Assessment of macular function, structure and predictive value of pattern electroretinogram parameters for postoperative visual acuity in patients with idiopathic epimacular membrane.

PURPOSE: To evaluate macular function and structure before and after epimacular membrane surgery and to estimate the usefulness of pattern ERG test parameters in predicting the postoperative visual acuity. METHODS: We evaluated 32 eyes of 32 patients (mean age 70.8 ± 6.7 years) before and 12 months after successful 25G pars plana vitrectomy with epimacular membrane removal and internal limiting membrane peeling. Distance best-corrected visual acuity (DBCVA-logMAR), foveal thickness (optical coherence tomography-OCT) and macular function [pattern electroretinogram-PERG (ISCEV standard): amplitudes (A) of P50- and N95-waves, implicit time (IT) of P50-wave] were assessed. To estimate the differences between the mean values of considered characteristics, the t test or Wilcoxon matched pair test was used. Correlation between preoperative data of PERG and preoperative and final DBCVA were investigated using Pearson correlation analysis. A receiver operating characteristic curve was constructed to obtain a cutoff value allowing prediction of visual prognosis. We tried to obtain the P50 and N95 amplitudes cutoff value in prediction of good visual outcome (DBCVA of 0.3 or less). RESULTS: Twelve months after surgery, mean of DBCVA significantly increased in comparison with preoperative value (0.31 ± 0.12 vs. 0.6 ± 0.15; p < 0.001) and 23/32 eyes (72 %) achieved visual improvement of two and more Snellen lines. In OCT test, the significant reduction in foveal thickness mean (313.34 ± 47.01 vs. 509.03 ± 93.88 µm; p < 0.001) was obtained. In PERG test, the significant increase in the mean amplitudes of P50- (AP50) and N95 (AN95)-waves as well as significant decrease in the mean implicit time (IT) of P50-wave were achieved (AP50: 3.41 ± 1.48 vs. 2.38 ± 1.23 µV; p < 0.001; AN95: 5.46 ± 1.72 vs. 3.75 ± 1.48 µV; p < 0.001; IT P50: 55.00 ± 3.60 vs. 56.75 ± 5.78 ms; p < 0.001). Twelve months postoperatively, DBCVA was significantly correlated with preoperative IT P50 (r = 0.39; p = 0.027), AP50 (r = -0.68; p < 0.001) and AN95 (r = -0.73; p < 0.001). CONCLUSION: Removal of idiopathic epimacular membranes with internal limiting membrane peeling not only provided increase in visual acuity and reduction in foveal thickness but also caused improvement of innermost retinal layer function in macular region. Pattern ERG test might be a valuable tool in predicting the postoperative visual acuity.

PARS PLANA VITRECTOMY IN ADVANCED CASES OF VON HIPPEL-LINDAU EYE DISEASE.

PURPOSE: To investigate spectrum of patients with Von Hippel-Lindau disease (VHL) that required pars plana vitrectomy and evaluate anatomical and functional outcomes of surgery. METHODS: Twenty-three patients who underwent surgery for advanced VHL eye disease were assessed by genetic tests, diagnostic tests for systemic lesions, and clinical eye examination. The vitrectomized eyes were divided into two groups: with or without retinotomy (group R vs. NR). Functional and anatomical outcome was analyzed and compared between the groups. RESULTS: All patients had central nervous system hemangioblastomas and 57% had other systemic tumors. Point germline mutations, large partial deletions, and complete vhl gene deletions were found in 64%, 27%, and 9% of patients, accordingly. Destruction of hemangioblastomas by retinotomy, laser, or cryotherapy and anatomical attachment of the retina were achieved in all eyes. Preoperative mean distance best-corrected visual acuity was logarithm of the minimum angle of resolution 2.66 (20/9,140) in group R and 1.76 (20/1,150) in group NR (P < 0.05). At 6 months postoperatively, distance best-corrected visual acuity improved in 20 eyes (83%). After over 24 months postoperatively, distance best-corrected visual acuity remained better than preoperatively in 36% in the R group and in 70% in the NR group of eyes. During 24 months postoperatively in 17 eyes, new retinal capillary hemangiomas developed. The mean number of new retinal capillary hemangiomas per eye was higher in group R than in group NR (3.14 vs. 0.70; P < 0.01). In group R, number of new retinal capillary hemangioblastoma was higher in retinal segments where retinotomy was performed (n = 29) than in other areas (n = 13) (P < 0.01). CONCLUSION: Advanced VHL eye disease correlates with occurrence of central nervous system and systemic lesions. Spectrum of vhl gene mutation in the patients corresponds to that of the general VHL population. Pars plana vitrectomy in advanced VHL eye disease can improve or preserve visual function, but postoperative progression of ocular VHL disease can be accelerated in cases where retinotomy is performed.

