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ImportanceSNRNP200is a recently identified genetic cause of autosomal dominant retinitis pigmentosa (RP). However, the associated retinal phenotype is not well characterized. ObjectiveTo describe the retinal phenotype in patients with RP secondary to variants inSNRNP200. Design, Setting, and ParticipantsThis retrospective, case-series study was performed at 2 tertiary referral centers for inherited retinal diseases. Participants included 9 consecutive patients from 8 families with RP attributed to variants inSNRNP200. Data were collected from August 2017 to March 2018 and analyzed from May to July 2018. Main Outcomes and MeasuresResults of clinical evaluation, multimodal retinal imaging, and molecular genetic testing using targeted next-generation sequencing. ResultsOf the 9 patients included in the analysis (4 female and 5 male; mean [SD] age at presentation, 19 [15] years), each presented with nyctalopia, typically in the first 2 decades of life, although 2 patients experienced symptom onset in middle age. None had any consistent systemic features suggestive of syndromic RP. Retinal imaging studies and electroretinography findings were typical of a rod-predominant dystrophy with later involvement of cone photoreceptors. Phenotypic heterogeneity was typified by 4 unrelated patients with the common c.2041C>TSNRNP200variant who demonstrated a variable age of disease onset (middle teenage years to the fourth decade of life). Disease progression was slow, with all but 1 patient maintaining visual acuity of better than 20/40 in the better-seeing eye in the fifth and sixth decades of life. Conclusions and RelevanceThese data suggest that variants inSNRNP200result in nonsyndromic RP with a typical phenotype of a rod-predominant dystrophy. Significant phenotypic heterogeneity and nonpenetrance were noted within some affected families. Symptom onset was typically within the first 2 decades of life, with slow progression and well-preserved visual acuities into the fifth and sixth decades.

Direct ophthalmoscopy (DO) is an examination technique used to inspect the retina and other structures of a patient ’ s eye. A skilled examiner may perform DO to identify clinical signs in the eye that may signify a sightthreatening disorder such as diabetic retinopathy, glaucoma and neovascular agerelated macular degeneration, or a lifethreatening disorder such as raised intracranial pressure or retinoblastoma/choroidal melanoma (Figure 1 ). These disorders are treatable, so it is important that all clinicians are competent at DO.

Neisseria meningitidis is a major cause of childhood morbidity and mortality worldwide. We describe an exceptional case of an immunocompetent 15-month-old child presenting with a unilateral anterior uveitis, hypopyon, and sepsis. Anterior chamber aspirate demonstrated gram-negative cocci before Neisseria meningitidis was identified in blood and cerebrospinal fluid. Meningococcal endophthalmitis presents variably with sepsis, meningitis, or isolated ocular symptoms. Diagnosis is a clinical challenge, requiring diagnostic sampling and treatment from both pediatricians and ophthalmologists. Delayed or incorrect treatment risks blindness, disability, or death. Simultaneous invasion of meningococcus across intact blood-brain and blood-ocular barriers in this child suggests antigenic correlates between meningeal and ocular endothelial interfaces. Meningococcus is an exclusively human pathogen; research is hampered by the lack of animal models. This clinical observation suggests the potential of a novel in vitro experimental approach of using ocular tissue from eye banks to further elucidate the meningococcal-endothelial interaction that underpins meningococcal disease.

Background: A 71-year-old female presented on 3 occasions with escalating pain in a congenitally blind eye. Examination revealed hypertensive uveitis with morning glory optic disc dysplasia and absence of a crystalline lens. There was no previous intraocular surgery or trauma. Intensive anti-hypertensive agents and topical steroids did not control intraocular pressure (IOP) or inflammation. Results: Dilated fundus examination on the third clinical review revealed a luxated cataractous lens on the retina. Pars plana vitrectomy and fragmatome lensectomy controlled inflammation and IOP, with resolution of ocular pain. Discussion: This is an exceptional case of phacogenic uveitis with secondary glaucoma occurring years after spontaneous crystalline lens luxation in a patient with morning glory syndrome. The embryological pathogenesis of morning glory syndrome and the significance of accelerated cataractogenesis and zonular weakness are discussed. Hypertensive uveitis with unexplained absence of a crystalline lens in a blind eye must prompt suspicion of delayed phacogenic uveitis following asymptomatic lens luxation.

A 49-year-old female presented to ophthalmology with intractable diplopia, secondary to decompensated esotropia. Her diplopia was controlled for several years with spectacle prisms, and thereafter with an occlusive contact lens until she became contact lens intolerant.

