Multifocal Serous Retinal Detachments Research Articles

Erdafitinib-induced bilateral multifocal serous retinal detachments and severe dry eye related unilateral peripheral ulcerative keratitis in a patient with metastatic urothelial carcinoma

Background: A case of bilateral multifocal serous retinal detachments and dry eye complicated with unilateral peripheral ulcerative keratitis (PUK) during erdafitinib therapy is described. Case description: A 76-year-old male underwent a baseline examination two months after initiating 8 mg erdafitinib therapy (April 2023) due to metastatic urothelial carcinoma. Left subfoveal serous retinal detachment was observed initially but the treatment was resumed as he was asymptomatic. In May 2023, bilateral multifocal subretinal fluid pockets were identified, and the patient was still asymptomatic. However, in June 2023 he complained of bilateral redness and a stinging sensation in his right eye. Bilateral severe dry eye and right PUK were diagnosed. He was prescribed dexamethasone eye drops and sodium hyaluronate artificial tears for both eyes. One week later corneal staining decreased, and progression of PUK ceased. Erdafitinib therapy was discontinued in June 2023 due to the planned transurethral prostatectomy. By July 2023, after discontinuation of the drug and administration of the topical treatment, the dry eye improved and the PUK became inactive. There was also resolution of subretinal fluid pockets in the right eye and a reduction of subretinal fluid pockets in the left eye. After the reinitiation of erdafitinib therapy, serous retinal detachments recurred in both eyes in September 2023, but both corneas remained stable with topical low-dose dexamethasone, cyclosporine-A and artificial tear usage. Conclusion: Erdafitinib therapy may lead to concurrent anterior and posterior segment complications. Multidisciplinary monitoring is crucial for patients undergoing erdafitinib therapy to prevent possible visual disturbances.

Vogt‐Koyanagi‐Harada‐like findings as the first manifestation of neuroleukaemia

Aims/Purpose: To report a well‐documented case of a 62‐year‐old woman presenting with VKH‐like findings as the first manifestation of neuroleukaemia.Methods: Case study.Results: A 52‐year‐old woman who had received a diagnosis of acute myeloid monoblastic leukaemia (AML) one month prior and was in remission after the induction phase presented with floaters and gradual visual loss in both eyes. Visual acuity was 20/200 and 20/25, respectively. Anterior segment exam was normal in both eyes. Funduscopy showed reduced foveal reflex. On spectral‐domain OCT, subretinal fluid was found in both eyes. Fluorescein angiography showed hypofluorescent areas compatible with multifocal serous retinal detachments and multiple hyperfluorescent dots surrounding the detached areas. Days after, the patient debuted with facial palsy and binocular diplopia due to bilateral VI cranial nerve (CN) palsy, left VII CN palsy and right complete III CN palsy. An MRI was performed with no signs of infiltration. A cerebrospinal fluid sample was drawn by lumbar puncture and its analysis denoted infiltration of AML cells with similar immunophenotype to that on initial bone marrow biopsy. Our patient presented with an atypical case of Vogt‐Koyanagi‐Harada (VKH) with multiple leakages along the periphery of the detached retinal area. This sparing of the macula should be considered a suspicious pattern, and combined with the finding of CN palsies was enough to suspect a relapse in the form of CSF infiltration, making the diagnosis of neuroleukaemia possible.Conclusions: Exudative retinal detachment can be a primary presentation of AML or denote a relapse. VKH is a diagnosis of exclusion, as one of the main criteria for its diagnosis is the absence of other systemic diseases. This becomes especially important in atypical presentations and older patients. So as not to miss life‐threatening underlying conditions, leukaemia and lymphoma should be considered in the differential diagnosis of VKH.

Bilateral multifocal serous retinal detachments after multiple trauma: a case report

We present a patient with bilateral multifocal serous retinal detachments after multiple trauma in a car accident without preceding corticosteroid treatment. A 37-year-old patient was admitted to our hospital after he had suffered multiple body injuries in a car accident. After being woken from an induced coma, he complained of blurry vision in his left eye, so he underwent a complete ophthalmic examination – several serous retinal detachments were found at the posterior pole of both eyes, along with thickened choroids. As there was no intraocular inflammation, accelerated hypertension or other changes indicating other causes of serous retinal detachments, and since we observed a steady spontaneous subretinal fluid resorption, we classified our case as an atypical central serous chorioretinopathy. Alternatively, serous retinal detachments could have occurred due to transient alterations in choroidal circulation following cerebral injury. In conclusion, this case report adds new data on the occurrence of serous retinal detachments and discusses their possible aetiology in the trauma setting. Care must be taken not to miss any other underlying pathology needing treatment.

