Clinical characteristics of intermediate uveitis in adults according to criteria of the SUN working group.

PURPOSE: To analyse the clinical characteristics of adult patients with pars planitis (PP-IU), non-pars planitis (NPP-IU) and multiple sclerosis-associated intermediate uveitis (MS-IU) and distinguish between groups. METHODS: Seventy-three adult patients with intermediate uveitis (IU) reviewed retrospectively and divided as PP-IU, NPP-IU and MS-IU according to 'The standardization of uveitis nomenclature working group classification criteria.' Demographic and clinical characteristics, OCT and fluorescein angiography (FA) findings, complications and treatments were recorded. RESULTS: A total of 134 eyes of 73 patients were included, and 42 of the patients were classified as PP-IU, 12 as NPP-IU, and 19 as MS-IU. If a patient presenting with blurred vision, or tent-shaped vitreous band/snowballs/snowbank on examination, or vascular leakage on FA and accompanying neurological symptoms, the frequency of demyelinating plaque detection on cranial MRI and the risk of MS-IU increased. Mean BCVA was increased from 0.22 ± 0.30 logMAR to 0.19 ± 0.31 logMAR (p = 0.021). Gender, initial BCVA, snowbank formation, disc oedema and periphlebitis on examination, and disc leakage/occlusion on FA were found predictive of decreased BCVA at final visit (p < 0.05). CONCLUSIONS: The clinical features of these three groups are similar, some features that can guide the differential diagnosis. It may be recommended to periodically evaluate "suspicious" patients with MRI for MS.

Intermediate Uveitis: A Review.

PURPOSE: This review aims to provide an update on the clinical presentation, etiologies, complications, and treatment options in intermediate uveitis (IU). METHODS: Narrative literature review. RESULTS: IU affects all age groups with no clear gender predominance and has varied etiologies including systemic illnesses and infectious diseases, or pars planitis. In some instances, IU may be the sole presentation of an underlying associated condition or disease. Management of IU and its complications include administration of corticosteroids, antimetabolites, T-cell inhibitors, and/or biologics, along with surgical interventions, with varying degrees of effectiveness across literature. In particular, increasing evidence of the safety and efficacy of immunomodulatory agents and biologics has seen greater adoption of these therapies in clinical practice. CONCLUSIONS: IU is an anatomical description of uveitis, involving intraocular inflammation of the vitreous, peripheral retinal vasculature, and pars plana. Various treatment options for intermediate uveitis are currently used in practice.

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.

Clinical Presentation, Management, and Long-Term Outcome of Pars Planitis, Panuveitis, and Vogt-Koyanagi-Harada Disease in Children and Adolescents.

OBJECTIVE: Chronic uveitis is a common manifestation of pediatric rheumatologic conditions and may result in irreversible blindness and long-term disability. While chronic anterior uveitis is the most commonly encountered ocular manifestation of rheumatic disease, little is known about the clinical presentation, management, and long-term outcome of more complex eye conditions such as pars planitis (PP), panuveitis (PU), and Vogt-Koyanagi-Harada disease (VKH). The present study was undertaken to comprehensively assess the long-term safety and efficacy of disease-modifying antirheumatic drugs (DMARDs) and biologics for the treatment of pediatric and adolescent patients with PP, PU, and VKH. METHODS: We retrospectively reviewed a cohort of 75 children and adolescents with idiopathic PP (n = 50), PU (n = 12), and VKH (n = 14) followed by the Pediatric Rheumatology Core at Children's Hospital Los Angeles and evaluated referral patterns, clinical presentation, treatment response, and long-term clinical outcome. RESULTS: Patients were followed for an average of 52 months. Their mean age at disease onset was 10 years. Bilateral eye involvement was seen in 87% of the patients. At first presentation to an ophthalmologist, glaucoma was noted in 21% of patients and vision loss (<20/40) in 87% of patients, while legal blindness (≤20/200 in the better-seeing eye) was diagnosed in 18 of 75 (24%) of patients (PP 22%, PU 36%, and VKH 21%). The average referral time to a pediatric rheumatologist was 13 months (range 1-96 months). Topical steroids were used in all patients, but 98% of patients required additional DMARDs, and 73% required therapy with biologics. After a mean of 52 months, 35% of patients across all disease groups had significant vision loss or were blind, and only 28% were in clinical remission without medications. The worst outcome was observed in children with PU. Regression analysis, young age at onset, delayed referral to a pediatric rheumatologist, and chronic disease were strong predictors for the risk of long-term blindness. CONCLUSION: PP, PU, and VKH involve a high risk of permanent vision loss and should be managed by a skilled rheumatologist as early and as aggressively as possible.