Purpose To describe the intraoperative use of the Heidelberg Spectralis for ultra-widefield fundus fluorescein angiography in infants. Design Retrospective observational case series. Methods A modified Heidelberg Spectralis was used to perform ultra-widefield fundus fluorescein angiography in infants undergoing an examination under general anesthesia for a range of retinal vasculopathies. Peripheral angiographic findings, angiographic image acquisition time, and any complications were reviewed. Results A total of 22 eyes of 11 infants underwent intraoperative ultra-widefield fundus fluorescein angiography using the modified Heidelberg Spectralis. Ultra-widefield fundus fluorescein angiography was successfully performed in all infants and permitted capture of the posterior pole as well as the peripheral retina in a single shot centered on the macula. Peripheral retinal pathologies captured include neovascularization, capillary nonperfusion, and skip areas from previous laser treatment. Capturing of angiographic images took a mean time of 7.09 minutes. Image artifact from condensation of the ultra-widefield lens was noted during imaging of 1 infant. Conclusions The modified Heidelberg Spectralis is an effective and reliable imaging tool for performing ultra-widefield fundus fluorescein angiography in infants. It is capable of capturing wide-angle images of high quality. The technique has advantages as an alternative to RetCam fluorescein angiography in infants undergoing an examination under general anesthesia.

Therapeutic occlusion of ocular media is indicated for patients with intractable diplopia that is refractory to optical, medical, and surgical treatment strategies1; for visual confusion where it is able to improve functional vision2; or for unsightly leukocoria, to achieve superior cosmesis.3–5 Occlusive strategies include permanent eye-patching or tarsorrhaphy, use of occlusive contact lenses, corneal tattooing (keratopigmentation),6,7 or insertion of an occlusive, or black intraocular lens (IOL).1–5,8,9 The use of black occlusive IOLs as a therapeutic solution, particularly for intractable diplopia, has been restricted by concern that such media prevent ophthalmologists from detecting important clinical signs and diseases including papilledema or choroidal melanoma of the optic disc, macula, and retina by slit lamp biomicroscopy and other instruments that use wavelengths of light across the visible spectrum. Interference with ocular examination and imaging may negatively influence ophthalmologists from offering—and patients from selecting—occlusive IOL implantation as a treatment modality. Despite these uncertainties, occlusive IOLs are offered and implanted in specific patient groups, particularly among those refractory to other treatment strategies such as prismatic correction or surgery in diplopia. The incidence of intractable diplopia has been estimated at 0.8% of patients after strabismus surgery,10 although the true prevalences of visual confusion and unsightly leukocoria remain unknown. However, these conditions are particularly disabling for patients who suffer from them,11 often requiring permanent occlusion to restore functional vision.12 In this patient group, occlusive IOL implantation has been shown to be an effective management strategy; it requires no maintenance and is inconspicuous once implanted, factors that contribute to the high levels of postoperative satisfaction, particularly in patients with intractable diplopia.1,2,8,9,13 Recently, it was discovered that macular imaging was possible through the 85F Morcher PMMA occlusive IOL with scanning laser ophthalmoscopy (SLO) and optical coherence tomography (OCT) scanner (Spectralis SLO/OCT; Heidelberg Engineering, Heidelberg Germany).12 The SLO/OCT imaging systems in clinical use have near-infrared (NIR) spectrum light (820–870 nm), and it was the assumption of this observation that the Morcher (Stuttgart, Germany) PMMA occlusive IOL transmits sufficient NIR light to permit retinal and macular imaging. Confirmation of NIR transmission and proof of SLO/OCT imaging capabilities across occlusive IOLs may allow more patients with intractable diplopia to benefit from this effective therapeutic strategy, as the reluctance to implant them due to difficulties in visualizing the retina would no longer influence clinical judgment.

UNLABELLED: We describe a 40-year-old woman with von Hippel-Lindau syndrome, secondary chronic exudative retinal detachment, and white cataract with consequent leukocoria. Because the cataract was contracted and calcified, a black Morcher 85F poly(methyl methacrylate) IOL was implanted in the ciliary sulcus anterior to the lens to prevent posterior capsule rupture and potential destabilization of the retinal detachment with a risk for phthisis. Excellent centration of the black IOL was achieved with good cosmetic outcome and without postoperative uveitis or glaucoma. We suggest that this is a rare indication for implantation of an IOL designed for the sulcus but in the presence of the crystalline lens. This approach makes IOL removal, if necessary at a later date, relatively straightforward. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

