3 clinical trial for SHP640, conjunctivitis. et al. A randomized, double-masked, a combination broad-spectrum placebo-controlled clinical trial of antiseptic and corticosteroid being Shire plans to enrol more than 2,700 two forms of omega-3 supplements for treating dry . evaluated for treating infectious patients to investigate the efficacy and Ophthalmology 2017; 124: 43-52. in adults and children. safety of SHP640 in adenoviral and 2. Foulks GN, Forstot SL, Donshik bacterial conjunctivitis. PC, et al. Clinical guidelines for management of dry eye associated According to an announcement by the with Sjögren disease. Ocul Surf 2015; biopharmaceutical company Shire, SHP640 is a broad-spectrum antiseptic 13: 2: 118-132. international clinical trial sites are (povidone-iodine, 0.6%) and anti- expected to open in the third quarter inflammatory steroid (dexamethasone, of 2017. 0.1%) combination. The treatment regimen being studied for SHP640 is SYNCHRONIZE, the phase 3 trial, will one drop, four times per day, for seven include four multicentre, randomised, days. double-masked, placebo-controlled studies, with two for adenoviral www.shire.com 16 SEPTEMBER 2017

Meningococcal conjunctivitis MenW is rising

Wesley Teoh Medical Student (MBBS V)

Monash University, VIC

INFECTIOUS conjunctivitis is a commonly presenting complaint to health professionals globally.1 Most conjunctivitis presentations are viral Figure 1. Patient presents with painful watery right eye in origin, self-limiting and require only topical palliative therapy such as lubricants. We present a case of non-invasive primary meningococcal presentation, which had resolved. He chloramphenicol, ciprofloxacin conjunctivitis (PMC) identified on had no meningism (symptoms similar and penicillin). The patient was retrospective Gram-stain and culture to those of meningitis but not caused completely asymptomatic at this time. diagnosis. by meningitis), fever, rashes or other His haematology and biochemistry symptoms. Apart from hypermetropia results were normal. He commenced Australia is currently experiencing a (he wore monthly contact lenses), he topical chloramphenicol 0.5% rise in the notifications ofNeisseria had no past medical history. He was an every two hours and 2 g intravenous meningitidis serogroup W (MenW).2 active cyclist and university student ceftriaxone twice daily. Contact tracing In 2014, there were 17 notified cases who lived with his family. and chemoprophylaxis with 500 mg of MenW. That number rose to 34 ciprofloxacin was initiated and the in 2015 and 110 in 2016.3 Although On examination, unaided Snellen health authorities were notified. the most common presentation of visual acuity was 6/6 bilaterally, MenW is septicaemia, localised with mild peri-orbital swelling and The patient was discharged following disease also occurs, including septic global scleral injection in the right normal ophthalmic review and blood arthritis, epiglottitis, pneumonia and eye. Slitlamp examination revealed cultures, after receiving two days of pericarditis.2,3 Although rare, especially widespread chemosis, significant intravenous ceftriaxone. He continued in adults, PMC is potentially vision and epiphora, marked hyperaemia, a minor topical chloramphenicol for a further life-threatening.4,5 This report reminds anterior chamber reaction (0.5+ of five days. health professionals of the current cells) and follicular response in the meningococcal W outbreak in Australia affected eye. Multiple corneal punctate Discussion and the possible consequences of PMC. epithelial erosions were present. There was no periauricular lymphadenopathy Neisseria meningitidis is an uncommon and vital signs were normal. His left but potentially dangerous cause of CASE REPORT eye was unremarkable. bacterial conjunctivitis.6 PMC is extremely rare in adults, with only 20 Due to the severity of his chemosis, cases reported before 1990.4 Even in A 19-year-old Caucasian male a swab was sent for Gram-stain and children, it is an uncommon cause of presented to a metropolitan emergency culture. The intensity of conjunctival bacterial conjunctivitis, accounting for department (ED) in Victoria, having chemosis and other signs indicated only two per cent of cases in a review experienced pain and copious watery that the pathology was not related to of 1,030 children with acute bacterial discharge from his right eye for six contact lens wear, but this could not conjunctivitis.7 Meningococcal hours. He complained of a unilateral be entirely ruled out. The patient was conjunctivitis can be divided into , mild visual disturbance and discharged on topical lubricants with primary (exogenous) and secondary foreign body sensation which worsened presumed viral conjunctivitis. (endogenous) disease, where the on removal of contact lenses which had latter is a complication of systemic been in place for two weeks. Five days later, the patient was meningococcal disease.6,7 recalled to the emergency department He had experienced a coryzal (catarrhal and admitted because the culture PMC typically presents abruptly with inflammation of the mucous membrane grew Neisseria meningitidis associated burning sensation, irritation, in the nose) illness a week prior to his serotype W (sensitive to ceftriaxone, epiphora and yellow-green purulent SEPTEMBER 2017 17

discharge from the affected eye, similar Neisseria meningitidis and about one in compared to those treated with topical to Neisseria gonorrhoeae.5 Low fevers 10 is an asymptomatic carrier.8,9 It is a and systemic antibiotics.7 Doctors are present in 24 per cent of PMC cases normal flora of the oropharynx but not should consult their local infectious and enlarged regional lymph nodes of the eye.10 Prevalence and duration of diseases physician or hospital in 14 per cent.4 Our patient presented carriage vary over time and in different guidelines for specific treatment atypically and mimicked epidemic populations and age groups, with peak options. This report reminds us of the viral conjunctivitis. However, one carriage rates (> 20 per cent) occurring current MenW outbreak in Australia and raises awareness of the possibility of atypical presentations of this disease.

