Evaluation of Optical Coherence Tomography Angiography Findings in Pars Planitis and Multiple Sclerosis Associated Intermediate Uveitis in Remission.

PURPOSE: To evaluate the microvasculature during remission in patients with pars planitis (PP-IU) and multiple sclerosis-associated intermediate uveitis (MS-IU) using optical coherence tomography angiography (OCT-A). METHODS: Single-center, descriptive, case-control study was conducted. Adult patients (≥16 years) with IU in remission (PP-IU and MS-IU) and healthy age-sex matched healthy controls (HC) were enrolled to the study. Demographic/clinical features, best-corrected visual acuity (BCVA), ocular findings, neurological symptoms and preferred treatments were recorded. The presence of cystoid macular edema (CME) during follow-up was recorded. All IU patients in remission and HC subjects were scanned with OCT-A. Foveal avascular zone (FAZ) areas of superficial and deep capillary plexus (SCP/DCP) and vascular densities of SCP, DCP and choriocapillaris were obtained from OCT-A and compared between the groups. RESULTS: Sixty-nine eyes of 37 IU patients in remission and 20 HC were included (44 eyes/23 patients in PP-IU, 25/14 in MS-IU, 40/20 in HC). No statistically significant differences were observed in terms of demographic or clinical characteristics of the patients. The vascular density in the SCP was significantly reduced in the PP-IU and MS-IU groups compared to the HC group (p < .05). Nevertheless, there were no significant changes in any of the OCTA parameters between the IU groups. Uveitis duration was found to be correlated with enlargement of the FAZ area in PP-IU (p = .039). CONCLUSION: OCTA may not be useful in differentiating between PP-IU and MS-IU. Nevertheless, the primary implication in SCP potentially elucidates the pathogenesis of these two subtypes of IU, which are characterized by a shared pathogenesis. The monitoring of the FAZ area in the PP-IU group is valuable in terms of chronicity.

Pediatric Pars Planitis: A Review.

PURPOSE: To provide an overview of pediatric pars planitis. METHODS: Narrative literature review. RESULTS: Pars planitis refers to the idiopathic subset of intermediate uveitis in which there is vitritis along with snowball or snowbank formation occurring in the absence of an associated infection or systemic disease. It is thought to be a T-cell mediated disease with a genetic predisposition. Pars planitis accounts for 5-26.7% of pediatric uveitis cases. Presentation is commonly bilateral but asymmetric, often with insidious onset of floaters and blurred vision. Although pars planitis is known to be a benign form of uveitis in most cases, severe complications secondary to chronic inflammation may arise, with cystoid macular edema being the most common cause of visual morbidity. Mild vitritis in the absence of symptoms, vision loss, or macular edema may be observed. Patients with severe vitritis and/or associated vision-threatening complications require prompt aggressive treatment. A stepladder approach including corticosteroids, immunosuppressive agents, anti­tumor necrosis factor­alpha and pars plana vitrectomy and/or laser photocoagulation is the most commonly used method for treatment of pars planitis. CONCLUSION: Timely diagnosis and adequate treatment of pediatric pars planitis and associated complications are crucial in order to improve visual outcomes.

Clinical characteristics and treatment of pars planitis: an adalimumab experience.

PURPOSE: This study aims to investigate the clinical and demographic characteristics, treatment outcomes and complications of patients with pars planitis. METHODS: This retrospective study included patients diagnosed with pars planitis between 1998 and 2019 and followed for at least 6 months. Demographics, best-corrected visual acuity (BCVA), anterior segment and fundus examination findings, intraocular pressure (IOP) values at baseline and final examination, treatments used during the follow-up, surgeries and complications were noted from medical records of the patients. The percentage of patients given adalimumab (ADA), the reasons for treatment switch and response to ADA were investigated. RESULTS: One hundred fifteen eyes of 59 patients were included in the study. Forty-seven percent of patients were female. The median age of the patients was 10 (4-44) years. The median follow-up time was 33 (6-252) months. The median BCVA at admission was 0.20 (0.00-2.00) logMAR. The most common complications were cystoid macular oedema, cataract, epiretinal membrane and inferior peripheral retinoschisis. Prophylactic laser photocoagulation for peripheral retinoschisis was the most common surgical intervention, followed by cataract surgery and pars plana vitrectomy. Approximately 80% of patients received immunosuppressive and corticosteroid therapy for initial treatment. ADA was initiated in 23 patients (38.9%) due to refractory uveitis and adverse effects to the corticosteroid and helped control intraocular inflammation and decrease the use of systemic steroids/immunosuppressives in 22 of 23 (95%) of patients who received ADA. The median BCVA at final examination increased to 0.00 (0.00-2.00) logMAR. CONCLUSIONS: Pars planitis is a chronic, progressive and insidious disease with several ocular complications and requires early and aggressive treatment. ADA appeared to be effective especially in patients' refractory to conventional treatment.