The place of ophthalmology in the undergraduate medical curriculum is slowly fading—with certainty, and yet without objection. Over the past 30 years, the duration of the ophthalmology placement has dwindled in the United States,1 the United Kingdom,2 and elsewhere.3 The inescapable truth is that without renewed activism from ophthalmologists interested in the education of tomorrow's doctors,1 the ophthalmology clerkship—exposed by poor representation from ophthalmologists on medical school committees—may soon disappear entirely.1, 2, 3 The likely outcome is a generation of clinicians who lack confidence and competence in basic ophthalmic examination,1 the management of simple eye complaints, and the safe triaging of patients under their care who develop serious ophthalmic disease.4 Skills in direct ophthalmoscopy among undergraduates have declined in parallel.5 Medical students lack confidence in direct ophthalmoscopy,6 and often request additional training to improve their performance.7 Several challenges exist for ophthalmologists and others involved in training of direct ophthalmoscopy: (1) reduced exposure to ophthalmology clinics where direct ophthalmoscopy is traditionally taught; (2) ownership of direct ophthalmoscopes in the United Kingdom has fallen since 1986 following withdrawal of grants traditionally used to fund them;8 (3) there is no clear consensus as to the standard of proficiency in direct ophthalmoscopy a medical student should satisfy;1, 9 (4) direct ophthalmoscopy is poorly represented in undergraduate examinations2—an influential determinant of student learning behaviour. With crisis comes opportunity, and approaches have been suggested to address these challenges:1, 10 to identify and prioritize essential ophthalmic clinical skills; to advocate for ophthalmology clerkships in the undergraduate curriculum; to integrate ophthalmology in the teaching of all basic sciences and allied clinical disciplines; and to innovate to create opportunities for clinical training in ophthalmology for undergraduates.10 One cannot advocate direct ophthalmoscopy without supporting undergraduate ophthalmology training around it: fundal findings are interpreted in the context of other elicited clinical signs, synthesized with relevant clinical knowledge to accurately triage or manage a patient's ocular complaint. It is common for patients with ophthalmic symptoms to present to primary care, minor injury units, and emergency departments.11 Immediate access to an ophthalmologist for clinical assessment is not universal. Non-specialist clinicians have a duty of care to perform a competent basic ophthalmic examination—of which direct ophthalmoscopy may be an essential component—to safely assess or triage a patient.11, 12 Omission of the components of a basic ophthalmic examination may be medico-legally indefensible, yet is common.4 Direct ophthalmoscopy adds a valuable weapon to the diagnostic armamentarium of a clinician. The identification of spontaneous venous pulsation may exclude raised intracranial pressure and prevent the need for acute neuroimaging in a child with meningococcal meningitis.13 The detection of papilloedema may be life-saving—potentially indicating a cerebral space occupying lesion or accelerated hypertension.14 Concealed systemic disorders may be elucidated on direct ophthalmoscopy, for example, endocarditis or cytomegalovirus retinitis—obviating the need for invasive investigations. Abusive head trauma may be supported by the finding of bilateral retinal haemorrhages on direct ophthalmoscopy in an infant with unexplained encephalopathy.15 Direct ophthalmoscopy may accurately distinguish causes of acute, painless visual loss into those requiring immediate treatment or referral (such as central retinal artery occlusion) from those which may be referred non-urgently (central retinal vein occlusion). Timely diagnosis of retinal artery occlusion could permit reversal of sight loss or prevent an impending stroke through identification of an embolic source. Swift recognition of leukocoria on direct ophthalmoscopy may improve visual outcome in congenital cataract or eye-saving in cases of retinoblastoma. To deprive medical students of a valuable clinical skill will leave them exposed in such contexts, and may endanger their patients.12 The cost of its omission is the additional time, investigation, anxiety, and travel required of a patient to arrive at a diagnosis—or worse—failure to make a diagnosis at all. Medical school committees would consider it unthinkable to omit training in cardiac auscultation simply because it is a difficult clinical skill. They would challenge any assertions that it is specialist skill undertaken only by cardiologists, or that an echocardiogram may safely circumvent it. Put simply, it is bad medicine to omit direct ophthalmoscopy in such contexts. Accordingly, the relegation of direct ophthalmoscopy from the undergraduate medical curriculum may be considered negligent. Technological advances may, in time, supersede the direct ophthalmoscope. The pan-ophthalmoscope circumvents the technical skill of direct ophthalmoscopy, but not the interpretation of the retinal image. Non-mydriatic digital fundus cameras promises accurate remote diagnosis by ophthalmologists using images captured by non-specialists at the point of care16 (emergency departments, primary care). It requires investment and training, and few ophthalmic units have established it. It is unlikely to be available in the foreseeable future in the developing world. The imperative for ophthalmologists to protect the teaching of direct ophthalmoscopy transcends the diagnostic value of the clinical skill itself. It strikes at the quintessence of the real issue of ophthalmology in the undergraduate curriculum: influential non-specialists contend—largely unchallenged—that the responsibility for ophthalmic assessment, diagnosis and treatment rests exclusively with the ophthalmologist. Defending the place of direct ophthalmoscopy in the undergraduate curriculum emphasizes our opposition to the global relegation of ophthalmology, and underlines a clear message: basic skills in ophthalmic assessment are mandatory. Students are required to learn them, doctors to practice and teach them, and patients to receive them. To forfeit direct ophthalmoscopy is to concede to this erroneous notion of diminished responsibility for ophthalmic health by non-specialists. Ophthalmologists are on occasion witnesses to mistakes in referral or assessment by non-specialists—errors whose root cause is the inadequate training at the undergraduate level, and where an irretrievable attitude of passivity to ophthalmology is ingrained. As ophthalmologists we are advocates for ophthalmic health. We must take action locally, regionally, and nationally to preserve direct ophthalmoscopy—and ophthalmology—in the undergraduate medical curriculum for the benefit of our future patients.1, 12 It is in the interests of the Royal College of Ophthalmologists to support it. Why? ‘Ut Omnes Videant': so that all may see.

