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Familial pars planitis.

Pars planitis is an intraocular inflammatory disorder which usually affects children or young adults and is characterized by vitreous cells and debris ('snowballs'), exudate, and 'snowbank' formation along the pars plana, variable periphlebitis, and cystoid macular edema. Although no inheritance pattern has been defined, familial cases of pars planitis have been reported. This report describes pars planitis in two sisters, one of whom had evidence of demyelinating disease at presentation. The literature on familial pars planitis is reviewed. To the author's knowledge this is the first case of familial pars planitis as the presenting sign of possible demyelinating disease.

Administration, Oral↗

Familial pars planitis.

Pars planitis has rarely been noted to occur in more than one member of a family. We report the occurrence of this disorder in more than one member of two unrelated families. The affected members of one of these families included a 37-year-old woman and three of her six children. The affected members of the other family were two teenaged brothers. No specific cause for the familial clustering of pars planitis has been identified in the affected members of these families.

Adolescent↗

Clinical symptoms and complications of pars planitis in childhood.

UNLABELLED: Pars planitis is the idiopathic form of intermediate uveitis; it most commonly affects persons between 5 and 40 years of age. In childhood, the disease is largely asymptomatic until in a well advanced stage with markedly reduced vision. Lack of early diagnosis and treatment may lead to permanent visual loss and amblyopia. AIM: To report on the clinical symptoms, complications, treatment and visual outcome of pars planitis in childhood. MATERIALS AND METHODS: Ten patients (8 boys and 2 girls) all younger than 16 years were diagnosed with and treated for pars planitis at our clinic between January 1993 and August 2004.

Adolescent↗

Visual outcomes after pars plana vitrectomy for epiretinal membranes associated with pars planitis.

OBJECTIVE: To evaluate the results of pars plana vitrectomy and membrane stripping for visually significant macular epiretinal membranes associated with chronic idiopathic pars planitis. DESIGN: Consecutive noncomparative case series. PARTICIPANTS AND METHODS: The records of all patients who underwent pars plana vitrectomy for pars planitis from 1988 through 1997 were retrospectively reviewed. Seven eyes of five patients who were diagnosed with visually significant epiretinal membranes associated with pars planitis and who underwent vitrectomy and membrane stripping were analyzed. Patients were diagnosed with pars planitis based on characteristic clinical signs and pertinent negative laboratory test results. INTERVENTION: Pars plana vitrectomy and epiretinal membrane stripping. MAIN OUTCOME MEASURES: Visual acuity and inflammatory grade were compared between the last preoperative visit and the most recent follow-up visit. Intraoperative and postoperative complications were also analyzed. RESULTS: The mean patient age was 31 years (range, 6 to 45 years). The mean duration of uveitis was 6.4 years (range, 6 months to 13 years). All patients were treated with combinations of periocular, topical, and oral corticosteroids before surgery. Five eyes had laser retinopexy, and two eyes had cryopexy to the inferior retina at the time of surgery. Five eyes had at least 3 Snellen lines of visual acuity improvement, and visual acuity in one eye worsened by 2 lines. Mean preoperative visual acuity was 20/73 (range, 20/50 to 20/300), and mean final visual acuity was 20/37 (range, 20/25 to 20/70). Five eyes had a final visual acuity of 20/40. Vitritis improved in all cases. Mean follow-up was 23 months (range, 3 to 54 months). Six of seven eyes had progressive cataract development, four of which underwent cataract extraction. No other intraoperative or postoperative complications occurred. CONCLUSIONS: Removal of epiretinal membranes associated with pars planitis can be safely performed and may result in improved visual acuity. Patients often require subsequent cataract extraction to obtain the best long-term final acuity.

Acute Disease↗

Familial pars planitis.

Ophthalmologists do not consider pars planitis to occur on a familial basis. The development of pars planitis in nine members of four different families is reported. Affected family members include twin sisters, a mother and daughter, two brothers, and a sister and two brothers from the same family. This suggests that a common hereditary and/or environmental factor contributed to the disease in these patients.

