Ocular outcomes in 4-year old prematurely born children.

Purpose: The aim of the study was to assess functional and structural ophthalmologic outcomes in 4-year old very low birth weight children (VLBW). Material and Methods: A group of 82 VLBW children including: (1) children without retinopathy of prematurity ­ group O (n = 30), (2) children with retinopathy of prematurity with no indications for laser coagulation- group 1 (n = 20 ), (3) children with retinopathy of prematurity treated with laser coagulation ­ group 2 (n = 32) were enrolled. Functional (visual acuity, visual evoked potentials, stereopsis, color vision test) and structural (anterior eye segment examination and fundoscopy) outcome, cycloplegic refraction, intraocular pressure and angle of squint were examined in all children. Developmental Test of Visual Perception was also assessed. Results: Very good visual acuity was presented in 56 (68.3%) patients, good visual acuity in 11 (13.4%) children, visual acuity between 0.4 and 0.2 in 13 (15.9%) and unfavorable function (equal or less than 0.1) was observed in 2 (2.4%) children. Twenty-three patients (28.1%) were myopic, 57 patients (69.5%) were hyperopic. Astigmatism > 1D occurred in 49 (59.8%) patients. Anisometropia larger than 2 D occurred in 7 patients (8.7%). There was no statistical difference between frequency of mentioned above complications between the groups. Abnormal VEP results were more common (14 children -54%) in group 2 as compared to other groups (group 0 - 7 children -25%, group 1 ­ 4 children -24%; p = 0.044). Moreover, the lower percentage of group 2 children presented stereopsis vision (46.9%, group 0 ­ 93.3%, group 1 ­ 90%; p<0.05). Children with retinopathy of prematurity treated with laser coagulation had significantly lower Developmental Test of Visual Perceptions scores. In group 1, in 1 child naevus pigmentosus of the eyelid was observed, in group 2 in one child eyeball atrophy in the right eye and aphakia in the left eye were diagnosed. In 1 child in group 0 optic disc drusen were observed, in 2 children optic discs were pale. In 2 children in group 2 retinal detachment stage 5 was diagnosed. Conclusion: An important factor affecting final ophthalomolgical assessment in prematurely born children is retinopathy of prematurity treated with laser coagulaion. Children with retinopathy of prematurity without laser coagulation had examinations results comparable wih children without retinopathy of prematurity. Diode laser photocoagulation is an effective method of treatment fot active stages of retinopathy of prematurity.

Association between Y402H, E318D and R102G polymorphisms of complement proteins genes and the response to intravitreal anti-VEGF treatment in patients with neovascular age-related macular degeneration. / Wplyw polimorfizmów Y402H, E318D oraz R102G genów kodujacych bialka ukladu dopelniacza na wyniki leczenia doszklistkowymi iniekcjami czynnika anty-VEGF u chorych na wysiekowa postac zwyrodnienia plamki zwiazanego z wiekiem.

Aim: To analyze the correlation between the E318D rs9332739 polymorphism of the C2 complement factor; R102G rs2230199 polymorphism of the C3 complement factor as well as the Y402H rs1061170 polymorphism of the CFH complement factor and risk of AMD as well as the response to anti-VEGF therapy. Material and methods: 106 patients with age-related macular degeneration treated with intravitreal ranibizumab or bevacizumab were enrolled. The response to treatment was assessed at 4 weeks intervals for 6 months and was based on the results of best corrected visual acuity and central retinal thickness measurements compared to the respective baseline values. The control group consisted of 58 healthy volunteers. The testing was performed using genetic probes (TaqMan Applied Biosystems) in all cases Results: E318D (C2) and R102G (C3) polymorphisms were not associated with age-related macular degeneration. The genotype CC of Y402H (CFH) polymorphism was more frequent in patients with age-related macular degeneration as compared to controls [OR=3.09 (1.28­7.49); p=0.0069]. At the last follow-up, patients with age-related macular degeneration positive for the CC rs1061170 CFH genotype presented with worse best corrected visual acuity and increased central retinal thickness as compared to their counterparts negative for this genotype [OR=7.67 (1.77­33.12), p=0.0052]. Among 25.47% of "non-responders", the CC rs1061170 CFH genotype was present in 51.8% of cases. In patients with the TT rs1061170 CFH genotype the final best corrected visual acuity was better and a significant reduction of central retinal thickness was demonstrated in all those cases, as compared to subjects with the CC rs1061170 CFH genotype [OR=0.31 (0.11-0.84), p=0.0194]. Conclusions: The study showed that the CC rs1061170 CFH genotype may be associated with the age-related macular degeneration. Additionally, the CC rs1061170 CFH genotype may promote a negative response to anti-VEGF treatment, while patients with the TT rs1061170 CFH genotype showed better functional and structural response to anti-VEGF agents.

