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.

Anti-tumor necrosis factor-alpha treatment in pediatric patients with pars planitis: a single-center experience from Turkey.

AIM: To evaluate the effectiveness and safety of anti-tumor necrosis factor-alpha (anti-TNF-alpha) treatment (Adalimumab [ADA]) combined with immunomodulatory agents (IMAs) in the treatment of pars planitis (PP). METHODS: The patients with PP who were treated with anti-TNF-alpha agents for at least six months were qualified for the chart review. The outcome parameters were the steroid-free remission state, the best-corrected visual acuity (BCVA) and the central macular thickness (CMT) of the patients at the last visit. RESULTS: After a mean total follow-up time of 15.5 ± 5.8 months (8-24 months), all the cases were in steroid-free remission at the last visit. The mean BCVA increased, and the mean CMT decreased significantly at the last visit (p < 0.001, p < 0.001, respectively). CONCLUSION: ADA combined with IMAs offers effective and safe treatment modalities in the control of chronic intraocular inflammation in PP cases.

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.

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.

Peripheral Cryoablation for Treatment of Active Pars Planitis: Long-Term Outcomes of a Retrospective Study.

PURPOSE: To compare the long-term outcomes of peripheral retinal cryoablation to conventional treatment for active pars planitis. DESIGN: Retrospective, interventional, comparative case series. METHODS: Review at a single institution was conducted to compare the effect of cryotherapy to eyes with pars planitis to those receiving conventional therapy (topical, regionally injected, or oral corticosteroid therapy). Best-corrected visual acuity (VA), complications, resolution of cystoid macular edema (CME), and anterior chamber and vitreous inflammation were assessed. RESULTS: One hundred thirty-six eyes were treated conventionally, 50 eyes were treated with cryotherapy. Median follow-up was 60.8 months (range 8.1-223.1 months) in the cryotherapy group and 45.0 months (range 3.1-339.0 months) in the controls. There were no significant differences in baseline VA, anterior chamber and vitreous inflammation, presence of CME, and prior use of regional corticosteroid injections. VA improved over time in the cryotherapy group (slope of -0.0018 logMAR units per month; P = .023) but declined in the controls (slope of +0.0011 logMAR units per month; P = .023). Kaplan-Meier survival estimates demonstrated faster times to resolution of anterior chamber cell, vitreous cell, and CME in the cryotherapy-treated eyes. Hazard ratios of remission (adjusted for confounding factors) for vitreous cell and CME for those treated with cryotherapy compared to controls were 4.73 (95% confidence interval 1.63, 13.63; P = .004) and 6.85 (95% confidence interval 1.06, 44.78; P = .044), respectively. No ocular complications were identified in the cryotherapy group. CONCLUSIONS: These data suggest that peripheral retinal cryoablation therapy is an effective treatment for active pars planitis and may be better than conventional regional corticosteroid injections and oral corticosteroid therapy for induction of remission.

Pacientes con pars planitis tratados con fotocoagulación selectiva: curso clínico / Clinical course of pars planitis in patients treated with selective photocoagulation

Introducción: La pars planitis es una uveítis intermedia, con una presentación bilateral y asimétrica. La etiología es desconocida y la patogenia no está clara. El tratamiento sigue el algoritmo de Foster, donde se incluye la fotocoagulación selectiva. El mecanismo de acción de la fotocoagulación es desconocido. Materiales y métodos: Estudio observacional, longitudinal, cohorte ambilectivo. El objetivo fue evaluar el curso de la inflamación en pacientes con pars planitis tratados con láser argón selectivo. Resultados: Se incluyó a 29 pacientes con diagnóstico de pars planitis tratados con láser selectivo; 10 pacientes eran del género femenino y 19 del masculino. La edad promedio de inicio de la enfermedad fue 11,37 años. De los pacientes, en el momento de recibir el láser selectivo, 18 (62,1%) no se encontraban con inmunosupresión y 11 (37,9%) estaban con inmunosupresor. Las indicaciones de láser fueron por: seguimiento del algoritmo 19 (65,55%), hemorragia vítrea siete (24,1%), vitrectomía dos (6,98%) y neovasos uno (3,4%). El tiempo promedio de disminución de la inflamación fue de 5,9 meses y 17 pacientes (58,6%) no presentaron recaída. Las agudezas visuales presentaron mejoría posláser (OD p = 0,025 y OI p = 0,022). Se observó mejoría clínica de la celularidad vítrea. Conclusión: El láser selectivo demostró ser efectivo en el 58,6%% de los pacientes (AU)

Introduction: Pars planitis is an intermediate uveitis with bilateral and asymmetric presentation. The etiology is unknown and pathogenesis is unclear. Treatment follows the algorithm of Foster, which includes selective photocoagulation. The mechanism of action of photocoagulation is still unknown. Material and methods: An observational, longitudinal, ambispective cohort study was performed with the objective of evaluating the course of inflammation in patients with pars planitis treated with a selective argon laser. Results: The study included 29 patients (10 female and 19 male) diagnosed with pars planitis and were treated with selective laser. The mean age of onset was 11.77 years. Eighteen (62.1%) patients were not immunosuppressed at the time of receiving the selective laser, and 11 (37.9%) were taking immunosuppressants. Indications for selective laser were; following the algorithm, 19 (65.55%), vitreous hemorrhage 7 (24.1%), vitrectomy 2 (6.98%), and neovascularization 1 (3.4%). The mean time for inflammation reduction was 5.9 months, and 17 patients (58.6%) had no relapse. Visual acuity showed improvement post-laser (OD P = 0.025 and OI P = 0.022). There was also an improvement in vitreous cells. Conclusion: Selective laser was effective in 58.6%% of patients (AU)

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)

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.