Adolescent↗

The association of HLA-B8, B51, DR2, and multiple sclerosis in pars planitis.

PURPOSE: To establish a human leukocyte antigen (HLA) association in a homogeneous population of patients with pars planitis. METHODS: A strict set of inclusion parameters was established for the diagnosis of pars planitis. Forty patients with pars planitis who met these criteria underwent HLA analysis of class I and II phenotypes. RESULTS: HLA-B8 was present in 15 (37.5%) of 40 patients versus 85 (19.7%) of 431 controls (relative risk, 2.44; P = 0.011). HLA-B51 was present in 9 (22.5%) of 40 patients versus 51 (11.8%) of 431 controls (relative risk, 2.16; P = 0.049). HLA-DR2 was present in 27 (67.5%) of 40 patients versus 121 (28.0%) of 431 controls (relative risk, 5.32; P < 0.0001). HLA-DR2 has been associated with multiple sclerosis (MS). Exclusion of five patients with pars planitis in whom MS subsequently developed did not change the significance of these findings. CONCLUSIONS: The strongest association of pars planitis with HLA-DR2 and the temporal development of MS in some patients with pars planitis further supports an association between pars planitis and MS.

Case-Control Studies↗

Long-term visual outcome and complications associated with pars planitis.

PURPOSE: To identify the ocular complications and to statistically evaluate the possible association of pars planitis with multiple sclerosis (MS) in a homogeneous population of pars planitis patients. METHODS: The authors reexamined 36 patients and reviewed the records of an additional 18 patients (total: 54 patients, 108 eyes) with idiopathic pars planitis. RESULTS: The initial mean visual acuity of 20/46 (logMAR: 0.36 +/- 0.50) was not statistically different from the final mean visual acuity of 20/44 (logMAR: 0.34 +/- 0.45; P = 0.73), after a mean follow-up of 89.2 months. Complications included neovascularization with or without associated vitreous hemorrhage (7 eyes, 6.5%), moderate to severe cellophane retinopathy (7 eyes, 6.5%), chronic cystoid macular edema (CME) (9 eyes, 8.3%), visually significant cataracts (16 eyes, 14.8%), and retinal detachment (9 eyes, 8.3%). Significant lens opacification was associated with a greater risk of retinal detachment (P = 0.004). In four patients (7.4%), optic neuritis developed, and in an additional eight patients (14.8%) MS developed. Kaplan-Meier analysis of these data showed a 16.2% +/- 6.2% risk of MS solely developing in patients, and a 20.4% +/- 6.7% risk of either MS or optic neuritis developing, after 5 years of disease. The presence of periphlebitis at the time of pars planitis diagnosis increased the rate of development of these conditions (P = 0.002). Six patients (11.1%) had a family history positive for MS in a first-degree relative. CONCLUSIONS: This study demonstrates the overall favorable visual prognosis in patients with pars planitis. Patients with significant cataract formation appear to be at greater risk for retinal detachment. Periphlebitis at the time of diagnosis of pars planitis increases the risk of development of optic neuritis or MS. The strong association demonstrated between pars planitis and MS in this study further supports a link between the two disease states.

Adult↗

Vitreous hemorrhage is a common complication of pediatric pars planitis.

OBJECTIVE: To report the prevalence of vitreous hemorrhage in pars planitis and to compare the prevalence of hemorrhage for children and adults with the disease. DESIGN: A retrospective, cross-sectional observational study. PARTICIPANTS: One hundred eighteen consecutive patients with pars planitis who were evaluated at the Oregon Health and Science University Uveitis Clinic between September 1985 and April 2000. METHOD: A review of clinical records. MAIN OUTCOME MEASURES: For all patients, we recorded presence or absence of vitreous hemorrhage, as well as laterality and cause. Children were defined as being age 16 years or younger at diagnosis, and adults were defined as being aged 17 years or older at diagnosis. RESULTS: Fourteen percent of patients with pars planitis experienced vitreous hemorrhage. Persons with hemorrhage were significantly younger at the time of disease diagnosis than persons without hemorrhage (P = 0.040). The difference in prevalence of vitreous hemorrhage between children (28%) and adults (6%) was statistically significant (P = 0.003). The difference in prevalence of hemorrhage as a presenting feature between children (20%) and adults (1%) was also statistically significant (P = 0.001). CONCLUSIONS: Children with pars planitis are more likely than adults to experience vitreous hemorrhage. Pars planitis should be considered in the differential diagnosis of pediatric vitreous hemorrhage.

