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Pars planitis in a family.

The familial occurrence of pars planitis is rare. We have found ten cases reported previously. We describe a new case of pars planitis in a family. The affected members included a mother and two of her four children. The family was tested for HLA antigens in order to establish a comparison with others HLA types by different authors. We have not identified any cause for the familial occurrence of this disease. We discuss the role of genetic and ambiental factors.

Child↗

Phacoemulsification with intraocular lens implantation in cases of pars planitis.

PURPOSE: To analyze the results of phacoemulsification cataract surgery with implantation of posterior chamber intraocular lenses (IOLs) of different biomaterials in eyes with pars planitis. SETTING: Medical and Vision Research Foundations, Sankara Nethralaya, Chennai, India. METHODS: The records of 86 patients (100 eyes) with pars planitis who had phacoemulsification with IOL implantation between January 1997 and April 2003 were retrospectively analyzed. The eyes were divided into 3 groups depending on IOL biomaterial: poly(methyl methacrylate) (PMMA) (n=32), heparin-surface-modified PMMA (n=39), or acrylic (n=29). The postoperative visual outcome and complications in each group were analyzed. RESULTS: Forty-seven patients were men and 39, women. The mean age was 38 years (range 10 to 65 years) and the mean follow-up, 19.67 months (range 3.00 to 54.53 months). At the final follow-up, 91 eyes (91%) had better visual acuity than preoperatively; 79 had an improvement of 2 or more Snellen lines, 12 had an improvement of 1 Snellen line, 4 had no change, and 5 had a decrease as a result of reactivation of the pars planitis and progression of cystoid macular edema (CME). Significant posterior capsule opacification occurred in 10 eyes (10%), CME in 50 eyes (50%), reactivation of pars planitis in 51 eyes (51%), IOL deposits in 29 eyes (29%), IOL decentration in 1 eye (1%), and anterior capsule fibrosis in 14 eyes (14%). The most frequent cause of poor visual recovery was CME, submacular fibrosis, and epiretinal membrane. There was no statistically significant difference in these complications between the 3 groups. CONCLUSIONS: Phacoemulsification with IOL implantation in eyes with pars planitis was safe and led to good visual outcomes in most cases. The factors in surgical success were control of inflammation, meticulous surgery, in-the-bag IOL implantation, and vigilant postoperative care.

Acrylic Resins↗

Genetic polymorphism of HLA DR in a Scottish population of patients with pars planitis.

PURPOSE: Human leucocyte antigen (HLA) class II influences the immunological susceptibility for a variety of diseases including many types of non-infectious intraocular inflammation. Previous studies on North American patients with pars planitis, a subtype of intermediate uveitis, reported an increased prevalence of HLA DR15 in this population. In contrast, two European studies could not find an association between HLA DR2 or its allelic subtype DR15 and various forms of intermediate uveitis. We therefore investigated the genotype frequency of HLA DR alleles in a Scottish population of patients with typical pars planitis. METHODS: Twenty patients with pars planitis were identified from the uveitis database of Grampian University Hospitals. Only patients with bilateral vitritis and snowbanks in at least one eye in the absence of systemic disease were included in the study. Fifteen patients and 34 healthy controls underwent HLA DR genotyping for all DRB genes using PCR sequence specific primers. RESULTS: HLA DR15 was found in 13% of patients with pars planitis and in 24% of controls. There was no statistically significant difference between these two groups. Furthermore, the frequencies of HLA DR 1, 3-14, and 16 did not differ significantly between patients and controls. CONCLUSIONS: There appears to be no association between the occurrence of pars planitis and the HLA DR15 or other known HLA DR genotypes in Scottish patients. However, the small sample size limits the power of this study.

Alleles↗

Cryopexy in pars planitis.

