Ophthalmology

This is the first reported case of eye injury caused by a crayfish antenna. A 20-year-old male crayfish diver sustained a scleral penetrating injury that led to a subconjunctival abscess. The foreign body was histologically similar to a crayfish antenna. Clinical recordA 20-year-old man who worked as a crayfish diver presented with a unilateral red watery right eye, initially developed while at sea. He was unable to continue diving, mainly due to photophobia, and on reaching land he was transferred to our hospital by aeroplane. He had no other medical history, regular medications or known allergies. Seven days before presentation, he had a sore ear on the right side with pain radiating down the right hand side of his neck and discharge from his nose; however, he denied any trauma to the right eye. At first presentation to the ophthalmologist, the patient’s vision was 6/36 in the right eye and 6/6 in the left eye. The vision in his right eye improved to 6/18 with pinhole. He had peripheral corneal superficial punctate keratitis, 3+ cells in the anterior chamber, and an elevation of the conjunctiva, inferior to the cornea, 4.5 mm in diameter. Examination of his retina was unremarkable, and he had a small nasal pterygium. Examination of the left eye was unremarkable. The intraocular pressure was 12 mmHg in both eyes. He was provisionally diagnosed with right eye acute anterior uveitis and treated with topical combined prednisolone acetate plus phenylephrine hydrochloride and 2% homatropine hydrobromide for mydriasis. At a scheduled review 2 days later, the condition of the patient’s right eye had deteriorated. He had developed an anteriorly discharging subconjunctival abscess. A combined duplex B-scan ultrasound showed a hypoechoic, hypervascular soft tissue mass in, or external to, the sclera and inferomedial to the iris. The mass was 7 mm deep and protruded into the globe. The patient was immediately taken to the operating theatre for excision and exploration of the subconjunctival abscess. Intraoperative examination with a three-mirror gonioscope lens before excision showed an unidentifiable membranous foreign body in the anterior chamber. The foreign body was thought to be a parasite due to its longitudinal appearance. The subconjunctival abscess was excised and explored. A tract from the subconjunctival space through the full scleral thickness and into the suprachoroidal space was explored, and the foreign body in the anterior chamber was removed. The scleral defect was covered with a conjunctival graft using cardinal and continuous sutures, and the overlying conjunctival defect was repaired with a conjunctival rotation. After surgery, the patient was treated with intravenous ceftriaxone and topical ofloxacin (3 mg/mL), prednisolone acetate plus phenylephrine hydrochloride and homatropine hydrobromide eye drops, oral analgesia, and three doses of albendazole (400 mg). Two days after surgery, the patient’s condition had improved considerably. His vision had improved to 6/6 with pinhole and the anterior chamber cells had improved to 2+ cells. He was discharged home on topical prednisolone acetate plus phenylephrine hydrochloride, ofloxacin and homatropine hydrobromide eye drops, and oral prednisolone. Histological results of the initial surgical specimen showed a small foreign body several microns in length intimately associated with deeply placed active inflammation (Box 1). There was fibroproliferative activity and an infiltrate of inflammatory cells; these were associated with tissue necrosis and microabscess formation. Culture of a preoperative conjunctival swab was negative for microbial growth. Three days after discharge, the patient returned to hospital with a recurrence of the subconjunctival abscess, for which he underwent emergency excision and drainage. The previous conjunctival graft was found to be necrotic and was removed. A thin fibrovascular membrane covering the scleral defect remained. A swab taken from the abscess was later positive for methicillin-resistant Staphylococcus aureus, which was successfully treated with intravenous vancomycin, oral clindamycin and topical chloramphenicol. At 4 weeks’ follow-up, the patient’s unaided visual acuity in his right eye had stabilised to 6/6 and the intraocular pressure was 7–8 mmHg. The conjunctival flap had dehisced, but there was a steady increase in the thickness of the fibrovascular covering of the scleral perforation. The original foreign body extracted during surgery was compared with a specimen of crayfish antennae and was found to be similar to the very outer layer (1–2 μm) of the specimen (Box 2). It most likely became lodged while the patient was handling crayfish or cleaning the crayfish tank without eye protection, which he was doing extensively during the morning that the red eye developed. The crayfish caught in this area are of several different types, although the Panulirus ornatus (tropical rock lobster) make up 95% of the local population.1 DiscussionPenetrating eye injuries (PEIs) are serious and can result in blindness, either from direct trauma to ocular structures or secondary infection and inflammation. Given the significant consequences of PEIs, early diagnosis and management is imperative to improve outcomes. Despite the vast majority of PEIs being obvious through history of trauma, some are less evident to both patient and clinician. We report a case of delayed diagnosis of PEI due to a unique mechanism. A population-based cross-sectional study of eye injuries in Australia found that they were more common among men, people living in rural areas and tradespeople, and that the workplace was the most common location where the injury occurred.2 A review of 109 penetrated or ruptured globes over 4 years in Adelaide identified that 80% of patients were men; the most common cause of injury was hammering metal; and the final visual acuity was 6/12 or better in 40% of patients, and no perception of light in 26%.3 Another group found that of 6308 patients treated at their hospital over 12 months, 6% were admitted for severe ocular trauma and most of these were young men.4 Workplace-related injuries accounted for 44% of all ocular injuries and 19% of cases of severe eye trauma.4 The annual medical costs for eye injuries have been estimated at $155 million for the projected 116 000 cases nationwide.4 In 2006, a review of ocular trauma over 7 years at Cairns Base Hospital estimated the incidence of open globe injuries to be 3.7 per 100 000 population.5 An international study of PEIs in rural areas found that 16% were work-related.6 Most injuries were related to maintenance or repair work, wood chopping or machine use. A small number were the result of cow butting. At the final review, 64% of these eyes were blind.6 Crayfish antenna penetration is a mechanism that has not previously been reported. Thorns of plants have been previously reported to cause PEIs in children, but the most common aquatic-related PEI is with fish hooks.7,8 Appropriate preventive measures, such as use of protective eyewear and implementation of education programs, could be effective in decreasing the incidence of these aquatic-related ocular traumas. Our case highlights the risk of PEIs for young men while at work. It demonstrates the possibility of a small organic foreign body associated with a large inflammatory reaction and a full-thickness scleral penetration that could have resulted in blindness. For clinicians, our case emphasises the importance of early suspicion of ocular trauma in high-risk patients with red eyes, particularly if they have an unexpected lack of response or deterioration to treatment despite an initially negative history of trauma. 1 Tip of crayfish antenna (arrow) embedded in scleral tissue 2 Tip of crayfish antenna

Khoi A Tran MMed, MB BS, BPharm · Matthew Green MSc, MB BS · Jayne Camuglia BSc, MB · Stephen O'Hagan MB BS, FRANZCO

A 21-year-old man sustained a high-velocity injury to his right orbit by a sinker, when his fishing line suddenly released from trapped vegetation. There was a medial canalicular laceration and inferonasal conjunctival entry wound with fishing line visible. Computed tomography showed the sinker lodged medially, deep within the orbit. There were no fractures and the globe appeared intact. In theatre, the sinker was pulled through the conjunctival laceration using the fishing line and the canaliculus repaired. Visual acuity was 6/6 postoperatively. There was a small nasal choroidal rupture and vitreous haemorrhage, but no major visual sequelae.

Esra Sanli · Jennifer J Danks

Bitôt spots on the temporal limbus of the left eye. Clinical record A 52-year-old man presented to the ophthalmology clinic with a 3-day history of tunnel vision and night blindness (nyctalopia). He reported recently needing to wear a headlamp to see adequately in dim lighting, notably while walking to work early in the morning. The patient had a history of hypertension, hypercholesterol-aemia and osteoporosis. Twice in the previous 2 years, he had seen a neurologist for symptoms of lower limb paraesthesiae. At that time, peripheral nerve conduction studies gave normal results, and a clinical diagnosis of peripheral neuropathy secondary to vitamin B12 deficiency was made. Supplement-ation with vitamin B12 and folate was initiated. He also described a 3-hour episode of tunnel vision 2 years before presentation. Examination revealed an otherwise well man with a body mass index of 27 kg/m2 (ie, in the overweight range). His best corrected visual acuity was 6/18 in the right eye and 6/9 in the left eye. Anterior segment examination revealed Bitôt spots, and no staining of the cornea with fluorescein (Figure). Pupillary examination revealed a relative afferent pupillary defect of the right eye, grade 1/4, indicating optic nerve dysfunction. Ocular motility and intraocular pressures were normal. Fundoscopy showed normal peripheral retinal pigment epithelium, and normal discs and maculae. Humphrey visual field 30-2 testing showed peripheral field constriction. Results of the Farnsworth–Munsell D-15 hue test for colour vision were normal. Dark-adapted electroretinography (ERG) revealed bilaterally decreased amplit-udes, consistent with diminished rod function. The results of computed tomography and magnetic resonance imaging of the brain and orbits, using contrast, were normal. Investigations revealed a markedly reduced vitamin A level (0.1 μmol/L [reference range, 1.4–4.0 μmol/L]). Results of a full blood count, electrolyte levels, and liver and thyroid function tests were normal. Vitamin B12 and folate levels were high, consistent with supplementation. Normal results of a malabsorption screen, including levels of fat-soluble vitamins D, E and K, were obtained, and test results for parietal cell and intrinsic factor antibodies were negative. Test results for antiendomysial antibodies were also negative, as were those for IgA and IgG antigliadin antibodies, thus excluding coeliac disease. A diagnosis of xerophthalmia (dryness of the conjunctiva and cornea) was made on the basis of the symptoms of nyctalopia and tunnel vision, as well as the findings of bilateral Bitôt spots, hypovitaminosis A, and diminished rod function on ERG. A dietary history revealed that the patient had suffered from self-diagnosed food intolerance for most of his life. Since childhood, his diet had consisted exclusively of potatoes, white bread (but he refused to eat butter and margarine) and cola. He described nausea and vomiting after eating any other foods. The patient underwent multidisciplinary evaluation by a neurologist, gastroenterologist, psychiatrist, dietitian and psychologist. Dietary supplements were prescribed and cognitive behaviour therapy was initiated. The patient was treated with 100 000 IU of vitamin A daily, given orally for 3 days, followed by 50 000 IU for 14 days. Humphrey visual field results and visual acuity improved within 3 days of commencing treatment. After a month, visual acuity had improved to 6/6 in both eyes, and the Bitôt spots had completely resolved. The relative afferent pupillary defect was no longer present, and Humphrey visual field and ERG results had returned to normal. Vitamin A deficiency is a systemic illness which can increase an individual’s risk of blindness, severe infections and mortality.1 It is rare in developed countries like Australia.2 Vitamin A deficiency and xerophthalmia in developed countries are reported in patients with malabsorption syndromes or liver disease, in those who have had major gastrointestinal surgery, in people with alcoholism, and in those with anorexia nervosa and other psychiatric disorders.3-6 This case is unique because our patient did not have any of these risk factors. This case also highlights the importance of clinicians regularly taking a thorough dietary history, especially in the context of other indications of nutritional insufficiencies, such as osteoporosis and previous vitamin B12 deficiency. Vitamin A is a fat-soluble vitamin found as retinol in dairy products and as provitamin A carotenoids in some fruits and green leafy vegetables.1,7-9 The first clinical evidence of vitamin A deficiency often occurs in the visual system and produces xerophthalmia. The ocular changes of xerophthalmia generally occur in a predictable pattern, as described by the World Health Organization.1 The first stage of xerophthalmia is nyctalopia, the result of defective regeneration of retinal rhodopsin.3,7-9 This responds rapidly to vitamin A therapy, and patients often report regaining their scotopic vision (vision under low-light conditions) within 24–48 hours after the initiation of treatment.1,3,7,9 The second stage of xerophthalmia is conjunctival xerosis, or drying, in which loss of goblet cells and squamous metaplasia of the conjunctiva occur.1,3,7,8 Bitôt spots are bilateral triangular patches of keratinised epithelium at the temporal limbus and nasal limbus of the eye.1,3,7 Colonisation of these patches by saprophytic bacilli, including Corynebacterium xerosis, results in a foamy appearance.1,3,8 Conjunctival xerosis and Bitôt spots respond to vitamin A therapy in 1–5 days.1,3 Corneal xerosis occurs primarily because of instability of the tear film as goblet cells are lost, with subsequent keratinising metaplasia of the ocular surface.1,8 Corneal xerosis usually responds to vitamin A therapy in 1–2 weeks.1,9 If left untreated, corneal drying can result in ulceration, with subsequent scarring and keratomalacia.2,3,7,8 Keratomalacia is a rapidly progressive and irreversible liquefactive necrosis of the cornea that can ultimately lead to perforation and spontaneous loss of intraocular contents.1,3,9 Lessons from practice Vitamin A deficiency is rare in developed countries like Australia. Visual symptoms can often be the first manifestation of a systemic illness, such as vitamin A deficiency. A thorough nutritional screen, including a dietary history, is essential, especially in the context of any other nutritional deficiency. Early diagnosis and treatment of vitamin A deficiency can be curative, preserving vision and life. Uncommonly, in vitamin A deficiency, the xerophthalmic fundus exhibits yellow and white dots peripherally, sometimes associated with a corresponding scotoma (area of diminished vision).1,7-10 These changes respond well to treatment, often returning to normal within 2–4 months.1,8,9 The diagnosis of vitamin A deficiency is made by a directed history and clinical findings, and confirmed by the presence of a low serum vitamin A level, and an abnormal electroretinography result.3,9 In our case, the diagnosis was made accordingly, and all the pathological findings resolved with vitamin A therapy. Vitamin A deficiency is usually treated by administering 200 000 IU of vitamin A orally on two successive days, followed by an additional dose 1–4 weeks later.1 Administration of intramuscular vitamin A is reserved for patients with malabsorption, or those who are unable to tolerate medications orally.1 Ocular vitamin A has not been shown to be beneficial because of the systemic nature of vitamin A deficiency.7 In summary, we present a unique case of vitamin A deficiency and xerophthalmia in an unlikely candidate for malnutrition. Hypovitaminosis A can have potentially devastating visual and systemic effects. To prevent this happening, it is important to maintain a high degree of suspicion, especially in the context of other nutritional deficiencies.