Optometrists in particular should consider this in any atypical anterior eye presentation and consider conjunctival swabbing as part of their work-up.

1. Daud J, Ishak SR, Deris ZZ, Hitam WHW. Excellent outcome of primary Neisseria meningitidis . Asian Pac J Trop Biomed 2011; 1: 5: 419-420. 2. Australian Government Department of Health. Meningococcal W Disease. Figure 2. Patient following two days of intravenous ceftriaxone and five days of topical Canberra: Commonwealth of Australia, chloramphenicol 2017. Accessed June 2017. Available from: http://www.health.gov.au/ internet/main/publishing.nsf/Content/ ohp-meningococcal-W.htm. case report in the ophthalmology in adolescents.3 Most meningococcal 3. Centre for Immunisation Research & Surveillance. Meningococcal vaccines literature reported a similar case with a cases in Australia tend to occur during for Australians fact sheet. New South retrospective culture diagnosis.4 early spring or winter.3 Meningococcal Wales: Centre for Immunisation bacteria are transmitted in respiratory Research & Surveillance, 2017. Accessed June 2017. Available from: http://www. There are 13 Neisseria meningitidis droplets and the risk of transmission ncirs.edu.au/assets/provider_resources/ serogroups: A, B, C, W and Y most increases with prolonged close contact fact-sheets/meningococcal-vaccines-fact- commonly cause disease.3 Until with those infected.3 sheet.pdf. 4. Andreoli C, Wiley H, Durand M, recently, serogroup B (MenB) was Watkins L. Primary meningococcal the most common cause of invasive Meningococcal disease tends to affect conjunctivitis in an adult. Cornea 2004; 23: 7: 738-39. meningococcal disease in Australia, people who are immunocompromised 5. Morrow G, Abbott R. Conjunctivitis. Am accounting for 63 per cent to 88 per (from age, disease or treatment), Fam Physician 1998; 57: 4: 735-746. cent of annual notified cases from 2006 asplenic, experiencing recent or current 6. Orden B, Martínez R, Millán R, Belloso 3 M, Pérez N. Primary meningococcal to 2015. However, in 2016 there were viral upper respiratory tract infection conjunctivitis. Clin Microbiol Infec 2003; 110 notified MenW cases, surpassing or live in crowded conditions.3 The 9: 1245-1247. MenB (92 cases).3 MenW also appears literature has also cited the extended 7. Barquet N, Gasser I, Domingo P et al. Primary meningococcal conjunctivitis: to have a higher case fatality rate than wear of contact lenses to be a risk report of 21 patients and review. Rev disease caused by other serogroups.3 factor for microbial keratitis, especially Infect Dis 1990; 12: 5: 838-847. with Gram-negative organisms.11,12,13 8. Swain CL, Martin DR. Survival of meningococci outside of the host: There are currently three types of Although not specific to conjunctivitis, implications for acquisition. Epidemiol meningococcal vaccines available this finding raises the possibility Infect 2007; 135: 2: 315-320. in Australia: meningococcal C of an increased risk of microbial 9. Centers for Disease Control and Prevention. Meningococcal Disease. conjugate vaccine, multicomponent conjunctivitis in our patient who had Atlanta: Centers for Disease Control meningococcal B vaccine had his contact lenses in place for two and Prevention, 2017. Accessed June and quadrivalent (A, C, W, Y) weeks. 2017. Available from: https://www. 3 cdc.gov/meningococcal/about/causes- meningococcal conjugate vaccines. transmission.html. It is important to note that there is no Possible local complications of 10. Davis CP. Normal flora. In: Baron single vaccine which covers all five PMC include corneal ulceration, S, ed. Medical Microbiology, 4th edn. Galveston: University of Texas common serogroups. iritis, keratitis and subconjunctival Medical Branch, 1996. Available from: haemorrhage.5,7 It can cause blindness https://www.ncbi.nlm.nih.gov/books/ NBK7617/. With the recent rise of MenW, the and progress to systemic disease (18 11. Dart JK. Predisposing factors in quadrivalent meningococcal vaccine per cent), being fatal in 13 per cent of microbial keratitis: the significance of is now being funded in Victoria, those with systemic disease.7 There is contact lens wear. Br J Ophthalmol 1988; 72: 12: 926-930. Queensland, New South Wales limited information in the international 12. Lim L, Loughnan MS, Sullivan LJ. and Western Australia in 2017 for literature regarding PMC and it raised Microbial keratitis associated with adolescents aged 15-19 years.3 Readers several questions regarding appropriate extended wear of silicone hydrogel contact lenses. Br J Ophthalmol 2002; should consult the respective state or treatment of the patient. 86: 3: 355-357. territory health department website for 13. Al-Mujaini A, Al-Kharusi N, Thakral A, further details. Patients treated solely with topical Wali UK. bacterial keratitis: perspective on epidemiology, clinico-pathogenesis, antibiotics have a 19-fold increased diagnosis and treatment. Sultan Qaboos Humans are the only natural hosts of risk of developing systemic disease Univ Med J 2009; 9: 2: 184-195. 18 SEPTEMBER 2017