A unique case report of Vogt‐Koyanagi‐Harada syndrome following COVID‐19 vaccination

AbstractPurpose: To report a case of Vogt–Koyanagi–Harada‐like disease (VKH) associated with COVID‐19 vaccination.Methods: Analysis of a single case report in a single tertiary institution.Results: A 48‐year‐old female presented with bilateral progressive blurring of vision 2 days after receiving her first dose of Pfizer‐BioNTech COVID‐19 vaccine. Clinical examination revealed multiple deep yellow lesions in the posterior pole and multifocal serous retinal detachments. Serological test for infective and autoimmune markers were negative. The patient was started on oral corticosteroids and had resolution of symptoms with near‐total improvement in visual acuity.Conclusions: There have been previous cases of vaccine associated uveitis from reported cases of hepatitis B vaccine, human papilloma virus vaccine and influenza vaccine1,2,3,4,5. The exact mechanism of vaccine‐associated uveitis is currently not clear. In this present case, because of the short time interval between COVID‐19 vaccination and the onset of ocular inflammation in our patient, this raises the possibility that COVID‐19 vaccination may be a possible trigger for VKH.

Vogt-Koyanagi-Harada Syndrome following influenza vaccination

Sir, Uveitis is a rare adverse event of vaccination with a low incidence rate as about 10.5 per 100,000.[1] Vogt-Koyanagi-Harada Syndrome is a very rare adverse event of vaccination, and there is only one case report in worldwide.[2] We recently experienced a case of Vogt-Koyanagi-Harada Syndrome following vaccination of influenza, thus herein report the case. A 52-year-old woman presented to our institution with bilateral red eyes and associated decrease in vision. There was no significant ocular or medical history. However, she had received the influenza vaccination 1 month previously. There was no history of trauma, and the presence of tinnitus was noted on review of the systems. On ocular examination, her best-corrected visual acuities were 20/30 (right) and 20/100 (left). Intraocular pressure was 15 mmHg bilaterally. She never suffered from uveitis previously. The anterior chamber revealed 2+ cells in the both eyes. The fundus appearances demonstrated multifocal serous retinal detachments, which were further confirmed with optical coherence tomography [Fig. 1]. Fluorescein angiography showed multiple hyperfluorescent spots [Fig. 2]. Laboratory examination showed that both IgM and IgG of cytomegalovirus (CMV), Epstein-Barr virus (EBV), and herpes simplex virus (HSV) were all negative in the serologic evaluation. Systemic steroid therapy was initiated promptly with intravenous methylprednisolone followed by systemic oral corticosteroids (1 mg/kg). Two months after treatment, anterior chamber got clear, and subretinal fluid disappeared. Her visual acuities were 20/20 (right) and 20/25 (left). Figure 1 Fundus photographs showing bilateral multiple serous retinal detachments (a). Optical coherence tomography demonstrates serous retinal detachment in the both eyes (b) Figure 2 Fluorescein angiography showing multiple hyperfluorescent spots This case is unusual in its etiology and presentation, with the development of Vogt-Koyanagi-Harada Syndrome in a woman who had received the influenza vaccination 1 month previously and the presentation of the syndrome with bilateral serous retinal detachments. That the negative finding of IgM and IgG of CMV, EBV, and HSV in the serum of a patient, and the effective use of systemic use of steroid, strongly suggested that the mechanism of uveitis and serous retinal detachments of the patient should be an autoimmune response rather than infectious. We encourage ophthalmologists, rheumatologists, and uveitis specialists to consider influenza vaccine as a cause of Vogt-Koyanagi-Harada Syndrome if the vaccine has been recently administered. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Multifocal retinopathy in Dachshunds with CLN2 neuronal ceroid lipofuscinosis