[Retinal vascular tumors in the material of the Department of Ophthalmology and Ocular Oncology of Jagiellonian University Collegium Medicum in Cracow]. / Guzy naczyniowe siatkówki w materiale Kliniki Okulistyki i Onkologii Okulistycznej Uniwersytetu Jagiellonskiego Collegium Medicum w Krakowie.

Retinal vascular tumours are congenital and acquired lesions of variable clinical manifestation. They are classified as benign, but their presence leads to vision impairment due to the development of complications. Each retinal vascular tumour possesses characteristic clinical features and is often associated with certain systemic disorders. Thus, a correct diagnosis is crucial for providing individualised treatment and expanded diagnostic management as well as prognostic assessment. The aim of the study was to present retinal vascular tumour cases of patients treated at the Department of Ophtalmology and Ocular Oncology of Jagiellonian University Collegium Medicum in Cracow.

[Utility of optical coherence tomography in assessing macular anatomy after conventional surgical repair of rhegmatogenous retinal detachment]. / Przydatnosc optycznej koherentnej tomografii do anatomicznej oceny plamki po konwencjonalnych zabiegach przedarciowego odwarstwienia siatkówki.

AIM: To determine the utility of optical coherence tomography in assessing macular anatomy of patients treated with conventional surgery for rhegmatogenous retinal detachment involving the macula. MATERIAL AND METHODS: 42 patients (42 eyes) treated for rhegmatogenous retinal detachment with macular involvement were enrolled in the study. 14 of them were treated with segmental scleral buckling, 8 patients--with encircling scleral buckling, and 20 others--with scleral explant placed along the horizontal or vertical meridian and encircling scleral band. The assessment in each case involved the assessment of best corrected visual acuity, Amsler grid test and optical coherence tomography of the macular region performed on day 1. following surgery as well as in months 1, 3 and 6 afterwards. RESULTS: On day 1. after the surgery, residual retinal detachment with macular involvement was confirmed in all patients with optical coherence tomography and no best corrected visual acuity improvement was noted. The central retinal elevation ranged from 315 microm to 480 microm (mean 387 microm). Subsequent follow-up assessments performed at months 1, 3 and 6 showed a gradual reabsorption of subretinal fluid and the reduction of central retinal elevation to 371 pm, 286 microm and 219 microm, respectively. At the final follow-up, the submacular fluid was completely resolved in 88.1% of eyes. The statistical analysis showed a significant correlation between the reduction of central retinal elevation and best corrected visual acuity improvement (p<0.01). CONCLUSIONS: Our results show that the optical coherence tomography may be a useful tool in assessing the residual retinal detachment responsible for the lack of visual acuity improvement after the uneventful conventional retinal detachment surgery.

[Familial retinoblastoma in the experience of the Department of Ophthalmology and Ocular Oncology in Kraków]. / Siatkówczak rodzinny w materiale Kliniki Okulistyki i Onkologii Okulistycznej Uniwersytetu Jagiellonskiego Collegium Medicum w Krakowie.