Therapeutic NEWS of note

large outbreaks much more likely. Adam Glassman of the Jaeb Center Associate Professor for Health Research, in an editorial in Mark Roth JAMA Paediatrics July 24, 2017. JAMA Ophthalmology. ‘There are now doi:10.1001/jamapediatrics.2017.1695. two large, randomised clinical trials BSc(Pharmacology) that show the benefits of anti-VEGF treatment compared with panretinal BAppSc(Optom) PGCertOcTher Vision loss in Indigenous photocoagulation,’ Glassman wrote. NEWENCO FAAO OAM Australians The trials are the Diabetic The National Eye Health Survey Clinical Research Network Protocols has found that vision loss is 2.8 and the CLARITY study, which times more prevalent in Indigenous reported successful outcomes with the Australians than in non-Indigenous use of anti-VEGF injections compared Australians. with panretinal photocoagulation Consequences of declines in treatment, the standard treatment for measles vaccinations Researchers conducted a nationwide, many years. Even a small decrease in the measles cross-sectional, population-based vaccination rate could lead to a three- survey of Indigenous Australians aged ‘Anti-VEGF was a more effective fold increase in cases, a study has 40 years or older and non-Indigenous treatment for preserving visual concluded. Australians aged 50 years and function and reducing complications older. Trained examiners conducted that can accompany proliferative Researchers looked into the public standardised eye examinations, ,’ Glassman health and economic impact of including visual acuity, perimetry, wrote. ‘Despite the promising results ‘vaccine hesitancy’, which is defined slitlamp examination, intraocular of the clinical trials, the barriers for as a ‘delay or refusal to accept pressure and fundus photography. wide-spread use of anti-VEGF agents vaccination based on personal beliefs persist.’ despite availability.’ Overall, the prevalence of vision loss in Australia was 6.6 per cent: 6.5 per Glassman said compliance was a Using Center for Disease Control cent in non-Indigenous Australians barrier. In the Protocol S trial, 12 data to simulate measles, mumps and 11.2 per cent in Indigenous per cent of participants in the anti- and rubella (MMR) vaccine coverage Australians. VEGF group failed to complete all for US children aged from two to visits through two years, and nine 11 years, researchers estimated In Indigenous Australians, the leading per cent of the aflibercept group in the number of measles cases and causes of vision loss were uncorrected the CLARITY trial were also lost to associated costs that would occur (60.8 per cent), follow-up. A second barrier noted with declining vaccine coverage due cataract (20.1 per cent) and diabetic by Glassman was cost, although to non-medical reasons. retinopathy (5.2 per cent). the use of bevacizumab (Avastin) was mentioned as a cost-effective At baseline, MMR vaccine coverage The study authors concluded that alternative. was 93 per cent, and the prevalence improvement in eye health care in of non-medical exemptions was two Indigenous communities is required. JAMA Ophth 2017; Jul 1; doi: 10.1001/ per cent. This would yield 48 measles The leading causes of vision loss jamaophthalmol.2017.1652. cases annually in this age group —uncorrected refractive error and across the USA. However, if vaccine cataract—are readily treatable. Efficacy of phacoemulsification coverage dropped by five per cent, the estimated number of measles Ophthalmology 2017 Jul 6. doi: Phacoemulsification (phaco) as a solo cases would increase to 150, costing http://dx.doi.org/10.1016/j. procedure lowers intraocular pressure the public sector an additional $2.1 ophtha.2017.06.001 and reduces patient dependency on million. glaucoma medications. Anti-VEGF for diabetic retinopathy The researchers concluded that Researchers conducted a retrospective remains a strong option even minor reductions in childhood review including 32 studies and 1,826 vaccinations, driven by vaccine While it’s too early to suggest subjects with comorbid cataract and hesitancy, will have substantial public that health-care providers should primary open angle glaucoma who health and economic consequences. eliminate the use of lasers for the underwent phaco and its effect on IOP In effect, when people opt out of treatment of proliferative diabetic and the required amount of topical vaccination, they are not just making retinopathy, recent research has glaucoma medications. a personal choice, they are affecting shown anti-VEGF injections to be a their community as a whole. Because strong option for some patients. Researchers found a 12 per cent, 14 vaccine hesitant parents tend to live per cent, 15 per cent and nine per cent in clusters, it makes the potential of ‘Is it time to retire your laser?’ asked reduction in IOP from baseline at six, SEPTEMBER 2017 19