Classification Criteria For Pars Planitis.

PURPOSE: To determine classification criteria for pars planitis. DESIGN: Machine learning of cases with pars planitis and 4 other intermediate uveitides. METHODS: Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set. RESULTS: Five hundred eighty-nine cases of intermediate uveitides, including 226 cases of pars planitis, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval 96.1, 99.9). Key criteria for pars planitis included unilateral or bilateral intermediate uveitis with either 1) snowballs in the vitreous or 2) snowbanks on the pars plana. Key exclusions included: 1) multiple sclerosis, 2) sarcoidosis, and 3) syphilis. The misclassification rates for pars planitis were 0% in the training set and 1.7% in the validation set, respectively. CONCLUSIONS: The criteria for pars planitis had a low misclassification rate and appeared to perform sufficiently well for use in clinical and translational research.

Classification Criteria for Intermediate Uveitis, Non-Pars Planitis Type.

PURPOSE: To determine classification criteria for intermediate uveitis, non-pars planitis type (IU-NPP, also known as undifferentiated intermediate uveitis). DESIGN: Machine learning of cases with IU-NPP and 4 other intermediate uveitides. METHODS: Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set. RESULTS: Five hundred eighty-nine of cases of intermediate uveitides, including 114 cases of IU-NPP, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval 96.1, 99.9). Key criteria for IU-NPP included unilateral or bilateral intermediate uveitis with neither snowballs in the vitreous humor nor snowbanks on the pars plana. Other key exclusions included multiple sclerosis, sarcoidosis, and syphilis. The misclassification rates for IU-NPP were 0% in the training set and 0% in the validation set. CONCLUSIONS: The criteria for IU-NPP had a low misclassification rate and seemed to perform well enough for use in clinical and translational research.

La esclerosis múltiple en oftalmología: más allá de la neuritis óptica / Multiple sclerosis in ophthalmology: beyond optic neuritis

Las alteraciones del sistema visual son uno de los principales rasgos de la esclerosis múltiple (EM) y conllevan un gran impacto en la calidad de vida de los pacientes. Aunque la neuritis óptica es la manifestación más frecuente, existen otros procesos oftalmológicos no relacionados con la neuritis, cuyo conocimiento es de gran utilidad en el manejo de los pacientes con EM. Se describen estas anomalías agrupadas en alteraciones de las vías aferente y eferente, así como de áreas cerebrales superiores. Adicionalmente, se describen los principales efectos oftalmológicos secundarios de los fármacos actualmente empleados en el control de la EM

Disorders of the visual system are one of the main features of multiple sclerosis (MS), and have a great impact on the quality of life of patients. Although optic neuritis is the most frequent manifestation, there are other ophthalmological processes not related to neuritis, a knowledge of which is very useful in the management of patients with MS. These abnormalities are described, grouped into impairments of the afferent pathway, efferent pathway, or upper cerebral areas. Additionally, the main ophthalmological side effects of the drugs currently used in the control of MS are described

Implications of pars planitis-associated cystoid macular edema on visual outcome and management in children.

PURPOSE: Pars planitis is a commonly observed type of pediatric uveitis. The aim of this study was to evaluate the implications of pars planitis-associated cystoid macular edema (CME) on visual outcome and treatment modalities. METHODS: A retrospective review of medical records in a single center with academic practice. RESULTS: Included were 33 children (mean age 8 years, 58 eyes). Eighteen eyes developed CME (31%): in 67% of them, CME was diagnosed at presentation and in 33%, it developed at a mean of 57 months after presentation. Anterior and posterior segment complications were more prevalent in eyes with CME. Papillitis was significantly associated with the development of CME (OR 12.4, 95% CI 2.3 to 65.6, p = 0.003). Patients with CME were 1.7 times more likely to be treated with systemic therapy. By the last follow-up, 50% of patients who never developed CME were without systemic therapy compared with 13% of patients who developed CME (p = 0.034). LogMAR visual acuity improvement between presentation and month 36 was 0.41 for eyes with CME compared with 0.14 for eyes that never developed CME (p = 0.009). CONCLUSION: Pars planitis-associated CME entailed higher prevalence of ocular complications, more frequent use of immunomodulatory therapy, and a lower rate of remission.