In this retrospective study we analyzed over 300 patients with diagnosed retinoblastoma treated in the Department of Ophthalmology and Ocular Oncology in Kraków in 1967-2011. Nine families (parents and offspring) with diagnosed familial retinoblastoma present in at least two generations were analyzed. A review of the age of onset, diagnosis and recurrence rate of tumors as well as the long-term results of applied therapy and advantages of prophylactic ophthalmic screening in children at high risk of familial retinoblastoma was performed. The results of our observations showed that in offspring the tumors were diagnosed earlier, and the therapy outcomes were better as compared to the group of Parents. We conclude that these observations were associated with performed genetic screening, early prophylactic ophthalmic examination of children born in families with diagnosed retinoblastoma and chemoreduction treatment.

Macular microcirculation blood flow after pars plana vitrectomy with silicone oil tamponade.

PURPOSE: To assess the macular microcirculation blood flow with Doppler laser scanning (HRF-Heidelberg Retinal Flowmeter), in patients with complicated rhegmatogenous retinal detachment (RRD), after pars plana vitrectomy (PPV), with silicone oil tamponade. MATERIAL AND METHODS: Eleven patients (6 males and 5 females), aged 38-64 years, mean age: 52.4 years, with unilateral macula-on complicated RRD were included in a study. In all cases conventional (20-gauge) pars plana vitectomy with silicone oil tamponade was performed. Control group consists of the fellow eyes. RESULTS: In the early postoperative period (1-3 days after surgery), in all operated eyes the mean values of macular microcirculation blood flow were significantly reduced in the comparison with those of the control eyes (p = 0.01). The reduction of mean values of macular microcirculation blood flow was still observed in all silicone-oil-filled eyes one month after surgery as compared to the contralateral eyes (p = 0.01). There was no correlation between the values of macular microcirculation blood flow and the duration of RRD, baseline BCVA, refractive error, number of retinal tears, and the patient age. CONCLUSIONS: The preliminary results of this study suggest that PPV with silicone oil tamponade influences over the retinal blood flow. We have observed that silicone oil may have a negative long-term effect on the retinal microcirculation as it was noted one month after surgery.

Ruthenium-106 plaque therapy for diffuse choroidal hemangioma in sturge-weber syndrome.

Diffuse choroidal hemangiomas associated with Sturge-Weber syndrome (SWS) are classically treated with external beam radiotherapy (EBR), but there are a few reports usually of single cases indicating the usefulness of plaque therapy. We present our observations on two cases of diffuse choroidal hemangiomas with exudative retinal detachment associated with SWS treated with Ruthenium-106 plaque therapy. Outcomes included best-corrected visual acuity (BCVA) and regression in tumor thickness measured by ultrasonography. The initial BCVA of the affected eyes was counting fingers at 1 meter and light projection. Pretreatment tumors thickness was 3.5 mm and 4.7 mm. In a follow-up period of 18-24 months, significant reduction in thickness of choroidal hemangiomas up to 1.2 mm and 1.4 mm with prompt resolution of exudative retinal detachment was observed. BCVA achieved 20/200 and 20/400, respectively. The findings in this paper indicate that Ruthenium-106 plaque therapy is effective in treatment of diffuse choroidal hemangiomas associated with SWS.

[Results of combined chemotherapy and local ophtalmic therapy for intraocular retinoblastoma]. / Wyniki chemioterapii skojarzonej z miejscowym leczeniem siatkówczaka wewnatrzgalkowego.

Until recently chemotherapy was used as adjuvant therapy after enucleation in cases with extraretinal spread of the disease (uveal extension, orbital extension, neoplastic infiltrates of the optic nerve at resection line, intracranial metastasis, generalized disease). Recent experience has proved that use of chemotherapy for intraocular retinoblastoma before local treatment (so called "chemoreduction") has allowed not only to decrease number of enucleations and indications for external beam irradiation or limit the extension of local therapy, but also increase chances for vision preservation and decrease the risk of severe complications. Seventy five children (with 106 involved eyes) aged 0.2 - 106 months with intraocular retinoblastoma diagnosed between January 1996 and June 2009 were the subject of this study. Among 106 involved eyes, in 70 (66%) the V stage according to Reese-Ellsworth classification (R-E) was established. Enucleation before chemotherapy was necessary in 9 (8.5%) cases, and in 22 more children the eye had to be removed after 1-2 courses of chemotherapy. In 68 remaining children (with 70 involved eyes) VEC (vincristine, etoposide, carboplatin) chemotherapy combined with delayed local therapy (cryotherapy, photocoagulation, brachytherapy) was employed. Out of 84 eyes treated by combined methods eye enucleation could be avoided in 47 (67%), including 18 (90%), 13 (87%) and 16 (46%) qualified to R-E group I-II, III-IV and V, respectively. First-line chemotherapy combined with the local treatment should be standard treatment for intraocular retinoblastoma groups I - IV. More effective therapy is required for R-E eye group V cases.

