Retinal Periphlebitis Research Articles

Bilateral uveitis, retinal periphlebitis, and optic neuritis associated with non-pineal central nervous system germinoma

Purpose: To report a case of bilateral uveitis, retinal periphlebitis, and optic neuritis associated with a non-pineal central nervous system (CNS) germinoma. Methods: Case report. Results: A 32-year-old male presented with episodes of acute painless visual disturbance in each eye, and was found to have decreased visual acuity, abnormal color vision, an afferent pupillary defect in the left eye, bilateral optic disc edema, perivenous sheathing, and candle-wax dripping exudates. Optical coherence tomography revealed bilateral intraretinal fluid and posterior vitreous hyperreflective opacities. Fluorescein angiography revealed bilateral optic disc leakage without active small vessel leakage. Magnetic resonance imaging of the brain and orbits revealed enhancing periventricular lesions and enhancement of the left optic nerve and bilateral perioptic nerve sheaths, posterior globes, and optic nerve heads. Brain biopsy was consistent with a CNS germinoma. His ocular signs and symptoms improved with chemotherapy for the germinoma. Conclusion: CNS germinomas, including those located outside the pineal region, can be associated with optic neuritis, uveitis, and periphlebitis including characteristic candle-wax dripping exudates. Ocular signs and symptoms typically improve with treatment of the underlying germinoma.

Retinal Periphlebitis May Be a Marker for Subphenotype in Multiple Sclerosis

Retinal periphlebitis (RPP) is a long-known entity in patients with multiple sclerosis (MS) and has not been revisited in the context of recent developments in MS pathogenesis and heterogeneity. We present six cases of RPP in three female and three male MS patients. They all have relapsing-remitting MS and did not have or had minor ocular symptoms. It is important to perform a thorough retinal examination in patients with MS, as peripheral and sectorial lesions could be unseen. A better knowledge on the concomitant involvement of retinal veins contributes to the understanding of immunopathology, with potentially distinct autoantigenic targets. RPP might serve as a subphenotype marker that may influence treatment choices in MS. Further research is needed.

Multiple sclerosis and occlusive retinal vasculitis: A case series

AbstractPurpose: Retinal vascular changes are a common finding in patients with multiple sclerosis (MS). While simple peripheral retinal periphlebitis is by far the most frequent manifestation, a spectrum exists that ranges up to the presence of proliferative retinal vasculitis.Methods: Case series of four patients with occlusive retinal vasculitis.Results: Three patients presented with complaints of visual loss and ocular discomfort: a 26‐year‐old man presented with vitreous inflammation, vitreous haemorrhage, macular edema and papillitis; a 44‐year‐old woman presented with intermediate uveitis and macular edema; and a 25‐year‐old woman presented with granulomatous panuveitis, papillitis and foveolar serous detachment. A fourth patient was addressed because of abnormal eye movements detected during a work‐up for dizziness. Examination revealed bilateral internuclear ophthalmoplegia with adduction lag. He had no visual complaints other than subtle floaters in the left eye. Anterior segment examination showed no signs of inflammation, whereas vitreous snowballs were present. Fluorescein angiography revealed occlusive retinal vasculitis in all patients, resulting in active neovascularization in two patients with vitreous haemorrhage in one of them. All patients required retinal laser photocoagulation. Extensive work‐up revealed multiple sclerosis as the underlying disease in three patients; the remaining patient had already been diagnosed with MS at the time of presentation. Nevertheless, additional work‐up was performed in this patient to exclude other possible causes.Conclusions: Occlusive retinal vasculitis is a rare manifestation in patients with MS and may result in neovascularization, vitreous haemorrhage, retinal detachment and neovascular glaucoma. MS should therefore be considered in the differential diagnosis of ischemic retinal disease. In addition, one should be alert for signs of retinal vasculitis in patients with MS, as it can present with few or no symptoms.

Ocular Manifestations of Buerger's Disease - A Review of Current Knowledge.