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.

Analysis of clinical features and visual outcomes of pars planitis.

PURPOSE: To evaluate the demographic characteristics, clinical features, treatment and outcomes of patients with pars planitis in a tertiary referral center in Turkey. METHODS: Medical records of patients with pars planitis were retrospectively reviewed. The data including demographic and ocular features and treatment outcomes were recorded. The distribution of clinical findings and complications were evaluated according to age and gender groups. The changes in final BCVA compared to the initial BCVA were noted. Statistical analysis was performed using SPSS software (Version 18.0, SPSS Inc., Chicago, USA). RESULTS: Twenty-seven patients (54 eyes) were included in this study. 16 patients were male (59.3%), and 11 were female (40.7%). Mean age at diagnosis was 12.84 ± 8.26 (range 4-36) years. Mean follow-up period was 61.3 ± 52.15 (range 9-172) months. Mean BCVA was 0.58 ± 0.36 (range 0.03-1.00) (0.40 ± 0.45 logMAR) at presentation, and 0.81 ± 0.28 (range 0.10-1.00) (0.14 ± 0.27 logMAR) at final visit (P = 0.001). Vitreous inflammation (100%), vitreous haze (92.6%), snowballs (74.1%), snowbanks (66.7%), anterior chamber cells (66.7%) and peripheral retinal vascular sheathing (48.1%) were the most common presentations. Ocular complications included vitreous condensation (51.9%), cystoid macular edema (22.2%), cataract (18.5%), inferior peripheral retinal detachment (11.1%), glaucoma (5.6%) and vitreous hemorrhage (3.7%). Treatments included topical, periocular, intravitreal and systemic corticosteroids, immunosuppressives, peripheral laser photocoagulation and pars plana vitrectomy when needed. CONCLUSIONS: Pars planitis is an idiopathic chronic intermediate uveitis mostly affecting children and adolescents. In spite of its chronic nature with high potential of causing ocular complications, adequate treatment and close follow-up lead to favorable visual outcomes.

Uveítis unilateral asociada a nefropatía IgA / Unilateral uveitis associated with IgA nephropathy

Caso clínico: Presentamos el caso de un varón de 42 años de edad con varios episodios de uveítis unilateral en su ojo derecho. El examen oftalmológico mostró una uveítis anterior granulomatosa con vitritis. El estudio sistémico puso de manifiesto una proteinuria de rango no nefrótico y microhematuria. La biopsia renal reveló una nefropatía IgA. Discusión: Las uveítis y las glomerulonefritis podrían tener mecanismos inmunológicos comunes. En el diagnóstico diferencial de los pacientes con uveítis y nefropatías debería incluirse la nefropatía IgA (AU)

Case report: The case is presented of a 42 year-old man with episodes of unilateral uveitis in his right eye. Ophthalmic examination showed a granulomatous anterior uveitis with vitritis. Systemic investigations revealed non-nephrotic proteinuria and microhaematuria. A renal biopsy showed IgA nephropathy. Discussion: Uveitis and glomerulonephritis may have common immunological pathogenesis. IgA nephropathy should be a differential diagnosis in patients with uveitis and nephropathy (AU)

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.

Retinoschisis in Pars Planitis.

