Macular Star Research Articles

PO-0811 Cat-scratch Disease: Still A Challenge

<h3>Introduction</h3> Cat-scratch disease (CSD)-neuroretinitis (CSDN) is an unusual pathology in immunocompetent individuals (1–2% of CSD). It typically presents with sudden vision loss following a febrile illness. <h3>Case report</h3> A 10 year-old healthy girl presented with fever, headache, vomits and abdominal pain for 48 h. Also, she referred myodesopsias on her right eye. She had daily contact with kittens. Physical examination showed bilateral cervical nodes. At presentation, her ophthalmologic examination revealed a right eye disc swelling with normal visual acuity. One-week later, unilateral sudden diminished visual acuity (RE=2/10) with macular serous detachment was detected. Blood analysis revealed normal white cell count, increased C-reactive protein (138 mg/L) and erythrocyte sedimentation rate (44 mm/h). A lumbar puncture showed pleocytosis (56/uL). Cranial and orbit MRI were normal. Blood and LCR cultures were negative. HIV, herpesviruses, <i>Toxoplasma gondii, Mycoplasma pneumoniae, Borrelia burgdorferi</i>, <i>Mycobacterium tuberculosis</i>, syphilis and autoimmune diseases were excluded. Serologic test (IFA) for <i>Bartonella spp</i>, in blood and CSF, was positive (antibodies titers: IgM 32; IgG 4096) with negative DNA amplification. She was treated with doxycycline and rifampicin. Moreover, intravenous corticosteroids were added in the first 3 days. She became asymptomatic and right visual acuity was 10/10 after 3 months. <h3>Discussion</h3> The absence of a macular star does not exclude and should not delay the diagnosis of CSDN. Serology remains the most common test to support the diagnosis. The role of antibiotics with or without steroids is not consensual. Still, patients usually have a favourable visual outcome.

Neuro-ophthalmic presentations and treatment of Cryptococcal meningitis-related increased intracranial pressure

ObjectiveTo illustrate three different ophthalmic presentations of cryptococcal meningitis (CM). IntroductionCM is the most common manifestation of extra-pulmonary cryptococcosis. Intracranial hypertension occurs in up to 75% of patients with CM and is associated with increased mortality. CM can present to the ophthalmologist as vision loss, papilledema, abducens palsy, and/or other cranial neuropathies. Participants and MethodsWe report three cases, two C. neoformans and one C. gattii, highlighting the various CM presentations. The first was a woman immunosuppressed following kidney transplantation in whom idiopathic intracranial hypertension (IIH) was initially suspected. The second was a man immunocompromised by previously undiagnosed HIV/AIDS who presented with signs and symptoms of increased intracranial pressure. The third case is an immunocompetent man with bilateral disc edema and an incomplete macular star diagnosed with presumed neuroretinitis. Further evaluation revealed positive CSF cryptococcal antigen with culture positive for C. gattii. ConclusionsOphthalmologists should be aware that cryptococcosis can mimic more benign etiologies including IIH and neuroretinitis. Additionally, C. gattii, an emerging organism, can infect immunocompetent patients. In contrast to the typical treatment of increased ICP, serial lumbar punctures are recommended while acetazolamide and surgical CSF shunting may be harmful.

Macular star formation in diabetic patients with non-arteritic anterior ischemic optic neuropathy (NA-AION)

BackgroundNA-AION is a condition that exhibits a number of unique characteristics in diabetics compared with the rest of the population. In some diabetic patients with NA-AION, lipid deposits can be observed around the macula forming an incomplete macular star. MethodsWe describe 12 case studies of patients with NA-AION observing the development of lipid deposits around the macula forming an incomplete macular star. ResultsAll our patients developed some level of lipid deposits around the macula in the form of a macular hemistar in the course of their illness. ConclusionSome authors have suggested that the macular star is formed by transudation from capillaries deep in the optic disk through the intermediary tissue of Kuhnt, which is located between the retina and the anterior portion of the lamina retinalis. However, the development of the macular star is currently understood not as a simple transudation but as a multifactorial process involving the presence of vascular damage around the optic disk, which is considered one of the most important factors leading to its occurrence.Although some studies mention the presence of a macular star in patients with NA-AION, we believe that this phenomenon may be significantly more common than the current literature suggests.