Esra Sanli MB BS, BMedSc · Edwin C Figueira MB BS, MSc, MS(Ophth) · Gaurav Bhardwaj MB BS · Stephanie L Watson MB BS, FRANZCO, PhD · Ian C Francis FRACS, FASOPRS, PhD

A 62-year-old woman with an autoimmune disease presented with panuveitis and was treated with immune suppression. She subsequently developed herpetic acute retinal necrosis and later died of herpes simplex encephalitis. Acute retinal necrosis usually occurs months to years after herpes simplex encephalitis. In our case, the ocular findings were present for 5 weeks before the encephalitis presented. To our knowledge, this is the first Australian case of acute retinal necrosis preceding herpes simplex encephalitis. (MJA 2011; 195: 87-88) Clinical recordA 62-year-old woman was referred to our hospital after a left-sided uveitis failed to respond to both topical and systemic steroids. She was known to have systemic lupus erythematosus (SLE) with severe arthritic changes, but had not received immunosuppressive therapy. She had right temporal lobe surgery in 1995 for a benign brain tumour. In 2005, she had Legionella pneumonia that had resulted in an intensive care unit admission with multiorgan failure and acute respiratory distress syndrome. As part of the diagnostic work-up during her previous admission, she was diagnosed as having herpes simplex virus (HSV) type 1 and was treated with aciclovir. She had no known history of genital or oral herpetic lesions. She had no significant ophthalmic history. When she was first reviewed at our hospital, she had been symptomatic for 3 weeks. She had been diagnosed as having an SLE-related panuveitis and had been treated with topical, regional and systemic steroids for 2 weeks with no response; on presentation, she was taking oral prednisolone, 1 mg/kg/day. She described a loss of appetite for 6 weeks but had no headache, fever, rash, pleuritic chest pain or shortness of breath suggestive of a lupus flare. Her initial visual acuity was 6/9 in her right eye and “hand movements” in her left eye. There was a relative afferent pupil defect in her left eye. Her anterior chamber had large mutton-fat keratic precipitates, fibrin and posterior synechiae — all indicative of a granulomatous uveitis. There was no fundal view of the left eye. An ultrasound scan of the left eye showed vitreous debris and a retinal detachment. Fundal examination of the right eye showed one or two small scattered intraretinal haemorrhages (Box 1, A). Fluorescein angiography (Box 1, B) of the right eye showed mild leakage from the disc and vessels in keeping with an early vasculitis. There was no view of the left fundus. An anterior chamber tap and vitreous biopsy were taken from the left eye. Bacterial and fungal microscopy and culture were negative; results of cytological analysis were normal. There was insufficient specimen for viral polymerase chain reaction (PCR). (This is a common occurrence for aqueous and vitreous samples, in which the amount taken averages 0.4 mL.) Blood sampling showed an antinuclear antigen positive to a titre of 1 : 2560 (> 1 : 160 considered positive for probable autoimmune disease), an erythrocyte sedimentation rate of 38 mm/hour (reference range, < 20 mm/hour for women aged > 50 years), and anti-DNA antibodies > 100 IU/mL (reference range, < 6 IU/mL); a white cell count and serum angiotensin-converting enzyme levels were normal, and human leukocyte antigen (HLA)-B27 was not detected. Serological testing was negative for syphilis and HIV. Blood culture was negative. An echocardiogram and chest x-ray were non-contributory. At this stage, the differential diagnosis included a masquerade syndrome (intraocular lymphoma), active lupus, or an endogenous, infective endophthalmitis. The patient was referred for a surgical vitreous biopsy. She continued to take oral prednisolone 1 mg/kg/day. Two days later, there was a marked deterioration in the patient’s vision. Her visual acuity was recorded as “no perception of light” in both eyes. She had no headache, but she was febrile. Her right eye now had anterior chamber and vitreous cells and widespread retinal haemorrhages (Box 1, C). Magnetic resonance imaging (MRI) of the patient’s brain showed an area of encephalomalacia in keeping with her previous surgery. Inflammatory changes and a retinal detachment were noted in the left globe. The optic nerves appeared normal. No orbital masses were noted, and no cavernous sinus pathological features were observed (Box 2, A). A surgical vitreous biopsy was performed on her left eye. The next day, she was found to have a deteriorating neurological status. She was delirious and seemed unable to hear and follow commands. She remained febrile. A lumbar puncture was performed. PCR of cerebrospinal fluid and vitreous fluid samples were positive for HSV type 1. In addition, the cerebrospinal fluid had 75 lymphocytes/mm3 and normal cytological features. She was commenced on intravenous aciclovir. A repeat MRI scan 8 days after the initial scan showed increased signal on flair involving the thalamus, occipital, temporal lobes and brainstem in keeping with an encephalitis (Box 2, B). She failed to improve clinically and died 3 weeks later. An autopsy was refused by the family, but the cause of death was pressumed to be herpes simplex encephalitis (HSE). DiscussionAcute retinal necrosis (ARN) associated with HSE has been well described. Most published reports describe the ocular findings following the encephalitis with a variable time course; the mean interval is 6 months but it can range between 10 days and 20 years.1-6 To our knowledge, there has been only one report published of HSE following ARN.7 When ARN follows HSE, it is assumed that a reactivated latent virus is axonally transmitted from the brain to the retina. A retrospective study published in 2008 examined the causative virus among 52 patients with ARN.8 It found that 14% had a history of previous HSE. There were no cases in which ARN preceded the encephalitis. In one case report, the authors noted that there have been 20 cases of ARN following HSE published in the past 20 years, and only one case of HSE following 3 weeks after ARN.9 HSE remains a serious illness with significant risk of morbidity and death. A high index of suspicion is required to diagnose HSE. Neurons undergo lysis associated with haemorrhage — a process similar to that seen in the eye with ARN. The exact mechanism of cell death is postulated to be a combination of direct virus-mediated and indirect immune-mediated processes.8,10 Without treatment, the brain undergoes severe inflammation and necrosis. ARN typically causes panuveitis with a distinctive pattern of retinal involvement. Patients may develop painful, severe visual impairment over a few days, or experience an insidious onset with mild visual symptoms such as floaters. Ophthalmic examination shows evidence of a granulomatous uveitis, vitritis, peripheral retinal periarteritis, retinal infiltrates, retinal necrosis, and sometimes retinal detachment. The posterior pole (macula) is usually spared until late; thus vision may remain fairly good despite surrounding necrosis. Diagnosis is made using PCR-based assays of aqueous and vitreous fluid. Treatment of ARN is intravenous aciclovir for 14 days followed by oral valaciclovir for 3 months. Systemic steroids are started a few days after initiation of antivirals to lessen the immune-mediated retinal necrosis. Our patient had a history of HSV years before the current presentation. There was, however, no history of preceding encephalitis. We hypothesise that the unguarded use of steroids may have caused reactivation of the virus and subsequent spread to the brain via retrograde axonal transport from the eye or from a generalised viraemia. This case highlights the importance of considering a viral aetiology in cases of atypical uveitis. ARN may herald systemic or cortical disease. Viral PCR should be performed before commencing systemic steroids. Herpetic disease may remain latent for many years before presenting in a previously uninvolved tissue. Intraocular inflammation remains a diagnostic challenge frequently encountered in ophthalmic practice. It remains imperative that all patients presenting with a possible uveitis be referred to an ophthalmologist. 1 Photographs of the patient’s right fundus A. Initial presentation of the right eye with small intraretinal haemorrhages. B. Fluorescein angiogram of the right eye at initial presentation. C. Two days after initial presentation. 2 Magnetic resonance imaging of the patient’s brain and orbits A. Old area of encephalomalacia on the right, and inflammatory changes in the left globe. B. Repeat scan showing increased signal on flair settings.

K Nadia Wittles MB ChB, FCOPHTH(SA), FRANZCO · Lucy A Goold MB BS · Jagjit S Gilhotra MB BS, MMed (ClinEpid), FRANZCO

Cosmetic lid margin tattoo (or cosmetic blepharopigmentation) is increasingly purveyed in Australia by specialist beauty salons and is marketed as a form of permanent make-up. Like other forms of modern tattoo art, it is applied using an electric needle tip which oscillates at 50–150 cycles per second. Each cycle embeds a small amount of pigment into the dermis. Typically, it is performed after application of a topical local anaesthetic. In this case, tattoo pigment was inadvertently embedded directly into the cornea. This resulted in severe conjunctivitis and a large epithelial defect. In this image, taken 1 month after the injury, the anterior corneal stroma remains stippled with tattoo pigment.

Adam K Rudkin · John L Crompton

The cost differential between these two drugs is no longer defensible Federal Health Minister Nicola Roxon recently met with an alliance of consumer, industry and other stakeholders to justify the government’s plan to indefinitely delay the listing of seven new medicines on the Pharmaceutical Benefits Scheme (PBS). She argued that, after considering the advice of the Pharmaceutical Benefits Advisory Committee (PBAC), it was the government’s responsibility to decide whether or not to list a new drug, taking into account other priorities across the health portfolio and current fiscal circumstances.1 Clearly, the cost of the PBS must be sustainable. However, there are other ways of reducing its cost apart from delaying the listing of drugs recommended by PBAC as cost-effective. The treatment of macular degeneration provides an illustrative example. Age-related macular degeneration (AMD) is responsible for almost half of all cases of blindness in Australia.2 In neovascular (or “wet”) macular degeneration, vision loss results from the abnormal growth and leakage of blood vessels in the macula. Ranibizumab (Lucentis), developed by Genentech and marketed by Novartis in Australia, is currently the only drug approved by the Therapeutic Goods Administration (TGA) and available on the PBS to treat wet AMD. It is administered as an intravitreal injection, usually 4–8-weekly, for 12 to 18 months or longer. The PBS-listed price of each injection is $1967. Ranibizumab is the antigen-binding fragment of a recombinant, humanised, monoclonal antibody that binds to vascular endothelial growth factor A (VEGF-A), the cytokine primarily responsible for blood vessel growth. The inhibition of VEGF-A reduces the permeability and growth of the neovascular vessels. Blindness is prevented in most patients, and the majority of treated patients go on to have some improvement in vision.3-5 Bevacizumab (Avastin) is an anti-VEGF-A humanised, monoclonal antibody (also developed by Genentech, and marketed in Australia by Roche; Genentech is a wholly owned subsidiary of Roche) that has been approved by the TGA for the systemic treatment of certain cancers. It was successfully used “off-label” for the treatment of wet AMD before ranibizumab became available, but has also been used to some degree since the availability of ranibizumab, especially in the United States, where patients bear more of the costs of drugs.5-7 It is prepared for ophthalmic use in a sterile pharmacy by taking a dose used in chemotherapy and splitting it for use in treating wet AMD for up to 25 patients. The cost of its off-label use has been significantly less than that of ranibizumab (around a 40th of the cost, at $50 per dose). Ranibizumab received PBS listing for use in treating wet AMD in 2007 and has since largely replaced bevacizumab for the treatment of AMD in Australia. Although therapy with ranibizumab has been successful, its PBS listing has come at great expense, costing taxpayers $237 million in 2010 (second only to atorvastatin and rosuvastatin). It is consistent with many of the principles of quality use of medicines (QUM), outlined in the National Medicines Policy,8,9 for ophthalmologists to select a PBS-listed therapy that has been demonstrated to be safe and effective. But advocates of QUM also emphasise the importance of choosing medicines that are cost-effective for individuals and the community. A recent study by the US National Eye Institute10 has raised the question of whether use of ranibizumab can be justified economically. The study compared bevacizumab and ranibizumab for the treatment of wet AMD, administered either monthly or as needed, in 1208 randomly assigned patients. At 1 year, bevacizumab and ranibizumab had equivalent effects on visual acuity, when administered according to the same schedule. Ranibizumab given as needed, with monthly evaluation, had effects on vision that were equivalent to those of ranibizumab administered monthly. The comparison between bevacizumab as needed and monthly bevacizumab was inconclusive. Differences in rates of serious adverse events were higher with bevacizumab but did not reach statistical significance, and require further study. Results from the second year of this study and from other comparative trials and experiential databases will provide additional information. Despite this new information having come to light, in Australia there is little motivation for clinicians, the pharmaceutical companies involved, or government bodies to pursue a broader economic agenda. There is no incentive for Australian ophthalmologists or patients to use bevacizumab off-label because the price to the patient for the PBS-listed ranibizumab is only that of the copayment, and the costs for the ophthalmologist visits are the same. There is little incentive for the sponsor of bevacizumab (Roche) to seek a new indication (wet AMD) for this drug — first from the TGA and then from PBAC — because of the substantial costs involved and the doubtful rewards. Also, the relationships between the companies involved appear to militate against moves that might change the present situation. Applications to both the TGA and PBAC are now fully cost-recovered by charges levied on the sponsor of the drug — a unique situation among equivalent developed countries. This provides a considerable disincentive for applications that are primarily in the public interest. While charges can be reduced or eliminated for so-called orphan drugs, this provision would not apply in this case because ranibizumab is currently available and patent-protected.11 And although it has been suggested that a third party, such as a Royal College, might sponsor an application to the TGA and PBAC in the public interest, this concept has foundered because the sponsor is also responsible for product liability. This leaves us with the question: what policy options might circumvent the difficulties outlined here and save taxpayers substantial amounts of money when treating neovascular AMD with anti-VEGF-A drugs? First, the drug committees and administrations of public hospitals with significant eye services could recommend off-label use of bevacizumab for AMD, in the light of the National Eye Institute study. Currently, in New South Wales public hospitals, there is no PBS access to ranibizumab. This would also accommodate public patients unable to pay for private ophthalmologist visits. Given the issues with bevacizumab of dose preparation, sterility and shelf life, combining services for efficiency and geographical coverage would make sense, as would amalgamating public clinics that already use bevacizumab off-label for other related indications (eg, diabetic retinopathy). Second, the government could consider ways in which it could withdraw the PBS listing for ranibizumab for the treatment of wet AMD, on the grounds that treatment with bevacizumab in public eye hospitals is likely to be more cost-effective. This is likely to be problematic for several reasons: the limited capacity of the public sector to provide this treatment; opposition by ophthalmologists operating privately and those who deliver public services; and opposition from the sponsor. Third, the government could negotiate with Novartis to reduce the cost of ranibizumab, or with Roche to apply to have bevacizumab approved and listed for use in treating AMD. It should be noted that the effort needed to register, list and distribute medicines internationally and in Australia should be considered in these negotiations, but the cost differential now extant in the light of the National Eye Institute study results is no longer defensible. Finally, the government could accept that it is the only body with the responsibility and capability of acting in the public interest in these matters. The Minister for Health and Ageing could ask the TGA and PBAC to consider listing bevacizumab for neovascular AMD in the public interest, perhaps with a temporary or provisional licence pending accumulation of more data, with the government accepting any liability that may accrue. Although the challenges of achieving an equitable solution to this problem are considerable, the significance to the PBS budget and subsequently for analogous situations is now a strong incentive for action.