12, 24 and 36 months, respectively, diluted bleach and mechanical However, the authors also suggested after phaco. A mean reduction in cleaning all lack the ability to that combined approaches, including glaucoma medications of 0.57, 0.47, remove cellular debris completely, outdoor activities, orthokeratology, 0.38 and 0.16 was noted per patient at which is necessary to prevent prion bifocals and possibly stem cells, might six, 12, 24 and 36 months after phaco, transmission. be necessary to better prevent the respectively. progression of . It was also determined that The IOP-lowering effects appear to disinfectants can cause tonometer JAMA Ophthalmol 2017; 135: last at least 36 months, with gradual tips to swell and crack by dissolving 6: 624-630. doi: 10.1001/ loss of the initial effect noted after the glue that holds the hollow tip jamaophthalmol.2017.1091. two years, according to researchers. together. The cracks can irritate the cornea, harbour microbes or allow Contact lenses risk no higher in A large variance in IOP reductions disinfectants to enter the interior of children after phaco, ranging from 1.2 per cent the tonometer tip. to 29.4 per cent, was also observed. The number of adverse effects in The researchers suggested that The report recommends the use contact lens wearing patients under the difference may be the result of of sodium hypochlorite against the age of 18 years is no higher than surgical variations or by differences adenovirus and HSV but encourages in adults, according to a review in patient characteristics between as clinicians to regularly check their published in Optometry and Vision well as within the studies. tonometers for signs of damage. Science.

The review implies that if phaco can Ophthalmology 2017; Jul 11. Authors of the research review temporarily reduce the medication doi: http://dx.doi.org/10.1016/j. collated data from a range of burden on patients, it may open a ophtha.2017.05.033 studies to estimate the incidence of window during which there is a complications in patients younger reduced risk of glaucoma surgical than 18 years. The analysis focused For children, low dose atropine is failure. on signs of corneal infiltrative events better than high dose measured against ‘patient years’ of soft J Glaucoma 2017; 26: 6: 511–522. doi: Low-dose atropine showed equal contact lens wear. 10.1097/IJG.0000000000000643 efficacy in slowing myopia progression in children and had fewer side-effects In three large prospective studies than higher doses, according to a large representing between 159 and 723 Disinfecting your tonometer meta-analysis of published studies. patient years of soft contact lens Sodium hypochlorite (diluted bleach) wear in patients eight to 14 years, the offers effective disinfection against Researchers reviewed 720 studies incidence of corneal infiltrative events adenovirus and herpes simplex virus that included 3,137 children younger was low: 136 per 10,000 years. (HSV) for tonometers, a report by the than 18 years; 268 were Asian, 201 American Academy of Ophthalmology were Caucasian. Studies with high, Data from a large retrospective study states. moderate and low-dose atropine showed similarly low rates of corneal (0.01%) were included, and while infiltrative events: 97 per 10,000 years Literature searches were conducted efficacy was not dose-dependent, side- in eight- to 12-year-olds (based on in the PubMed and the Cochrane effects were shown to be positively 411 patient years of wear) and 335 Library databases for original research correlated with concentration. per 10,000 years in 13- to 17-year- investigations. After exclusion criteria olds (based on 1,372 patient years were applied, 10 laboratory studies The pooled data showed significantly of wear). None of the prospective remained for this review. less progression of myopia with all studies reports any cases of microbial doses of atropine compared with keratitis. The infectious agents covered in control groups, but no correlation was the assessment were adenovirus found between dose and treatment One retrospective study found no 8 and 19, HSV 1 and 2, human effect. Ethnicity also had no impact on cases of microbial keratitis occurred immunodeficiency virus 1, hepatitis the effect of treatment. in eight- to 12-year-olds (411 patient C virus, enterovirus 70, and variant years) and an incidence of 15 per Creutzfeldt-Jakob disease. A total of 308 adverse events occurred 10,000 patient years in 13- to 17-year- in 2,425 patients in the atropine olds (1,372 patient years), which is no All four studies of adenovirus groups (12.7 per cent). The difference higher than the incidence of microbial 8 concluded that after sodium with control groups was statistically keratitis in adults wearing soft contact hypochlorite disinfection, the virus significant. The most common effects lenses on an overnight basis. was undetectable, but only two of the were , poor vision at four studies found that 70% isopropyl near and allergy, and their incidence Although they emphasised the need alcohol (alcohol wipes or soaks) significantly increased with dose for further research, the authors eradicated all viable viruses. escalation. concluded that the overall data support the safety of prescribing All three HSV studies concluded that The study authors recommended the contact lenses for children. both sodium hypochlorite and 70% use of the lowest dose of atropine isopropyl alcohol eliminated HSV. (0.01%) for therapy, but called Optom Vis Sci 2017; 94: 6: 638–646. Ethanol, 70% isopropyl alcohol, for clinical trials with that dose. doi: 10.1097/OPX.0000000000001078 20 SEPTEMBER 2017

Did cataract surgery cause this patient’s NAION?