Cryotherapy has been reported to be of benefit in pars planitis. We studied 16 eyes with classic pars planitis unresponsive to corticosteroid therapy. Eight eyes continued with systemic and periocular steroid therapy, and in the remaining eight eyes transconjunctival cryopexy of the peripheral retina and vitreous base was done as an additional procedure. At 6 months four of the eyes that received cryopexy showed an improvement in Snellen visual acuity, and in the other four the acuity was unchanged; none of the eyes showed any vitreous base neovascularization. Among the eyes that received steroid therapy only, the acuity was unchanged in five, improved in one and reduced in two.

Adolescent↗

A fluorescein angiographic study of patients with pars planitis and peripheral exudation (snowbanking) before and after cryopexy.

BACKGROUND: Transconjunctival cryopexy of the vitreous base is clinically effective in patients with steroid nonresponsive pars planitis who have peripheral neovascularization. Cryoablation of the region previously has been thought to eliminate the areas of permeable neovascular tissue, thus removing the focus of exudation. The authors report the first study of patients with refractory pars planitis in which fluorescein angiography of the inferior pars plana snowbanks was performed both before and after cryopexy of the vitreous base. METHODS: Twelve consecutive phakic eyes with pars planitis and prominent peripheral snowbanks had fluorescein angiograms of the pars plana. Clinical involvement was graded on the basis of visual acuity, levels of vitreous inflammation and cystoid macular edema, and on the extent of peripheral snowbanking. Transconjunctival cryopexy of the vitreous base was performed. Fluorescein angiography of the pars plana snowbanks was repeated 2 months after the procedure, and clinical involvement again was graded. RESULTS: Early hyperfluorescence with late leakage was present in all eyes and was limited to the area of the pars plana snowbanks. After cryopexy of the vitreous base, all eyes demonstrated hypofluorescence and diminution of late-phase dye leakage in the treated areas. Eighty-three percent of all eyes demonstrated clinical improvement, consistent with previously reported studies. CONCLUSION: Cryopexy of the vitreous base decreases peripheral exudation in patients with refractory pars planitis and snowbanking. This suggests that elimination of peripheral neovascular tissue may be directly related to clinical improvement in these patients.

Cryosurgery↗

Pars planitis in father and son.

The authors examined a family in which father and son presented with pars planitis. Both of them and the paternal grandparents were HLA typed; no association between HLA antigens and pars planitis was found. Present and previous data suggest a relation between pars planitis and allergic predisposition.

Adrenal Cortex Hormones↗

[Origin of macular edema caused by pars planitis].

In four cases with clinical diagnosis of "pars planitis" fluorescein angiography was performed and macular area observed. Permeability disturbances were noted in retinal capillary area. This points to the vitreous changes being secondary to an inflammation of superficial retinal layers. For this reason in those conditions the name "hyalo-retinitis" used by Gärtner seems to be more correct than the limiting diagnosis of "pars planitis".

Adult↗

[Diathermy and cryotherapy of the pars plana in the treatment of pars planitis. Long-term evaluation].

Seventeen eyes of 14 patients affected with severe pars planitis underwent surgical treatment with either cryotherapy - 3 eyes - or diathermy - 14 eyes. The indications for surgery were selected as follows: ten eyes of 9 patients showed recurrence(s) or increase of ocular inflammation following tapering of systemic corticoids; 5 patients had contra-indications to systemic corticoids. Ten eyes had cystoid macular edema. The follow-up after surgery varies from 2,5 years to 10 years with an average of 5 years. Complete and permanent recovery from ocular inflammation was achieved in 7 eyes (41,2%). Permanent although incomplete improvement was achieved in 4 eyes (23,5%). Recurrences of ocular inflammation after incomplete improvement occurred in 4 eyes (23,5%). Complications - cystoid macular degeneration and severe ocular hypotension - occurred in 2 eyes (11,8%). Cystoid macular edema cleared in 9 out of 10 eyes. The final visual acuity was the same or better as compared to the initial visual acuity in 10 eyes, and worse in 7 eyes. It is concluded that the long term evaluation of cryotherapy and diathermy in the treatment of pars planitis confirms the results achieved in other series with a shorter follow-up. The treatment is beneficial in selected cases of severe pars planitis. However complete and permanent recovery from ocular inflammation should be expected in less than 50% of the cases.