Ken J Harvey MB BS, FRCPA · Richard O Day MD, FRACP · William G Campbell MB BS, FRANZCO · Wendy Lipworth MB BS, MSc, PhD

To the Editor: Indigenous Australians have a higher risk of vision loss from preventable and treatable causes than non-Indigenous Australians1 and have been reported to attend eye care services at a lower rate than non-Indigenous Australians.2 Here, we report results from the National Indigenous Eye Health Survey1 which indicate that many Indigenous Australians with vision problems have accessed eye care services but not as frequently as recommended by the National Aboriginal Community Controlled Health Organisation (NACCHO) and the National Health and Medical Research Council (NHMRC), particularly for high-risk groups of patients with diabetes.3,4 The survey methods have been reported elsewhere.1 Briefly, 1694 Indigenous children and 1189 Indigenous adults from 30 communities across Australia had a standardised eye examination and completed a questionnaire in 2008. Recruitment rates were 84% for children aged 5–15 years and 72% for adults aged ≥ 40 years, and 96% of responses to questionnaire items were complete. Seventy-nine per cent (936/1189) of Indigenous adults reported vision problems, of whom 83% (778/936) had sought care from an eye care service (Box 1). Similar to previous studies,5 we found use of eye care services increased with increasing age, but being male and having no education were barriers to accessing services. There was a significant association between higher education levels and higher rates of using of eye care services, with the odds for using eye care services being the highest among those with the highest level of education (data not shown). These factors should be considered when designing public health messages on the importance of using eye care services. As elsewhere in Australia, optometric services were the most frequently used facilities (49%, 378/778) across all regions except very remote inland, where primary health care services had the highest reported usage (33%, 49/149). Participants from very remote coastal and very remote inland regions were twice as likely to consult an ophthalmologist compared with the other regions (Box 2). Twenty-three per cent (179/769) of participants with vision problems reported that they had last seen someone about their vision problem within the previous year, 67% (519/769) within the previous 3 years, and for 33% (250/769) it had been ≥ 3 years. Only 20% (87/444) of participants with self-reported diabetes had seen someone about their vision problem within the previous year. NACCHO recommends that Indigenous adults aged ≥ 40 years should be screened for reduced visual acuity at least every 2 years,3 and NHMRC guidelines recommend that Indigenous adults with diabetes have their eyes checked every year.4 Our results show that we are far from reaching these targets. As regular eye examinations have the potential to reduce the incidence of vision loss, this is a matter of great concern. The importance of regular eye examinations and follow-up, particularly for high-risk groups, should be emphasised to health care providers and the community. Reasons given for not seeking eye care were: not enough time (41%, 62/153); condition not severe enough (22%, 33/153); too expensive (17%, 26/153); eye care not available in area (14%, 22/153); decided not to seek care (14%, 22/153); transport or distance issues (10%, 15/153); and waiting time too long (10%, 15/153). The two most common reasons for not seeking care indicate a lack of awareness about the importance of regular eye examinations, possibly because of a lack of culturally appropriate public health messages. 1 Self-reported history of vision problems, facilities used and resolved vision problems for Indigenous adults 2 Indigenous adults with self-reported vision problems, by facility used and region Primary health care (n = 255) Hospital (n = 48) Optometrist (n = 378) Ophthalmologist (n = 91) Not specified (n = 6) No consultation (n = 156) Total χ2 P Major city 34 (31%) 6 (6%) 48 (44%) 7 (6%) 0 13 (12%) 108 22.5 < 0.01 Inner regional 39 (28%) 4 (3%) 71 (52%) 8 (6%) 1 (1%) 14 (10%) 137 17.7 < 0.01 Outer regional 51 (34%) 6 (4%) 56 (37%) 13 (9%) 1 (1%) 25 (16%) 152 18.3 < 0.01 Remote 55 (28%) 9 (5%) 79 (40%) 12 (6%) 2 (1%) 42 (21%) 199 — 0.16* Very remote coastal 27 (14%) 8 (4%) 81 (43%) 30 (16%) 2 (1%) 41 (22%) 189 — 0.87* Very remote inland 49 (33%) 15 (10%) 43 (29%) 21 (14%) 0 21 (14%) 149 12.7 0.03 * Fisher exact test.

Anna-Lena M R Arnold · Lucy Busija · Jill E Keeffe · Hugh R Taylor

Visual field defects typically caused by lesions in the optic nerve or optic chiasm may be caused indirectly by subdural haematomas. A 61-year-old man survived transtentorial herniation caused by subdural haematomas that resulted from shaking his head in an attempt to remove a cockroach impacted in his external auditory canal. Bilateral incomplete posterior cerebral artery infarction of both inferior tips of the occipital lobe resulted in bilateral superior quadrantanopia. There are no previous reports of tentorial herniation causing this permanent outcome. (MJA 2011; 194: 420-422) Clinical recordA 61-year-old man presented to the emergency department with a 3-day history of worsening headache and drowsiness. He had a past medical history of renal calculi and gout and was not taking any regular medication. He had a history of consuming 5–6 standard units of alcohol per day, but no antecedent falls or head injury were reported. On physical examination, his Glasgow Coma Scale (GCS) score was 15/15 and his vital signs were normal, apart from mild hypertension (154/80 mmHg). His right pupil measured 4 mm, and his left pupil, 3 mm. His reflexes were documented as brisk in the upper limbs. Results of liver and coagulation studies were normal. A cerebral computed tomography (CT) scan showed large bilateral frontoparietal subdural haematomas (Box 1, A). The right-sided haematoma had mixed hyperdense and isodense areas suggestive of acute on subacute bleeding, while the left frontal haematoma was isodense. The CT scan also showed marked mass effect (Box 1, B) and early tentorial herniation of the uncus (Box 1, C) without tonsillar descent. There was a delay of 8 hours, including transfer of the patient to a neurosurgery centre, before surgery. The patient’s medical notes showed that before undergoing surgery he had developed bilateral extensor Babinski responses and had a GCS score of 14/15. One hour before the operation, the nursing records indicated “GCS 13/15, patient too drowsy and disoriented to consent [to surgery]”. No further imaging was undertaken and no further notes were made before surgery. The patient made an uneventful recovery after bilateral craniotomy and drainage. A CT scan of the brain on discharge about a week later confirmed evacuation of the haematomas and normalisation of brain position. During routine follow-up 10 weeks later, the patient reported persistent difficulty with reading since the operation and that he was experiencing problems differentiating colours. His visual acuity was found to be 6/5 in the right eye and 6/18 in the left, with symptoms of defective colour vision (3/14 Ishihara plates correctly identified with the right eye, and 4/14 with the left). A minor cataract was found in his left eye, but there was no other intraocular abnormality in either eye. His optic discs appeared healthy without pallor in both eyes (and remained so on further follow-up several years later). Computerised visual field perimetry testing conducted at 10 weeks showed bilateral superior altitudinal defects (Box 2). Routine visual evoked potentials showed normal latencies. However, multifocal visual evoked potentials showed normal latencies with decreased amplitude in superior fields bilaterally. Magnetic resonance imaging (MRI) of the brain and orbits conducted 6 months after the surgery showed chronic infarction involving the inferior tip of both occipital lobes medially, more marked on the right side (Box 1, D and E). There was evidence of residual blood products from the evacuated bilateral subdural haematomas, with no evidence of new bleeding. There was no abnormality of the optic nerves or optic chiasm, and venous drainage on magnetic resonance venography was normal. Upon further questioning at this time, a reason for the apparent spontaneous subdural haematomas became evident. The patient described an incident 7 weeks before admission when he awoke with an insect inside his ear. Despite, in his words, “shaking my head from side to side and banging on the side of my head with my fist”, the patient was not able to get the insect out. He was seen at the emergency department, where his ear was syringed and, eventually, a German cockroach (Blatella germanica) was removed with further shaking of the head (which was documented in the hospital records). He recalled a marked headache for the next 2 weeks, which gradually subsided. Recurrence of the headache 5 weeks later prompted him to present to the emergency department again, and the subdural haematomas were detected. DiscussionThis is an unusual and to the best of our knowledge a novel case of bilateral visual field defects related to earlier impaction of a cockroach in the external auditory canal. Subdural haematoma is an uncommon cause of bilateral homonymous hemianopia.1 It can occur as the result of direct local injury to the occipital lobes, or alternatively, mass effect from subdural haematoma can result in tentorial herniation, potentially compromising the blood supply of the posterior cerebral artery (PCA) and placing the regions it supplies at risk. Visual field defects as a consequence of this indirect effect of subdural haematoma have been reported in some surviving patients, but posterior cerebral infarction is more commonly recognised in postmortem examinations of the brain.2-4 Altitudinal visual field defects are typically due to optic nerve lesions and are usually unilateral. Theoretically, the minimal lesion required to produce a bilateral superior quadrantanopia is one located in the inferior optic chiasm; however, this occurs rarely. Typically, injury causes splaying of the chiasm, which is more commonly associated with bitemporal hemianopia. Transtentorial herniation is a well recognised cause of uncal and brainstem displacement and third cranial nerve palsy, but it can also cause stretching and compression of the PCA — thereby compromising the blood supply and leading to infarction.4,5 Survival from tentorial herniation with residual bilateral homonymous hemianopia has been occasionally reported in the literature.6-9 To date, there is no documentation of it causing bilateral superior quadrantanopia. The regions of the brain at risk secondary to PCA ischaemia or infarction are the inferior and medial temporal and occipital cortices. The actual area of involvement depends on which tributaries (and distributions) are affected. The calcarine branch of the medial occipital artery, which supplies the medial occipital lobe, is most commonly involved.10,11 In our patient, there was evidence of mass effect and tentorial herniation secondary to the enlarging subdural haematoma. This would have placed the PCA blood supply at risk. The MRI findings of bilateral infarction of the inferior tip of the medial occipital lobe, together with field test findings of bilateral superior altitudinal defects, confirm the involvement of the visual cortex, which is topographically organised with the cortical representation of the superior field localised inferiorly.5,12 The affected region corresponds to the striate cortex and most posterior part of the prestriate cortex. Both areas are involved in aspects of colour visual processing, suggesting an explanation for the patient’s defective colour vision despite a normal optic chiasm and optic nerves, although a congenital cause cannot be excluded.13 The patient’s history of daily alcohol consumption may have predisposed him to bleeding tendencies and subdural haematomas, although results of his liver and coagulation studies were normal. A diagnosis of acute on subacute subdural haematoma fits well with the timing of the patient’s violent attempts to remove a foreign body from his ear about 7 weeks previously, and with subsequent delayed re-bleeding and increasing supratentorial pressure resulting in uncal herniation and infarction of the part of the primary visual cortex known to correlate with the superior visual fields. A preferred method of removing a cockroach from the ear canal is to drown it with non-toxic vegetable or mineral oil so it can then be easily extracted with forceps. Cautious suction can also be used, taking care not to cause barotrauma by occluding the canal. Lignocaine 2% also rapidly kills the insect or results in it leaving the canal, but entry of lignocaine through any perforation into the middle ear can produce severe sustained vertigo with nystagmus for some hours.14-17 1 Initial computed tomography scan of the brain (A–C), and magnetic resonance images 6 months later (D, E) A: Isodense subdural haematomas (arrows) extending over the convexities longitudinally with effacement of sulci. B: Right hyperdense frontal acute component of a subdural haematoma (arrows) consistent with delayed re-bleeding. C: Uncal herniation, particularly on the left (arrow). D, E: Sagittal fluid-attenuated inversion-recovery magnetic resonance image 6 months later, left and right of midline, respectively (arrows), showing inferior occipital pole infarction. 2 Computerised visual field perimetry of the right eye (A) and left eye (B) The left superior and right superior visual fields are abnormal for both eyes (black areas). The left superior quadrant is slightly worse for both eyes, suggesting this is homonymous and that the lesion is posterior to the chiasm. We describe this as a bilateral superior quadrantanopia rather than a hemianopia to distinguish this circumstance from the normally lateral hemianopias, and because the lesion required to cause this finding is bilateral.