Sight-threatening pseudotumour cerebri associated with excess vitamin A supplementation

A 17-year-old girl presented with a 2-week history of increasingly severe generalised headache that was worse on waking, with blurring of vision and transient visual obscurations. Her visual acuity was...

Neuroretinitis in ocular bartonellosis: a case series.

We report a case series of neuroretinitis in ocular bartonellosis and describe the serologic verification for Bartonella henselae. This is a retrospective interventional case series of four patients who presented in the ophthalmology clinic of Hospital Universiti Sains Malaysia from June 2012 to March 2013. All four patients had a history of contact with cats and had fever prior to ocular symptoms. Each patient presented with neuroretinitis characterized by optic disc swelling with macular star. Serology analysis showed strongly positive for B. henselae in all of the patients. All patients were treated with oral azithromycin (except case 4, who was treated with oral doxycycline), and two patients (case 1 and case 3) had poor vision at initial presentation that warranted the use of oral prednisolone. All patients showed a good visual outcome except case 3. Vision-threatening ocular manifestation of cat scratch disease can be improved with systemic antibiotics and steroids.

Changing Perspectives in Classifications of Hypertensive Retinopathy.

Hypertensive retinopathy represents the ophthalmic findings of end organ damage secondary to systemic arterial hypertension. The eye is a target organ as well as an established prognostic indicator of systemichypertension. Based on ophthalmoscopically visible alterations, several classifications, the majority of them grading hypertensive fundus changes into four stages have been suggested. The recognition of hypertensive retinopathy is important in cardiovascular risk stratification of hypertensive individuals. The review evaluates the changing perspectives in classification & prognostic significance of fundal lesions. consists of retinal vascular changes that are pathologically related to both transient & persistent microvascular damage from elevated blood pressure. Ocular changes can be the initial finding in an asymptomatic patient with hypertension necessitating primary referral. In other instances, asymptomatic patient may be referred to an ophthalmologist for visual problems caused by hypertensive changes. Elevation of systemic blood pressure causes both focal and generalized constriction of retinal arterioles mediated by autoregulation. These findings are relatively common in long standing hypertension. A prolonged duration of high blood pressure can be associated with a breakdown of inner blood retinal barrier with extravasation of plasma & RBCs . Retinal haemorrhages, cotton wool spots, intraretinal lipids & in severe cases , the development of macular star configuration of intraretinal lipid can be seen. Although its name implies only retinal involvement, changes in both choroid & optic nerve are observed. When choroidal vessels are severely affected by elevated blood pressure, as in acute hypertension, fibrinoid necrosis of choroidal arterioles can cause occlusion of areas of choriocapillaris, with a subsequent breakdown of outer blood retinal barrier. Ocular findings in accelerated hypertension are divided into three distinct categories: hypertensive retinopathy, hypertensive choroidopathy & hypertensive optic neuropathy. Ophthalmic findings in acute malignant hypertensive retinopathy include focal arterial narrowing, cotton wool spots, intraretinal transudates , macular edema and retinal haemorrhages. Cystoid macular edema, lipid deposits and arteriolar changes are signs of malignant hypertensive retinopathy (2) . Cotton wool spots are fluffy, elevated, tan white areas of retinal opacity occurring within a few disc diameters of optic nerve, caused by occlusion of terminal retinal arterioles. Cotton wool spots typically resolve in 3-6 weeks and are associated with permanent nerve fiber layer loss in the vicinity of the lesion (2) . Periarteriolar intraretinal transudates are tan white retinal lesions which resolve without residual retinal damage in 2-3 weeks (3) . Macular edema and subretinal fluid are retinal findings related to hypertensive choroidal changes affecting the retinal pigment epithelium, with alterations in the blood retinal barrier. Clinical changes from hypertensive choroidopathy are directly related to the release of endogenous vasoconstrictor agents (eg.,angiotensin II, epinephrine, vasopressin) during systemic hypertension. Angiographically, there is delayed, patchy choroida lfilling

Diagnosis of Malignant Hypertension with Ocular Examination: A Child Case

ABSTRACTA 12-year-old female patient was admitted to our clinic complaining about low vision. Bilateral optic disc edema, macular star, and preretinal hemorrhages were found in fundoscopic examination. In fundus fluorescein angiography, massive leakage in the late phase was seen in the optic nerve head and macular area. These findings were compatible with high-grade hypertensive retinopathy. The patient consulted with pediatrics and a diagnosis of vesicourethral reflux and malignant hypertension was made.