Sir, We thank Professor MacEwen and coauthors1 for responding to our letter describing the factors influencing primary black intraocular lens (IOL) selection.2 They present the first study describing the clinical outcomes following primary implantation of a large (>7 mm optic) black IOL within the capsular bag. The Ophtec Black Ani II IOL (Ophtec BV, Groningen, The Netherlands) is a novel device produced from a material that is identical to the Artisan 201 iris-claw IOL, which has been proven to be occlusive to near infra-red (NIR) light.3 This series adds to the growing experience with black IOL implantation, and several observations warrant further comment. The mechanism of light perception following black IOL implantation is a curious phenomenon and its identification is critical to minimize the risk of treatment failure.4, 5 High rates of postoperative satisfaction are reported in patients with NIR-transmitting black IOLs,6 despite near universal postoperative light perception.5 We have suggested previously that trans-optical NIR light transmission across NIR-transmitting black IOLs may underlie intractable ghosting and treatment failure.4, 5 Such symptoms are extinguished by secondary NIR-blocking Ophtec black iris-claw IOL implantation.4, 7 Postoperative light perception in this series suggests para-optical light leakage despite a 9 mm occlusive optic, exceeding the scotopic pupil diameter. It is unlikely that formed, foveal images would result as a consequence: perceptions of ‘ghosting' following Ophtec Ani II Black IOL implantation are unlikely. Numerous black IOLs are available for clinical use, each with specific dimensions and properties that must be tailored to lens status/pupil size and the risk of retinal or optic nerve disease in each individual patient.2 We have recently described ultra-widefield imaging in a patient with a NIR-transmitting black IOL using the Heidelberg Spectralis Scanning Laser Ophthalmoscope (Heidelberg Engineering, Heidelberg, Germany). This novel discovery may drive primary implantation of NIR-transmitting black IOLs to achieve a balance of efficacy and safety – permitting detection of retinal and optic nerve pathology across the life of the implant. All black IOLs are likely to alleviate symptoms in patients with intractable diplopia and represent valid treatment modalities. However, it is essential that both patient and surgeon are informed of the risks of treatment failure and the possibility of ultra-widefield retinal imaging when a primary black IOL is selected.

Purpose To evaluate the feasibility of ultra-widefield retinal imaging in patients with near infrared (IR)–transmitting black intraocular lenses (IOLs). Setting Oxford Eye Hospital, Oxford, United Kingdom. Design Laboratory evaluation of a diagnostic technology with interventional case report. Methods The field of retinal imaging through a Morcher poly(methyl methacrylate) (PMMA) black IOL was determined in a purpose-built adult schematic model eye with the HRA2 Spectralis confocal scanning laser ophthalmoscope using standard imaging, Staurenghi retina lens–assisted imaging, and ultra-widefield noncontact imaging. Retinal imaging using each modality was then performed on a patient implanted with another Morcher PMMA black IOL model. Results Ultra-widefield noncontact imaging and lens-assisted imaging captured up to 150 degrees of field (versus 40 degrees with a standard confocal scanning laser ophthalmoscope). Ultra-widefield retinal images were successfully acquired in a patient eye with a black IOL. Conclusions This study has identified the first ultra-widefield retinal imaging modalities for patients with near IR-transmitting black IOLs. Should larger studies confirm this finding, noncontact ultra-widefield confocal scanning laser ophthalmoscopy might be considered the gold standard imaging technique for retinal surveillance in patients with near IR-transmitting black IOLs. Financial Disclosure No author has a financial or proprietary interest in any material or method mentioned.

SUMMARY Persistent hyperplastic primary vitreous (PHPV) is an ocular developmental disorder resulting from incomplete apoptosis of the embryonic hyaloid vasculature. Unilateral PHPV is traditionally associated with a poor prognosis because of the challenges associated with managing progressive anisometropic amblyopia. We report a child with unilateral PHPV who underwent cataract extraction, primary posterior capsulotomy with anterior vitrectomy and intraocular lens implantation followed by combined trabeculectomy/trabeculotomy within the first 8 weeks of life. Intensive optometric and orthoptic input was required for many years to manage the increasing anisometropic amblyopia with final visual acuity of 20/40 unaided in the affected eye and without evidence of glaucomatous optic neuropathy. This case illustrates the excellent visual outcome possible in a child with complex, unilateral PHPV using an intensive management approach comprising: early surgical intervention for congenital cataract and secondary glaucoma, meticulous monitoring of refraction, visual acuity and intraocular pressure and motivated parents who engaged in the management. BACKGROUND Persistent hyperplastic primary vitreous (PHPV)— or persistent fetal vasculature—results from persistence of the embryonic hyaloid vasculature manifesting variably with a combination of anterior and posterior segment abnormalities. Unilateral PHPV was often managed conservatively or associated with poor outcomes due to difficulties in the longterm management of progressive anisometropic amblyopia. We report a child with unilateral PHPV —who had an excellent visual outcome beyond visual maturity—with early combined cataract extraction, cauterisation of the hyaloid stalk and primary trabeculectomy/trabeculotomy undertaken within the first 8 weeks of life. This case demonstrates the excellent long-term visual outcome in children with unilateral PHPV with an intensive management approach. We suggest a similar approach be considered in this patient group.