Buerger’s disease, also known as thromboangiitis obliterans, is a disorder of primarily small and medium arteries and veins of the arms and legs. We have failed to find a comprehensive review discussing a possible link between the disease and the eyes. The aim of this study is to review current knowledge on the topic of ocular manifestations in the course of Buerger’s disease. The Medline and Web of Science databases were searched without a time or language limit. We have managed to review 13 articles, describing the involvement of the eyes in thromboangiitis obliterans. It appears that patients suffering from Buerger’s disease may develop non-arteritic anterior ischemic optic neuropathy (NAION), occlusive retinal vasculitis and periphlebitis, papillophlebitis, central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO), normal tension glaucoma (NTG), uveitis, chorioretinal atrophy, retinitis, papillitis, optic atrophy, changes typical for hypertensive retinopathy. Additionally the abnormalities in electroretinography might be present. The treatment options and the possible outcome depend on the type of ocular manifestations, so it seems impossible to propose a universal therapy. We would like to raise awareness of the possible ocular manifestations in the course of Buerger’s disease.

Tuberculosis related Neuroretinitis and Retinal Vasculitis in an Immunocompetent Patient

Introduction: This study aimed to describe the occurrence of bilateral neuroretinitis and retinal vasculitis in an immunocompetent female with disseminated tuberculosis. Case description: A 25 year old female diagnosed with disseminated tuberculosis presented with sudden painful loss of vision bilaterally. On examination she was found to have bilateral neuroretinitis and retinal periphlebitis involving the posterior pole. She was prescribed systemic steroids in addition to the anti-tuberculous therapy that she was receiving. The fundus lesions gradually resolved over four months with modest improvement in visual acuity. There were no recurrences in the follow up period of 14 months. Conclusions: Ocular involvement in the form of neuroretinitis with concomitant retinal vasculitis can occur in patients with disseminated tuberculosis. Addition of systemic steroids to anti-tuberculous therapy leads to anatomical resolution with improvement in visual acuity.

Insights into multiple sclerosis-associated uveitis: a scoping review.

This paper is a scoping review of research on multiple sclerosis (MS)-associated uveitis to determine its epidemiology, pathophysiology, clinical features and treatment. A comprehensive search of the medical databases MEDLINE (PubMed), EMBASE, Web of Science and Cochrane was carried out on 25 November 2019, to identify papers published between 1980 and 2019 that focus on patients with MS-associated uveitis. Based on large cohort studies (n≥1000), the prevalence of uveitis in patients with MS is estimated to be 0.53-1.34% (mean=0.83%), and MS is diagnosed in 0.52-3.20% (mean=1.30%) of patients with uveitis. The condition is most frequent among middle-aged women. Patients usually complain of floaters and/or blurred vision, with bilateral intermediate uveitis (with retinal vasculitis) as the most frequent ophthalmological finding. Both MS and intermediate uveitis are associated with HLA-DRB1*15:01 and IL-2RA gene polymorphism rs2104286 A>G, suggesting a common genetic background. T cells, and possibly B cells, play an important role in both autoimmune disorders. Multiple sclerosis (MS)-related uveitis is classically treated as non-infectious uveitis, with corticosteroids as the first treatment step. Other treatments include immunosuppressants, cryotherapy, laser photocoagulation and vitrectomy. These treatment options have a limited, if any, effect on the course of MS and can be complicated by side-effects. As treatment strategies for MS have increased in the last decade, it would be interesting to evaluate the efficacy of these new treatments during the course of uveitis. Moreover, the correlation between retinal periphlebitis and MS could be established more accurately with the recently developed techniques of wide-field fluorescein angiography in a large cohort of MS patients. MS-associated uveitis is a rare, highly discussed pathology about which much is still unknown. Large epidemiological studies and extrapolation of new MS treatments to this condition are warranted.

Ocular tuberculosis: A case series from a London hospital

Background: Ocular TB is defined as an infection of Mycobacterium tuberculosis involving any part of the eye. Estimates of 1.4% of patients with pulmonary tuberculosis have ocular involvement and though rare, this presentation can have devastating consequences if not recognised. We present a case series of ocular TB diagnosed at London North West Healthcare trust which serves a multicultural community with the highest rates of TB infection in the UK. Methods and materials: Retrospective analysis of laboratory and medical records, radiology and microbiology results of patients with ocular TB identified from the London TB register from September 2012 to July 2019 from London North West Hospitals University NHS Trust. Results: 28 patients with ocular TB were identified: 23 uveitis (10 anterior uveitis, 6 posterior uveitis, 7 panuveitis), 1 maculopathy, 1 optic neuritis, 1 epislceritis, 1 orbital fibrovascular TB lesion and 1 retinal periphlebitis. All were presumptive diagnoses with only one having ocular microbiological samples obtained; which were negative. 54% of cases were female, average age at diagnosis 48 years (range 22 to 72). 89.3% were migrants; 29% African and 61% South Asian, particularly Indian (39%). Average time from symptoms presenting to treatment commencing was 186 days. Only 2 patients had evidence of TB at other sites; both extra-pulmonary (mediastinal and extra thoracic lymph nodes). 1 patient was HIV positive. Nine patients (32.6%) received steroids (3 eye drops and 6 systemic therapies). Nearly all patients had either a reactive Mantoux test (10) or Interferon Gamma Release Assay (17). Treatment duration was usually the standard 6 months (57%) followed by 9 months (19%), 12 months (19%) and 13 months (5%) with 2 patients lost to follow up. 19 patients (67.9%) were treated with ethambutol sparing regimens usually replacing with moxifloxacin. 1 patient had blindness as a long term sequaela of ocular TB. Conclusion: Our case series demonstrates that ocular tuberculosis frequently occurs in the absence of pulmonary tuberculosis and should be considered in all cases of uveitis in patients from endemic regions.