Pars planitis in children: epidemiologic, clinical, and therapeutic characteristics.

PURPOSE: To report the demographics and clinical characteristics, therapy logarithm, and prognosis of children with pars planitis. PATIENTS AND METHODS: The medical records were reviewed of all patients diagnosed with pars planitis between June 1995 and December 2005 in the Department of Pediatric Ophthalmology at Hospital Universitario La Paz, Madrid, Spain. A retrospective, descriptive, and longitudinal study of 30 eyes in 16 children was performed. RESULTS: Pars planitis was bilateral in 87.5% and more frequent in males (68.8%). Average age at onset was 9.2 years. The main ophthalmologic findings recorded were snowballs (96.7%) and vitritis (93.3%). Cataract formation was the most prevalent complication (36.7%). Mean initial and final best-corrected visual acuities were 0.640 and 0.840, respectively. Periocular corticosteroids were used in 33.3% of cases and cryotherapy or laser photocoagulation in 16.7%. Complications requiring surgical management occurred in 4 eyes (13.3%). CONCLUSION: Pars planitis treated with adequate medical and surgical procedures has a good prognosis in most cases.

Peripheral retinoschisis and exudative retinal detachment in pars planitis.

PURPOSE: To review and describe bullous retinoschisis and exudative retinal detachment in patients with pars planitis. METHODS: Retrospective, multicenter study of patients with pars planitis who presented with retinoschisis and exudative retinal detachments. RESULTS: The authors describe 13 eyes of 9 patients with pars planitis who presented with inferior peripheral retinoschisis and/or exudative retinal detachment. Four patients were male; five were female. Patients' ages ranged from 8 years to 35 years (median, 12 years). The follow-up interval ranged from 1 month to 10 years (median, 4 years). These peripheral retinal elevations had a tendency to remain stable, although those with telangiectatic vessels or vasoproliferative tumors occasionally demonstrated an increase in accumulation of exudate. Treatment of such eyes with cryotherapy, or low-dose plaque radiotherapy, resulted in vasoproliferative tumor and telangiectatic vessel regression, absorption of the hard exudate, and resolution of the retinal elevation in four of five eyes. One patient had spontaneous regression of the retinoschisis cavity over a 4-year period. CONCLUSION: Patients with pars planitis may present with bullous retinoschisis and/or exudative retinal detachment. These findings may be related to a Coats disease-like vascular response (telangiectatic vessels and vasoproliferative tumors) secondary to chronic inflammation. Treatment of the vascular leakage tended to result in resolution of the detachment and/or schisis.

Clinical findings in posterior uveitis [abstract]

Posterior uveitis which is an important cause of visual loss, needs careful work-up and aggressive management to minimize morbidity and reduce the incidence of blindness. Uveitis may be classified with regards to its presentation (acute or chronic) and also depending on its anatomic location. Patient symptoms are often helpful in determining the type of inflammation. It is important to carefully evaluate patients with posterior uveitis to determine the aetiology. Inflammation may be in the par plana (pars planitis), retina (retinitis) and the choroid (choroiditis). Some patients have inflammation in many layers (chorio-retinitis). In addition to specific agents to treat aetiologic factors (penicillin for syphilis), steroids are important agents for treating inflammation in these patients. Appropriate laboratory investigations help to determine systemic disorders. Chest X-rays are important to evaluate granulomatous diseases such as sarcoidosis and tuberculosis. Close follow-up is critical. (AU)

Pars planitis: epidemiology and clinical outcome in a large community hospital in Mexico City.

Medical records of 51 patients with a diagnosis of pars planitis (97 eyes) were reviewed. Based upon the severity of vitreous inflammation, in 34 eyes (35%) the condition was classified as mild, in 38 (39%) as moderate, and in 25 eyes (26%) as severe. Vascular sheathing was present in 51 eyes (53%), snowballs in 42 (43%), snowbanks in 29 (30%), cataract in 19 (20%), cystoid macular edema in 26 (27%), retinal detachment in 4 (4%) and glaucoma in 3 (3%) eyes. Treatment consisted of prednisone and periocular injections of deposteroids or immunosuppressive agents; 13 patients required surgery for retinal repair, glaucoma, vitreous opacities or cataract. After treatment the visual acuity improved in 58% of the eyes, did not change in 33%, and worsened in 9%.