[Signs of retinoblastoma]. / Objawy siatkówczaka.

PURPOSE: Retrospective analysis of retinoblastoma symptoms in patients treated in Ophthalmological Clinic in Krakow. MATERIAL AND METHODS: A retrospective chart review was conducted of 293 retinoblastoma patients from our center (1969-2008). RESULTS: A total of 170 (58%) of 293 patients presented with leukocoria, 81 (27.6%) patients had strabismus, 22 (7.5%) had atypical sings, 12 (4.1%) was detected by chance. Eight (2.8%) patients who had a family history of retinoblastoma and were clinically screened for retinal tumors from birth were diagnosed younger. CONCLUSIONS: We recommend routine dilated fundoscopic examination by an ophthalmologist for early detection of retinoblastoma before leukocoria and strabismus.

[Ocular outcomes in 3-year-old premature infants with retinopathy treated with cryotherapy and laser photocoagulation]. / Ocena narzadu wzroku u 3-letnich wczesniaków z retinopatia leczonych krioterapia i laserokoagulacja.

PURPOSE: The aim of the study was to compare functional and structural outcomes in 3-year-old premature children treated in acute phase of retinopathy of prematurity with diode laser photocoagulation and cryotherapy. MATERIAL AND METHODS: We examined 106 premature children aged 3 years, treated for retinopathy of prematurity, by transpupillary diode laser photocoagulation (group II) or cryotherapy (group I). Functional (visual acuity using Snellen charts or preferential looking test - Teller acuity Card) and structural (anterior segment slit lamp and indirect Fison fundus examination) outcome and cycloplegic refraction using 1% Tropicamide were examined in all children. RESULTS: Favorable functional results occurred in 81.9% of eyes in patients after cryotherapy and in 83.9% of eyes in patients after diode laser photocoagulation. Structural outcome was favorable in 90.1% of eyes treated with cryotherapy and in 95.3% of eyes treated with diode laser photocoagulation. CONCLUSION: Diode laser photocoagulation appliance in retinopathy of prematurity treatment allows to obtain better functional and structural outcomes than cryotherapy.

Alström syndrome--a case report and literature review.

PURPOSE: To report a case of Alström syndrome referred as bilateral macular degeneration. MATERIAL AND METHODS: A 52 years old man was diagnosed with an over 30 years history of progressive visual acuity worsening in both eyes, with the presence of night blindness and photophobia. Since childhood the right eye has been positioned in a divergent deviation. General history revealed: high grade obesity, dilated cardiomyopathy with mitral insufficiency, diabetes mellitus type 2, hepatic cirrhosis with elevated serum enzymes, systemic hypertension. Family history: one patient's brother died at the age of 2 years because of a congenital heart disease, and the second brother was diagnosed for the congenital organic heart disease. The basic ophthalmic examination was performed with additional diagnostic methods including: kinetic visual field examination, Amsler grid test, panel D-15 test, fundus photography, ERG, EOG and VEP. RESULTS: Best corrected visual acuity of both eyes was 0.1. Amsler grid and color vision tests were normal. Visual field revealed concentric contraction in both eyes. The funduscopy showed pale optic discs, atrophic maculopathy, golden appearance of peripheral and midperipheral fundus, coarser pigmentary changes with a "bone-spicule" configuration and arterioral narrowing. The red free pictures demonstrated the atrophy of internal retinal layers and the infrared pictures revealed the atrophy of the external layers of the retina in posterior pole of the fundus. The flash ERG showed reduced amplitude of photopic and scotopic b-wave. The multifocal ERG demonstrated the normal function of the central retina. EOG revealed decreased Arden ratio in both eyes; 1.68 in the right and 1.32 in the left. The pattern VEP revealed the P100 amplitude reduction by 80% and elongation of latency by 120% in the right eye and normal in the left eye. The flash VEP showed normal latency and amplitude reduction by 50% in both eyes. CONCLUSIONS: Based on the results of performed tests the diagnosis of Alström syndrome was established. This rare congenital autosomal recessive condition is characterized by progressive cone-rod retinal dystrophy associated with obesity, sensorineural deafness, type 2 diabetes, congenital cardiac insufficiency secondary to dilated cardiomyopathy, systemic hypertension and kidney failure.