Wan Yi Ng BBus, MB BS · Brian E Chua MB BS, BSc, MPH · Todd A Hardy MB BS, PhD · David Wechsler MB BS · Stephen W Reddel MB BS, PhD

To the Editor: Trachoma control guidelines from the World Health Organization1 and Communicable Diseases Network Australia (CDNA)2 recommend the “SAFE” strategy that includes surgery for trichiasis, antibiotic treatment, facial cleanliness and environmental improvement. Lack of access to antibiotics in isolated areas should not be a major contributor to the persistence of trachoma in Australia because special Pharmaceutical Benefits Scheme arrangements (SPBSA) under section 100 of the National Health Act 1953 for the supply of medicines to remote-area Indigenous health services should enable a ready availability of azithromycin. The 1999–00 to 2001–02 evaluation of the SPBSA suggested that the program had led to major increases in the supply of medicines in remote areas, but that the supply of azithromycin did not change as a result of the introduction of the program.3 We assessed the relationship between reported azithromycin treatment of people with trachoma, their household contacts, and community members; levels of treatment recommended by WHO and CDNA guidelines; and the total number of courses of azithromycin available through the SPBSA. The National Trachoma Surveillance and Reporting Unit (NTSRU) provided 2008 data on treatment with azithromycin and trachoma prevalence in the Northern Territory, South Australia and Western Australia (refer to the report for limitations of the data).4 Data for 2007–08 on the supply of azithromycin to health services under SPBSA were obtained from Medicare Australia. The WHO guidelines recommend treatment of an entire community if the prevalence of active trachoma among children is above 10%.1,5 The CDNA guidelines2 recommend that contacts (> 6 months of age) of infected children within a household be treated. Our estimate of trachoma treatment according to CDNA guidelines is based on multiplying the number of infected children (from the NTSRU data) by the average number of members in remote Indigenous households.5 The impact of shared and multiple residence on estimates of household contacts could not be taken into account. The Box shows the numbers of azithromycin courses available through the SPBSA to remote-area Aboriginal and Torres Strait Islander health services in the three states. In the NT, the reported number of courses given (3069) fell well below the level recommended by both WHO (by 34%) and CDNA (by 41%) guidelines. In SA, reported courses of azithromycin given (7) fell well below the 45 suggested by the CDNA guidelines. The prevalence of trachoma did not exceed 10% in any SA community, so no treatment was required under WHO guidelines. In WA, the reported number of courses given (2917) also fell below recommended levels, although the deficit (WHO, 35%; CDNA, 81%) varied substantially depending on which guidelines were used. Reported treatment with azithromycin was below levels recommended by the CDNA and the WHO despite health services having sufficient courses available to them to mostly meet these targets. All aspects of the SAFE strategy are important in the eradication of trachoma. However, improving the supply and distribution of azithromycin should be relatively easy to implement, fund and monitor. Azithromycin courses* available, 2007–08;† courses given, 2008;‡ and WHO- and CDNA-recommended courses,§ in three Australian states with remote-area Indigenous health services WHO = World Health Organization. CDNA = Communicable Diseases Network Australia. * Azithromycin courses are given to those with active trachoma, their household contacts, and community members. † Under special Pharmaceutical Benefits Scheme arrangements and reported by Medicare Australia. ‡ Reported by the National Trachoma Surveillance and Reporting Unit (NTSRU). § Based on NTSRU data and using community population estimates.

Margaret Kelaher · Angeline S Ferdinand · Hugh R Taylor

To the Editor: I note the letter in the 7 June issue of the Journal by Ojaimi and colleagues, in which they express their concerns about the lack of uniform screening guidelines for patients on hydroxychloroquine therapy.1 The most obvious problem was the apparent failure of the treating rheumatologist to ensure that the patient was adequately followed up for ocular side effects associated with this treatment. The potential for these side effects has been documented for many years and, while more pertinent in earlier times when chloroquine was used more frequently, isolated case reports about retinal toxicity related to hydroxychloroquine continue to be presented in the medical literature. All patients need to be warned that there is a risk to their eyesight before they start treatment. The pertinent questions are, however, what is the risk and what is the cost of screening? One study found a single case of retinal toxicity in a series of over 1200 patients being treated long-term with hydroxychloroquine.2 Using this information and the data quoted by Ojaimi and colleagues on hydroxychloroquine use,1 there would currently be about 20 000 patients in Australia using hydroxychloroquine, and 17 potential patients with toxicity in the whole nation. Screening guidelines vary, with most recommending a baseline screen and then, depending on the presence or absence of “high risk factors” (dosage > 6.5 mg/kg/day, treatment for > 5 years, renal or hepatic impairment, concomitant eye disease, age > 60 years), testing 2 years after the baseline test and then annually,3 or 5 years after the baseline test and then annually.4,5 The cost of screening the 20 000 Australian patients being treated with hydroxychloroquine according to these guidelines and assuming a standard initial specialist consultation (Medicare Benefits Schedule item 104) and field test (Medicare Benefits Schedule item 11222) was performed would be between $20 174 000 and $28 820 000 over a 10-year period. One really has to ask, can we afford it? The patient described by Ojaimi and colleagues1 falls clearly into the high-risk group in terms of her daily dose and duration of treatment. The slit lamp findings of vortex keratopathy also indicate she had a high risk of retinal toxicity. Rather than screening all patients on hydroxychloroquine therapy, doctors prescribing the medication should be more aware of the potential consequences, and monitor the dosage carefully so that patients in the high-risk group can be referred for screening. Multifocal electroretinography and fundus autofluorescence could potentially be used for screening, but these modalities are not available routinely and their utility in screening is yet to be established. Until then, patients at high risk of hydroxychloroquine retinopathy should be referred to an ophthalmologist for examination. The minimum examination would include measurement of visual acuity, slit lamp biomicroscopy, dilated fundus examination and automated perimetry, looking specifically for changes in the central 10 degrees of visual field.

Trevor J P Hodson

Clinical record In 2008, an 88-year-old woman with a background of well controlled systemic hypertension and high myopia presented to an emergency department with a 5-day history of left periorbital swelling, erythema and mild discomfort, without visual loss. She was seen in the ophthalmology clinic on the same day. Her visual acuity was 6/12 in each eye. She was afebrile, with left upper and lower lid erythema, oedema closing the palpebral fissure, and conjunctival and scleral injection. Slit-lamp biomicroscopy revealed normal anterior chambers; clear, well centred intraocular lens implants from previous uncomplicated cataract surgery; and posterior staphylomata (an incidental finding of bulging of the globe seen in some highly myopic patients). A computed tomography (CT) scan revealed left periorbital soft tissue thickening, with stranding confined to the preseptal area (Figure A). Radiologically, the patient appeared to have periorbital cellulitis, although clinical examination, being more sensitive to ocular signs, revealed postorbital septal spread of infection as evidenced by scleral injection (not expected to be seen on a CT scan), leading to the diagnosis of orbital cellulitis. Intravenous antibiotics were administered for 3 days, with moderate resolution of the periorbital swelling, although the scleral injection remained. Three days after discharge on oral antibiotics, the patient re-presented to the emergency department with recurrence of left periorbital swelling, erythema and discomfort. After administration of intravenous antibiotics for five days, her lid oedema and erythema resolved, but conjunctival and scleral injection persisted. Investigations during this second admission revealed raised erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) level, with a normal blood leucocyte count. Soon after her second discharge, the patient presented again with persisting and worsening erythema of the left eye, with no associated lid swelling or erythema. A clinical diagnosis of non-necrotising anterior scleritis was made (Figure B), and it was thought that conjunctival lymphoma should be excluded. B-scan ultrasonography revealed fluid in Tenon’s capsule and scleral thickening, consistent with anterior and posterior scleritis (Figure B), and investigations again revealed a raised ESR and CRP level. Screening for tuberculosis (TB) (Mantoux test and chest x-ray), antinuclear antibodies, extractable nuclear antigen antibodies, rheumatoid factor, syphilis, and angiotensin-converting enzyme (an indicator of sarcoidosis) were negative. Screening for Wegener’s granulomatosis with perinuclear antineutrophil cytoplasmic antibodies was positive, but attempted confirmation by screening for antiproteinase 3 antibodies and antimyeloperoxidase antibodies was negative. Magnetic resonance imaging revealed marked high T2 signalling and enhancement of the left sclera, extraocular muscle insertions and distal optic nerve (Figure C). A scleroconjunctival biopsy was performed to exclude conjunctival lymphoma or other uncommon diagnoses, such as tuberculosis and fungal infection. The biopsy revealed a dense inflammatory infiltrate consisting of B and T lymphocytes, plasma cells and occasional neutrophils, with no granulomatous inflammation. Flow cytometry; immunohistochemistry; bacterial, fungal and mycobacterial staining and culture; and TB polymerase chain reaction tests were all negative. Haematological consultation excluded systemic or central nervous system lymphoma. A short course of oral prednisolone (1 mg/kg/day for 14 days) resulted in complete resolution of the patient’s signs and symptoms, and she remained well at 6-week follow-up. A: Sagittal computed tomography scan showing left periorbital soft tissue thickening and posterior staphyloma. B: Superior scleral injection and B-scan ultrasound showing fluid in Tenon’s capsule and scleral thickening, consistent with anterior and posterior scleritis. C: Magnetic resonance images showing high T2 signalling and enhancement of the left sclera, extraocular muscle insertions, and distal optic nerve. To our knowledge, this is only the second reported case of posterior scleritis mimicking orbital cellulitis. We believe that a diagnosis of posterior scleritis should always be considered when patients with presumed periorbital or orbital cellulitis do not respond appropriately to intravenous antibiotic treatment. In the previous report of posterior scleritis mimicking orbital cellulitis, the authors hypothesised that inflammation had spread anteriorly, involving the upper lid structures, causing lid swelling and simulating cellulitis.1 Orbital cellulitis has an incidence of 5.8 per 100 000 population in people aged 65 years or older.2 Posterior scleritis is much less common. McCluskey and colleagues3 published a single-centre case series of patients with posterior scleritis seen at Moorfields Eye Hospital in London that shed much light on the nature of the disease. Between 1974 and 1996, only 137 patients were diagnosed with posterior scleritis. Interestingly, in this series, only 31% of patients had visual loss, little more than half (56%) had pain, and 17% had no physical signs of posterior scleritis on examination. Anterior scleritis at or after presentation occurred in 60% of patients, with 36% having combined anterior and posterior scleritis on presentation. Only 28% had an associated systemic disease, and no patient had necrotising posterior scleritis. Our patient had no pain, no visual loss, and no physical sign of posterior scleritis on clinical examination. Her initial examination appeared to be consistent with the diagnosis of orbital cellulitis. With respect to diagnostic criteria, there were many signs in her clinical presentation that are shared by orbital cellulitis and posterior scleritis. Features that favoured the eventual diagnosis of posterior scleritis were the presence of physical and radiological signs in the absence of an appropriate response to intravenous antibiotic treatment, and a positive response to oral prednisolone treatment. Patients presenting with signs of periorbital or orbital cellulitis should always be treated with intravenous antibiotics immediately, pending the results of any radiological investigation. However, when a systemically well patient with no radiological or clinical evidence of an orbital abscess requiring surgical drainage does not respond positively to intravenous antibiotic treatment, ophthalmologists should suspect non-infective aetiology. Lessons from practice Periorbital cellulitis can spread posteriorly into the orbit, cavernous sinus and brain, and is therefore potentially lethal. Intravenous antibiotic treatment for probable cellulitis should be started immediately, without waiting for the results of diagnostic radiological investigations. When a systemically well patient with no radiological or clinical evidence of an orbital abscess requiring surgical drainage does not respond positively to intravenous antibiotic treatment, ophthalmologists should suspect non-infective aetiology. Any patient with periorbital or orbital cellulitis should be assessed by an ophthalmologist.