Large deletion causing von Hippel-Lindau disease and hereditary breast cancer syndrome.

Patients with intragenic mutations of the VHL gene have a typical disease presentation. However in cases of large VHL gene deletions which involve other genes in the proximity of the VHL gene a presentation of the disease can be different. To investigate whether large VHL deletions that remove the FANCD2 gene have an effect on the disease phenotype, we studied a family with a 50 kb large deletion encompassing these two genes. Four patients in this family were affected by VHL-related lesions. However one carrier of the deletion also had bilateral ductal breast cancer at age 46 and 49. Both tumors were of ~2 cm in diameter. On one side lymph nodes were affected. One tumor was ER- and PR-negative (HER2 s unknown) and the second was ER- and PR-positive, and HER2-negative. Our study suggests that a deletion of FANCD2 gene, an important gene in the DNA repair pathway, may be associated with an increased risk of breast cancer, but further studies are needed in this regard.

[Terson syndrome--a literature review]. / Zespól Tersona - przeglad pismiennictwa.

Tersone syndrome was first described in 1900 as haemorrhages in the eye that occur as a consequence of intracranial hemorrhage. The main cause of the syndrome in adults is a subarachnoid hemorrhage following the rupture of intracranial aneurysm. The pathogenesis has not been fully understood. It is believed that the increase of intracranial pressure results in the hemodynamic changes in ocular vessels. Reported symptoms include decrease of visual acuity following generalized symptoms such as headache, loss of consciousness and meningealsigns. Physical examination usually reveals vitreous hemorrhage (classic Terson's syndrome) and other forms of intraocular hemorrhage. The authors reviewed the available literature on the Terson's Syndrome. Clinical presentation, pathogenesis, diagnosis, and available treatment options are presented and discussed, considering the rare occurrence of this disease.

[Combined photodynamic therapy and intravitreal injection of triamcinolone acetonide in patients with wet form of AMD. Introductory report]. / Zastosowanie terapii laczonej PDT z iniekcjami doszklistkowymi octanu triamcinolonu u pacjentów z wysiekowa postacia AMD. Doniesienie wstepne.

PURPOSE: To evaluate the efficacy of combined PDT and 4 mg intravitreal triamcinolone acetonide injection, performed 48-72 hours after PDT, in patients with wet form of AMD. MATERIAL AND METHODS: Nonrandomised, interventional case series, 13 eyes of 13 patients with subfoveal CNV due to AMD that did not respond to PDT monotherapy - 7 females, 6 males - at the age of 65-85 (average age 76.6 +/- 6.7 years); standard PDT was performed in all patients followed by a 4 mg intravitreal injection of triamcinolone acetonide given 48-72 hours after PDT. Follow up visits were scheduled 1 and 7 days after the injection and then every 3 months afterwards and included: BVCA (Snellen chart), IOP measurements, FA, OCT, slit lamp and eye fundus examination. Lesions with active CNV leakage in FA were retreated every 3 months. RESULTS: Average observation time was 10.8 +/- 3.5 months. Baseline visual acuity before PDT monotherapy was applied (Vo) was 0.17 +/- 0.12 (0.06-0.5), and after the therapy decreased to (V1) 0.14 +/- 0.13 (0.05-0.2). After combined PDT and Tc treatment BVCA increased to (V2) 0.21 +/- 0.13 (0.06-0.5), p<0,03. 76,9% of patients gained or maintained visual acuity after combined therapy in the observation time. In 70% of eyes no signs of active CNV was observed in AF and OCT after 1 session of combined PDT and Tc treatment. Only 4 patients required 1 repeated treatment session. CONCLUSIONS: 1. Combination of PDT and IVTA may be effective in patients with wet AMD with no response to PDT alone and significantly reduces the repeated treatment rate. 2. Intravitreal Tc injection performed 48-72 hours after PDT may improve the final functional effects in treated eyes as compared with PDT monotherapy. Our results need further investigation.

Comparison of polypropylene and silicone Ahmed® glaucoma valves in the treatment of neovascular glaucoma: A 2-year follow-up.