PURPOSE: To describe the course of retinoschisis in patients with pars planitis (PP). METHODS: Chart review of PP patients seen July 2012-September 2014 at a single institution. RESULTS: Included were 34 patients (68 eyes). Uveitis was bilateral in all cases. Thirteen eyes (19%) developed retinoschisis. In six patients (86%), the schisis was bilateral. The average follow-up of patients with schisis was 7 years; the average best-corrected visual acuity (BCVA) was 20/22 at last follow-up. Schisis was noted to develop or progress in patients with both active and inactive inflammation. Five eyes of five patients underwent vitrectomy; three for disease control, with scleral buckle to reduce residual traction. Two eyes required vitrectomy for retinal detachment with progressive schisis, despite inactive uveitis. Seven eyes remained stable without intervention. CONCLUSIONS: Retinoschisis is a common complication in patients with PP. It is typically bilateral, and may develop or progress, despite control of uveitis.

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)

Asociación de membranas epirretinianas con edema macular en pars planitis / Association of epiretinal membranes with macular edema in pars planitis

INTRODUCCIÓN: La pars planitis (PP) es una uveítis intermedia idiopática que presenta múltiples complicaciones en el segmento posterior, las cuales incluyen el edema macular quístico o cistoideo (EM) y la formación de membranas epirretinianas (MER). Comúnmente se decide realizar tratamiento antiinflamatorio o inmunosupresor ante la presencia de EM. Sin embargo, este puede coexistir con la presencia de MER y, por lo tanto, existe la posibilidad de que el enfoque del tratamiento deba ser diferente. OBJETIVOS: Determinar la asociación que existe entre la presencia de MER con EM en PP. MATERIALES Y MÉTODOS: Serie de casos y controles donde se analizaron de manera retrospectiva los expedientes de pacientes con PP, todos ellos seguidos con angiografía con fluoresceína (AF) y tomografía de coherencia óptica de dominio espectral (SD-OCT). Se determinó la presencia de MER por SD-OCT, mientras que el EM fue determinado por AF. Se realizaron tablas de contingencia para determinar cómo influyen las MER como factores de riesgo para desarrollar EM. RESULTADOS: 31 ojos presentaron MER. 16 ojos presentaron EM. La razón de riesgo para presentar EM por MER fue de 0,971 con un valor de p determinada por χ2 de 0,77. CONCLUSIONES: No existe una asociación significativa entre la formación de MER y el desarrollo de EM. No existe evidencia que sugiera considerar un abordaje quirúrgico como primera línea de tratamiento ante la presencia de MER en PP

INTRODUCTION: Pars planitis (PP) is a form of intermediate uveitis that manifests with several posterior segment complications, including cystoid macular edema (CME) and epiretinal membrane formation (ERM). On the presence of CME the patient is usually treated with anti-inflammatory and/or immunosuppressive drugs. However the presence of CME may coexist with ERM formation, and therefore the treatment could be different. PURPOSE: To determine the association between ERM and CME in PP. MATERIALS AND METHODS: Case control series. The charts of patients diagnosed with PP were retrospectively reviewed. All patients had fluorescein angiogram (FA) and spectral domain optical coherence tomography (SD-OCT). Presence of ERM was determined by SD-OCT, while CME was determined by FA. Contingency tables were used to determine the risk of developing CME with ERM. RESULTS: 31 eyes presented ERM. 16 eyes presented CME. Relative risk to have CME and ERM was 0.971, with a P value of 0.77 (χ2). CONCLUSIONS: There is no association between ERM formation and the development of CME. There is no evidence to suggest a surgical approach as first line of treatment with the presence of ERM in PP

[Association of epiretinal membranes with macular edema in pars planitis]. / Asociación de membranas epirretinianas con edema macular en pars planitis.

INTRODUCTION: Pars planitis (PP) is a form of intermediate uveitis that manifests with several posterior segment complications, including cystoid macular edema (CME) and epiretinal membrane formation (ERM). On the presence of CME the patient is usually treated with anti-inflammatory and/or immunosuppressive drugs. However the presence of CME may coexist with ERM formation, and therefore the treatment could be different. PURPOSE: To determine the association between ERM and CME in PP. MATERIALS AND METHODS: Case control series. The charts of patients diagnosed with PP were retrospectively reviewed. All patients had fluorescein angiogram (FA) and spectral domain optical coherence tomography (SD-OCT). Presence of ERM was determined by SD-OCT, while CME was determined by FA. Contingency tables were used to determine the risk of developing CME with ERM. RESULTS: 31 eyes presented ERM. 16 eyes presented CME. Relative risk to have CME and ERM was 0.971, with a P value of 0.77 (χ(2)). CONCLUSIONS: There is no association between ERM formation and the development of CME. There is no evidence to suggest a surgical approach as first line of treatment with the presence of ERM in PP.