Michael Rossiter-Thornton BMedSc(Hons), MB BS(Hons) · Lia Rossiter-Thornton BMedSc, MB BS(Hons) · Raf Ghabrial MB BS, FRANZCO · Domit A Azar MB BS(Hons), MPH(Hons), FRANZCO

Using shared care to tackle the complexity of optimal patient management Globally, the burden of disease has shifted from acute to chronic illnesses and the management of multiple comorbidities. To address the challenges this creates, integrated care has received growing attention as a means of improving health care delivery and outcomes.1 Integrated care (frequently equated with “disease management” and “shared care”) is defined by the World Health Organization as a concept bringing together inputs, delivery, management and organization of services related to diagnosis, treatment, care, rehabilitation and health promotion. Integration is a means to improve services in relation to access, quality, user satisfaction and efficiency.2 For the first time, Australia has a shared care model for the management of a chronic condition — namely, glaucoma — launched under the Pharmaceutical Benefits Scheme (PBS) in January 2008. Under the new PBS guidelines (Box),3 authorised optometrists can co-manage patients with glaucoma in a shared care arrangement with an ophthalmologist. Also, similarly to optometrists in the United Kingdom, Canada, and the United States, authorised optometrists in Australia may now prescribe therapeutic agents for certain eye conditions under the PBS. In January 2008, prescribing rights for topical ophthalmic medications were extended to certified optometrists as a result of a legislative change (National Health Amendment [Pharmaceutical Benefits] Act 2007 [Cwlth]).4 The range of medications that authorised optometrists may prescribe under the PBS includes lubricants and therapeutic agents for treating allergies, infection, inflammation, and glaucoma. Glaucoma is a heterogeneous group of diseases leading to a progressive optic neuropathy. It is the most common cause of preventable blindness in developed countries.5 Commonly, glaucoma is managed with topical hypotensive medications that include prostaglandin analogues, carbonic anhydrase inhibitors, selective α-adrenoceptor agonists and topical β-blockers. Under the shared care model, the patient’s ophthalmologist and optometrist together develop a written management plan that specifies the treatment goals and the roles and responsibilities of the two practitioners, create a review schedule, and communicate clinical information to the patient’s general practitioner to promote an integrated approach. The PBS guidelines also recommend that a pharmacist be involved in providing medicines information, such as advice related to administration and techniques to limit systemic absorption and side effects of ophthalmic medications, as well as potential interactions with concomitant systemic medications for comorbidities.3 Quality integrated care practices require effective communication and coordination among the involved health care providers and with patients. Decision making in relation to medicines for optimal patient management is increasingly complex because of more pharmacotherapeutic options for more conditions, increasing exposure and age at exposure of older patients to a wider range of medications, and rising numbers of affected patients as the population ages. Ophthalmic medications are often overlooked in medical history taking. Almost half the medical records kept by GPs have been reported to have no record of eye drops being used by patients with glaucoma.6 Ophthalmologists, GPs, optometrists and pharmacists need to consider a patient’s comorbidities and concurrent treatments to be able to provide a holistic approach to patient care. Topical ophthalmic medications may have effects on other diseases and their management: topical β-blockers may cause bronchospasm in those with reactive pulmonary disease,7 and their prescription may warrant an additional bronchodilator. Topical ophthalmic medications may interact with systemic medications. Polypharmacy, especially in the older population, may have serious consequences.8 For example, cimetidine, an over-the-counter H2-receptor antagonist for gastrointestinal irritation, may increase the effect of β-blockade when used in conjunction with β-blockers and should be used with caution in patients with underlying cardiac conditions.9 Australia’s new integrated care model promises to increase patient access to eye health care services, particularly in rural areas, and thereby enhance continuity and quality of care in these regions.2 Involvement of optometrists offers the opportunity for increased detection of glaucoma and patient access to subsidised ocular therapeutic agents. However, the processes and clinical outcomes of this new model of care need to be evaluated rigorously to determine its quality, feasibility and durability. Are anticipated improvements being realised? Are there unintended consequences? Important measures will include the rate of medication-related problems among glaucoma patients before and after the introduction of this shared care arrangement; patient adherence to and persistence with therapy; detection of undiagnosed glaucoma (prevalence of which has been reported to be high10); patient satisfaction; rates of visual field deterioration; and intraocular pressure levels. Some of these outcomes, such as medication use outcomes recorded in computerised health care datasets, will be easier to measure than others. Commitments by government and public agencies to rigorous research and funding to allow prospective studies and direct measures of health outcomes would seem a sound investment. This would provide valuable information not only for Australia but also for other countries that develop and implement integrated care models. Guidelines for the shared care of glaucoma patients under Australia’s Pharmaceutical Benefits Scheme (PBS)3 Confirmation of diagnosis and development of a management plan An authorised optometrist who makes a provisional diagnosis of glaucoma is to refer the patient to an ophthalmologist for confirmation of the diagnosis. With the consent of the patient, the optometrist and the ophthalmologist are to develop a management plan together, including the sharing of care between the two practitioners, and the communication of clinical information to the patient’s nominated general practitioner. Patients being considered for anti-glaucoma therapy with a β-blocking agent should be assessed for any potential cardiovascular or respiratory risk by a medical practitioner (eg, the patient’s GP) before initiating therapy. This assessment should be repeated if a change in dose of the β-blocker is proposed. Once a treatment plan is established with the ophthalmologist, the optometrist can prescribe topical medications under the PBS and perform ongoing reviews to monitor the patient. Changes to the management plan are only made following consultation between treating practitioners. A written patient management plan must specify: all the agreed components of treatment, including any pharmacotherapy; target intraocular pressures and actions to be taken if these are not achieved within a specified time frame; an agreed approach to monitoring visual fields and optic disc imaging and actions to be taken following changes in visual fields; triggers for referral for immediate ophthalmological and GP review; likely side effects from agreed treatment and the action to be taken to address these; an agreed schedule for patient review by both practitioners; who is responsible for performing each of the required tests and the required frequency for performing them; an agreed method for timely communication of clinical findings and patient management between the two practitioners and the patient’s nominated GP.

Christine Y Lu BPharm, MSc, PhD · Vicky H Lu MB BS, MPH · Ivan Goldberg MB BS, FRANZCO, FRACS · Richard O Day MD, FRACP

Charles Bonnet syndrome (CBS) is a disorder in which psychologically normal people, often with vision impairment, experience complex visual hallucinations. The hallucinations are purely visual and do not occur in any other sensory modality, and people with CBS have full insight into the unreal nature of the hallucinations. This report describes the case of a CBS sufferer who experienced a distressing change in the nature of her visual hallucinations following a stressful event — the Black Saturday bushfires of February 2009. Clinical recordA healthy and alert 80-year-old woman presented for an orthoptic consultation in September 2009. The patient was legally blind (ie, her best corrected visual acuity was less than 6/60), with diagnosed age-related macular degeneration and closed-angle glaucoma. She had a known 4-year history of Charles Bonnet syndrome (CBS), a condition that caused her to have complex visual hallucinations, usually triggered when she was in unfamiliar surroundings. She reported that the nature of her hallucinations had changed significantly. Previously, the patient had experienced images such as “an elephant walking down the street with a child on its back” and intricate blue designs when looking at white plates or coloured carpets. While the patient was aware that the images were not real, occasionally her insight was delayed if the hallucination fit into the surrounding environment. For example, she would see a truck while travelling as a passenger in a car and, upon alerting the driver that they were about to collide with the truck, would be informed that it was a letterbox by the side of the road. When the hallucinations had first appeared, the patient had thought “there is something going on in my head” and reported her symptoms to her husband and then to her ophthalmologist, who explained the benign nature of the disorder. Since that time, she had not been troubled by the hallucinations, explaining “usually I can laugh at it”. Initially, the patient’s experience with CBS followed the typical pattern — she experienced hallucinations weekly that usually lasted only for several seconds. The patient’s hallucinations became atypical on “Black Saturday”, 7 February 2009, a day of intense heat and bushfires in the southern Australian state of Victoria. Bushfires raged in 14 regions across the state, resulting in 173 deaths.1 The patient lives in an area affected by the fires and was evacuated from her home on Black Saturday. That day and subsequently, she experienced hallucinations that were horrific in nature to her, such as seeing a prickly coat on her short-haired dog, spiral wire-like hair protruding from the heads of bald family members, and their faces beginning to melt, “like wax dripping”. She saw people’s legs covered with curly, black, wiry hair. Despite her visual impairment, the patient is a talented artist and has drawn her disturbing hallucinations (Box 1). The hallucinations that commenced on Black Saturday persisted for days, rather than seconds, and the images frightened the patient for the several days during which she experienced them. She reported experiencing great stress during the bushfires and also recalled earlier distressing episodes with significant fires as a child, while living in London: a chimney caught fire in her home; she witnessed the burning of the Crystal Palace in 1936; and she was present during the “Blitz” between September 1940 and May 1941, when London was subjected to intense aerial firebombing. She reported that these stressful episodes relating to fire increased her fear on Black Saturday and explained that “fire has a particularly bad effect on me”. Following discussion about CBS and her experiences, a review was not considered necessary from an orthoptic point of view, but she was encouraged to make contact as necessary if symptoms changed. She was also instructed to continue seeing her ophthalmologist and her psychologist. DiscussionThis case demonstrates that a stressful life event can change the nature of hallucinations experienced in CBS, with an accompanying change in emotional experience (from non-distressing to distressing). In general, hallucinations can result from false sensory input to the brain and occur in the absence of external stimuli. When a person presents with visual hallucinations, an underlying psychiatric disorder, neurological abnormality or drug intake can be suspected.2 Visual hallucinations can also occur as a result of lesions in the visual system, from the cornea to the cortex. The occurrence of complex visual hallucinations, as reported in this case, was first described by Swiss philosopher, naturalist and lawyer Charles Bonnet in 1760, after whom the disorder was later named. CBS is characterised by vivid, elaborate and recurrent visual hallucinations in psychologically normal people, who have full insight into the unreal nature of the hallucinations.3,4 The hallucinations are purely visual and do not occur in any other sensory modality. Images of complex patterns and people are most common, and the hallucinations often fit into the surrounding situation. They can appear for several minutes every week, and can continue to occur for 12 months or more. Often CBS occurs upon waking, but not exclusively so.5 Key features of CBS are listed in Box 2. Two theories have been proposed to explain CBS: the “release” and “sensory deprivation” theories. The release theory postulates that a lesion at any level of the visual pathway leads to the release of defective electrochemical impulses, thereby causing visual hallucinations. The sensory deprivation theory proposes a similar mechanism, except that it is reduced sensory input to the brain as a result of an ocular lesion that causes spontaneous discharge of neurones at the level of the retina or cortex.6,7 Triggers for the onset of CBS have not been clearly identified, and the circumstances the person finds themselves in at the onset of an episode can vary. It is possible that this patient suffered with acute stress disorder, as she had a past history of exposure to fire-related trauma and reported distressing recollection of the bushfires, but she did not report other symptoms of acute stress disorder.8 The change in nature of her hallucinations may represent interplay between CBS and an acute or post-traumatic stress disorder. The number of cases of CBS in Australia is not known. Reticence to discuss symptoms of CBS out of fear of being labelled insane is common. Most people only tell a family member about their symptoms and very few discuss the problem with a medical professional.5 Further, the symptoms of CBS are probably not always recognised and therefore correctly identified. As in this patient, CBS is usually associated with vision impairment, and it is important for clinicians to be aware that these types of hallucinations may occur in patients with advancing age and early vision impairment. The prevalence of CBS in people with impaired vision has been reported to be between 10% and 40%, with a much lower prevalence in Asian populations (< 1%).9-13 It is estimated that almost half a million Australians have vision impairment,14 and the prevalence of CBS hallucinations among these patients has been found to be 17.5%,5 suggesting that about 85 000 people in Australia may have CBS. However, while there is an association between CBS and loss of vision, it can also occur in individuals with no obvious ocular abnormality. There is no treatment of proven effectiveness for CBS, but the use of selective serotonin reuptake inhibitors has been associated with a reduction in hallucinations.15 Sporadic reports of effective medications can be found in the literature, but no controlled clinical trials have been published. Some sufferers indicate that closing their eyes or blinking may make the hallucinations stop.5 This case report highlights the limited knowledge about the underlying causes and triggers of CBS and the need for further investigation into this disorder. 1 Patient’s drawings of her disturbing hallucinations following the Black Saturday bushfires Family members with melted faces and spiral wire-like hair on their heads and legs, and a prickly coat on the patient’s short-haired dog. 2 Key features of Charles Bonnet syndrome Vivid, elaborate and recurrent visual hallucinations3 Experienced by psychologically normal people who have insight into the unreal nature of the hallucinations3 Risk factors include vision impairment, old age, hearing impairment, living alone, and female sex5 Reported prevalence is 10%–40% of the vision-impaired population; prevalence is skewed by non-reporting of the hallucinations for fear of being labelled with a psychiatric disorder5 Hallucinations typically last for several minutes and commonly occur upon waking5 There is no treatment of proven effectiveness6