Inferior Corneal Haze and Inflammatory Endotheliopathy Related to Pars Planitis.

PURPOSE: To describe the finding of inferior corneal haze secondary to presumed inflammatory endothelio-10 pathy in a series of patients with pars planitis. METHODS: Single-center retrospective observational consecutive case-series. RESULTS: Seven patients with an established diagnosis of pars planitis are described in this case series including four females and three males. The ages ranged from 5 to 31 years at presentation. Pars planitis was bilateral in six patients and unilateral in one patient. Fundus examinations revealed vitreous opacities and pars plana exudates in all seven patients, cystoid macular edema in four patients, and peripheral retinal vasculitis in two patients. Corneal examination revealed opacification of the posterior cornea in an inferior location in 10 of the 13 eyes with pars planitis. Only one of these eyes had keratic precipitates. For a given patient, corneal involvement was more commonly seen in the more inflamed eye. In one patient with active inflammation, microcystic corneal edema was noted to predate the formation of inferior corneal endothelial opacification, suggesting that physical proximity to the site of inflammation at the inferior pars plana is the cause of this notable physical finding. CONCLUSIONS: Inferior posterior corneal haze related to inflammatory endotheliopathy may occur in eyes with pars planitis. As patients with pars planitis may be otherwise asymptomatic, this corneal finding shouldprompt a detailed funduscopic examination to rule out this form of uveitis.

Progression of retinal pigmentation mimicking unilateral retinitis pigmentosa after bilateral pars planitis: a case report.

BACKGROUND: To report our findings in a young patient with unilateral retinitis pigmentosa (RP)-like appearance who developed pigmentary changes in his left retina after an episode of bilateral pars planitis. CASE PRESENTATION: A 17-year-old man presented with 6 months of blurry vision in both eyes. He was diagnosed with bilateral pars planitis. Progressive, intraretinal bone crepuscule pigmentation developed in his left retina during the following three months. An electroretinogram showed subnormal response only in the left eye, suggesting the diagnosis of unilateral pseudoRP. CONCLUSION: An inflammatory disease like pars planitis can accelerate the pigmentation of the retina and mimic a RP in young patients. Causes of pseudoRP may be considered, especially in those rare cases with unilateral affection.

Ojo rojo en tatuaje: Un curioso ojo rojo de etiología tuberculosa / Red-eyed-tatoo: An unusual red eye of tuberculous etiology

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Outcomes of cataract surgery with/without vitrectomy in patients with pars planitis and immunosuppressive therapy.

BACKGROUND: The purpose of this study was to evaluate the characteristics and outcomes of cataract surgery with/without vitrectomy in patients with pars planitis who received immunosuppressive therapy. METHODS: This was a retrospective case series, single-center study. Twenty-two patients with pars planitis who received immunosuppressive therapy were included, with a median age at presentation of 9.5 years, having had cataract surgery. The following data was collected: age at presentation and at cataract surgery, time of follow-up, best-corrected visual acuity (BCVA) before the surgery and at 1 week, 1 and 6 months after the procedure, immunosuppressive therapy, complications and causes for failed visual improvement. The variables associated with an improvement in visual acuity were evaluated. RESULTS: All patients had phacoemulsification with intraocular lens implantation. The most common immunosuppressive therapy used for the patients was methotrexate in nine patients (40.9%). The BCVA improved from a median of 20/400 to 20/100 after 6 months of follow-up (p = 0.0005); 14 patients (63.6%) improved two lines of vision or more. No significant risk factors were found for the association with improvement in visual acuity after the surgery. No improvement in visual acuity was attributed to posterior segment manifestations or amblyopia; the most common complication was posterior capsule opacification in 11 eyes (50%). The median follow-up after the surgery was 32 months. CONCLUSION: Phacoemulsification was the procedure for all the patients. Visual acuity improved in patients with pars planitis treated with immunosuppressive drugs who underwent cataract surgery, except for the patients with posterior segment complications or amblyopia.

Two masquerade presentations of retinoblastoma.

Masquerade syndromes are disorders occurring with intraocular inflammation misdiagnosed as uveitis. The underlying causes may be benign or malignant conditions, and one of the most important diagnoses to take into consideration in children is retinoblastoma. We present two cases with uncertain early misdiagnosis whose definite diagnosis eventually was retinoblastoma.