Retinal vasoproliferative tumors: comparative clinical features of primary vs secondary tumors in 334 cases.

OBJECTIVE: To compare the clinical features of primary vs secondary retinal vasoproliferative tumors (VPTs). METHODS: Retrospective case series of 334 tumors in 295 eyes of 275 patients. RESULTS: Of 275 patients with VPT, 41% (n = 113) were male and 59% (n = 162) were female, with a mean age of 44 years at presentation. Primary VPT occurred in 80% (n = 219) and secondary VPT, in 20% (n = 56) of patients. Secondary VPT (n = 67) occurred in eyes with retinitis pigmentosa (n = 15, 22%), pars planitis (n = 14, 21%), Coats disease (n = 11, 16%), previous retinal detachment surgery (n = 8, 12%), idiopathic peripheral retinal vasculitis (n = 4, 6%), familial exudative vitreoretinopathy (n = 3, 4%), and others (n = 12, 18%). The mean interval between diagnosis of underlying ocular condition and secondary VPT was 160 months. Statistically significant differences (P < .05) in clinical features (primary vs secondary VPTs) included mean age at presentation (46 vs 38 years), visual symptoms (74% vs 87%), poor visual acuity worse than 20/200 (15% vs 28%), bilaterality (4% vs 20%), multifocality (5% vs 15%), postequatorial tumor location (20% vs 33%), tumor basal dimension (6 vs 7 mm), anterior chamber cells (16% vs 30%), and vitreous cells (19% vs 48%). CONCLUSIONS: Retinal vasoproliferative tumor can be primary (80%) or secondary (20%). Compared with primary VPT, secondary VPT is more often bilateral, multiple, and larger and occurs at an earlier age associated with poorer visual acuity.

[Vitrectomy surgery for the treatment of the vitreo-retinal complications of the pars planitis]. / Cirugía de vitrectomía para el tratamiento de las complicaciones vítreo-retinianas asociadas a pars planitis.

OBJECTIVE: To evaluate the effect of pars plana vitrectomy in the management of patients with pars planitis. METHODS: A retrospective analysis of the clinical course, post-operative complications and recurrent uveal inflammation following pars plana vitrectomy in patients with pars planitis. RESULTS: The study included 22 eyes of 19 patients. The mean follow-up was 55.7 (± 39.6) months (range 7 - 144 months). The surgical indications were, persistent vitreous opacities in 10 eyes, vitreous haemorrhage in 9 eyes, and epiretinal membrane in 3 eyes. There was an improvement in the clinical course of the uveitis in 19 of the 22 eyes (86.4%), allowing the suspension of the systemic treatment in 16 patients. An improvement of the visual acuity was observed in 20 eyes (90.9%). The most common post-operative complications were, lens opacities in 9 eyes (40.9%), and glaucoma in 4 eyes (18.2%). CONCLUSIONS: The results of this study suggest that pars plana vitrectomy has a beneficial effect on the course and visual function of patients with vitreo-retinal complications associated with pars planitis.

[Pars plana vitrectomy as anti-inflammatory therapy for intermediate uveitis in children]. / La vitrectomía como terapia antinflamatoria de la uveítis intermedia en niños.