Macular Hole

A 37-year-old woman presented with an anterior optic neuropathy related to Bartonella henselae. Twenty-nine days after symptom onset, a partial thickness macular hole developed in the involved eye. Fundus photography and optical coherence tomography confirmed the conversion to a full-thickness macular hole in 2 months. Macular hole as a complication of cat scratch disease is a rare entity, with 2 prior reported cases in children. The development of a macular hole following cat scratch disease can appear without the clinical picture of multiple white chorioretinal lesions, macular star, or vitritis.

Molecular Diagnosis and Ocular Imaging of West Nile Virus Retinitis and Neuroretinitis

To describe the ocular features of West Nile virus (WNV) infection proven by serology and molecular diagnostic techniques. Prospective case series. Fifty-two patients who presented to the uveitis clinic with ocular inflammatory signs and history of fever preceding ocular symptoms between January 2010 and January 2012 were enrolled for laboratory diagnosis. Serum samples were collected from 30 healthy controls from the same geographic area. Patients were tested for all endemic infectious diseases that can cause ocular inflammation by serology or molecular diagnostics. When patients had positive antibodies for WNV, serum/plasma samples were tested by real-time reverse transcription (RT) polymerase chain reaction (PCR) and RT loop-mediated isothermal gene amplification assays. The PCR product was subjected to nucleotide sequencing. Fundus fluorescence angiography (FFA), optical coherence tomography (OCT), and indocyanine green angiography were performed. Visual prognosis was analyzed. Clinical signs (retinitis, neuroretinitis, and choroiditis) and ocular complications (decrease in vision). A total of 37 of 52 patients (71%) showed positive results for at least 2 laboratory tests for WNV. Fundus examination revealed discrete, superficial, white retinitis; arteritis; phlebitis; and retinal hemorrhages with or without macular star. The FFA revealed areas of retinal inflammation with indistinct borders, vascular and optic disc leakage, vessel wall staining, or capillary nonperfusion. Indocyanine green angiography confirmed choroidal inflammation in 1 of the patients who was diabetic. The OCT scan of the macula revealed inner retinal layer edema in active inflammation and retinal atrophy in late stage. At the final visit, 43% of patients had visual acuity better than 6/12. In addition to previously reported clinical signs, retinitis, neuroretinitis, and retinal vasculitis were seen in this population. Atrophy of the inner retinal layer was seen on OCT after resolution of inflammation. Visual prognosis was good in patients with focal retinitis and poor in patients with occlusive vasculitis. The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Neuroretinitis: Update on a visual emergency and role of technology in its diagnosis

Neuroretinitis is one of the forms of optic neuritis characterized by swelling of optic nerve head and adjoining retinal nerve fiber layer resulting in a macular star configuration. The underlying pathophysiology involves increased permeability of disc vasculature, but the etiology is not fully defined. Neuroretinitis may occur due to an infectious process involving the disc, a postviral or autoimmune mechanism or sometimes idiopathic. Technological advances like ophthalmoscopy, slit-lamp examination, fluorescein angiography, magnetic resonance imaging and immunodiagnostic tests all come handily and are often imperative in making an accurate diagnosis. Conditions mimicking neuroretinitis include papilledema, anterior ischemic optic neuropathy, and infiltration of the optic disc by tumor and systemic hypertension. Neuroretinitis is usually a self-limited disorder with a good visual prognosis. Treatment of neuroretinitis is required only when there is an underlying infectious or inflammatory condition.