Pars Planitis en la infancia / Pars Planitis in childhood

Objetivo: Evaluar la respuesta al tratamiento con corticoides e inmunomoduladores en niños con pars planitis. Materiales y método: Se realiza un estudio retrospectivo y observacional de nueve historias clínicas de pacientes con diagnóstico de pars planitis atendidos en el servicio de oftalmología del Hospital de Pediatría Juan P. Garrahan desde el año 2010. Resultados: De los nueve pacientes 6 eran niños y 3 niñas. El motivo de consulta principal fue disminución de la visión. La mayoría de los pacientes fueron derivados por otros oftalmólogos para tratamiento. El 100% sufrió afectación ocular bilateral. Las complicaciones fueron: catarata, queratopatía en banda, hipertensión ocular y edema macular cistoide. El 100% de los pacientes recibió tratamiento con corticoides por vía oral e inmunomoduladores. A algunos se les efectuó inyecciones de triamcinolona subtenoniana y criocoagulación. Conclusiones: con el tratamiento con prednisona e inmunomoduladores se logró mejoría en la agudeza visual en 15 ojos y 3 ojos mantuvieron igual agudeza visual. La pars planitis es una patología crónica con un pronóstico visual reservado que requiere de un seguimiento estrecho y tratamiento prolongado por parte de un equipo interdisciplinario (AU)

Objective: To assess response to steroid and immunomodulating treatment in children with pars planitis. Material and methods: A retrospective observational study was conducted reviewing nine clinical charts of children with a diagnosis of pars planitis seen at the Department of Ophthalmology of the Pediatric Hospital Juan P. Garrahan since 2010. Results: Of nine patients, six were boys and three were girls. Main complaint was loss of vision. The majority of patients were referred to our hospital by other ophthalmologists for treatment. All children had bilateral eye involvement. Complications observed were: cataracts, band keratopathy, ocular hypertension, and cystoid macular edema. All patients received oral steroids and immunomodulating treatment. In some subtenonian injection of triamcinolone and cryocoagulation was performed. Conclusions: Prednisone and immunomodulating treatment resulted in an improvement of visual acuity in 15 eyes and three eyes visual acuity remained unchanged. Pars planitis is a chronic disease with an uncertain visual prognosis that requires close follow-up and prolonged treatment by an interdisciplinary team (AU)

Chronic candida endophthalmitis as a cause of intermediate uveitis.

Intermediate uveitis is a subset of intraocular inflammation where vitritis is the most consistent sign, with or without snowball opacities or snow banks over the pars plana. Some patients will have an associated underlying systemic disease such as sarcoidosis, multiple sclerosis, ocular tuberculosis, inflammatory bowel disease, possibly Behçet's disease and intraocular lymphoma, whereas some will be classified as pars planitis in case of the lack of an identifiable systemic disease association. Our patient, a 47-year-old woman, developed intermediate uveitis after cataract surgery in her right eye, was misdiagnosed as pars planitis, and received steroid monotherapy for 8 months. Her inflammation only fully resolved after vitrectomy with removal of the intraocular lens (IOL) and capsular bag. Oral fluconazole and intravitreal amphotericin B injection had failed to resolve her inflammation when Candida albicans was identified as the cause of her persistent intermediate uveitis.

Treatment with immunosuppressive therapy in patients with pars planitis: experience of a reference centre in Mexico.

AIM: To evaluate the clinical course of the patients with pars planitis that received immunosuppressive drugs. METHODS: We retrospectively analysed the data of 10 years from 374 patients with pars planitis in a large reference centre in Mexico City and included 49 patients (92 eyes). RESULTS: Median age at presentation was 8 years. 35 patients (71.4%) were male and 43 patients (87.7%) had bilateral disease. Diverse immunosuppressive medications were used, mainly methotrexate (69.4%) and azathioprine (63.3%) with 18 patients requiring more than one drug. The main indications for starting immunosuppressive therapy were lack of response to initial treatment and advance disease at presentation. The results showed good response with steroid reduction (69.3% of patients), visual acuity improvement (51% of patients) and inflammatory disease reduction (59.1% of patients). In 25 patients (51%), steroids were started previous to immunosuppressors and in 24 (49%) at the same time without significant difference in clinical improvement (p=0.210) or visual outcome (p=0.498). Thirteen patients (26.5%) presented mild adverse effects. The median of the final visual acuity was 20/40. The median follow-up time was 44 months (range 13-115 months). CONCLUSIONS: Immunosuppressive therapy allows an adequate control of inflammatory disease in pars planitis, with clinical and visual improvement and steroid dose reduction.

Rheumatic inflammatory eye diseases of childhood.