The CLN2 form of neuronal ceroid lipofuscinosis is an autosomal recessively inherited lysosomal storage disease that is characterized by progressive vision loss culminating in blindness, cognitive and motor decline, neurodegeneration, and premature death. CLN2 disease results from mutations in the gene that encodes the soluble lysosomal enzyme tripeptidyl peptidase-1. A null mutation in the TPP1 gene encoding this enzyme causes a CLN2-like disease in Dachshunds. Dachshunds that are homozygous for this mutation serve as a model for human CLN2 disease, exhibiting clinical signs and neuropathology similar to those of children with this disorder. Affected dogs reach end-stage terminal disease status at 10–11 months of age. In addition to retinal changes typical of CLN2 disease, a retinopathy consisting of multifocal, bullous retinal detachment lesions was identified in 65% of (TPP1−/−) dogs in an established research colony. These lesions did not occur in littermates that were heterozygous or homozygous for the normal TPP1 allele. Retinal changes and the functional effects of this multifocal retinopathy were examined objectively over time using ophthalmic examinations, fundus photography, electroretinography (ERG), quantitative pupillary light response (PLR) recording, fluorescein angiography, optical coherence tomography (OCT) and histopathology. The retinopathy consisted of progressive multifocal serous retinal detachments. The severity of the disease-related retinal thinning was no more serious in most detached areas than in adjacent areas of the retina that remained in close apposition to the retinal pigment epithelium. The retinopathy observed in these dogs was somewhat similar to canine multifocal retinopathy (CMR), a disease caused by a mutation of the bestrophin gene BEST1. ERG a-wave amplitudes were relatively preserved in the Dachshunds with CLN2 disease, whether or not they developed the multifocal retinopathy. The retinopathy also had minimal effects on the PLR. Histological evaluation indicated that the CLN2 disease-related retinal degeneration was not exacerbated in areas where the retina was detached except where the detached areas were very large. DNA sequence analysis ruled out a mutation in the BEST1 exons or splice junctions as a cause for the retinopathy. Perfect concordance between the TPP1 mutation and the retinopathy in the large number of dogs examined indicates that the retinopathy most likely occurs as a direct result of the TPP1 mutation. Therefore, inhibition of the development and progression of these lesions can be used as an indicator of the efficacy of therapeutic interventions currently under investigation for the treatment of CLN2 disease in the Dachshund model. In addition, these findings suggest that TPP1 mutations may underlie multifocal retinopathies of unknown cause in animals and humans.

Canine multifocal retinopathy caused by a BEST1 mutation in a Boerboel

A 7-month-old male intact Boerboel presented to the Cummings School of Veterinary Medicine at Tufts University for the evaluation of ocular discharge. Bilateral multifocal serous retinal detachments were noted on fundus examination as an incidental finding. Genetic testing confirmed the dog to be homozygous for a mutation in the BEST1 gene, where a C73 T/R25 X change results in premature termination codon. Further testing, including electroretinography and optical coherence tomography (OCT), demonstrated that there was no evidence of retinal photoreceptor dysfunction and confirmed that observed lesions were characteristic of canine multifocal retinopathy. No progression of the lesions was noted 3 months after the initial examination. To the authors' knowledge, this is the first report of canine multifocal retinopathy in the Boerboel breed.

Sympathetic ophthalmia after 23-gauge transconjunctival sutureless vitrectomy

PurposeWe report a case of a sympathetic ophthalmia that occurred after 23-gauge transconjunctival sutureless vitrectomy for a retinal detachment.Case reportA 41-year-old Japanese woman underwent combined phacoemulsification with intraocular lens implantation and 23-gauge transconjunctival sutureless vitrectomy for a rhegmatogenous retinal detachment in the right eye. Endolaser photocoagulation and silicone oil tamponade were used to manage inferior retinal holes. Four weeks after the surgery, she returned with a 5-day history of reduced vision and metamorphopsia in her left eye. Slit-lamp examination showed a shallow anterior chamber in the right eye and moderate anterior uveitis bilaterally. Silicone oil bubbles and pigment dispersion were observed in the subconjunctival space adjacent to the right eye’s superonasal sclerotomy site. Fundus examination showed multifocal serous retinal detachments in both eyes. A diagnosis of sympathetic ophthalmia was made and the patient was treated with intensive topical and systemic steroids. The subretinal fluid cleared in both eyes following treatment. Twelve months after the onset of inflammation, the patient’s condition was stable on a combination of oral cyclosporine and topical steroids. Sunset glow retinal changes remain, but there has been no evidence of recurrent inflammation.ConclusionSympathetic ophthalmia can develop after 23-gauge transconjunctival sutureless vitrectomy despite its smaller sclerotomy size. We recommend that special care should be taken to inspect for adequate closure of sclerotomy sites at the end of this operation.