B-Scan and “En-Face” Spectral-Domain Optical Coherence Tomography Imaging for the Diagnosis and Followup of Acute Retinal Pigment Epitheliitis

Purpose. To report B-scan and “En-face” spectral-domain optical coherence tomography (SD-OCT) findings in acute retinal pigment epitheliitis (ARPE). Methods. Two patients (3 eyes) with ARPE were examined. Fluorescein and indocyanine green (ICGA) angiography, B-scan, and “En-face” SD-OCT were performed in each patient at initial and follow-up visits. Results. Both patients presented with acute onset of blurred vision, and one with bilateral involvement. B-can OCT revealed disruption of the macular retinal pigment epithelial (RPE) inner band layer and photoreceptors' inner and outer segment (IS-OS) junction. Hyperreflective dots were observed in the outer nuclear layer (ONL) above the RPE/IS-OS disruption. Just around these hyperreflective dots, slight thickening of the hyperreflective IS/OS junction was observed. During the late phase, indocyanine green angiography (ICGA) showed a macular cockade-like hyperfluorescent halo. “En-face” OCT showed the same cockade-like appearance with a hyporeflective center and a hyperreflective border matching the pattern observed on ICGA. At followup, as vision improved without treatment, B-scan OCT demonstrated progressive resolution of the hyperreflective and disrupted lesions; “en-face” OCT also showed disappearance of the macular cockade-like halo with a transient discrete hyperreflective macular star at the RPE level in one eye. Conclusion. “En-face” OCT associated with B-scan SD-OCT analysis appears to be very helpful in the diagnosis and followup of ARPE. The pathophysiology of ARPE remains complex and still poorly understood. These techniques help define the location and extent of structural damage occurring in this disease.

Intravitreal bevacizumab in persistent retinopathy secondary to malignant hypertension

PurposeTo evaluate the efficacy and safety of intravitreal bevacizumab (IVB) injection in persistent retinopathy secondary to malignant hypertension (MHT). Patients and methodsSingle IVB injection of 1.25mg/0.05ml in 12 cases with persistent retinopathy secondary to MHT more than one month after control of MHT with pre and post injection evaluation of best corrected visual acuity (BCVA) and anatomical outcome up to sixth month and postinjection complications were evaluated. ResultsProgressive reductions in retinal hemorrhages, exudates, cotton-wool spots, and macular star were documented by photography, angiography, and central macular thickness (CMT) measured by optical coherence tomography (OCT) imaging. Decreased macular edema was the most common finding. Improvement or stabilization of visual acuity was noted in all cases. ConclusionsIn addition to proper medical management of MHT, IVB injection is an effective and safe approach to treat persistent retinopathy associated with MHT.

Ruthenium-106 brachytherapy and intravitreal bevacizumab for retinal capillary hemangioma

The purpose of this study is to report a case of retinal capillary hemangioma treated with ruthenium-106 brachytherapy combined with intravitreal bevacizumab. A 58-year-old woman presented with blurred vision in her left eye for 15 days. Best-corrected visual acuity (BCVA) was counting fingers at 3 m in her left eye. Examination showed a peripheral endophytic retinal capillary hemangioma, with associated macular edema and diffuse hard exudates in a macular star pattern. B-scan ultrasonography revealed a solid, highly reflective retinal tumor measuring 7.1 × 7.5 mm in basal dimension and 3.5 mm in thickness. The hemangioma and macular edema were treated with ruthenium-106 brachytherapy (500 Gy) followed 1 month later with an intravitreal injection of 1.25 mg (0.05 ml) bevacizumab. After 10 months of follow-up, examination demonstrated involution of the hemangioma, resolution of the macular edema, less hard exudates, and improvement of BCVA to 20/25. Ruthenium-106 brachytherapy combined with intravitreal bevacizumab was associated with significant involution of the choroidal hemangioma with resolution of the macular edema and improvement in visual acuity.