A 61-year-old woman presented with an 18-year history of a debilitating ghosting phenomenon and light perception in her right eye following implantation of a Morcher black intraocular lens (IOL) for intractable diplopia. On 5 occasions, she was referred to hospital ophthalmology, repeatedly requesting right optic nerve transection to alleviate the symptoms. The mechanism of light perception across the occlusive IOL remained enigmatic, preventing appropriate management. Morcher occlusive IOLs transmit high levels of near infrared (IR) light, which may cause light perception under photopic conditions by long-wavelength cone photoreceptors. Considering this, we implanted a near IR–blocking Artisan black iris-claw IOL anterior to the Morcher occlusive IOL. Postoperatively, the light perception and ghosting disappeared and the visual quality of life score (VF-14) increased from 41 to 86. We suggest consideration of this surgical approach of black-on-black IOL implantation for patients with refractory light perception despite implantation of a near IR–transmitting occlusive IOL. Financial Disclosure No author has a financial or proprietary interest in any material or method mentioned.

Pyridoxine-dependent epilepsy (PDE) is a cause of neonatal epileptic encephalopathy not previously known to cause ophthalmic disease. We describe the novel observation of a 5-year-old girl with pyridoxine-dependent epilepsy and bilateral cataracts. PDE is the result of mutations in the ALDH7A1 gene encoding antiquitin, an enzyme protective against cellular dehydration and osmotic stress. Accumulating metabolic precursors in PDE have been shown to be cataractogenic in vitro, and experimental pyridoxine deficiency has been associated with lenticular opacities in vivo. The association of ALDH7A1 haploinsufficiency in PDE and congenital cataract may offer insight into the relationship between osmotic stress and fetal cataract development. Bilateral progression of cataracts in this child suggests ongoing metabolic dysregulation within the crystalline lens despite pyridoxine supplementation at doses sufficient to control seizure activity.

An infant born at 24-weeks gestation weighing 750 grams 33 underwent examination with indirect ophthalmoscopy and scleral indentation for retinopathy of prematurity (ROP) at 14 weeks post-natal age. A second examiner noted widespread retinal hemorrhages. Ultra-widefield non-contact retinal imaging and fundus fluorescein angiography (0.1mL/kg of 10% fluorescein) was performed (Optos PLC, Dunfermline, Scotland, UK) using the “flying baby position”).

IMPORTANCE: Patients with neuromyelitis optica who have aquaporin-4 antibodies are being identified and receiving immunosuppressant treatment earlier and more aggressively as a result of increasing awareness of the importance of preventing relapses responsible for the high morbidity and mortality associated with the disease. To our knowledge, opportunistic retinal infection in patients with aquaporin-4 antibodies who are receiving immunosuppressants has not been reported to date. OBSERVATIONS: We describe 2 patients with aquaporin-4 antibodies who were receiving conventional doses of first-line immunosuppressive therapy. Both patients presented with vision loss that was initially thought to be optic neuritis attacks. The subsequent diagnoses were ocular toxoplasmosis and cytomegalovirus retinitis. CONCLUSIONS AND RELEVANCE: Retinal opportunistic infections can occur in patients with aquaporin-4 antibodies who are receiving relatively low levels of immunosuppression, may mimic optic neuritis, and are a potentially reversible cause of vision loss when treated promptly.

Sir, We read with interest the recent report by Shonibare and Lochhead1 describing a patient with visual confusion who suffered troublesome visual ghosting following primary implantation of a near-infrared (NIR)-transmitting black IOL. Secondary implantation of a NIR-blocking Artisan black IOL abolished the patient's symptoms. Simultaneously—and independently—we reported an identical experience in a patient with intractable diplopia who required a secondary NIR-blocking Artisan IOL to suppress debilitating visual ghosting.2 NIR light is assumed imperceptible to humans, yet perception of light is almost universal in patients following NIR-transmitting black IOL implantation.3 In all, 91% of patients perceived light post-operatively, the remainder had acquired optic neuropathies with impaired long-wavelength spectral sensitivity and consequently could not perceive NIR light.3 Taken together, these observations suggest that careful and systematic evaluation of both patient factors and black IOL properties are necessary to identify the optimal primary black IOL implant pre-operatively in each patient to minimize the risk of treatment failure. First, patient factors should be considered including the necessity of posterior segment monitoring, need for absolute light occlusion, scotopic pupil size, lens status, and optic nerve pathology (Table 1). Second, the properties of each black IOL, including optimum surgical configuration, occlusive optic size, and utility in primary and secondary IOL implantation must then be considered (Table 2). Black intraocular lenses are variably occlusive to NIR light and may be usefully categorized by this property.4 Table 1 Summary of patient factors relevant to primary Black IOL selection in eligible patients Table 2 Technical details of all black intraocular lenses in clinical use High levels of post-operative satisfaction have been reported with NIR-transmitting black IOL implantation,5 although emerging reports of treatment failure do advise some caution.1, 2, 3 Predicting this risk pre-operatively is critical to the long-term efficacy of surgical intervention. Design of a NIR-transmitting black contact lens would permit a therapeutic trial to determine pre-operative NIR light sensitivity where uncertainty exists. Primary implantation of a NIR-blocking IOL would be advised if troublesome ghosting occurred. SLO/OCT posterior segment imaging remains a distinct clinical advantage in patients with NIR-transmitting black IOLs and may improve long-term safety. However, informed consent in this context must raise the possibility of treatment failure and need for secondary NIR-blocking IOL implantation in this patient group.