Blinding meningitis: frosted branch angiitis: A rare presentation

Frosted branch angiitis (FBA) is typically a bilateral diffuse retinal periphlebitis which occurs in a number of conditions. Here we report an unusual case of a 20 year old female patient who presented with bilateral blindness with a fundus picture of FBA following four days of fever and headache. A series of investigations were carried to rule out the possible underlying diseases and significant recovery of vision was seen on treatment with intravenous steroids and intravitreal Acyclovir therapy.

Granuloma-like formation in deeper retinal plexus in ocular sarcoidosis.

Sarcoidosis is a multi-system disease characterized by the presence of non-caseating epithelioid granulomas in affected tissues and the most frequent cause of noninfectious uveitis in Japan. Ocular manifestations of sarcoid uveitis vary enormously and commonly characterized by granulomatous anterior uveitis, intermediate uveitis, and posterior uveitis such as retinal periphlebitis, choroiditis, optic nerve granuloma and papillitis. However, case reports of multiple granuloma formation related to sarcoid uveitis is uncommon. This report describes the use of optical coherence tomography angiography to visualize changes occurring in granuloma formation in deeper retinal plexus before and after systemic corticosteroid treatment.

Ocular sarcoidosis: new diagnostic modalities and treatment.

Ocular involvement in sarcoidosis is present in up to 80% of patients and is frequently manifested before diagnosis of the underlying systemic disease. Considering the therapeutic consequences, early diagnosis of the underlying disease is advantageous in patients presenting with ocular inflammation. There are several ocular findings suggestive of underlying sarcoidosis, such as granulomatous keratic precipitates, iris nodules, cells in the vitreous humor known as snowballs and snowbanks, and retinal periphlebitis. High suspicion is crucial for the diagnosis of sarcoidosis. This review on ocular sarcoidosis will mainly focus on new diagnostic and treatment modalities. Recent studies found possible new diagnostic indicators for the diagnosis of ocular sarcoidosis which include not only serum profiles but also vitreous sample analysis. Ophthalmologic imaging techniques have improved to investigate the ocular structure in detail. Results from recent uveitis clinical trials have included sarcoidosis as an underlying cause and have reported positive results. The diagnosis of ocular sarcoidosis can be challenging in some cases. High suspicion is important to diagnose ocular sarcoidosis with various laboratory and ophthalmic tools. There are many possible options for the treatment of ocular sarcoidosis including various biologic agents.

A case of frosted branch angiitis

Frosted branch angiitis (FBA) is typically a bilateral diffuse retinal periphlebitis that may occur in a number of varying conditions. Here, we report a case of a young female who presented with bilateral sudden diminution of vision with a fundus picture of FBA. A series of investigations were carried to rule out the possible underlying diseases, and significant recovery of vision was seen on treatment with oral steroids.