Paradoxical response in ocular bartonellosis

IntroductionCat scratch disease is an infectious disease caused by thebacteria Bartonella henselae [1] and is transmitted by catscratches or exposure to cat saliva. The spectrum of itsocular manifestations includes Parinaud's oculoglandularsyndrome, iritis, vitritis, neuroretinitis, panuveitis, retinitisappearing as small white intraretinal infiltrates, and choroi-dal white lesions [1, 2]. B. henselae is reported to be themost common cause of neuroretinitis; typically character-ized by optic disk edema in association with a partial orcomplete macular star. The majority of infected patientspresenting with neuroretinitis reported in the literature aretreated with antibiotics. In some cases steroids were added.However several reports showed that even patients who didnot receive treatment had an excellent visual outcome [1].We describe here a paradoxical response to treatment in ayoung female presenting with bilateral neuroretinitis andinflammatory optic disk lesions.Case reportA 19-year-old healthy female presented with an abruptdeterioration of left eye vision of 1-day duration. For10 days prior to presentation, she was suffering from drycough; 5 days later she had intermittent fever up to 41°Cwith shaking chills. There was a history of contact with acat, but she denied being scratched. On admission, physicaland neurologic examinations were unremarkable. Ophthal-mologic assessment disclosed visual acuity of 4/4 in theright eye and 4/60 in the left eye. She had normal anteriorsegments and intraocular pressures. A left afferent pupillarydefect was noted. Funduscopy of the right eye showed asmall white optic disk lesion in its temporal aspect with adeep retinal white lesion along the superonasal arcade(Fig. 1). The left optic disk had blurred margins with biggerwhite lesions in its nasal and temporal aspects with anassociated localized vitreous opacity on its surface (Fig. 1).There was marked macular edema and two deep retinalwhite lesions along the superotemporal arcade and infer-onasal to the optic disk. Optical coherence tomography(OCT) revealed intraretinal fluid in the right papillomacularbundle and left exudative macular detachment (Fig. 2).Visual field examination showed right nasal step andsuperonasal arcuate defect while there was enlargement ofthe left blind spot with nasal step (Fig. 3). Work-uprevealed mild anemia with hemoglobin of 11.7 g% andleukocytosis of 14,800 (lymphocytes, 31%). Kidney andliver functions were within normal limits except for aslightly elevated lactate dehydrogenase at 768 (normalrange, 300–620 u/l). She had elevated erythrocyte sedi-mentation rate (ESR) (90 mm/h) and C-reactive protein(5.3 mg%; normal range, less than 1). Chest X-ray did notreveal any pathological findings, and CT scan and MRI ofthe head and brain showed mild thickening of the opticnerves bilaterally and hyperintense foci in the flairsequences in the intraocular aspects of the optic nerves,respectively. Cerebrospinal fluid cytology was normal.Serological tests were negative for HIV, syphilis, Brucella,Coxiella burnetti, toxoplasma, and toxocara. Serologicaltests for B. henselae revealed positive IgM titer at 75 units

Clinical Characteristics in 53 Patients with Cat Scratch Optic Neuropathy

To describe the clinical manifestations and to identify risk factors associated with visual outcome in a large cohort of patients with cat scratch optic neuropathy (CSON). Multicenter, retrospective chart review. Fifty-three patients (62 eyes) with serologically positive CSON from 5 academic neuro-ophthalmology services evaluated over an 11-year period. Institutional review board/ethics committee approval was obtained. Data from medical record charts were collected to detail the clinical manifestations and to analyze visual outcome metrics. Generalized estimating equations and logistic regression analysis were used in the statistical analysis. Six patients (9 eyes) were excluded from visual outcome statistical analysis because of a lack of follow-up. Demographic information, symptoms at presentation, clinical characteristics, length of follow-up, treatment used, and visual acuity (at presentation and final follow-up). Mean patient age was 27.8 years (range, 8-65 years). Mean follow-up time was 170.8 days (range, 1-1482 days). Simultaneous bilateral involvement occurred in 9 (17%) of 53 patients. Visual acuity on presentation ranged from 20/20 to counting fingers (mean, 20/160). Sixty-eight percent of eyes retained a visual acuity of 20/40 or better at final follow-up (defined as favorable visual outcome). Sixty-seven percent of patients endorsed a history of cat or kitten scratch. Neuroretinitis (macular star) developed in 28 eyes (45%). Only 5 patients had significant visual complications (branch retinal artery occlusion, macular hole, and corneal decompensation). Neither patient age nor any other factor except good initial visual acuity and absence of systemic symptoms was associated with a favorable visual outcome. There was no association between visual acuity at final follow-up and systemic antibiotic or steroid use. Patients with CSON have a good overall visual prognosis. Good visual acuity at presentation was associated with a favorable visual outcome. The absence of a macular star does not exclude the possibility of CSON. The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Neuroretinitis: Review of the Literature and New Observations