The patient’s one-day post-operative visual field. On further questioning, Andy Mackner BA examination was unremarkable. his wife reported that he had noticed Post-operative medications included a spot in his vision prior to his first Pacific University College of prednisolone acetate 1%, moxifloxacin cataract extraction but they had not Optometry, Forest Grove, Oregon 0.5% and ketorolac 0.5% four times thought anything of it and attributed it a day in the right eye. Uncorrected to his cataracts. acuities were 6/18-1 in the right eye Dr Leonid Skorin Jr and 6/18+2 in the left eye. IOPs were Because of this new complaint, the OD DO MS FAAO FAOCO 17 mmHg right eye and 16 mmHg left left eye surgery was cancelled and the eye. The right eye anterior chamber patient was re-evaluated. His right eye Department of Ophthalmology, was deep with trace cells, posterior acuity was 6/21 with pinhole 6/12. Mayo Clinic Health System, Albert chamber IOL was stable, surgical The IOP was 11 mmHg in the right Lea, Minnesota incisions were intact and there was no eye. Pupils were equal, round, reactive Siedel sign. to light with no relative afferent pupillary defect. Confrontation The patient was then scheduled for fields suggested a superior field left eye cataract surgery the following defect in the right eye. Anterior week. segment findings were unremarkable. Fundus examination revealed CASE REPORT As the patient was being checked inferior-nasal oedema and into surgery the following week, he no haemorrhages (Figure 1). The reported that the vision in his right computerised visual field exhibited A 78-YEAR-OLD Caucasian male eye seemed to be better but he was a superior altitudinal defect. The left presented for a cataract evaluation. unable to see anything in his superior eye visual field was unremarkable. The patient reported that his right eye seemed worse than his left eye. The patient was scheduled for cataract extraction of the right eye. His ocular history was significant for cataracts and blepharoplasty. His medical history was significant for Alzheimer’s disease with moderate dementia.

At the initial visit, the patient’s best corrected acuities were 6/45-2 in the right eye and 6/20+2 in the left eye. Pupil testing was unremarkable and his eye movements were full, as were confrontation fields. His intraocular pressures (IOPs) were 13 mmHg in both eyes. Slitlamp examination of the right eye revealed grade 2+ nuclear sclerotic and grade 2+ posterior subcapsular cataracts. The left eye exhibited grade 2+ nuclear sclerotic and grade 1+ posterior subcapsular cataracts. All other anterior structures were unremarkable. The posterior examination of both eyes revealed maculae that were flat and dry, optic nerves were perfused with a cup- to-disc ratios of .45 x .45 and the peripheral retinas were flat and intact. The patient underwent successful phacoemulsification cataract surgery with a posterior chamber IOL in the Figure 1. Fundus examination revealed inferior-nasal optic disc oedema and no right eye. haemorrhages SEPTEMBER 2017 21