Sarcoidosis and uveitis : An update

Ocular involvement in sarcoidosis is present in up to one third of patients and is frequently manifested before the underlying systemic disease has been diagnosed. With aview to the therapeutic consequences an early diagnosis of the underlying disease is advantageous. In cases of visual loss early diagnostic measures include invasive procedures, such as ultrasound-guided bronchoalveolar lavage and transbronchial biopsy, if nodular conjunctival or cutaneous manifestations which would confirm the diagnosis are not present. Sufficiently sensitive biomarkers of disease activity have not yet been identified. As elevated activity of angiotensin-converting enzyme (ACE) and lysozyme are associated with disease activity they may be supportive of but not specific for the diagnosis. Other indicators include calcemia, calciuria, augmented activity of hepatic enzymes, cutaneous anergy and hilar changes in the lungs. In patients with newly diagnosed anterior uveitis presenting with granulomatous mutton-fat corneal precipitates, snow balls and vitreous body base condensates with mild retinal periphlebitis in intermediate uveitis as well as multifocal choroiditis (creamy choroidal lesions at different stages of activity and approximately 500 µm in diameter), adiagnosis of sarcoidosis has to be considered and tuberculosis should be excluded, even in the absence of aknown systemic disease. Anterior uveitis is frequently a self-limiting condition, which can be well-controlled with topical steroids. On the other hand, intermediate, posterior and panuveitis are typically associated with a chronic course and require systemic therapy to prevent severe loss of vision. If the response to systemic corticosteroids is insufficient, treatment with immunomodulatory agents and biologics is initiated at progressively earlier stages, with aview to averting permanent organ damage and frequently with a positive impact on the short and long-term outcomes, even in therapy-refractive cases.

MicroRNAs and their effects on retinal neovascularization

Retinal neovascularization occurs in various ischemic retinopathy, including diabetic retinopathy, retinopathy of prematurity, retinal periphlebitis and retinal vein occlusion. The tortuous, disordered and leaky new blood vessels produced in this pathological process of diseases, are the main cause of intraocular hemorrhage, retinal detachment and even visual loss. Although current clinical treatments, including laser photocoagulation and vitrectomy, have certain curative effect to delay the progress of neovascularization, they are invasive treatments. Recently, along with the deep studies of microRNAs, it is found that different microRNAs play important roles in the angiogenesis process by regulating the specific target genes, therapy that based on microRNAs provides a new idea for effective inhibition of retinal angiogenesis. This article reviews on microRNAs and its function in retinal neovascularization. Key words: Retinal neovascularization; MicroRNAs; Target gene; Treatment

Bilateral frosted branch angiitis as the presenting sign of antiphospholipid antibody syndrome.

Background“Frosted branch retinal angiitis” is an encompassing term for a rare, typically bilateral diffuse retinal periphlebitis that may occur in a number of varying conditions. To our knowledge, we report the first case of frosted branch angiitis as the presenting sign of antiphospholipid antibody syndrome in a 28-year-old woman.FindingsThis study is a retrospective case report and literature review. Serial fundus photos, fluorescein angiogram, and ocular coherence tomography taken were before and after treatment, showing resolution of diffuse retinal perivascular sheathing and macular edema along with marked improvement in visual acuity 4 months after the treatment with corticosteroids.ConclusionsFrosted branch angiitis can be seen in association with antiphospholipid antibody syndrome. Prompt recognition and treatment with corticosteroids may result in good visual prognosis, and long-term immunosuppression and additional anticoagulation may be beneficial to prevent recurrence.

A Case of Eales Disease in Postpartum Period

A 31-year-old female presented with visual field defect and floaters in right eye. Symptom occurred just before labor and worsened after labor. The best corrected visual acuity was 1.0 in the right eye. Fundus examination revealed retinal hemorrhage and periphlebitis in the affected eye. After 2 weeks, newly developed retinal vasculitis was observed in the fellow eye as well as in the affected eye. She was treated with systemic corticosteroid and disease activity increased while tapering corticosteroid. After adding systemic immunomodulating agent, disease activity was controlled. Retinal neovascularization was observed in previous non-perfusion area. She underwent vitrectomy in the right eye due to recurrent vitreous hemorrhage, and prophylactic laser photocoagulation was performed in the left eye.

Peripheral Vasculitis, Intermediate Uveitis and Interferon Use in Multiple Sclerosis.

Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system. A 40-year-old female patient with a 12-year history of MS was admitted to our clinic with blurred vision and floaters in her right eye for about 1 month. Here, we share the findings and the management of intermediate uveitis and retinal periphlebitis in an MS case being treated with interferon beta-1a for 7 years.

Post-cataract endophthalmitis caused by multidrug-resistant Stenotrophomonas maltophilia: clinical features and risk factors.