Sympathetic ophthalmia manifesting with conjunctival chemosis

A 13-year-old girl was referred to us with the complaints of severe headache and decreased visual acuity (VA) in the right eye, of 1 week duration. She had a history of penetrating ocular trauma in the left eye 1 month earlier. Her best-corrected VA was counting fingers in the right eye and no light perception in the left eye. External examination revealed a massive inferior conjunctival chemosis bilaterally (Fig. 1a). Slitlamp examination of the right eye showed 0.5? cells, no corneal precipitates, and mild vitritis. There were a cloud cornea and an iris distortion, and no posterior segment structures were visible in the traumatized eye. Fundus examination of the right eye revealed focal yellow–white lesions at the level of the retinal pigment epithelium (RPE), multifocal serous retinal detachment (SRD), and hyperemic optic disc (Fig. 1b). Fluorescein angiography showed multifocal leaks at the level of the RPE with pooling of dye in the area of SRD (Fig. 1c). Ultrasonography of the right eye showed marked, diffuse choroidal thickening (3 mm), and diffuse retroscleral edema with T-sign in the peripapillary region (Fig. 1d). In the traumatized eye, there was choroidal and ciliary body detachment. Optical coherence tomography of the right eye showed SRD with subretinal septa. A diagnosis of SO was made, and the patient was treated with intravenous methylprednisolone (1 g/day for 3 consecutive days) followed by oral administration of prednisone (1.5 mg/kg). Three days after presentation, intraocular inflammation had improved and conjunctival chemosis had completely resolved. One week later, the chemosis had recurred and the SRD became more extensive and bullous. Cyclosporin (5 mg/kg/day) and then infliximab (5 mg/kg/ 0–2–4–8 weeks) were added. After a follow-up of 3 months, conjunctival chemosis had disappeared (Fig. 2a) and VA was 20/200. SRD had resolved, and there were subretinal fibrosis with RPE proliferation (Fig. 2b). Ultrasonography showed resolution of choroidal thickening and retroscleral edema (Fig. 2c). S. Attia S. Jenzeri I. Chtioui B. Jelliti S. Zaouali S. B. Yahia M. Khairallah (&) Department of Ophthalmology, Fattouma Bourguiba University Hospital, 5019 Monastir, Tunisia e-mail: moncef.khairallah@rns.tn

Acute Vogt–Koyanagi–Harada Disease in Enhanced Spectral-Domain Optical Coherence Tomography

To demonstrate unique membranous structures seen on enhanced spectral domain optical coherence tomography (SD-OCT) images of eyes with acute Vogt-Koyanagi-Harada (VKH) disease. Retrospective, observational case series. Twenty eyes of 10 consecutive patients diagnosed with acute VKH disease. We reviewed fundus photographs, conventional OCT images, fluorescein angiograms (FA), and enhanced SD-OCT images, including serial sections through the macula, obtained at the initial visit and periodically after steroid treatment until complete resolution of serous retinal detachment. Findings on enhanced SD-OCT imaging and FA in eyes with VKH disease. All 20 eyes had multifocal serous retinal detachment in the posterior fundus that appeared as multilobular dye pooling on late-phase FA. In 17 of the 20 eyes (85%), some areas of dye pooling corresponded to cystoid spaces on enhanced SD-OCT images and in 14 of the 20 (70%) eyes, there were areas of dye pooling and cystoid spaces in the fovea. The floors of the cystoid spaces consisted of a membranous structure of uniform thickness (mean thickness in the fovea of 14 eyes, 62.5+/-3.3 microm). The membranous structure, on SD-OCT, seemed to include a highly reflective line, which was continuous with the line representing the junction of the photoreceptor inner and outer segments (IS/OS) in attached areas of the retina. Intraretinal split was seen to overlie the abnormal IS/OS line in the vicinity of cystoid spaces involving the fovea in 9 of the 20 eyes (45%). During the course of steroid therapy, the membranous structure changed to a granular structure. The membranous structure that can be seen in eyes with acute VKH disease seems to represent a portion of the outer segment layer that has become separated from the inner segment layer by cystoid spaces. We hypothesize that the membranous form of the outer segment is bound with the action of inflammatory products, such as fibrin, and this membranous structure changes to a granular structure as steroid therapy "dissolves" the fibrin holding the outer segments together. The authors have no proprietary or commercial interest in any of the materials discussed in this article.