Although the patient denied any jaw visually devastating complications.6 history of NAION who are interested claudication, scalp tenderness or Past studies have shown that there in undergoing cataract surgery, we temple pain, we proceeded to order is an increased risk of NAION with should educate them on the associated an erythrocyte sedimentation rate cataract surgery that is greater than risks and reassure them that risk of and C-reactive protein (CRP) test to the overall incidence of NAION.7 The NAION in the fellow eye is low.9 rule out temporal arteritis. Laboratory incidence of NAION following cataract Ultimately, it is the patient’s decision results showed an elevated ESR at 62 extraction is estimated to be one case to undergo cataract surgery but it mm/hr and a normal CRP at 4.2 mg/L. per 2,000 whereas studies suggest is our responsibility to be aware of that the overall incidence of NAION associated risks and provide guidance We started the patient on 60 mg oral is 2.3 to 10.2 cases per 100,000 in to our patients. prednisone daily and scheduled a individuals over the age of 50 years.7,8,9 temporal artery biopsy. The temporal artery biopsy was negative for However, a more recent study inflammation. The oral prednisone suggests that the increased incidence 1. Tamhankar M, Volpe N. Nonarteritic anterior ischemic : was discontinued and the patient was may reflect the use of retrobulbar Clinical features and diagnosis. In: diagnosed with non-arteritic anterior and peribulbar anaesthesia as well UpToDate, Post TW (Ed), Waltham, MA. ischaemic optic neuropathy (NAION). as intracapsular and extracapsular Accessed June 26, 2017. 2. McCulley T, Lam B, Feuer W. A The patient was then scheduled for surgical techniques. More comparison of risk factors for cataract extraction of his left eye. The modern surgical methods using postoperative and spontaneous patient underwent successful cataract phacoemulsification and topical nonarteritic anterior ischemic optic neuropathy. Neuro-Ophthalmology extraction of the left eye with no anaesthesia have a prevalence and 2005; 25: 22-24. complications. incidence of NAION after cataract 3. Hata M, Oishi A, Muraoka U, et al. surgery comparable to that of the Structural and functional analyses in nonarteritic anterior ischemic general population.9 Additionally, this Discussion optic neuropathy: Optical coherence study found no evidence suggesting tomography angiography study. Neuro- This case report highlights the that patients who have experienced Ophthalmology 2017; 37: 140-148. 4. Kabat AG, Skorin L: Nonarteritic importance of listening to our patients NAION in one eye have an increased ischemic optic neuropathy. In: and the rare association of cataract risk of NAION following a non- Onofrey B, Skorin L, Holdeman N, extraction and non-arteritic anterior complex cataract surgery in the fellow eds. Ocular Therapeutics Handbook: A 9 Clinical Manual, 3rd ed. Philadelphia: ischaemic optic neuropathy. eye. Lippincott Williams & Wilkins, 2011; 591-593. Anterior ischaemic optic neuropathy Although new evidence suggests that 5. McCulley T, Lam B, Feuer W. Nonarteritic anterior ischemic optic can present in two forms: arteritic concern for NAION in the fellow neuropathy and intraocular surgery. (AAION) and non-arteritic (NAION).1,2 eye following cataract surgery may Amer J Ophthalmol 2017; 175: 14-16. Although the exact mechanism is still be unwarranted, it is still important 6. Ruskiewicz J. NAION follows cataract surgery. Review Optometry 2013; 18: 58- unclear, it is thought that NAION is a to educate our patients about the 70. result of acute ischaemia to the optic associated risks. Currently, there is 7. MCulley T, Lam B, Feuer W. Incidence 1-3 of nonarteritic anterior ischemic optic disc. NAION is characterised by a no treatment for NAION. Surgical neuropathy associated with cataract sudden, painless decrease in vision intervention with optic nerve sheath extraction. Ophthalmology 2001; 108: with optic disc oedema and visual decompression has been shown to be 1275-1278. 1,3 10,11,12 8. Lam B, Jabaly-Habib H, Al-Sheikh, et al. field defect. The most common ineffective and possibly harmful. Risk of non-arteritic anterior ischemic visual field defect is an altitudinal Other strategies to improve recovery optic neuropathy (NAION) after cataract defect. However, other common visual by improving perfusion to the nerve extraction in the fellow eye of patients with prior unilateral NAION. Brit J field defects may include central, have been attempted. Aspirin, Ophthalmol 2007; 91: 585-587. centrocecal, arcuate or a generalised vasodilators, systemic steroids and 9. Moradi A, Kanagalingam S, Diener-West depression.1 intravitreal steroids have been trialled M, Miller N. Post-cataract surgery optic neuropathy: prevalence, incidence, but none has been proved to be temporal relationship, and fellow eye The patient may also present effective.12 involvement. Amer J Ophthalmol 2017; with reduced visual acuity, 175: 183-195. 10. Yee R, Selky A, Purvin V. Outcomes of dyschromatopsia, afferent pupillary During the patients pre-operative visit optic nerve sheath decompression for defect, peripapillary splinter there were no signs of ischaemic optic nonarteritic ischemic optic neuropathy. J haemorrhages and a small or neuropathy. The patient explained Neuro-Ophthalmology 1994; 14: 70-76. 11. Dickerson K, Everett D, Feldon S, et crowded disc, also referred to as to us that he noticed the decrease al. Optic nerve decompression surgery a disc at risk.4,5 Other risk factors in vision after the initial visit, prior for nonarteritic anterior ischemic optic include sleep apnoea, diabetes to his first eye cataract surgery, and neuropathy (NAION) is not effective and may be harmful. J Amer Med Assoc mellitus, hypertension and attributed the change in his vision 1995; 273: 625-632. hypercholesterolaemia.4 In addition, to the cataracts. However, after right 12. Atkins EA, Bruse BB, Newman NJ, Biousse V. Treatment of non-arteritic the use of phosphodiesterase-5 eye cataract surgery, he became more ischemic optic neuropathy. Survey inhibitors and amiodarone have been aware of the visual field defect in Ophthalmol 2010; 55: 47-63. associated with the development of his right eye and brought it to our NAION.5 attention prior to the surgery in his left eye. A less commonly cited association with NAION is cataract extraction. In this case the NAION was not The association with cataract surgery related to the cataract surgery but is rare but it is also one of the most when consulting our patients with a 22 SEPTEMBER 2017