Adolescent↗

The association of HLA class II with pars planitis.

PURPOSE: To investigate the association of HLA class II alleles with pars planitis. METHODS: Blood samples were obtained from 28 patients with pars planitis seen in the Department of Ophthalmology and Visual Sciences and the Barnes Retina Institute at Washington University, St. Louis, Missouri. RESULTS: HLA-DR15, one of the allelic subtypes of HLA-DR2, was present in 18 (64.3%) of 28 patients vs. 10 (20%) of 50 controls (OR = 7.20, CI = 2.28--23.20, P =.0001). HLA-DR51 (HLA-DRB 5) was present in 16 (57.1%) of 28 patients vs. 6 (12%) of 50 controls (OR = 9.78, CI = 2.79--36.42, P =.0001). HLA-DR17 was present in eight (28.6%) of 28 patients vs. one (2%) of 50 controls (OR = 19.60, CI = 2.29--886.7, P =.0001). CONCLUSION: Pars planitis is associated with an increased frequency of the HLA-DR2 suballele, -DR15, HLA-DR51, and HLA-DR17. These results suggest an immunogenic predisposition exists to pars planitis.

Adolescent↗

Pars planitis: epidemiology, treatment, and association with multiple sclerosis.

During retrospective and prospective studies, we attempted to determine the clinical characteristics, treatment, and visual outcome of patients with pars planitis and to evaluate the association between pars planitis and multiple sclerosis (MS). The retrospective study included 44 patients with pars planitis, who had been examined between October 1986 and January 1999. We analyzed age, sex, visual acuity (VA), median follow-up time, and medical and surgical treatments. The prospective study, which included 21 consecutive patients with pars planitis, was performed to determine the presence of MS. In the retrospective study, the mean patient age was 22.4 years (SD +/- 11.5) and the median follow-up was 34.9 months (SD +/- 27.2). Complications included macular edema (47.7%), vitreous opacities (38.6%), papillitis (38.6%), vasculitis (36.4%), and cataract (20.5%). Forty patients (90.9%) had a final bilateral VA better than 20/40. In the prospective study, magnetic resonance imaging (MRI) was performed. Demyelinating lesions were found in 10 (47.6%) of the 21 patients and relapsing-remitting clinically definite MS was diagnosed in seven (33.3%). With the exception of age, no significantly statistical differences were observed when the visual prognosis and the clinical and epidemiologic characteristics were compared between the two groups of patients with and without associated MS; a diagnosis of MS was more frequently made in patients over 25 years of age. With appropriate treatment, patients with pars planitis have a good visual prognosis. Because the presence of demyelinating lesions seems to be high among patients with pars planitis, MRI should be considered, especially in patients over 25 years of age.

Adolescent↗

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.

Adolescent↗

The appearance of pars planitis in multiple sclerosis.

The aim of the study was to investigate the clinical association of multiple sclerosis and pars planitis (or intermediate uveitis), as well as to determine the incidence of pars planitis in multiple sclerosis patients. During the period of one year authors examined 42 patients with multiple sclerosis divided into two groups. First group consisted of 23 patients with history of optic neuritis and the second group consisted of 19 patients who have never had optic neuritis. The mean age of patients in the first group was 31.7 +/- 5.1 years and in the second group 29.1 +/- 8.1 years. Pars planitis was found in 12 patients with multiple sclerosis. Age, sex and degree of neurological disability had no influence on the appearance of pars planitis. Although optic neuritis is considered to be the most common ocular manifestation of multiple sclerosis, the significant number of patients with multiple sclerosis has pars planitis.