Neuroretinitis (NR) is an inflammatory disorder characterized by optic disc edema and subsequent formation of a macular star figure. The underlying pathophysiology involves increased permeability of disc vasculature, but the etiology is not fully defined. In some cases, NR is probably due to an infectious process involving the disc; in others, a postviral or autoimmune mechanism is more likely. Cases can be divided into those in which a specific infectious agent has been identified, those considered idiopathic, and those with recurrent attacks. Some reports have not distinguished among these subgroups, and it is unclear if their clinical features vary. We reviewed the literature and our own patients looking particularly at features that might better distinguish these subtypes. Features common to all 3 groups included age, absence of pain, and fundus appearance. Preceding systemic symptoms were more common in patients with cat scratch disease (CSD) and uncommon in those with recurrence. The pattern and magnitude of visual field loss differed, more commonly confined to the central field in CSD cases and more severe in recurrent cases. Recovery of visual acuity and field was less substantial in recurrent cases even after the initial episode. MRI was usually normal in all 3 groups. Enhancement confined to the optic disc was found in all 3 groups, but enhancement of the retrobulbar optic nerve was seen only in recurrent cases. Findings that are strongly suggestive of CSD include very young age, preceding systemic symptoms, and poor visual acuity but with a small or absent relative afferent pupil defect (RAPD). In contrast, the following are suggestive of idiopathic NR with a high risk of recurrence: absence of systemic symptoms, visual field defect outside the central field, preserved visual acuity with a large RAPD, and poor recovery of vision. Decisions regarding evaluation and treatment should be made with these features in mind.

Neuroretinitis as an Unusual Manifestation of Leptospirosis: A Case Report

Leptospirosis is a zoonotic infection caused by spirochetes leptospira. It presents with both ocular and systemic manifestations. Neuroretinitis has been reported in the few cases of leptospirosis. We present a case of leptospirosis with unilatertal neuroretinitis presenting with sudden loss of vision, optic disc edema and macular star. Leptospirosis was confirmed by serological test and the disease responded optimally to specific therapy.

Optic neuropathy secondary to cat scratch disease: case report

Optic neuropathy due to cat scratch disease is a relatively infrequent occurrence associated with macular star formation and is characterized by sudden painless loss of vision mostly unilateral. Bartonella henselae is well recognized as the etiologic agent in cat scratch disease. Ocular complications of the disease occur in up to 10% of patients and include neuroretinitis. Ocular bartonelosis is usually self-limited with complete or near-complete recovery of vision in otherwise healthy patients. A case of a boy with neuroretinitis caused by B. henselae is reported.

Intravitreal Bevacizumab in a Patient with a Macular Star in Malignant Hypertension

To report a case of retinopathy secondary to malignant hypertension showing a macular star, which responded favorably to a single intravitreal injection of bevacizumab (IVB). A 46-year-old man with malignant hypertension showing incomplete macular star in his right eye with best-corrected visual acuity (BCVA) of 20/32 underwent IVB. Subsequent examinations revealed a progressive resolution of the macular star with functional recovery over the follow-up. In particular, at the 9-month examination BCVA was 20/20, fundus biomicroscopy disclosed resolution of the macular star, and the retinal sensitivity improved remarkably. Intravitreal injection of bevacizumab can be a useful approach to treat the exudative manifestations of malignant hypertension.

Characteristics of optic disc melanocytomas presenting with visual dysfunction

Objective and Design:A retrospective review study was designed to describe five cases of optic disc melanocytomas with tumor-related visual impairment.Participants:Five patients with optic disc melanocytoma presented with visual complaints to a tertiary eye hospital in Saudi Arabia.Materials and Methods:Demographic and clinical data were analyzed, including the results of ocular examination, lesion laterality, best-corrected Snellen visual acuity, pupillary reflex, visual field testing, color fundus photography, fundus fluorescein angiography, and ophthalmic ultrasound.Results:Visual dysfunction secondary to optic disc melanocytoma was identified. Case 1 had macular star edema with mild tumor enlargement, Case 2 had optic atrophy, Case 3 had juxtapapillary choroidal neovascular membrane with macular involvement, Case 4 had optic disc swelling with an enlarged blind spot, and Case 5 had a large altitudinal visual field defect.Conclusion:Although melanocytomas of the optic disc tend to have a benign behavior with slow evolution and stable vision, they may adversely affect visual function through a variety of mechanisms.