Adolescent↗

Blood serum interleukin-1 receptor antagonist in pars planitis and ocular Behçet disease.

PURPOSE: To determine the levels of interleukin-1 receptor antagonist (IL-1ra) in the blood serum of patients with idiopathic bilateral pars planitis and Behçet disease. METHODS: Five milliliters of blood was with-drawn from the cubital vein of 91 patients (58 with the ocular type and five with the combined type of Behçet disease; 28 with pars planitis) and 36 volunteers. Serum was separated from these samples and stored at -70 C until assayed. Interleukin-1 receptor antagonist levels were determined by human IL-1ra enzyme-linked immunosorbent assay kits. In patients not receiving any systemic medication, one serum sample was obtained before initiating treatment and another when the patients had been under full medical treatment for 6 weeks or more. RESULTS: Pretreatment mean +/- SD serum IL-1ra levels were 320 +/- 32 pg/ml for the patients with pars planitis, 380 +/- 54 pg/ml for patients with Behçet disease, and 271 +/- 29 pg/ml for the control subjects (no statistical significance). During treatment, a mean serum level of 352 +/- 37 pg/ml was observed in patients with pars planitis (not significantly different from control subjects) and 538 +/- 79 pg/ml in patients with ocular Behçet disease (P = .0116 compared with control subjects). The greatest increase in IL-1ra levels was observed in patients with Behçet disease who received a combination of cyclosporine and corticosteroids. CONCLUSIONS: Because IL-1ra is one of the natural immunomodulating molecules, the significant increase of serum IL-1ra levels, especially after combined treatment with cyclosporine and corticosteroids, could indicate that the therapeutic effects of this regimen may be mediated through its effects on this molecule.

Behcet Syndrome↗

Pars planitis: a comparison of childhood onset and adult onset disease.

PURPOSE: To compare the symptoms, visual acuities and complications found in childhood onset pars planitis, with those seen in adult onset disease. METHOD: The records of 26 patients (52 eyes) with idiopathic pars planitis seen at a tertiary care clinic were reviewed. The study design allowed comparison of follow-up visual acuities for the adult onset and childhood onset groups at 2 years and 5 years from the time of initial diagnosis. RESULTS: The visual acuity at the time of initial diagnosis, at 2 years' follow up and at 5 years' follow up was worse for children than for adults, but this difference only reached statistical significance at 2 years' follow up (6/10 vs 6/7; P = 0.026). When looking at correlation coefficients between age and acuity, the same trend was observed at all time intervals, only reaching statistical significance with initial visual acuities (r = 0.32; P = 0.019). Adults had a tendency to complain more at presentation of blurred vision and floaters than children. Complication rates were similar for both groups. CONCLUSION: The study presents data supporting the idea that patients presenting with idiopathic pars planitis in childhood have a worse visual acuity both at initial diagnosis and at follow up than those presenting in adulthood. This is a long-suspected finding that has not previously been demonstrated.

Adolescent↗

Molecular cloning, sequencing, and expression of the 36 kDa protein present in pars planitis. Sequence homology with yeast nucleopore complex protein.