Chronic inflammatory eye diseases are a common manifestation of pediatric rheumatologic diseases, potentially leading to lifelong vision impairment and disability. The mechanisms leading to the breach of the blood eye/brain barrier and the subsequent immune attack against a variety of intraocular mostly unidentified antigens remains poorly understood. Pediatric rheumatologists need to be familiar with the various inflammatory eye diseases because they are often responsible for selecting and supervising treatment in close collaboration with the ophthalmologist. This article provides an update of recent developments in the pathogenesis and treatment of the most relevant ocular diseases encountered in rheumatologic practice.

Aproximación diagnóstica a las uveítis / Uveitis: diagnostic approach

Mujer de 32 años, sin antecedentes epidemiológicos relevantes salvo contacto tuberculoso en la infancia, remitida desde el Servicio de Oftalmología para valorar estudio de enfermedad sistémica. Refiere miodesopsias y disminución de agudeza visual en ambos ojos de varios meses de evolución, acompañados de artralgias y tos seca, sin otra sintomatología extraocular. La exploración oftalmológica muestra agudeza visual de 0,3 en el ojo derecho y 0,4 en el ojo izquierdo. En la exploración de la cámara anterior se observa una uveítis anterior bilateral granulomatosa con discreta inflamación (Tyndall 1+). Se observa vitritis (3+) bilateral, con exudados en banco de nieve y flebitis periférica. La angiografía con fluoresceína no muestra signos de vasculitis central, y la tomografía de coherencia óptica confirma la existencia de edema macular quístico bilateral. La autofluorescencia es normal. ¿Cómo debe ser evaluada inicialmente esta paciente para decidir cuál es el estudio sistémico más adecuado teniendo en cuenta las manifestaciones oftalmológicas?(AU)

A 32 year-old woman was referred from the Ophthalmology Department to rule out a possible systemic disease. Her only past medical history of relevance was a tuberculosis contact during childhood. She complained of floaters and progressive blurring of vision in both eyes for some months, as well as arthralgia and cough. Her visual acuity was 0.3 in the right eye and 0.4 in the left eye. Biomicroscopy showed bilateral anterior granulomatous uveitis (1+ cells). Funduscopy showed bilateral vitritis 3+, snow banking and peripheral phlebitis. Fluorescein angiography did not show central vasculitis, and optical coherence tomography showed bilateral cystoid macular oedema. Fundus autofluorescence was normal. How would you initially assess this patient in order to decide which systemic examination should be performed, bearing in mind the ophthalmological manifestations?(AU)

Different ophthalmologic manifestations of sarcoidosis.

PURPOSE OF REVIEW: Sarcoidosis can manifest with different ocular findings. Three different cases have been presented, each of which showed different ocular problems. The literature has also been reviewed as to find out other eye signs and treatment strategies of the disease. The diagnosis may be difficult and the treatment may include combination of different immunosuppressors. RECENT FINDINGS: Recent findings include a genetic basis, and certain human leukocyte antigens may affect the course of the disease. Sarcoidosis can influence the eye and the optic nerves in the beginning, and biopsy of the involved tissue may be necessary for the diagnosis. Laboratory investigation may be unyielding. Once the diagnosis is made, steroids are generally started. Other than the classical corticosteroid treatment, other immunosuppressive agents show promise in the atypical cases. SUMMARY: Our cases show different manifestations of the disease like bilateral optic neuropathy, Horner's syndrome, pars planitis, and anterior and posterior uveitis. Patients recovered with steroid treatment, but especially in young patients other agents like methotrexate were needed because of the sideeffects of steroids.

[Clinical and epidemiological features and HLA association in patients with pars planitis]. / Características clínicas, epidemiológicas y asociación con el sistema HLA en pacientes con pars planitis.

BACKGROUND AND OBJECTIVES: Epidemiological studies on North American patients reported an association between HLA DR15 and pars planitis. This association has not been studied in the Spanish population. The objectives of the present study were to describe the clinical and epidemiological features of patients with pars planitis diagnosed in our hospital as well as the prevalence of multiple sclerosis and HLA class I and II. PATIENTS AND METHODS: Twenty four patients with pars planitis were identified among 226 patients with uveitis diagnosed in the Ophtahlmology Department of our center from January 1992 to October 2006. Twenty four patients and 194 healthy controls underwent HLA A, B and DR genotyping. RESULTS: The most frequent complication was cystic macular edema. Most patients needed many medical treatments. No statistical association was found between pars planitis and HLA. CONCLUSIONS: Epidemiological data were consistent with previously reported studies. There appears to be no association between the occurrence of pars planitis and HLA DR 15 or other known HLA genotypes in Spanish patients. However, the small sample size could have limited the power of this study.