BACKGROUND: Inflammation associated with biomaterials of Ahmed® glaucoma drainage devices may cause the formation of a capsule around the device and can thus have a significant influence on the level of intraocular pressure reduction. OBJECTIVES: The objective of this study was to compare the clinical outcomes after the implantation of a polypropylene or silicone Ahmed® glaucoma valve in patients with neovascular glaucoma. MATERIAL AND METHODS: In the study, 27 eyes with neovascular glaucoma (group 1) received silicon Ahmed® valves and 23 eyes (group 2) received polypropylene valves. The best corrected distance visual acuity (BCDVA), intraocular pressure (IOP) and number of anti-glaucomatous drugs were recorded preoperatively and during a follow-up period of 24 months after surgery. Success was defined by the following criteria: 1) intraocular pressure in the rage of 6-21 mm Hg; 2) IOP reduction of at least 30% relative to preoperative values. All complications were registered. RESULTS: One month postoperatively, the mean BCDVA increased significantly in both groups compared to preoperative values (p < 0.001). These values did not change during the 24 months of follow-up examinations. The probability of success defined by criterion 1 at 24 months of observation was 66.7% for silicone and 27.3% for propylene valves group (p < 0.007). According to criterion 2, the difference in success between the groups was not statistically significant. The total number of complications that occurred in both groups during the 24 months of follow-up examinations was similar, except for a higher occurrence of Tenon's cyst formation in the group with a polypropylene valve (18% vs 35%; p < 0.04). CONCLUSIONS: In patients with neovascular glaucoma, the implantation of a silicone valve is associated with a significantly higher probability of long-term reduction of IOP below 21 mm Hg and with a lower risk of valve encapsulation in comparison to polypropylene valves. The obtained results suggest that silicone Ahmed® valves are more effective in the treatment of patients with neovascular glaucoma.

Serum selenium levels are associated with age-related cataract.

OBJECTIVE: The aim of the study is to analyse correlations between age-related cataract (ARC), serum selenium levels and glutathione peroxidase gene 1 and 4 (GPX-1 and GPX-4). MATERIAL AND METHODS: A total sample of 275 participants were enrolled into the study: group A, 94 subjects elligible for ARC surgery, and group B, 181 volunteers without ocular symptoms, gender-, age-, and smoking- status and volume-matched at 1:2 with subjects in group A. All participants (n=275) were divided according to the Lens Opacities Classification System III (LOCS III) into: 1) study group (subjects with clinically significant cataract; N≥3 or C≥3 or P≥2), 2) control group (controls with clinically non-significant cataract; N<3 and C<3 and P<2). The single nucleotide polymorphisms of GPX-1 and GPX-4 were assessed using Real Time PCR. Serum selenium levels were assayed using Inductively Coupled Plasma Mass Spectrometry. RESULTS: Low selenium levels significantly predicted any age-related cataract (OR 7.969; p<.01), nuclear cataract (OR 12.823; p<.01) and cortical cataract (OR 3.31; p<.01). There was no significant effect of gender, age, SNP GPX-1 and SNP GPX-4 on the prevalence of age-related nuclear, cortical and posterior sub-capsular cataract. Serum selenium levels of 75-85 µg/L were associated with the lowest prevalence of ARC. CONCLUSIONS: Due to a confirmed association between serum selenium levels and age-related cataract, low serum selenium levels may constitute a potential risk factor of age-related cataract.

Fine needle aspiration biopsy and molecular analysis in differential diagnosis of lymphoproliferative diseases of the orbit and eye adnexa.

The aim of the investigation was the assessment of the role of routine cytology and clonality evaluation using PCR in differential diagnosis of lymphoproliferative diseases of the orbit and eye adnexa. The investigations were carried out in cellular material collected via fine needle aspiration biopsy (FNAB) from 29 patients aged 31-82 years, including 17 women and 12 men. Apart from routine cytology, molecular-genetic studies were performed employing the PCR technique. In 21 cases histopathology was performed. In 2 patients, despite several attempts, FNAB failed to provide any diagnostic material for routine cytology. Based on cytology, non-Hodgkin's lymphoma was diagnosed in 11 patients and suspected in three. In 13 patients no firm diagnosis was possible based on cytological smears. The employment of PCR allowed for rendering the diagnosis more precise in 13 cases, confirming it in 13 patients, while in 3 cases the results of the above tests did not affect the final diagnosis. Clonality studies by PCR may be performed in material obtained through FNAB. Clonality assessment by PCR technique is very useful in differential diagnosis of lymphoproliferative disordes.