Meri Vukicevic BOrth, PGDiplHlthResMthds, PhD

To the Editor: In February 2009, a 68-year-old man presented to the Royal Victorian Eye and Ear Hospital within hours of developing an itchy, red left eye. The patient, who was otherwise healthy, lived in suburban Melbourne, usually with his pet dogs, but the last of his dogs had recently died. The patient was unsure if all his dogs had been dewormed regularly because he spends about 6 months a year in Europe. General inspection of the eye suggested subconjunctival haemorrhage. However, slit-lamp examination showed a mobile, tightly coiled structure within the subconjunctival blood. It grew increasingly agitated with higher slit-lamp light intensity (Box, A). Assessment of the patient’s visual acuity and the anterior and posterior chambers of the eyes was unremarkable. Blood tests revealed a positive filarial serology and eosinophilia. The patient was transferred to the operating theatre and, under topical anaesthesia, a 5 mm conjunctival incision was made and the mobile structure removed (Box, B and C). The patient was discharged with a prescription for prednisolone acetate 1% and chloramphenicol 0.5% eye drops (one drop four times a day). He made a full recovery. The extracted specimen was reviewed by one of us (D M S). The 150 mm worm was identified as a young adult female filarioid nematode, Dirofilaria immitis (commonly named dog heartworm) after comparisons with laboratory specimens of D. immitis and Pelecitus roemeri. Infection with either P. roemeri (kangaroo and wallaby knee worm) or Loa loa (loiasis) was excluded. Our specimen did not have lateral alae and the distance from anus to tail was shorter than would be expected for the kangaroo worm. In addition, the patient had never been to Africa where loiasis is endemic to several countries. Subconjunctival dog heartworm is rare, but its incidence is increasing in parts of the world.1,2 Dogs are the natural hosts and transmission to humans occurs through mosquito bites of the skin (into which the third-stage infective larva may escape). For an unknown reason, the worm sometimes takes an abnormal migratory route and ends up in the eye of the host. Ophthalmic cases have been reported in dogs.3,4 Careful measures to exterminate mosquitoes and deworm dogs and cats are important in limiting its transmission. Surgical extraction is the definitive treatment and further treatment with systemic anthelmintics is unnecessary.5 Humans are non-natural hosts for this parasite and, therefore, its life cycle cannot be completed within the human body. When a larva does evade the human immune system, as in the case of our patient, the chances of another larva being present elsewhere in an immunocompetent person seems remote. Furthermore, unless the larva becomes clinically apparent, it would be impossible to find. Subconjunctival Dirofilaria immitis infection in a 68-year-old man A: A whitish mobile structure coiled in the haemorrhagic subconjuctival space B: The female Dirofilaria species measuring about 150 mm C: Day 1 after removal of worm and necrotic temporal conjunctiva, exposing bare sclera

Elaine W Chong · Harsha Sheorey · Cheng Hean Lo · David M Spratt · Enrique Graue-Hernández

To the Editor: Hydroxychloroquine is used infrequently (in less than 0.1% of Australians) for the long-term management of chronic conditions (eg, rheumatoid arthritis).1 Hydroxychloroquine retinopathy is a rare but sight-threatening side effect that is usually not reversible.2-5 Various eye screening recommendations for hydroxychloroquine toxicity have been proposed overseas,3-5 but there is no recommended consensus for eye screening in Australia. As a result, screening is currently not uniform or universal, and this sometimes leads to significant consequences. A 36-year-old woman with a longer than 10-year history of hydroxychloroquine therapy (400 mg/day; body weight, 62–67 kg) for rheumatoid arthritis presented to her general practitioner after a year of central visual disturbance and photopsias (a sensation of flashes of light). She was referred to the Medical Retinal Clinic at the Royal Victorian Eye and Ear Hospital in August 2009 with suspected hydroxychloroquine toxicity. She had undergone screening by her optometrist for an initial period, but had not been screened within the past 4 years because she failed to attend; she appeared unaware of the potential serious side effect of the medication on her vision. On examination, her visual acuity was 6/9 in the right eye and 6/12 + 2 letters in the left. Her colour vision (assessed by Ishihara plates for colour blindness) was normal. She had left vortex keratopathy (a whorl-like corneal epithelial deposit) and examination of her fundus revealed subtle macular pigment change and retinal arteriolar attenuation (Box, A). Fundus autofluorescence showed significant changes at the level of the retinal pigment epithelium (Box, B). Dense bilateral paracentral field defects were detected on visual field testing. The rheumatologist was notified of the hydroxychloroquine retinal toxicity, and therapy with the drug was ceased. At 6-month eye review, the patient’s vision was stable. Recommendations on the timing of eye screening vary.3 The manufacturer’s product information recommends quarterly ophthalmological examinations, but this is impractical and not cost-effective.3,5 The Royal College of Ophthalmologists (RCO) in the United Kingdom found no evidence-based justification for a systematic screening program.4 They recommend ophthalmological referral only when there are visual symptoms (eg, distorted or patchy central vision, reading difficulties), or eye disease is detected at baseline and confirmed by an optometrist. In contrast, the American Academy of Ophthalmology (AAO) suggests a systematic approach, determined by risk status, that is based on factors such as hydroxychloroquine dose and duration of intake.5 Despite their differences, these protocols both aim to detect toxicity early and minimise the degree of visual loss, rather than to prevent visual loss. This is because there are currently no established criteria to identify toxicity at a reversible stage.4 We recommend adoption of the AAO or RCO protocols and alerting patients to the symptoms of toxicity. Careful counselling of patients at commencement of hydroxychloroquine treatment and on an ongoing basis is essential to promote early presentation and minimise toxicity. Hydroxychloroquine toxicity, although rare, can lead to severe loss of vision if therapy is not stopped. By stopping treatment, the condition may stabilise, and further irreversible vision loss can potentially be avoided. Hydroxychloroquine retinopathy in a 36-year-old woman A: Subtle macular pigment change (black arrows) and some arteriolar narrowing (white arrows). B: Autofluorescence imaging showing symmetrical changes at the level of the retinal pigment epithelium. Mottled loss of autofluorescence (black arrows) indicates loss of retinal pigment epithelium cells, and the adjacent increased autoflourescence (white arrows) indicates cell abnormality.

Elvis Ojaimi · Robyn H Guymer · Tien Y Wong · C Alex Harper

Aim: To determine the prevalence and causes of vision loss in Indigenous Australians.Design, setting and participants: A national, stratified, random cluster sample was drawn from 30 communities across Australia that each included about 300 Indigenous people of all ages. A sample of non-Indigenous adults aged ≥ 40 years was also tested at several remote sites for comparison. Participants were examined using a standardised protocol that included a questionnaire (self-administered or completed with the help of field staff), visual acuity (VA) testing on presentation and after correction, visual field testing, trachoma grading, and fundus and lens photography. The data were collected in 2008.Main outcome measures: VA; prevalence of low vision and blindness; causes of vision loss; rates of vision loss in Indigenous compared with non-Indigenous adults.Results: 1694 Indigenous children and 1189 Indigenous adults were examined, representing recruitment rates of 84% for children aged 5–15 years and 72% for adults aged ≥ 40 years. Rates of low vision (VA < 6/12 to ≥ 6/60) were 1.5% (95% CI, 0.9%–2.1%) in children and 9.4% (95% CI, 7.8%–11.1%) in adults. Rates of blindness (VA < 6/60) were 0.2% (95% CI, 0.04%–0.5%) in children and 1.9% (95% CI, 1.1%–2.6%) in adults. The principal cause of low vision in both adults and children was refractive error. The principal causes of blindness in adults were cataract, refractive error and optic atrophy. Relative risks (RRs) of vision loss and blindness in Indigenous adults compared with adults in the mainstream Australian population were 2.8 and 6.2, respectively. By contrast, RRs of vision loss and blindness in Indigenous children compared with mainstream children were 0.2 and 0.6, respectively.Conclusion: Many causes of vision loss in our sample were readily avoidable. Better allocation of services and resources is required to give all Australians equal access to eye health services.

Hugh R Taylor AC,MD, FRANZCO · Jing Xie PhD · Sarah Fox BA · Ross A Dunn BAppSc(AppChem), GradDipBIT · Anna-Lena Arnold BSc · Jill E Keeffe OAM, PhD

Clinical record Patient 1 A 90-year-old man with bilateral neovascular age-related macular degeneration (AMD) presented with sudden onset of painful visual loss in his left eye. Visual acuity in his right eye was 1/60 due to scarring associated with AMD, and visual acuity in his left eye had deteriorated to hand movements; it had previously been 6/120. He had been taking warfarin for about 2 years for atrial fibrillation and a transient ischaemic attack. The international normalised ratio (INR) had previously been measured almost weekly, but as it had been stable at 2.5–2.9 (target INR, 2.0–3.0), it had not been measured for 6 weeks. B-mode ultrasonography and dilated fundus examination revealed massive suprachoroidal haemorrhage (Figures A and B). The INR was 6.0, which was reversed with intravenous administration of two units of fresh frozen plasma and 1 mg of vitamin K. Visual acuity in the patient’s left eye deteriorated to light perception. As there were no signs of spontaneous improvement and this had been his better eye, surgery was performed to drain the haemorrhage. After surgery, the haemorrhage decreased in size but his vision did not improve. Patient 2 An 82-year-old man presented with a 3-week history of a shadow in his left eye. He had been taking low-dose aspirin for ischaemic heart disease and stroke, but had no known ocular history. On examination, visual acuity was 6/5 and hand movements in his right and left eye, respectively, and massive subretinal haemorrhage was noted in the left eye. Non-neovascular AMD was present in his right eye, and the haemorrhage in his left eye was presumed to be due to a combination of neovascular AMD and the effects of aspirin. He was offered surgery, but declined as there was little chance of improvement in central vision. Subsequently, he gradually lost all vision (including light perception) in his left eye. Aspirin was continued because of his significant cardiovascular history. Over the following year, neovascular AMD developed in his right eye and visual acuity dropped from 6/5 to 6/36 (Figure C). He was treated with regular intravitreal ranibizumab injections, and his vision stabilised. However, several months later, vision in his right eye suddenly deteriorated to vague perception of light. On examination of his right eye, there was no view of the fundus due to a dense cataract and vitreous haemorrhage, but B-mode ultrasonography once again revealed massive choroidal, subretinal and vitreous haemorrhage. The patient was still taking low-dose aspirin therapy, which was subsequently ceased. In an attempt to preserve vision in his only seeing eye, he underwent vitrectomy and silicone oil insertion, but there was no improvement and he subsequently lost light perception in his right eye. A: B-mode ultrasonogram of Patient 1’s left eye, showing massive suprachoroidal haemorrhage. B: Fundus photograph of Patient 1’s left eye, showing massive suprachoroidal haemorrhage bulging forward (therefore largely out of focus). C: Fundus photograph of Patient 2’s right eye, showing right neovascular AMD with subretinal haemorrhage, prior to development of massive suprachoroidal, subretinal and vitreous haemorrhage. Age-related macular degeneration affects about one-third of people aged over 75 years.1 Of those with AMD, 10%–15% develop the neovascular (“wet”) form,2 characterised by abnormal new blood vessel formation in the choroid, under the retina. These abnormal vascular membranes are prone to rupture, leading to subretinal bleeding, fibrous scar formation and severe visual loss. Many older patients who have AMD also take medications that can exacerbate or promote bleeding, such as anticoagulants or antiplatelet agents. In rare cases, intraocular bleeding — in the form of subretinal, suprachoroidal, or vitreous haemorrhage — can be catastrophic and blinding. Previous reports link systemic anticoagulation therapy to intraocular haemorrhage and blindness in AMD patients,3-7 including a recent report in this Journal.8 In two of these reports, patients taking warfarin had very high INRs (4.1 in one case;3 6.3 in the other4). Other reports link systemic anticoagulation therapy to spontaneous suprachoroidal haemorrhage, even in the absence of neovascular AMD.9-11 Additionally, patients with neovascular AMD can develop massive submacular haemorrhage, even if they are not taking antiplatelet or anticoagulant agents. Unfortunately, as many patients with AMD have one eye with poor visual acuity due to macular scarring, it is all the more catastrophic when a massive haemorrhage leads to blindness in their “good” eye. We suggest that the risk of catastrophic bleeding may be stratified, with the greatest risk being associated with anticoagulants (eg, warfarin), followed by antiplatelet agents (eg, clopidogrel and ticlopidine, with aspirin conferring a lower risk). In addition, we would expect a greater risk with combination therapy comprising simultaneous use of multiple antiplatelet and/or anticoagulant agents. In the cases reported here, we suggest that haemorrhage is likely to have been more severe and blinding than it would have been in a patient who was not taking those medications. Although these medications should not be withheld in cases where they are warranted to reduce cardiovascular morbidity and mortality, they are not without risk, and a careful risk–benefit analysis should be performed for each patient before they are prescribed. For example, the risk of stroke is different among patients with simple atrial fibrillation compared with aortic valve replacement. It has been previously noted that if a patient has only one functioning eye, the patient’s general practitioner or cardiologist should seek an ophthalmologist’s opinion to assess the risk of neovascular AMD in the seeing eye before, or soon after, commencing warfarin.12 Further, ophthalmologists should ask their patients whether they take warfarin, and should communicate to the treating doctor whether a patient has, or is at high risk of developing, neovascular AMD.12 Patients taking warfarin who develop neovascular AMD should be advised to maintain an INR at the lower end of the recommended range.3 In addition, we recommend that it would be prudent for INR monitoring to be at the more frequent end of the spectrum. Lessons from practice Patients taking warfarin who develop neovascular age-related macular degeneration (AMD) should maintain an international normalised ratio (INR) at the lower end of the recommended range. INR monitoring should be more frequent for patients with neovascular AMD. If a patient has only one functioning eye, an ophthalmologist should assess the risk of developing neovascular AMD in the seeing eye before, or soon after, commencing warfarin or an antiplatelet agent, including aspirin. Ophthalmologists should ask all patients whether they take warfarin, and should communicate to the treating doctor whether this patient has, or is at high risk of developing, neovascular AMD.