[Pregnancy and the eye]. / Wplyw ciazy na narzad wzroku.

During pregnancy periodical hormonal, metabolic, hematological, vascular and immunological changes can be observed, that can influence the function of the eye. Some of them can be observed in an eye due to pregnancy itself and would resolved spontaneously postpartum. Pregnancy can also induce or aggravate the preexisting conditions such as diabetic retinopathy or uveal melanoma. The autoimmune intraocular inflammation is modified by pregnancy manifesting by regression of the ocular symptoms. Pregnancy can have also beneficial effects on glaucoma because intraocular pressure has been reported to decrease during pregnancy. The presence of any ocular symptoms in a pregnant woman requires urgent ophthalmic examination and further management.

[Diffuse atrophy of the choroid--a case report]. / Rozlany zanik naczyniówki--opis przypadku.

PURPOSE: The authors present a case of the 66-year-old female patient suffering from the diffuse atrophy of the choroid. MATERIAL AND METHODS: The 66-year-old woman was referred to the Ophthalmological Clinic in Krakow with the diagnosis of choroideremia. The patient developed central vision problems (mainly in the left eye) and night blindness. We performed following investigations: distance and near visual acuity with Snellen charts, Amsler grid test, the investigation of the anterior and posterior segments of both eyes, and the fluorescein angiography as well as the infrared photo of the fundus. The assessment of ornithine serum level was performed. RESULTS: The ophthalmological examination revealed: the distance visual acuity in the RE=0.5, and in the LE=0.25. The near visual acuity in the RE=0.5, in the LE=0.75. The Amsler grid test in the RE showed no pathology but in the LE revealed in the nasal aspect of the central visual field a relative scotoma. Fundoscopy, fluorescein angiography and infrared imaging revealed the broad patch form atrophy of the RPE and choriocapillaris within the whole fundus and also within the macula. The serum level of ornithine was within the normal limits. CONCLUSIONS: Based on the results of performed investigations the definitive diagnosis of a very rare dystrophy--a diffuse atrophy of the choroid was established.

[Diode laser photocoagulation for retinopathy of prematurity--outcomes in one-year observation]. / Zastosowanie lasera diodowego w leczeniu retinopatii wczesniaków--wyniki rocznej obserwacji.

PURPOSE: To evaluate structural and functional outcomes in one-year old patients, treated for retinopathy of prematurity (ROP) with transpupillary diode laser photocoagulation. MATERIALS AND METHODS: 58 children (116 eyes) 12 months old, after treatment for ROP with diode laser photocoagulation, were examined. Both, functional outcome (visual acuity--basing on the examination with Teller Acuity Card Procedure), and structural outcome were evaluated. The cycloplegic refraction was examined in all cases. RESULTS: We have found good functional outcome in 81.9%, and good structural outcomes in 96.5% of examined eyes. 64.6% of eyes had hyperopic refractive error, and 31% were myopic. CONCLUSIONS: Diode laser photocoagulation is an effective method of treatment for ROP.