BackgroundTo report clinical features and risk factors of post-cataract surgery endophthalmitis (PE) due to Stenotrophomonas maltophilia.MethodsA retrospective case review from December 10, 2010 to April 7, 2011 was performed at the Eye & ENT Hospital, Fudan University. Data were collected for surgical details, disease characteristics, antibiotic sensitivity of the pathogen, and treatment response. Visual outcomes were examined with a minimum follow-up of 12 months.ResultsFourteen cases of S. maltophilia endophthalmitis were identified. The onset of infection occurred from 1–56 days postoperatively (median, 13.5 days). Obvious cellular reactions were found in all patients in the anterior chamber, along with the absence of pupil synechia. Retinal periphlebitis was an early sign of PE. S. maltophilia was positive in eight patients (57.1%). The fluids from aspiration tubes revealed the same bacteria, which were resistant to multiple drugs (e.g., amino glycosides, most of the β-lactams, aztreonam, imipenem, and ciprofloxacin), except levofloxacin. Compared with the culture-negative group, the infection was more rapid, more severe, and more difficult to control in the culture-positive group. Among 14 patients, 11 patients (78.6%) underwent pars plana vitrectomy (PPV) with intravitreal injection. Three patients had PPV twice, and three patients had intraocular lens and capsular bag removal. A final visual acuity of ≥20/100 was achieved by 13/14 patients (92.9%). Complications included retinal detachment in three cases (21.4%) and recurrence of infection in two cases (14.8%). Statistical analysis showed that age over 90 years and posterior capsule rupture were risk factors of infection (P = 0.034 and P = 0.034, respectively). The phacoemulsifier allowed potential contamination between the aspiration and irrigation tubes.ConclusionsS. maltophilia should be considered a pathogenic organism of PE. The infection often occurs in older patients with posterior capsule rupture. Intravitreal or systemic administration of effective antibiotics and earlier initial PPV may contribute to better clinical outcomes. Tubes with connections between aspiration and irrigation should be avoided during surgery.

Negative Mantoux test in a patient with definite pulmonary and ocular tuberculosis

The case is reported of a patient with pulmonary and ocular tuberculosis presenting with blurred vision in both eyes. A 27-year-old well-nourished male nursing-home resident with a previous history of traumatic intracerebral hemorrhage was brought to the ophthalmological clinic due to progressively blurred vision. His best-corrected visual acuity was 20/400 in the right eye with only light perception in the left eye. Fundus examinations revealed retinal segmental periphlebitis and hemorrhagic retinitis in the right eye and dense vitreous hemorrhage in the left eye. The Mantoux test was negative; however, the results of an interferon gamma release assay were positive. Ocular tuberculosis was suspected. Although he had never had any respiratory symptoms, his chest radiograph and computed tomography scan showed a multiple centrilobular glandular and ground-glass appearance with air-space consolidations and atelectasis in both lower lobes. Pulmonary tuberculosis was confirmed by a positive acid-fast stain of a bronchial alveolar lavage sample. A GEN-PROBE amplified Mycobacterium tuberculosis direct test of the vitreous fluids was also positive. Ocular tuberculosis was confirmed. After treatment for tuberculosis and vitrectomies, his final best-corrected visual acuity improved to 20/30 in the right eye and 20/200 in the left eye. Ocular tuberculosis is rarely reported as the primary presentation of systemic tuberculosis in young patients. A negative Mantoux test may lead to misdiagnosis and delayed treatment. Doctors should become more familiar with the manifestations of systemic tuberculosis and use advanced diagnostic tools in cases of clinical suspicion.

Unilateral Chorioretinal Finding: A Presumptive Diagnosis in a Case of 5 Years Ago Treatment of Extrapulmonary Tuberculosis

The most common manifestation of ocular tuberculosis in patients with pulmonary tuberculosis is choroiditis. Retinal periphlebitis is rarely caused by direct invasion of the retina by tubercle bacilli. A 27-year-old woman with complaints of decreased vision one month ago demonstrated with presume ocular tuberculosis.

Infectious and noninfectious granulomatosis in patient with multiple sclerosis: diagnostic dilemmas and followup.

Patient was followed up over the course of 30 years. In 1978, after severe systemic infection followed by fever, pulmonary edema, and numerous neurological manifestations, patient was differentially diagnosed with apoplectic form of multiple sclerosis (MS), which was confirmed a year later via neurological and MRI findings. Approximately 20 years following the initial attack, sarcoidosis was diagnosed during the regular preoperative procedures required for cataract surgery. As consequence of lower immune system, infectious granulomatosis in form of pulmonary tuberculosis developed. Ophthalmological findings revealed bilateral retrobulbar neuritis (RBN) approximately six years after initial attack. This developed into total uveitis with retinal periphlebitis and anterior granulomatous uveitis—all of which are clinically similar in both MS and sarcoidosis.