Transient retinal artery occlusion: the potential utility and limitations of electroretinography

Purpose To determine the feasibility of Ultra wide-field intravenous fundus fluorescein angiography (UWF-IV-FFA) in infants. Methods A retrospective review of infants who underwent UWF-IV-FFA with the Optos P200MA was performed. The ability to capture different phases of the angiogram, the total image acquisition time and the presence of image artefacts were analysed. Results Twelve infants with a variety of proliferative retinopathies underwent UWF-IV-FFA over a 7-month period. The mean age was 3.4 months (range 2–6 months) and the mean image acquisition time was 4 min (range 3–5). Pseudocolour fundus images and the venous, recirculation and late phases of UWF-IV-FFA were captured successfully in all infants (100%). Choroidal and arterial phases were captured in one (8.3%) and 10 (83.3%) infants, respectively. Image artefacts due to eyelashes and corneal desiccation occurred in nine (75%) and six (50%) infants, respectively. No adverse medical problems were noted. Conclusions We have shown that UWF-IV-FFA can be successfully and safely performed in infants using the Optos P200MA system. It is a feasible new alternative to RetCam for obtaining an IV-FFA in the outpatients setting.

Krohn et al describe a novel photographic technique using near-infrared (NIR) transillumination to detect peripheral anterior choroidal and ciliary body melanomas not visible on slit lamp examination.1 The described imaging technique uses a broad spectrum background light source to illuminate the fundus and external photography with a 720–1100 nm NIR filter to evaluate anterior uveal masses, permitting topographical description of tumour location in relation to the ciliary body and ora serrata.1 ,2 NIR transillumination photography may serve another essential clinical application as the first diagnostic technique described capable of detecting anterior uveal melanomas in patients implanted with black intraocular lenses (IOLs) who cannot undergo slit lamp examination. Black IOLs are indicated in patients with intractable diplopia, visual confusion, unsightly leucocoria and a range of neuro-ophthalmic disorders.3 Despite high rates of postoperative satisfaction in patients,4 their use has been restricted by concerns from ophthalmologists that an inability to visualise the retina may prevent the identification of life-threatening pathology—principally uveal melanoma—behind the occlusive IOL.5 Morcher black IOLs have been demonstrated to transmit high levels of NIR light.5 This property permits high quality imaging of the macula and optic nerve using scanning …

Children treated for toxoplasma retinochoroiditis may experience a range of severe adverse drug responses. Drug reaction with eosinophilia and systemic symptoms syndrome is a life-threatening idiosyncratic drug reaction with a 10% mortality. We present a case of drug reaction with eosinophilia and systemic symptoms syndrome in a child on standard combination treatment with oral sulfadiazine, pyrimethamine, folinic acid, and steroids for toxoplasma retinochoroiditis. Early clinical recognition and appropriate treatment led to a complete recovery and no longterm sequelae. The parents of children during sulfadiazine treatment should be counseled on the potential significance of nonspecific illness.

A 64-year-old man presented to casualty with blurred vision and pain in his left eye, 2 h after inadvertently instilling clear nail glue into his eye instead of postoperative Timolol eye drops. The glue was removed at the slit lamp revealing a corneal abrasion, which was managed with topical antibiotics. Inadvertent ocular cyanoacrylate, or ‘superglue’, instillation has been reported frequently since 1982 when superglue was repackaged into ophthalmic style dropper bottles. This case report highlights the continuing problem of cyanoacrylate eye injuries and serves as a reminder to healthcare professionals to report such incidents to manufacturers and regulatory bodies, on behalf of their patients, to promote the introduction of universal safety mechanisms on all household chemical containers. Failure of glue manufacturers to introduce safety cap mechanisms has resulted in significant ocular morbidity over the last 27 years, and such incidents are expected to occur until superglue bottles are redesigned.