PURPOSE: Intermediate uveitis represents between 2 and 26% of uveitis in children. The spectrum of the disease is highly variable, ranging between mild cases that resolve spontaneously and chronic, severe forms that develop multiple episodes and complications. The purpose of this study is to evaluate the efficacy of vitrectomy to control inflammation in children with recurrent intermediate uveitis. METHODS: Retrospective evaluation of patients with at least six months of follow-up. All patients under 16 who had undergone vitrectomy for intermediate uveitis were included. Vitrectomy was performed after at least two episodes of intermediate uveitis in children that had had no previous prophylactic systemic immunosuppressant treatment. Data recorded were visual acuity (VA), recurrences and surgical complications. RESULTS: Seven eyes of five children with intermediate uveitis who underwent vitrectomy were included. After a mean follow-up of 34 months, VA improved in all eyes after surgery. Four eyes developed mild subcapsular posterior cataracts. Post-surgical recurrences were anterior and responded to topical treatment, except for an episode of intermediate uveitis that required a periocular injection of triamcinolone. Only one patient is being treated with systemic immunosuppressants, due to the presence of repeated episodes of uveitis in the non-vitrectomised eye and since his parents were unwilling to have him undergo new surgery. CONCLUSIONS: Vitrectomy with inferior cryotheraphy controls inflammation in intermediate uveitis in children with good mid-term results avoiding the secondary side-effects of systemic immunosuppressants.

Decompression retinopathy and possible risk factors.

PURPOSE: To discuss the underlying mechanisms in decompression retinopathy. METHODS: Report of two cases. RESULTS: Retinal hemorrhages secondary to decompression retinopathy occurred following combined trabeculotomy-trabeculectomy with mitomycine-C (MMC) in one eye of a case of congenital glaucoma and pars plana vitrectomy-lensectomy in a case of vitreous condensation secondary to pars planitis. Both cases were operated under general anesthesia. Postoperative hypotony did not take place in either eye. The same surgical procedure was performed on the other eye of the patient with congenital glaucoma 1 week apart. Postoperative decompression retinopathy was not seen in this eye despite hypotony was recorded. CONCLUSION: Valsalva manoeuvre, hypotony and other factors may play a role individually or in combination in the pathogenesis of decompression retinopathy.

Endoteliopatía corneal autoinmune como manifestación de uveitis intermedia / Corneal autoimmune endotheliopathy as manifestation of intermediate uveitis in children

Background: Intermediate Uveitis (IU) is an important cause of uveitis in children. It is considered a chronic intraocular inflammation that mainly affects the anterior vitreous and peripheral retina. Pars Planitis is a subtype of IU, consisting of a white opacity that covers the pars plana and ora serrata plus vitreous condensations in the eye. Corneal endothelium disease is a rare clinical finding associated with pars planitis. Objective: Describe the corneal manifestations in IU and its associated treatment between ophthalmology and pediatric rheumatology. Case-report: A 5 years-old boy with autoimmune endotheliopathy and unilateral pars planitis in the right eye. Initially, he was treated topically, but evolved with intraocular complications that required systemic medication. Long-term follow-up was performed, searching for associated systemic diseases, until one of these entities appeared. Conclusion: It is very important to search for inflammation in the anterior vitreous and pars plana in all children with corneal endotheliopathy, considering that an early and integral management of IU could diminish the risk of visual impairment as a complication.

Introducción: La Uveitis Intermedia (UI) es una causa importante de uveitis infantil. Se considera como una inflamación intraocular crónica que afecta principalmente a la retina periférica y vitreo anterior. La Pars Planitis es un subtipo de UI caracterizada por opacidades blanquecinas sobre la pars plana y ora serrata más condensaciones vitreas. La enfermedad del endotelio corneal es un hallazgo clínico infrecuente asociado con la Pars Planitis. Objetivo: Destacar la manifestación corneal de una UI y el tratamiento de la enfermedad entre oftalmólogo y reumatólogo infantil. Caso clínico: Se presenta caso clínico de niño de 5 años de edad con endoteliopatía autoinmune asociada a Pars Planitis unilateral del ojo derecho, que en principio se trató localmente, pero evolucionó con complicaciones intraoculares que requirieron medicación sistémica y se efectuó un seguimiento clínico por varios años buscando enfermedades sistémicas asociadas a UI hasta que ella apareció. Conclusión: Es necesario buscar la presencia de inflamación de pars plana y vitreo en todo niño que se presente con endoteliopatía corneal, considerando que el tratamiento oportuno y manejo integral del paciente con UI puede disminuir el riesgo de pérdida visual asociado a las complicaciones de esta enfermedad.