Three steps for third nerve palsy: pupil, palsy and patient

patient’s unique presentation, the risk aneurysm causing third nerve palsy. Dr Brandon Powell OD of aneurysm may be much higher or The ‘rule of the pupil’ is widely taught lower in any given situation. as the best way to determine if an Nova Southeastern University, aneurysm should be suspected. College of Optometry Microvascular diseases, such as diabetes and hypertension, should Pupil fibres superficially travel with Florida USA be of concern if they are causing cranial nerve three and are vulnerable neurological deficits but an aneurysm- to compression from an aneurysm of Professor Joseph Sowka causing compression on cranial nerve the posterior communicating artery. OD FAAO DIPL three is a cause of grave concern. The These fibres are relatively immune 30-day mortality for patients with to ischaemia from vascular infarct. Director, The Glaucoma Service subarachnoid haemorrhages caused However, this rule should apply only Nova Southeastern University, by cerebral aneurysm rupture is as in cases of complete third nerve palsy. College of Optometry high as 45 per cent.2 Knowing the Florida USA proper approach for this condition Lee et al describe situations where is paramount for any eye-care the ‘rule of the pupil’ cannot be practitioner. applied. If a patient has an incomplete third nerve palsy, which the authors describe as at least one extra ocular Step 1: Look at the PUPIL muscle having some function left or The earlier mentioned patient had an incomplete , then the rule can AN 89-YEAR-OLD man with poorly- his pupils thoroughly examined for no longer be applied and the patient controlled diabetes presented with reaction to light directly, indirectly, should be worked up for a possible complaints of constant for and an afferent pupillary defect was aneurysm. Additionally, third nerve the past few days. The patient’s right searched for; the same testing was done palsies in conjunction with other lid had partial ptosis and his extra when the patient returned two weeks non-ocular, neurological symptoms ocular motility in that eye was limited later. On both visits, it was thoroughly should no longer have the rule applied. in adduction, supraduction and confirmed that both pupils were briskly In addition, evidence of aberrant infraduction. reactive to light directly and indirectly, regeneration means that the rule cannot and that there was no afferent pupillary be applied.3 This was all consistent with incomplete defect in either eye. third nerve palsy. Neuroimaging was If complete third nerve palsy is obtained through the patient’s primary Thus, he had ‘pupil sparing’. Pupil present, then the rule can be cautiously care physician but the study was testing is an important way to applied to determine if an aneurysm inadequately directed. Nevertheless, no determine the amount of suspicion should be suspected. If the pupil evidence of aneurysm or subarachnoid there should be for a suspected reacts poorly to light, the patient haemorrhage was seen. Two weeks later, the patient returned for follow- up with now complete ptosis and no movement in any of the previously mentioned gazes.

When a patient presents with a suspected palsy of cranial nerve three, it is imperative that the clinician knows all of the appropriate steps that need to be taken. There is almost no case where this condition does not suggest some underlying pathology that is either morbid or even mortal.

A recent study found causes of a third nerve palsy to be microvascular (42 per cent), trauma (12 per cent), neoplasm (11 per cent), post-surgery (10 per cent) and aneurysm (six per cent).1 Be aware that given an individual Figure 1. Ptosis in complete third nerve palsy. SEPTEMBER 2017 23

should be immediately evaluated for non-invasive method of detecting an Microvascular aetiology should not an aneurysm. However, Kissel et al aneurysm is computerised tomography be strongly suspected in patients not determined that it is not enough to look angiography and should be the first having ischaemic vascular diseases. only for presence of pupillary light test that is requested. However, if the reaction.4 If there is partial internal patient is pregnant or is a child, they In a study conducted by Watanabe et al, dysfunction of the pupil with greater should not be exposed to the radiation on patients with cranial nerve three or than 1 mm of or diminished and magnetic resonance angiography is four palsies caused by diabetes, about light reaction, then the suspicion of the next best option.5 70 per cent of these patients had other aneurysm should be high. diabetic complications elsewhere in Non-invasive scans may be performed the body.7 A microvascular aetiology or interpreted incorrectly. It is warrants referral to the patient’s Step 2: Look at the PALSY imperative to explain to a radiologist primary care physician because Patients with complete third nerve all thoughts on potential aetiologies so hypertension, diabetes or high palsy without pupil involvement have that the neuroimaging may be properly cholesterol that may be damaging a a low risk of harbouring an intracranial directed. Still, errors can be made. If cranial nerve is probably affecting other aneurysm. If the palsy is incomplete the nerve and artery are anatomically organ systems elsewhere in the body. or partial, then the patient should far apart in a patient, then a very large It may also be prudent to recommend be suspected of having a developing aneurysm may be required to cause erythrocyte sedimentation rate and aneurysm regardless of the pupillary compression. C-Reactive Protein testing, since giant state. Patients with partial third nerve cell arteritis (GCA) can cause pupil palsy can have a pupil that is initially Conversely, if the artery and nerve sparing third nerve palsy that would normal in function. Any partial third are anatomically close together in a mimic a microvascular aetiology.8 nerve palsy must be emergently patient, then a very small aneurysm evaluated for aneurysm regardless of which may be missed on non-invasive Improvement of microvascular palsy pupillary state. imaging may result in palsy. In should be seen over time and the cases where an aneurysm is strongly condition should resolve nearly If a patient falls into any of the suspected and non-invasive scans completely. However, if there is previously mentioned categories provide no evidence of such, digital no improvement over time, then where an aneurysm is suspected, then subtraction angiography is required.6 a different aetiology should be neuroimaging needs to be done. To be considered. Presumed microvascular on the safe side, even complete third palsy may progress throughout one Step 3: Look at the PATIENT nerve palsy with absolutely no pupil week and be unimproved at two involvement may be best served by Age and related medical conditions weeks. However, microvascular palsy ruling out an intracranial aneurysm. are important when assessing patients will not get progressively worse over Although extremely rare, it has been with third nerve palsy. Microvascular the course of two weeks. Patients published that pupillary involvement palsies are uncommon in younger with presentation and risk factors may not appear in the case of an patients and alternative aetiologies characteristic of microvascular palsy aneurysm until four months after initial should be considered in patients can still have alternative causes, such paresis.4 younger than 50 years. While older as neoplasm, inflammation, GCA and age and the presence of ischaemic brain stem infarction.9 The most common place for an vascular diseases such as diabetes, aneurysm causing a third nerve palsy is atherosclerosis, hypercholesterolaemia An aetiology for the earlier mentioned on the posterior communicating artery, and hypertension, favour patient was determined most likely to because the nerve runs parallel to this microvascular aetiology, they are be microvascular due to his type 2 artery. The most sensitive and reliable not protective against an aneurysm. diabetes. The patient was taking insulin four times a day, was on maximum metformin therapy, and his medical records revealed blood sugar values that fluctuated from around 400 and dipped down to about 40. The patient was also being medically managed for hypertension and high cholesterol. The patient’s primary care physician was alerted and recommended to follow tighter diabetic control. The patient was informed that he should wear a patch over his affected eye to relieve the diplopia and that his condition should resolve within three months. Within several weeks, his palsy recovered.