Purpose To evaluate whether occlusive intraocular lenses (IOLs) produced by several manufacturers for clinical use equivalently transmit near-infrared (IR) light for scanning laser ophthalmoscopy (SLO) or optical coherence tomography (OCT) imaging. Setting Nuffield Laboratory of Ophthalmology, Oxford University, United Kingdom. Design Evaluation of diagnostic test or technology. Methods The study evaluated 6 black IOLs of 2 designs: 3 poly(methyl methacrylate) (PMMA) and 3 iris-claw anterior chamber IOLs. Each IOL was placed between a broad-spectrum white light source and a spectroradiometer to generate transmission spectra. Transmission in the near-IR range was examined using an 850 nm light-emitting diode. Scanning laser ophthalmoscopy or OCT imaging using Spectralis spectral-domain SLO or OCT was attempted through occlusive IOLs in a model eye. Results Artisan iris-claw and MS 612 PMMA occlusive IOLs totally occluded all wavelengths of light, including in the near IR range in which SLO and OCT imaging systems operate. It was not possible to capture SLO or OCT images through the iris-claw and PMMA occlusive IOLs in a model eye. Conclusions Results suggest the property of near-IR transmission that permits SLO or OCT imaging through occlusive IOLs is restricted to the Morcher range of occlusive IOLs. Patients with non-near IR transmitting IOLs will not be able to receive detailed posterior segment monitoring with SLO or OCT. This finding may have a significant impact on preoperative occlusive IOL selection and the management of current patients with occlusive IOLs. Financial Disclosure No author has a financial or proprietary interest in any material or method mentioned.

TDP-43 immunoreactive (TDP-43-ir) pathological changes were investigated in the temporal cortex and hippocampus of 11 patients with autosomal dominant familial forms of Alzheimer's disease (FAD), 169 patients with sporadic AD [85 with early onset disease (EOAD) (i.e before 65 years of age), and 84 with late onset after this age (LOAD)], 50 individuals with Down's Syndrome (DS) and 5 patients with primary hippocampal sclerosis (HS). TDP-43-ir pathological changes were present, overall, in 34/180 of AD cases. They were present in 1/11 (9%) FAD, and 9/85 (10%) EOAD patients but were significantly more common (p = 0.003) in LOAD where 24/84 (29%) patients showed such changes. There were no demographic differences, other than onset age, between AD patients with or without TDP-43-ir pathological changes. Double immunolabelling indicated that these TDP-43-ir inclusions were frequently ubiquitinated, but were only rarely AT8 (tau) immunoreactive. Only 3 elderly DS individuals and 4/5 cases of primary HS showed similar changes. Overall, 21.7% of AD cases and 6% DS cases showed hippocampal sclerosis (HS). However, only 9% FAD cases and 16% EOAD cases showed HS, but 29% LOAD cases showed HS. The proportion of EOAD cases with both TDP-43 pathology and HS tended to be greater than those in LOAD, where nearly half of all the cases with TDP-43 pathology did not show HS. The presence of TDP-43-ir changes in AD and DS may therefore be a secondary phenomenon, relating more to ageing than to AD itself. Nevertheless, a challenge to such an interpretation comes from the finding in AD of a strong relationship between TDP-43 pathology and cognitive phenotype. Patients with TDP-43 pathology were significantly more likely to present with an amnestic syndrome than those without (p < 0.0001), in keeping with pathological changes in medial temporal lobe structures. HS was also associated more commonly with an amnestic presentation (p < 0.005), but this association disappeared when TDP-43-positive cases were excluded from the analysis. TDP-43 may, after all, be integral to the pathology of AD, and to some extent determine the clinical phenotype present.

Angle closure glaucoma, both acute and chronic, is a major cause of blindness worldwide. Transscleral cyclophotocoagulation (TSCP) is conventionally undertaken non-urgently in patients with advanced glaucoma and poor visual potential with poor control of intraocular pressure (IOP). We describe a case of a patient with refractory acute angle closure glaucoma and severe pain in whom emergency TSCP was undertaken 12 h after presentation, reducing the IOP from 68 to 10 mm Hg. Further, a patient with chronic angle closure glaucoma underwent TSCP, reducing the IOP from 78 to 14 mm Hg. Both patients consequently underwent uneventful phacoemulsification cataract surgery with preservation of visual acuity and long-term IOP control. TSCP may achieve prompt IOP control and symptomatic relief in the acute setting in patients with acute and chronic forms of angle closure glaucoma refractory to medical therapy. TSCP may reduce the risk of definitive surgical intervention by temporising phacoemulsification or trabeculectomy surgery until the IOP is well controlled.