Adult↗

Ultrasound biomicroscopy in pars planitis.

PURPOSE: To report the diagnosis of pars planitis on the basis of ultrasound biomicroscopy images. METHODS: The Humphrey ultrasound biomicroscope was used for examination of the right eye of a 17-year-old boy, referred to our clinic because of blurred vision. The lens showed a posterior subcapsular cataract precluding fundus visualization. RESULTS: Ultrasound biomicroscopy showed a homogeneous mass of medium reflectivity over the pars plana. Lensectomy and vitrectomy confirmed the diagnosis. CONCLUSION: This brief case report points out the utility of ultrasound biomicroscopy in diagnosing pars planitis in cases of media opacities.

Adolescent↗

Pars planitis: clinical features and class II HLA associations.

OBJECTIVE: To describe a cohort of patients with pars planitis followed at a single tertiary care institution, determine the frequency of multiple sclerosis and/or optic neuritis in patients with this disorder, and calculate gene frequencies of human leukocyte antigen (HLA) class II alleles in these patients. DESIGN: Fifty-three patients with the diagnosis of pars planitis underwent clinical record review or telephone interview for follow-up or both; 32 of these underwent phlebotomy for analysis of HLA class II alleles. MAIN OUTCOME MEASURES: Outcomes included visual acuity, occurrence of multiple sclerosis and/or optic neuritis, and HLA class II gene frequencies. RESULTS: With a mean follow-up of 2 years, approximately 90% of patients maintained a visual acuity better than 20/40 in at least one eye. The most frequently encountered ophthalmic complications included cystoid macular edema, cataract, and epiretinal membrane formation. Of 37 patients with pars planitis who had medical or neurologic follow-up evaluations, 6 (16.2%) developed multiple sclerosis. The HLA-DR15 allele, coding for one of the two HLA-DR2 subtypes, was associated with pars planitis (odds ratio = 2.86, 95% confidence interval = 1.42-5.78, P = 0.004). CONCLUSIONS: A common immunogenetic predisposition to multiple sclerosis and pars planitis may be associated with the HLA-DR15 allele. This association may represent genetic linkage to the HLA-DR locus or a role for the HLA-DR15 gene product in the pathogenesis of both of these diseases.

Adolescent↗

Cataract extraction in patients with pars planitis.

BACKGROUND: The authors analyzed the results of cataract surgery performed on patients with pars planitis from January 1985 through August 1992. METHODS: One hundred twenty-six patients with pars planitis were evaluated and treated during this period. Cataracts that warranted surgery developed in 12 patients (18 eyes) from this tertiary referral population. These 12 patients were evaluated with respect to pars planitis duration, systemic disease association, treatment regimens, macular and disc pathology, and final visual result. RESULTS: The average final visual acuity of these 18 eyes was 20/38, and 83% of the patients achieved a final visual acuity better than or equal to 20/40. The factors that limited visual recovery to this level were primarily macular and optic nerve pathology (cystoid macular edema [CME], macular epiretinal membrane, and optic atrophy). Control of inflammation required regional steroids in all patients, systemic steroids in ten patients, and immunosuppression in four patients. Posterior chamber lens implantation accompanied the surgery in 14 eyes (10 patients). Recurrent episodes of inflammation in two patients (3 eyes) resulted in accumulation of deposits on the posterior chamber intraocular lens (IOL) surface. Deposits were removed by a YAG laser lens "polishing" session. CONCLUSION: Absolute control of inflammation in patients with pars planitis through a stepladder approach may reduce the incidence of cataract development, and can certainly improve visual rehabilitation after cataract extraction. Implantation of a posterior chamber lens can be well tolerated in selected cases.

Adult↗

Identification, quantitation, and purification of a 36 kDa circulating protein associated with active pars planitis.