[Results of 10 years observation of organ of sight in prematurity]. / Wyniki dziesiecioletniej obserwacji narzadu wzroku u wczesniaków.

PURPOSE: (1) To compare the incidence of refractive errors and the orthoptic condition in ten-year-old children, born prematurely and treated with cryotherapy versus children, whom did not apply such a procedure. (2) To estimate condition of the extraocular muscles. MATERIAL AND METHODS: A group of 60 prematurely born children was divided into 2 subgroups: I--30 children treated with cryotherapy, II--30 no treatment was applied. All the children had anterior segment, fundus and visual acuity examined. Refraction following cycloplegia was evaluated. Visual acuity and orthoptic status was assessed. RESULTS: In the group of prematurely born children who had retinopathy in the infantile age and were treated with cryotherapy, refractive errors were frequently observed. In over half of those children squint or impaired eye movement were found. In the group of children who required no cryotherapy the percentage of those with refractive errors was similar. CONCLUSIONS: In prematurely born children with retinopathy refractive errors and squint occur quite often. Prematurely born children should be subject to ophthalmologic follow-up throughout their childhood.

[Results of combined chemoreduction and local treatment for intraocular retinoblastoma]. / Wyniki leczenia siatkówczaka--chemioterapia skojarzona z leczeniem miejscowym.

PURPOSE: Of this study was to evaluate the results of combined treatment for intraocular retinoblastoma. MATERIALS AND METHODS: We examined 23 children (32 eyes), 14 with unilateral and 9 with bilateral disease. 53% of eyes had the stage V of the disease, according to Reese-Ellsworth. RESULTS: In I-III group according to R-E, it was possible to save more than 90% of eyes. In group IV-V we saved 20% of eyes. CONCLUSIONS: Chemoreduction combined with local treatment is an optimal method of treatment for retinoblastoma, especially in I-III group.

[Risk factors of retinopathy of prematurity in newborns treated in neonatal intensive care unit of University Children's Hospital Collegium Medicum Jagiellonian University]. / Czynniki ryzyka rozwoju retinopatii wczesniaków u pacjentów Oddzialu Patologii i Intensywnej Terapii Noworodka Uniwersyteckiego Szpitala Dzieciecego Collegium Medium Uniwersytetu Jagiellonskiego.

BACKGROUND: A persistent progress in perinatal care and a large increase in infants' survival rate have been observed recently. As a result, the number of neonates requiring ophthalmologic examinations due to retinopathy of prematurity (ROP) increased as well. METHOD: A retrospective survey including 206 neonates with mean birth weight 1342 g and mean gestational age 30 weeks. MATERIAL: Three groups: 1) without ROP--165 infants (80.1%); 2) with ROP not requiring treatment--13 infants (6.3%); 3) with prethreshold and threshold ROP requiring cryotherapy--28 infants (13.6%) were analysed. RESULTS: Threshold ROP was not observed in infants with gestational age > 32 weeks and/or birth weight > 1500 g. Cryotherapy was applied between the 6th and 18th weeks of life (Mo: 12). The procedure was applied at postmenstrual age between the 34th and 45th weeks of life (Mo: 38). The correlation of ROP with the following factors was observed: length of mechanical ventilation (Me in the studied groups: 6; 22; 33 days respectively; p < 0.01), length of oxygen therapy (Me: 27; 58; 70 days; p < 0.01), number of blood transfusions in first month of life (Me: 2; 4; 4; p < 0.01), minimum pO2 (average: 37.2; 30.2; 29.2 mmHg p < 0.01), pO2 oscillation (measured as SD) (Me: 10.25; 13.2; 15.4 mmHg p < 0.016) and maximal pCO2 (average: 52.5; 56.8; 66.5 p < 0.01) between the 2nd and 4th weeks of life. CONCLUSION: Monitoring of pO2 and pCO2 in infants in the 1st month of life reduce the risk of ROP development. Infants born before the 32nd week of gestational age belong to high risk group and require very systematic ophthalmologic examination. The current prophylactic examination schedule for ROP for more mature infants may be changed.