Sir, I read with interest the article by Yusuf et al1 on transient retinal artery occlusion (TRAO) during cataract surgery. I would like to highlight an avoidable but often overlooked cause of TRAO during cataract surgery. After administration of local anaesthesia, some surgeons or anaesthetists use a Honan balloon or similar device to compress the eye, so as to spread the anaesthetic and reduce the incidence of positive vitreous pressure and its attendant complications.2 The use of such devices is often poorly documented in the case notes, and if applied incorrectly, they may cause the vitreous pressure to exceed the central retinal artery pressure. This can lead to a total interruption of the blood supply to the retina that only resolves after the globe has decompressed itself with aqueous drainage. Instances of this type of TRAO have been described following intravitreal injections (especially if 0.1 ml or more of fluid is injected), scleral buckling surgery, and when patients are placed in the prone position during neurosurgical procedures.3, 4 As will all neuronal tissues, if circulation is not restored within a few minutes, irreversible hypoxic damage to the retina will follow. The amount of time the retina can survive with complete interruption to its blood flow is not known and may vary between patients but is thought to be no more than 5 min.5 A different situation exists in thrombo-embolic central retinal artery occlusions, where there may still be some blood flow, albeit small, as evidenced by delayed filling on fundus fluorescein angiography. In these situations, the retina may survive for several hours. The judicious use of compressive ocular devices for local anaesthesia requires that clinicians pay due attention to the pressure applied and the duration of compression. The intraocular pressure may, however, be difficult to gauge. As a precautionary measure, clinicians would be well advised to release such devices every 60–90 s to ensure there is adequate circulation to the retina and avoid irreversible ischaemic damage.

Pseudoexfoliation syndrome and cardiovascular disease: studies must control for all cardiovascular risk factors

Intractable diplopia occurs in approximately 0.8% of patients following strabismus surgery, with considerable detriment to visual quality of life. Long-term therapeutic occlusion is required to permit functional vision. Black intraocular lens (IOL) implantation is an effective therapeutic option associated with high rates of postoperative satisfaction.1 Black IOL implantation entails a cost to the public sector or patient but this may be offset by long-term clinical and social benefits and fewer hospital visits. A distinction has emerged between black IOLs that transmit near-infrared (NIR) light and those producing total occlusion of all wavelengths of light (figure 1).2 Morcher black IOLs transmit NIR light exponentially from 720 nm, with 100% transmission over 820 nm, permitting posterior segment imaging with scanning laser ophthalmoscopy (SLO) and optical coherence tomography (OCT).2 This property offers a distinct clinical advantage; SLO/OCT may detect life-threatening retinal and optic nerve diseases, such as choroidal melanoma.2 Figure 1 Black IOLs in clinical use. Near-infrared (NIR)-transmitting black IOLs: (A) 85F PMMA IOL (6 mm optic, 12 mm overall), (B) 6S PMMA IOL (10 mm optic, 12 mm overall), (C) 80D …

Intravitreal silicone oil achieves an effective endotamponade in patients with complex retinal detachments. Silicone oil displacement into the anterior chamber risks glaucoma and endothelial failure. We describe a 52-year-old patient with pseudophakia with silicone oil endotamponade presenting with visual loss and intraocular pressure of 60 mm Hg. Inferior YAG iridotomy was undertaken to repatriate silicone oil to the posterior segment. Despite normal intraocular pressure, acute corneal oedema occurred postiridotomy, resolving spontaneously over 2 weeks. Pupil block glaucoma secondary to silicone oil requires a management approach based on an understanding of silicone oil fluidics. Careful selection of inferior laser iridotomy site is critical to effectively reverse pupil block. Anterior migration of silicone oil in patients with pseudophakia is rare. We offer an hypothesis to explain unanticipated transient corneal oedema following silicone oil displacement from the anterior chamber. Clinicians must discuss the possibility of transient or permanent endothelial failure preoperatively in this patient group.

Dermatology offers an interesting clinical or surgical career in combination with a balanced and flexible working life, write Imran Yusuf and colleagues

Objectives To audit the use, indications, complications and patient information regarding hydroxychloroquine (HCQ) treatment in rheumatology clinics in a tertiary referral centre. Methods During a 9-month period, we identified all patients prescribed HCQ and attending rheumatology clinics in one hospital. We established: (1) the indication for HCQ (2) the prevalence of HCQ overdosing based on absolute body weight (ABW) (3) documentation of warning of risk of retinal toxicity (4) systemic and ocular co-morbidities (5) ocular symptoms during treatment (6) reasons for stopping HCQ. Results We identified 427 patients (104 male, 323 female). The cumulative dose of HCQ was lower in rheumatoid arthritis (RA; median 365 g; range 6-1752 g) compared to systemic lupus erythematosus (SLE; 450 g; 66-1788 g) (p = 0.105). The median duration of HCQ therapy was four years (range 0.1-13); 28% of patients with RA and 29% with SLE continued HCQ beyond five years. After adjusting for ABW and renal function, 10% (31/312) had been prescribed doses exceeding recommendations. Formal documentation of counselling on ocular complications was only found in one third of patients. Three cases of HCQ retinopathy were identified (all of whom had RA). Conclusion HCQ therapy is being used for more than five years in 29% of patients with rheumatic diseases, with higher than recommended doses in approximately 10% of patients. We recommend more rigorous scrutiny of the use of HCQ to reduce the risk of retinopathy.