PURPOSE: Patients with active pars planitis have increased levels of a 36 kDa protein (p-36) in their circulation. The current studies were undertaken to determine the primary structure of this protein. METHODS: A degenerate oligonucleotide probe based on the amino terminal sequence of p-36 was used to identify a clone from a human spleen cDNA library. The cDNA insert was subcloned into the EcoR1 site of pUC-19, and both strands were sequenced. Southern blot analysis was used to study the genomic hybridization pattern. p-36 cDNA was subcloned in a pSG5 expression vector, and the construct was used to transfect COS-7 cells. RESULTS: The cDNA sequence contained an open reading frame of 966 base pairs encoding a protein of 322 amino acids, an untranslated region of 322 base pairs, and 2693 base pairs at the 5' and 3' ends, respectively. The deduced amino acid sequence showed 96.8% identity with the carboxy-terminal region of a yeast nucleopore complex protein, nup 100. Southern blot analysis of human genomic DNA revealed a simple hybridization pattern. Transfection of p-36 cDNA in COS-7 cells resulted in the presence of p-36 mRNA and expression of protein. CONCLUSIONS: The 36 kDa protein (p-36) detected at increased levels in the blood of patients with active pars planitis was cloned from a human spleen cDNA library. Its deduced amino acid sequence is homologous with the carboxy-terminal region of a nucleopore complex protein. Thus, we refer to this protein as nup36.

Amino Acid Sequence↗

Pars planitis presenting with vitreous hemorrhage.

A 31-year-old woman complained of a sudden, painless decrease in vision of the right eye. Ophthalmic examination demonstrated a vitreous hemorrhage in the right eye secondary to peripheral neovascularization. The left eye demonstrated findings consistent with pars planitis. Before this episode, vision was good in both eyes. Although rare, vitreous hemorrhage can occur in the setting of pars planitis and may be a presenting symptom. Pars planitis should be considered in the differential diagnosis of young patients with vitreous hemorrhage.

Adult↗

Pathology of pars planitis.

Seven eyes and one vitrectomy specimen from seven patients with pars planitis and complications such as secondary glaucoma and phthisis bulbi were studied histopathologically. Two of the specimens were also examined by electron microscopy. All cases showed a typical intravitreal "snowbank" opacity overlying the pars plana. On light microscopy, these "snowbanks" appeared to consist of a loose fibrovascular layer containing occasional fibrocyte-like cells and scattered mononuclear inflammatory cells adjacent to the hyperplastic nonpigmented epithelium of the pars plana. Within the vitreous base, an extensive fibroglial proliferation had developed, often drawing the peripheral retina anteriorly into the "snowbank." Electron microscopy showed this fibroglial tissue to be composed of condensed vitreous collagen and probable fibrous astrocytes which had produced larger-diameter (about 24 mm) collagen fibrils. The fibroglial proliferation also appeared continuous with an ultrastructurally similar preretinal fibroglial membrane. All eyes showed prominent lymphocytic cuffing and mural infiltration of retinal veins, with sparing of the arterioles. Several cases showed cystoid macular edema. Only mild choroiditis or cyclitis could be shown in some cases. We believe that in pars planitis the fibroglial "snowbank" may reflect a common inflammatory process involving both the peripheral retina and vitreous base.

Adolescent↗

Pars planitis.

We have studied seven eyes from six patients with longstanding pars planitis. Two eyes were phthisical. The remaining five eyes from four patients showed similar findings and included retinal phlebitis, disc and macular oedema, and the presence of a 'snowbank' over the inferior peripheral retina and pars plana. The snowbank consisted of posteriorly detached and collapsed vitreous in which cellular proliferation from the retina with non-pigmented ciliary epithelium was present. Electron microscopy showed the presence of fibrous astrocytes and collagen in two eyes. Retinal neovascularization extended into the snowbank in one case. We have also studied vitreous and preretinal membranes and a portion of a snowbank obtained by pars plana vitrectomy from two eyes of two young patients with active pars planitis. The snowbank and preretinal membranes contained fibrous astrocytes and new collagen and the vitreous snowballs consisted of granulomatous inflammation.

Adolescent↗

Neovascularization of the disc in pars planitis.