Helen M Garrott FRANZCO,MB BS(Hons), BMedSc · Richard J Haynes MB BCh, FRCOphth, MD

Objective: To determine the prevalence of trachoma among Indigenous Australians.Design, setting and participants: A national, stratified, random cluster sample survey of Indigenous children (5–15 years) and adults (≥ 40 years) in 30 communities across Australia. Data collection was undertaken in 2008.Main outcome measures: Results based on a standardised protocol that included trachoma grading and double grading of photographs of the tarsus.Results: 1694 Indigenous children and 1189 Indigenous adults were examined. Recruitment rates were 84% for children and 72% for adults. The overall rate of follicular trachomatous inflammation among children was 3.8%, ranging from 0.6% in major cities to 7.3% in very remote areas; 50% of communities in very remote areas had endemic rates (> 5%). Trachomatous scarring (TS) occurred among 15.7% of adults, trachomatous trichiasis (TT) among 1.4% and corneal opacity (CO) among 0.3%. TS was found in all regions and TT in all except major cities and inner regional areas. The highest community rates for TS were 58.3%; for TT, 14.6%; and for CO, 3.3%.Conclusion: Blinding endemic trachoma remains a major public health problem in many Aboriginal and Torres Strait Islander communities. Although active trachoma is predominantly seen in very remote communities, scarring and blinding sequelae occur among Indigenous people across the country. The Australian Government’s recent commitment to eliminate blinding trachoma is welcomed and much needed.

Hugh R Taylor AC, MD, FRANZCO · Sarah S Fox BA · Jing Xie PhD · Ross A Dunn BAppSci(App Chem), GradDip(BIT) · Anna-Lena M R Arnold BSc · Jill E Keeffe OAM, PhD

Clinical record An 88-year-old man presented to our emergency department with sudden loss of vision in his right eye. Past ocular history included amblyopia in his right eye and bilateral neovascular age-related macular degeneration (AMD). Previously recorded best corrected visual acuities were 6/120 and 6/18 in his right and left eye, respectively. The patient’s past medical history included acute myocardial infarction, atrial fibrillation, hypertension, hypercholesterolaemia and polymyalgia rheumatica. He had no history of diabetes, cerebrovascular accident or transient ischaemic attack. His medications included warfarin, aspirin, metoprolol, perindopril and simvastatin. His international normalised ratio (INR) had ranged between 1.3 and 2.3 over the preceding 12 months, with a target of 2.0. Visual acuity in the right eye was count fingers. Dilated slit lamp examination revealed a large submacular haemorrhage with associated vitreous haemorrhage (Figure A). An electrocardiogram showed atrial fibrillation with a heart rate of 72 beats/min. His blood pressure was 110/80 mmHg. His INR was 2.8. The eye was managed conservatively and, following resolution of the vitreous haemorrhage, vision remained at count fingers. Four months later, the patient reported sudden loss of vision in his left eye. Visual acuity was light perception, and dilated fundus examination revealed dense subretinal and associated vitreous haemorrhage (Figure B). The patient was still taking warfarin, and his INR at this time was 2.7. As this had been his better eye, he underwent pars plana vitrectomy and clearance of the vitreous haemorrhage. After surgery, his visual acuity was count fingers. After further discussion with the patient’s cardiologist, a decision was made to cease warfarin. A: Fundus photograph of the right eye at presentation, showing macular subretinal and intraretinal haemorrhage. The mild haziness of the photograph is indicative of vitreous haemorrhage. B: Fundus photograph of the left eye at the time of presentation of visual loss in this eye. Extensive subretinal haemorrhage is seen in the macula. Again, the haziness of the photograph is indicative of vitreous haemorrhage. This report highlights a potential interaction between a commonly used anticoagulant, warfarin, and an increasingly common ocular condition — AMD. The risk of AMD increases with age, with prevalence reaching 20% of people aged over 75 years in white populations.1 Neovascular (“wet”) AMD accounts for 10%–20% of cases,2 and for 80%–90% of patients who become legally blind from AMD.3 Peripheral vision is typically retained, and most patients are able to maintain a degree of functional independence. Development of large subretinal and vitreous haemorrhages in neovascular AMD is uncommon. Treatment options are limited and, even with surgical intervention, visual outcomes are in the range of light perception to counting fingers only, with significant loss of paracentral and peripheral vision.4 The functional effects are therefore profound. Previous studies have demonstrated an association between the use of anticoagulant medication, particularly warfarin, and large intraocular haemorrhages among patients with neovascular AMD.5,6 The largest of these, a retrospective case–control study comprising 100 patients, found that those with massive intraocular haemorrhage were 11.6 times more likely to be taking anticoagulant medication.6 Among these patients, INR ranged from 3.0 to 4.0. Patients with massive haemorrhage were twice as likely to be taking aspirin, although the significance of this finding is less certain, as the lower limit of the 95% confidence interval was less than 1. No patient was taking anticoagulants and aspirin concurrently.6 In our patient, the role of concurrent aspirin therapy is unclear, as there is no evidence in the literature to support or refute the hypothesis that concurrent antiplatelet therapy may have contributed to the development of haemorrhage. However, both instances of haemorrhage were noted to occur at times when his INR was high compared with those recorded over the previous 12 months. Our patient was taking aspirin at all times during this period. Although it is possible that intraocular haemorrhage may have occurred purely as a result of his underlying neovascular AMD, the temporal relationship between the development of the haemorrhages and the high INRs strengthens the case for the implication of warfarin therapy as a contributing factor. Application of the Naranjo probability scale7 indicates that this adverse drug event was probable (Naranjo score, + 5). The association between anticoagulant therapy and intraocular haemorrhage is of key importance for several reasons. Firstly, massive intraocular haemorrhage is an important diagnosis to consider in an anticoagulated patient who presents with loss of vision, and who has a background of neovascular AMD. Secondly, patients with neovascular AMD in one eye are at risk of developing neovascular AMD in the second eye,8 and therefore at risk of developing large intraocular haemorrhages in both eyes if long-term anticoagulation is continued. There are several clinical situations in which the indication for anticoagulation is relative. There are key roles for the ophthalmologist, cardiologist, and general practitioner to ensure that an appropriate risk–benefit evaluation is made before initiating anticoagulant therapy for patients with neovascular AMD. Patients taking anticoagulants who develop neovascular AMD, and in particular those with neovascular AMD who are taking anticoagulants and who develop intraocular haemorrhage in one eye, should have their therapy carefully re-evaluated. Importantly, awareness of the poor outcomes of intraocular haemorrhage in neovascular AMD, the role of anticoagulants as a risk factor, and effective communication between health professionals may help reduce the incidence of this devastating complication. Lessons from practice Patients with neovascular age-related macular degeneration (AMD) have a small but significant risk of intraocular haemorrhage, which may be increased in severity if patients are taking anticoagulant medication. The outcomes of intraocular haemorrhage are poor, with significant deterioration in paracentral and peripheral vision, and subsequent implications for ability to maintain functional independence. When considering anticoagulation therapy for patients with neovascular AMD, liaison between the general practitioner, cardiologist and ophthalmologist will ensure that an appropriate risk–benefit evaluation is made. If patients who take anticoagulant medication develop signs of neovascular AMD, it is essential that the ophthalmologist liaise with the GP and/or cardiologist to ensure that the need for anticoagulation, and the target international normalised ratio, are carefully reviewed.

Rajeev Chalasani MB BS · Salmaan Qureshi FRANZCO

To the Editor: Rudkin and colleagues1 remind readers of the importance of detecting congenital glaucoma early to reduce the risks of permanent eye damage, including blindness. The first clinical signs of congenital glaucoma are reported to be blepharospasm, photophobia and excessive tears, all difficult to discriminate in an infant. If the condition is untreated, the cornea progressively loses clarity, and diagnosis becomes more obvious. In giving this account of my personal experience, I remind general practitioners, paediatricians and ophthalmologists that early oedema of the cornea may be detectable before other signs. Our daughter was born uneventfully and without medical problems. Four weeks after the birth, my wife, while gazing into her newborn’s eyes, commented, “Do you think her right eye is . . . more “shiny” than the left?” Looking at all angles, the anxious medical parents were convinced it was. Various medical friends were consulted. “Maybe, possibly”, they indulged us. A call to the senior paediatric ophthalmology registrar at our local children’s hospital was made along the lines of, “Is there such a thing as loss or increase in shine to the eye of a newborn?” In the absence of any other signs, such as inflammation, misery or excessive tears, we were told not to worry. Not reassured, we prevailed upon another ophthalmology registrar who, in a fit-in appointment, confirmed subtle corneal oedema caused by bilateral glaucoma, worse in the left eye. In retrospect, the diagnosis was obvious. “Couldn’t have been anything else”, except the presenting sign was not a cloudy cornea, blepharospasm or misery — it was simply light reflecting off one eye less brilliantly than the other. “It ain’t fine, if it don’t shine.”

Peter J Lewindon

Joffre Bartholomew Cowle was born in Sydney on 28 June 1931. He was educated at Waverley College and the University of Sydney, graduating in medicine in 1956. While still an undergraduate, Joffre had spent 2 years in the university’s Department of Pharmacology under Professor Roland Thorp studying the mode of action of cardiac glycosides. Their research, showing that cortisone increased the rate of polymerisation of skeletal actin but had no effect on cardiac actin, was published in the journal Nature,1 and Joffre was awarded a Bachelor of Science in Medicine with first class honours. After a 2-year residency at the Mater Misericordiae Hospital in Sydney (1956–1957), Joffre spent some time at the Peter MacCallum Cancer Centre in Melbourne and also worked as a Flying Doctor. In 1959, newly married to Colette, a casualty nurse, he went to the United Kingdom, where he worked at the Glasgow Eye Infirmary and later at the Cambridge Regional Hospital. From 1960 to 1961, he held the position of Assistant Professor of Pharmacology at the University of Ottawa in Canada, where he was involved in the pharmacology teaching program for medical students. He obtained a Diploma in Ophthalmology in 1961. Returning to Australia in 1962, Joffre worked at Hornsby District Hospital and the Medical Eye Service of New South Wales, specialising in glaucoma, and then at the Department of Ophthalmology and Eye Health of the University of Sydney. He also did experimental and clinical ophthalmology work in conjunction with the Department of Veterinary Medicine. While working in clinical practice, he continued to do research, and was involved in developing neutral pilocarpine eye drops. Joffre held a number of prestigious positions and awards. He was a Foundation Fellow of the Royal Australian College of Ophthalmologists, established in 1970. In 1980, he set up and became Director of the Research Establishment of Engineering; Biology and Medical Sciences in Sydney. In 1989, he was made a member of the Instituto Barraquer in Barcelona, Spain, a world renowned ophthalmological centre. He was also a Knight of the Military and Hospitaller Order of St Lazarus of Jerusalem and became President of the NSW Commandery. One of Joffre’s great loves was music. He played the Hawaiian steel guitar and built his own recording studio. With his wife and seven children, who played numerous instruments, he performed on radio and television. Although suffering from diabetes, Joffre continued to do research until his death from a heart attack on 21 May 2009. He is survived by Colette, his six sons (who work in various fields of engineering) and his daughter (a musician).