PURPOSE: To establish a correlation between the presence of a 36 kDa protein in the blood of patients with pars planitis and to characterize and purify this protein. METHODS: Blood samples were obtained from patients with pars planitis and other types of uveitis and from various controls. Samples were treated with polyethelene glycol and protein A and were analyzed on 10% SDS-PAGE for the presence of a 36 kDa protein. Quantitative estimation of the level of this protein was determined by densitometric tracing of the stained gels. Polyclonal antibodies were raised by immunizing New Zealand White rabbits with a mixture of the gel fragment containing the 36 kDa protein (p-36) and complete Freund's adjuvant. These antibodies were used in the immunoaffinity purification of this protein. RESULTS: The levels of p-36 were sixfold to eightfold higher in 81% of the patients with active pars planitis than in controls (P < 0.05). Furthermore, the levels of this protein correlated with disease activity. A partial amino terminal sequence analysis revealed that p-36 may be a novel protein. It has been purified from the patient's blood using affinity chromatography. CONCLUSIONS: A 36 kDa protein (p-36) is found in elevated concentrations in the blood of many patients with active pars planitis. Its putative role in the etiopathogenesis of pars planitis is unknown.

Amino Acid Sequence↗

Results of peripheral laser photocoagulation in pars planitis.

PURPOSE: To determine the effect of peripheral retinal laser photocoagulation (PLP) on visual acuity, intraocular inflammation, and other ocular findings, including retinal neovascularization in eyes with pars planitis. METHODS: A retrospective chart review of eyes with pars planitis that had undergone PLP. RESULTS: Twenty-two eyes in 17 patients with pars planitis had undergone treatment with PLP at 2 centers. The mean age at the time of treatment was 19.3 years. Following treatment, mean follow-up was 16.3 months (range, 6 to 37 months). Mean visual acuity was 20/60 preoperatively and 20/50 postoperatively. This level of improvement was not statistically significant (P > .10), but there was a statistically significant decrease in the use of corticosteroids between the preoperative examination and the last postoperative examination (86% versus 27%, P < .05). There was also a statistically significant decrease in vitritis at the last follow-up (P = .0008) and a decrease in neovascularization of the vitreous base (P = .03) and in clinically apparent cystoid macular edema (P = .02). Epiretinal membranes were noted in 23% of eyes preoperatively and in 45% of eyes postoperatively. Only one of these epiretinal membranes was considered to be visually significant. One eye developed a tonic dilated pupil, which slowly improved. CONCLUSIONS: Although the long-term natural history of clinical findings in pars planitis is not well documented, PLP appears to decrease the need for corticosteroids while stabilizing visual acuity. It also appears to decrease vitreous inflammation. PLP has few complications and should be considered in patients with pars planitis who are unresponsive or have adverse reactions to corticosteroids.

Adolescent↗

Grading of pars planitis by ultrasound biomicroscopy--echographic and clinical study.

OBJECTIVE: To determine the value of high frequency ultrasound biomicroscopy (UBM) in the assessment of pars planitis, and in particular to correlate UBM findings and ophthalmoscopy findings. METHODS: All patients with pars planitis were identified from the uveitis database of the Department of Ophthalmology, University of Aberdeen. Fifteen consecutive patients (age 14-52 years) underwent complete ophthalmological examination. UBM was performed at a sound frequency of 50 MHz on 17 eyes of 10 patients to determine the extent of disease. UBM findings were evaluated by two investigators in a blinded fashion and graded from 0 to 3 according to the following grading criteria: 0=no cells, 1=mild cells, 2=marked cells, 3=organization of cells. Opthalmoscopy findings were also graded using the same criteria. UBM and ophthalmoscopy findings were independently graded and compared. RESULTS: We found a good inter-observer correlation for the UBM grading of pars planitis (rho=0.86). There was no significant difference in the grading of pars planitis by indirect ophthalmoscopy as compared to grading by UBM (P>0.05). CONCLUSION: UBM appears to be a valuable and reliable diagnostic technique for the evaluation of patients with pars planitis and may be useful especially in patients with media opacities to diagnose and/or monitor efficacy of treatment.

Adolescent↗