Retinopathy of Coton de Tulear dogs: clinical manifestations, electroretinographic, ultrasonographic, fluorescein and indocyanine green angiographic, and optical coherence tomographic findings

To document the clinical manifestations, development, progression, and mode of inheritance of the retinopathy of Coton de Tulear dogs. Multiple Coton de Tulear dogs were examined with biomicroscopy, indirect ophthalmoscopy, photopic and scotopic electroretinography, fluorescein and indocyanine green angiography, optical coherence tomography, ultrasonography, and fundic photography for 3 years. The retinopathy of Coton de Tulear dogs is inherited as an autosomal recessive condition and manifests as multifocal serous retinal detachments in homozygous puppies, between 3 and 4 months of age. Optic coherence tomography and ultrasonography confirm multiple focal serous retinal detachments. Serial fundic photographs confirmed minimal progression of lesions beyond 1 year of age. Electroretinography identified diminished scotopic and photopic amplitudes; however, the only significant differences between affected and age-matched control Coton de Tulear dogs were noted during photopic flicker electroretinograms. Leakage of fluorescein or indocyanine green through the blood ocular barriers was not detected during repeated angiograms prior to, during, and after development of the retinopathy. There was no focal pooling of fluorescein in any of the dogs examined. Focal retinal thinning was detected with optical coherence tomography over each detachment and the serous content of some of the bullae diminish after several years leaving focal areas of hyper-reflectivity. Multifocal retinopathy of Coton de Tulear dogs is inherited as an autosomal recessive condition. The retinopathy manifests early in life as nonprogressive multifocal bullous retinal detachments. The blood ocular barrier remains intact. Photopic and scotopic electroretinography are minimally diminished compared to age-matched dogs.

Retinopathy of Great Pyrenees dogs: fluorescein angiography, light microscopy and transmitting and scanning electron microscopy

Investigation of the pathogenesis of Great Pyrenees retinopathy. One male and two female puppies of parents who were affected with Great Pyrenees retinopathy and one 4-year-old affected adult male Great Pyrenees dog. The puppies were examined daily from 7 weeks of age by indirect ophthalmoscopy and their fundi were photographed until the lesions were static. Fluorescein angiography was completed at 7 weeks of age, within 24 h of detection of ophthalmoscopic lesions, and then weekly. The eyes of a 4-year-old and two 20-week-old puppies were examined with light microscopy, and transmitting and scanning electron microscopy. Blocked choroidal fluorescence was detected at 7 weeks of age. The blocked fluorescence enlarged, when the characteristic serous retinal detachments developed at 11 weeks of age. The detachments enlarged in size and number until the puppies were approximately 20 weeks old. Fluorescein pooling confirmed the presence of transient retinal pigment epithelial detachments. Leakage of dye into serous retinal detachments was not detected in this study. Light microscopy and transmitting and scanning electron microscopy confirmed the presence of multifocal serous retinal detachments with focal retinal degeneration that extended to the inner nuclear layer in a 4-year-old dog. The retinal detachments were accompanied by hypertrophy, hyperplasia, increased pigmentation, and vacuolation of the retinal pigment epithelium. Great Pyrenees retinopathy is manifested by multifocal serous retinal and retinal pigment epithelial detachments. These detachments are similar to those noted with central serous retinopathy of humans. The sudden development of multifocal retinal and retinal pigment epithelial detachments, and the serous nature of these detachments, supports a theory that they develop secondary to focal secretion and absorption defects in retinal pigment epithelial cells. Given the age of the puppies when the blocked choroidal fluorescence was noted and maturation of the dog retina at 8 weeks postpartum, this retinopathy is considered to be a retinal pigment epithelial dysplasia. This unique inherited retinopathy offers an opportunity to study retinal pigment epithelial secretion.

A Report of Two Cases Suggesting Positive Influence of Pregnancy on Uveitis Activity

Background: Little is known about the influence of pregnancy on uveitis activity. We report two cases suggesting a favorable influence of pregnancy on the clinical course of uveitis. Case: A 30-year-old woman who was three months pregnant was suspected Vogt–Koyanagi–Harada disease based on the systemic symptoms and ocular findings of iritis and multi-focal serous retinal detachment. She was positive to human leukocyte antigen (HLA)-DR 4. She was treated only with topical corticosteroids. One month later, the retinal detachment disappeared. Six months later, a healthy baby was born. The fundus of both eyes took on a sunset glow appearance and there has been no recurrence of uveitis. The other case was a 23-year-old woman with Behçet's disease who had several episodes of uveitis in a year even on tacrolimus. Because of pregnancy, all systemic drugs including tacrolimus were discontinued since then. Interestingly, the frequency of uveitis was remarkably decreased during the pregnancy. A normal healthy baby was born. The uveitis has almost completely disappeared since parturition until now. Conclusion: It is considered that the increase of intrinsic hormone, especially corticosteroid, and some other factors with pregnancy may give the suppressive influence on uveitis in our cases.