Figure 2. Pupil test determines a suspected aneurysm causing third nerve palsy Continued page 24 24 SEPTEMBER 2017

the appropriate practitioners, while palsies with internal carotid: posterior keeping other aetiologies in mind. communicating artery aneurysms. Ann Third-nerve palsy Neurol 1983; 13: 1: 149-154. 5. Chaudhary N, Davagnanam I, Ansari From page 23 SA, et al. Imaging of intracranial Acknowledgement aneurysms causing isolated third cranial nerve palsy. J Neuro-Ophthalmol 2009; The authors thank Dr Sherrol Reynolds 29: 3: 238-244. for her contribution to this article. 6. Elmalem VI, Hudgins PA, Bruce BB, Conclusion et al. Underdiagnosis of posterior communicating artery aneurysm in If a patient presents with third nerve non-invasive brain vascular studies. J palsy, three steps should ensure that 1. Fang C, Leavitt JA, Hodge DO, et al. Neuro-Ophthalmol 2011; 31: 2: 103-109. Incidence and etiologies of acquired 7. Watanabe K, Hagura R, Akanuma Y, et the patient receives the appropriate third nerve palsy using a population- al. Characteristics of cranial nerve care needed to rule out any cases of based method. JAMA Ophthalmol 2017; palsies in diabetic patients. Diabetes aneurysm aetiology and that other 135: 1: 23-28. Research Clin Practice 1990; 10: 1: 19- 2. Broderick JP, Brott TG, Duldner JE, et 27. aetiologies are properly managed. al. Initial and recurrent bleeding are 8. Thurtell MJ, Longmuir RA. Third nerve If an aneurysm is suspected, urgent the major causes of death following palsy as the initial manifestation of giant hospital evaluation with appropriate subarachnoid hemorrhage. Stroke 1994; cell arteritis. J Neuro-Ophthalmol 2014; 25: 1: 1342-1347. 34: 3: 243-245. neuroimaging is required. If 3. Lee AG, Hayman LA, Brazis PW. The 9. Tamhankar MA, Biousse V, Ying G-S, aneurysmal cause is completely ruled evaluation of isolated third nerve palsy et al. Isolated third, fourth and sixth out and there are microvascular risk revisited. Survey Ophthalmol 2002; 47: cranial nerve palsies from presumed 2: 137-157. microvascular versus other causes: a factors, then patients should have 4. Kissel JT, Burde RM, Klingele TG, prospective study. Ophthalmology 2013; these systemic diseases managed by Zeig HE. Pupil-sparing oculomotor 120: 11: 2264-2269.

Clinical Quiz From page 12

DIAGNOSIS: Large conjunctival dermoid Conjunctival dermoid can occur as an isolated lesion, as in this case, or it can be a component of Goldenhar syndrome.

Dermoids can appear in various sizes as a yellow-white limbal mass, usually inferotemporally but can appear in other meridians. The size can range from 2 mm to 15 mm in diameter, and 0 to 10 mm in thickness. Fine white hairs often protrude from the lesion as well as a yellow-white lipid line in the adjacent corneal stroma. Conjunctival lesion Image: Associate Professor Mark Roth Larger dermoids can cause ocular surface irritation and . surgical removal can be considered. Jerry A Shields and Carol L Shields. Eyelid, Histopathologically, dermoid is lined Reasons for surgical removal include: Conjunctival and Orbital Tumors: An Atlas and Textbook, 3rd edition. Wolters Kluwer by stratified squamous epithelium secondary astigmatism, encroachment 2016. and deep to the epithelium is dense on visual axis, ocular surface irritation, collagenous tissue. dellen formation and cosmetic reasons.

For excision a combination of lamellar Management keratoplasty, amniotic membrane Smaller, asymptomatic lesions can be and stem cell replacement can be observed and monitored. Alternatively, employed. Achieve success in your own time

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