James B Roche

Clinical record A 4-month-old girl was referred to an ophthalmology clinic in May 2008 for assessment of a left convergent squint (esotropia). First noticed 3 weeks previously, the squint had become increasingly prominent. The child’s parents had also noted increased watering of the eyes, ascribed, by the child’s paediatrician, to congenital nasolacrimal duct obstruction. The infant was born preterm (at 36 weeks’ gestation), and had a history of congenital hypothyroidism and a perimembranous ventricular septal defect. There was no family history of glaucoma, and no history of intrauterine infection or birth trauma. Examination confirmed left esotropia. The infant’s eye movements were full, implying a concomitant squint. Her pupils were reactive to light, and with no afferent defects. She was photophobic when her pupillary light reflexes were tested. Aversion to light prevented assessment of visual function. There was tearing (epiphora) in both eyes. The corneas were mildly cloudy and appeared enlarged (buphthalmos); the horizontal corneal diameter measured 13.0 mm in the right eye and 12.5 mm in the left (Figure). A more detailed examination was performed under general anaesthesia. Intraocular pressure (IOP) was elevated at 27 mmHg in the right eye and 28 mmHg in the left (IOP reference range [RR] for an infant under general anaesthesia, 5–14 mmHg).1 The angles were open, and the optic nerve cup-to-disc ratio was increased bilaterally (0.6; RR for infants, < 0.4). Bilateral primary congenital glaucoma was diagnosed. Treatment began immediately with topical ocular antihypertensives and oral acetazolamide, and bilateral trabeculotomy was performed in a staged manner over the next month. At follow-up 13 months after diagnosis, ocular antihypertensive agents had been discontinued, and IOP was acceptable (16 mmHg bilaterally). Horizontal breaks in the basement membrane of the corneal endothelium (Haab striae) were present. Although visual outcome could not be assessed until the child was older, acuity (measured using Teller Acuity Cards) was subnormal bilaterally and worse in the right eye (20/380) than in the left (20/190). To treat amblyopia, the left eye is currently being patched for 2 hours a day. The patient will be reviewed at regular intervals to assess visual function and measure IOP. This case of primary congenital glaucoma (PCG) illustrates the typical clinical features of this condition, which, if untreated, results in blindness. In this case, the infant was referred to our ophthalmology service by a general paediatrician, who was reviewing her for thyroid and cardiac disease; neither of these conditions has a known association with congenital glaucoma. The difficulty in recognising PCG lies partly in its rarity; PCG has a reported incidence of 1 in 30 000 live births in Australia.2 Congenital glaucoma can also be mistaken for a number of common, benign conditions: conjunctivitis, corneal injury, in-turning eyelashes from associated conditions such as epiblepharon (a congenital anomaly in which a fold of skin lies across the lower lid margin), or, as in this case, congenital nasolacrimal duct obstruction. The significance of our patient’s squint is uncertain, although most likely it was a result of her deteriorating visual function. The pathophysiology of PCG appears to be dysgenesis of the trabeculum or its surrounding structures, which results in impaired aqueous outflow and increased intraocular pressure (IOP).3 The conventional outflow path for aqueous humour is shown in the Box. Elevated IOP results in breakdown of corneal endothelial function and an influx of aqueous humour into the normally anhydrous corneal stroma. Prolonged elevation of IOP results in excavation and undermining of the neural and connective tissue of the optic disc, and the development of optic neuropathy. The first clinical signs of PCG include the triad of blepharospasm, photophobia and excessive tearing. Progressively, the corneas become oedematous, lose clarity, and, if untreated, may become opaque. Elevated IOP in an immature eye also results in progressive enlargement of the cornea and sclera, known as buphthalmos (“ox eye”). Potential for corneal enlargement generally ceases when the child reaches 3 years of age, although scleral enlargement can increase beyond this age.3,4 The horizontal corneal diameter in full-term newborn infants averages 9.8 mm; a corneal diameter greater than 11 mm is enlarged.5 A horizontal corneal diameter greater than 12 mm in a 12-month-old child suggests abnormality.6 Progressive enlargement of the cornea may also result in horizontal breaks in the basement membrane of the corneal endothelium (Descemet membrane), known as Haab striae.3 Lessons from practice Congenital glaucoma is a sight-threatening differential diagnosis in an infant who presents with watering eyes. Key clinical features of congenital glaucoma are a cloudy cornea, excessive tearing, blepharospasm, photophobia and an enlarged globe. In infants, corneal size is a surrogate marker of intraocular pressure. Enlargement and asymmetry in corneal diameter requires further investigation. Patients with suspected congenital glaucoma require urgent referral to an ophthalmologist. The mean age of diagnosis is 4.4 months.2 Severe disease is occasionally observed in neonates, although in its early stages PCG tends to have few signs. An urgent referral to an ophthalmologist is required if tearing is associated with photophobia or blepharospasm; the cornea is hazy; or the eye or cornea is enlarged or asymmetrical. It is important to distinguish PCG from congenital nasolacrimal duct obstruction: infants with the latter also have excessive tearing, but, in contrast to PCG, the cornea remains clear, there is no enlargement of the globe and no photophobia or blepharospasm. Nasolacrimal duct obstruction is common, typically innocuous, and frequently resolves spontaneously within the first 12 months of life. It can be safely assessed and managed by the primary-care physician. Diagnosis of PCG relies on IOP measurement. The patient is usually assessed under general anaesthesia, not only to aid IOP measurement, but to permit accurate measurement of corneal diameter and thickness, detection of refractive errors, viewing of the angle structure and a detailed examination of the optic disc. Surgery is the first-line treatment for PCG. The two most common procedures are goniotomy and trabeculotomy, both of which involve microsurgical dissection of the trabecular meshwork, with the aim of increasing aqueous outflow. Ocular antihypertensive agents are an important adjunct to surgery, and are often used as a temporising measure. Screening for amblyopia and correction of asymmetrical refractive errors are undertaken until the child’s vision is developmentally mature to ensure the best visual outcome achievable for the child. Lifelong follow-up is often required. With modern treatment, a visual outcome of better than 6/15 is achieved in up to 79% of cases of PCG; whereas, if untreated, this condition carries an exceedingly poor visual prognosis.3,7 Normal production and outflow of aqueous humour Aqueous humour is produced by the ciliary body and passes from the posterior chamber through the pupil into the anterior chamber. Its outflow path includes the trabecular meshwork, Schlemm canal and the episcleral venous system. The proposed pathophysiology of primary congenital glaucoma is dysgenesis of the trabecular meshwork.

Adam K Rudkin BM BS, BJuris, BA(Hons) · Jwu J Khong MB BS(Hons), MMed · Theresa M Casey MB BS, FRANZCO

To the Editor: The Therapeutic Goods Association (TGA) has informed Australian eye banks that nucleic acid testing (NAT) of donor sera will be required in addition to routine serological tests for hepatitis B virus (HBV), hepatitis C virus (HCV) and HIV. NAT can detect viral genome in the window period between infection and the appearance of antigen or antibody, and is to commence in Australia as soon as possible. However, we believe the benefit of NAT would be small and the cost considerable. Importantly, it would be likely to have an adverse effect on the availability of donor corneas for transplantation. HBV, HCV or HIV have not been transmitted from seronegative donors by means of corneal transplantation. In the mid 1980s, HBV was almost certainly transmitted by corneal tissue to two recipients in the United States who developed acute hepatitis B; HBV surface antigen was detected subsequently in the donor sera.1 The transmission of HCV or HIV has not been reported despite corneal transplantation from infected donors.2,3 The American Red Cross instigated HCV/HIV (but not HBV) NAT for blood donors in 1999, and the US Food and Drug Administration mandated HCV/HIV NAT for eye donors in 2007. So, how many window-period eye donors could be identified by NAT? It has been estimated that 7.2 per 100 000 American tissue donors are in this period for HBV, HCV or HIV.4 With NAT, the number reduced to 1.8 per 100 000 (most assays, including NAT, have sensitivity limitations). Let us assume that the prevalences of HBV and HCV are the same in Australia and the US, the prevalence of HIV in Australia is half that of the US, and that prescreening by medical and social history is equally effective in both countries. At 700 corneal donors (1200 grafts) per year in Australia, one window-period donor would be expected every 23 years, falling to one every 93 years with NAT. Thus, one window-period donor would be detected by NAT every 30 years at an estimated cost of $9 million ($50 per donor averaged up for out-of-hours testing and kit wastage) plus any charges for specimen transport. If only HCV/HIV NAT is performed, such a donor would be detected every 52 years. What is the actual risk of infection? Assuming 25 000 corneal donors (43 000 grafts) per year in the US, the figures4 suggest that eyes were collected from one window-period donor every 7 months between 1990 (when a serological test for HCV became available) and 2007, but no infections have been reported. Perhaps there was no virus in the corneal tissue; even among donors who are seropositive for HBV, HCV or HIV, few have detectable viral genome in the cornea.5,6 It is not known whether these viruses can invade the cornea before the appearance of antibody, but the risk that infection will occur in recipients of corneas from HCV/HIV-seronegative, NAT-positive donors appears to be very small indeed. The TGA’s decision may have been based on factors other than this type of analysis. We believe the decision should be reconsidered.

Paul R Badenoch · Douglas J Coster

To the Editor: Following the letter by Kitson et al, about a patient with asymptomatic ocular syphilis,1 we describe another unusual presentation of syphilis, with perioptic neuritis that mimicked papilloedema. A 28-year-old man presented with blurry central vision of his right eye on waking. He had had flu-like symptoms 2 days before presentation. There were no symptoms suggestive of meningitis, no raised intracranial pressure and no obvious ocular infection or inflammation. He had been diagnosed with type 2 diabetes mellitus at age 15 years, and had no known microvascular complications. His only medication was metformin. His visual acuity was 6/12 in the right eye and 6/6 in the left. The right eye had an enlarged blind spot with a small paracentral scotoma, with a central relative scotoma to red. The visual field in the left eye was normal. There was no relative afferent pupillary defect, and the anterior chambers were normal. Fundoscopy confirmed bilateral swelling of the optic discs (Box). Computed tomography and magnetic resonance imaging of the brain excluded space-occupying lesions and demyelination. Examination of the cerebrospinal fluid (CSF) showed: normal opening pressure; a white cell count of 66 × 109/L (reference range, 4–11 × 109/L), 100% lymphocytes; a protein concentration of 1.12 g/L (reference range, 0.15–0.45 g/L); a glucose concentration of 8.9 mmol/L (reference range, 2.7–4.4 mmol/L); and no bacterial growth. Findings of a baseline blood analysis were normal, but with an elevated glycated haemoglobin (HbA1c) level of 10.5% (reference range, < 6%), indicating poorly controlled diabetes. We thus initially considered diabetic papillopathy and non-arteritic ischaemic optic neuropathy, but the patient subsequently (about a week later, after most other investigations gave negative results) reported having had unprotected sexual intercourse with different men about 5 months previously. He recalled having a transient palmar maculopapular rash 2 months before presentation. Neurosyphilis was then confirmed with a strongly positive result on an absorbed fluorescent treponemal antibody (FTA-ABS) test in CSF. Results of serological tests for syphilis were positive (reactive [3+] on a Treponema pallidum particle agglutination test and a markedly elevated titre on a rapid plasma reagin test [1:128]), but results of tests for HIV and hepatitis were negative. Contact tracing was initiated. After 21 days of treatment with intravenous penicillin, the vision in his right eye was completely restored. At 9-month follow-up, the bilateral swelling in his optic discs had resolved, and there was a tenfold decline in the rapid plasma reagin test titre (1:8). This case illustrates syphilitic perioptic neuritis with optic disc swelling that is indistinguishable from papilloedema. Perioptic neuritis describes inflammatory involvement of the optic nerve sheath without inflammation of the nerve itself. The affected eye often has normal visual acuity with no visual field defect except for an enlarged blind spot.2,3 The prevalence of infectious syphilis has been rising in recent years, especially in Victoria and New South Wales, predominantly among men who have sex with men.4,5 Endemic syphilis should also be considered in patients with HIV co-infection and Indigenous patients, particularly those in the remote communities of Western Australia. Physicians should be vigilant in looking for neurosyphilis in any sexually active individual with unexplained eye or neurological signs. The swollen optic discs of a patient with syphilitic perioptic neuritis

Grace S H Low · Robert H Edis

To the Editor: The article by Statham and colleagues raises important issues about the accuracy of diagnosis by primary eye care providers, with all professionals in the study recording a diagnostic accuracy rate of less than 50%.1 From the general practice perspective, the authors raise a number of important contributors — lack of equipment, ophthalmological expertise and time. Additional factors, such as undergraduate and postgraduate exposure, and targeted training in the diagnosis of sight-threatening acute eye conditions, are also crucial considerations. From a postgraduate point of view, the Royal Australian College of General Practitioners offers a comprehensive continuing professional development program to support broad-based GP training, including in eye disease.2 The Master of Medicine (GP) offered by the University of Queensland also includes a dedicated subject on primary eye care, with particular emphasis on sight-threatening presentations.3 The Division of General Practice in which one of the sites in the report by Statham and colleagues1 sits is currently working with that hospital’s Department of Ophthalmology to institute an education/continuing professional development reform program to better target hospital eye referrals from primary care (Shelley Kleinhans, Health Systems Improvement Program Manager, GPpartners [Brisbane North] Division of General Practice, personal communication). It is very important to describe accurately the dimension of missed diagnosis within primary care — the ensuing challenge is to address it by harnessing the significant momentum within the primary care community for quality improvement.

Claire L Jackson