Neovascularization of the disc (NVD) was present in 9 of 163 patients with pars planitis. In all cases, NVD was unilateral and observed in eyes with recent exacerbation of the inflammatory process. To reduce intraocular inflammation, all nine eyes were treated with varying combinations of topical, periocular, and systemic corticosteroids. In addition to corticosteroids, one eye received Argon laser photocoagulation, two eyes underwent peripheral cryotherapy, and one eye was treated with both Argon laser photocoagulation and peripheral cryotherapy. With decrease or disappearance of intraocular inflammation, NVD resolved in all cases without recurrence during follow-up study, which ranged from 6 to 189 (mean, 81) months. Rhegmatogenous retinal detachments developed in two eyes treated with peripheral cryotherapy. Both detachments were successfully repaired with surgery. Control of intraocular inflammation appears to be the key factor for regression of NVD in pars planitis. If NVD does not regress or vitreous hemorrhage occur, photocoagulation and peripheral cryotherapy may be beneficial.

Adolescent↗

Fibroglial proliferation in pars planitis.

In a 51-year-old patient with long-standing pars planitis, light and electron microscopical examination of the intravitreal 'snowbank' revealed a fibrovascular layer adjacent to the hyperplastic nonpigmented epithelium of the pars plana and an extensive fibroglial proliferation within the vitreous base. The fibrovascular layer consisted of well-differentiated capillaries, probably emanating from the peripheral retina, interspersed with aggregated vitreous fibrils. The fibroglial portion of the 'snowbank' was composed of fibrous astrocyte-like cells which had secreted basement membranes and larger diameter collagen fibrils. This fibroglial tissue was in direct continuity with an ultrastructurally similar preretinal membrane. No significant choroiditis or cyclitis could be demonstrated. It is hypothesized that, in pars planitis, a primary inflammatory process of the peripheral retina and vitreous base may stimulate the observed preretinal and intravitreal fibroglial proliferation.

Ciliary Body↗

Pars planitis.

The records on thirty-three patients (58 eyes) with the diagnosis of pars planitis were reviewed. The patients were divided into 4 groups: Group 1 had bilateral vitreous cells and pars plana 'snowbank' formation (13 patients, 26 eyes), Group 2 had bilateral vitreous cells and 'snowbank' formation in one eye only (8 patients, 16 eyes), Group 3 had vitreous cells and 'snowbank' formation in one eye only (8 patients, 8 eyes), and Group 4 had bilateral vitreous cells without 'snowbank' formation (4 patients, 8 eyes). Frequent findings and complications were tabulated; cystoid macular oedema was the most common cause of decreased vision. Although pars plana 'snowbank' exudates are usually present in pars planitis, they may not be necessary to make the diagnosis. An approach to therapy is outlined.

Adolescent↗

2 peripheral scatter photocoagulation for neovascularization associated with pars planitis.

BACKGROUND: Peripheral cryotherapy appears to be efficacious in the treatment of neovascularization of the vitreous base in patients with pars planitis, although it may be associated with the development of rhegmatogenous retinal detachments. OBJECTIVE: To evaluate the safety and efficacy of peripheral scatter photocoagulation for treatment of neovascularization of the vitreous base when used alone or combined with pars plana vitrectomy. METHODS: Six patients (10 eyes) presented with vitritis, cystoid macular edema, and neovascularization of the vitreous base, unresponsive to corticosteroid therapy. Three patients (five eyes) received scatter diode or argon photocoagulation treatment alone. The other three patients (five eyes) underwent pars plana vitrectomy coupled with argon or diode photocoagulation, placed in three rows, posterior to the area of inferior neovascularization of the vitreous base. RESULTS: Pretreatment visual acuity ranged from 20/20 to 20/200. All patients were followed up for a minimum of 6 months. After placement of photocoagulation (with or without concurrent pars plana vitrectomy), the neovascularization regressed, inflammation was stabilized, and cystoid macular edema improved in all eyes. There were no retinal detachments or other complications of treatment. Posttreatment visual acuity ranged from 20/20 to 20/100. When final visual acuity was 20/40 or less, cataract formation was generally responsible. CONCLUSIONS: Peripheral scatter photocoagulation is efficacious and appears at least equal to peripheral cryotherapy in causing regression of neovascularization of the vitreous base in patients with pars planitis.

Adult↗