Flow cytometry in the diagnosis of lymphoproliferative lesions of the orbit and eye adnexa in fine needle aspiration biopsy.

The goal of the investigation was to evaluate the validity of routine cytology and flow cytometry in the differential diagnosis of lymphoproliferative disorders of the orbit and eye adnexa. The investigations were carried out on materials originating from fine needle aspiration biopsy performed in 14 patients, including 9 females and 5 males aged 31-81 years. Apart from routine cytology, cytometric studies were also performed. Based on cytology, non-Hodgkin's lymphomas were diagnosed in six patients, while one was suspected of NHL. In seven patients the diagnosis was ambivalent, since based on cytology it was impossible to conclusively determine the biological character of the lesion, i.e. state whether it was benign or malignant. Flow cytometry was performed in 14 patients, but ultimately the results were available in 12 individuals, since in two cases the material was scant enough to exclude any assessment. Thanks to using a panel of monoclonal antibodies against light chains kappa and lambda, as well as against CD surface antigens, the authors demonstrated clonality in 90% (9/10) of NHL cases; of this number, in 7 instances the test confirmed the preliminary diagnosis and in 2 cases rendered the diagnosis more precise. On the other hand, in 3 cases no clonality was noted; of this number, in two instances the diagnosis was specified as a benign lesion (BLPL) and in one case the assessment of clonality had no impact on the final diagnosis. Out of 12 investigated aspirates, in 11 cases the result concerning clonality affected the final diagnosis. The evaluation of cellular phenotype in flow cytometry in materials obtained in the course of FNAB is a fast and sensitive method and in many cases allows for avoiding a surgical biopsy.

A low selenium level is associated with lung and laryngeal cancers.

PURPOSE: It has been suggested that selenium deficiency is a risk factor for several cancer types. We conducted a case-control study in Szczecin, a region of northwestern Poland, on 95 cases of lung cancer, 113 cases of laryngeal cancer and corresponding healthy controls. METHODS: We measured the serum level of selenium and established genotypes for four variants in four selenoprotein genes (GPX1, GPX4, TXNRD2 and SEP15). Selenium levels in the cases were measured after diagnosis but before treatment. We calculated the odds of being diagnosed with lung or laryngeal cancer, conditional on selenium level and genotype. RESULTS: Among lung cancer cases, the mean selenium level was 63.2 µg/l, compared to a mean level of 74.6 µg/l for their matched controls (p<0.0001). Among laryngeal cancer cases, the mean selenium level was 64.8 µg/l, compared to a mean level of 77.1 µg/l for their matched controls (p<0.0001). Compared to a serum selenium value below 60 µg/l, a selenium level above 80 µg/l was associated with an odds ratio of 0.10 (95% CI 0.03 to 0.34; p = 0.0002) for lung cancer and 0.23 (95% CI 0. 09 to 0.56; p = 0.001) for laryngeal cancer. In analysis of four selenoprotein genes we found a modest evidence of association of genetic variant in GPX1 with the risk of lung and laryngeal cancers. CONCLUSION: A selenium level below 60 µg/l is associated with a high risk of both lung and laryngeal cancer.

Retinal function in the von Hippel-Lindau disease.

PURPOSE: To assess the retinal function in patients with von Hippel-Lindau disease (VHL). PATIENTS: Studies were undertaken in 12 patients (17 eyes) with detected VHL gene mutation and 12 normal healthy controls (17 eyes). METHODS: Pattern ERG (PERG), standard flash electroretinogram (ERG) recordings were performed in accordance with the International Society for Clinical Electrophysiology of Vision (ISCEV) standards. RESULTS: In VHL patients, electrophysiological statistically significant changes were found. In PERG examination, increased latency of P50 was found in the total VHL group (p < 0.02) and in the VHL subgroup with retinal angiomas (p < 0.04). In ERG examination, photopic b-wave latency was increased in the total VHL group (p < 0.03) and also in the VHL subgroup without retinal angiomas (p < 0.05). In OPs, latency increase of OP2, OP3 waves and reduced amplitude of OP3 wave in the total VHL group (OP2 latency, p < 0.05; OP3 latency, p < 0.01; OP3 amplitude, p < 0.03) and in the VHL subgroup with retinal angiomas (OP2 latency, p < 0.02; OP3 latency, p < .008; OP3 amplitude, p < 0.02) were obtained. CONCLUSIONS: It can be hypothesized that dysfunction of the inner retinal layer is present in individuals with VHL disease even in patients without retinal angiomas.