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Małgorzata Wichrowska,1,2 Przemysław Wichrowski,1,3 Jarosław Kocięcki1 1Department of Ophthalmology, Poznan University of Medical Sciences, Poznań, Poland; 2Doctoral School, Poznan University of Medical Sciences, Poznań, Poland; 3I Clinic of Anesthesiology and Intensive Therapy, Poznan University of Medical Sciences, Poznań, PolandCorrespondence: Małgorzata Wichrowska, Department of Ophthalmology, Poznan University of Medical Sciences, Ul. Szamarzewskiego 84, Poznań, 60-569, Poland, Tel +48 61 101 98 00, Fax +48 61 101 98 13, Email malgorzata.wichrowska@skpp.edu.plPurpose: The aim of the study comprised the anatomical and functional assessment of the optic nerves and macular ganglion cells in the population of patients with unilateral wet age-related macular degeneration (wAMD) and comparison of its results to those obtained from the fellow eye with non-advanced dry age-related macular degeneration (dAMD). Furthermore, we aimed to determine if the number of administered injections contributed to the potential differences between the examined eyes.Methods: The study was based on 104 eyes of 52 patients in a cross-sectional study. The eyes with chronically treated wAMD were the main subject of the study, while fellow eyes affected with non-advanced dAMD served as the control group. Primary morphological outcomes comprised differences in peripapillary retinal nerve fiber layer (RNFL) and GCC layers between the studied groups (treated and control). In turn, primary functional outcomes included differences in implicit times and amplitudes of the P100 wave of pattern visual evoked potentials (PVEP) and N95 wave of pattern electroretinogram (PERG) between treated and control groups.Results: We did not find any differences in total RNFL thickness between wAMD and dAMD groups. The number of injections only affected the RNFL in the nasal quadrant of the optic disc (p = 0.023). We did not find any differences regarding GCL thickness in both groups. In the study group, a longer implicit time of the P100 of PVEP (p = 0.014) and a shorter amplitude of the P50-N95 wave of PERG (p = 0.005) were detected. The total number of injections had no significant effect on these two values.Conclusion: We detected worse functional parameters of the optic nerve and retinal ganglion cells in eyes with wAMD, with lack of significant differences in anatomical (RNFL, GCL) parameters compared to the control group. However, the number of injections did not contribute to the differences found.Keywords: age-related macular degeneration, anti-VEGF, retinal nerve fiber layer, RNFL, PVEP, PERG

Pregnancy is associated with numerous changes affecting all organs. Ophthalmological changes in pregnant women are most often physiological and resolve spontaneously after delivery. However, the possibility of progression of previously diagnosed ophthalmic diseases or the occurrence of ophthalmological complications in the course of diseases characteristic for pregnancy should always be considered.

Purpose: The aim of this study was to identify the molecular genetic basis of cone-rod dystrophy in 18 unrelated families of Polish origin. Cone-rod dystrophy is one of the inherited retinal dystrophies, which constitute a highly heterogeneous group of disorders characterized by progressive dysfunction of photoreceptors and retinal pigment epithelium (RPE) cells., Methods: The study group was composed of four groups of patients representing different Mendelian inheritance of the disease: autosomal dominant (AD), autosomal recessive (AR), X-linked recessive (XL), and autosomal recessive or X-linked recessive (AR/XL). The combined molecular strategy included Sanger sequencing of the RPGR-ORF15 gene (three families with XL and three families with the AR/XL mode of inheritance), mutation-specific microarray analysis of the ABCA4 gene (five families with the AR mode of inheritance and two families with the AR/XL mode of inheritance), targeted next-generation sequencing (NGS) of inherited retinal disease-associated (IRD) genes (seven families with the AD mode of inheritance and five families with the AR mode of inheritance), and whole exome sequencing, performed in select families who had been mutation-negative in the analysis with the targeted NGS panel (one family with the AD mode of inheritance, one family with the AR mode of inheritance, and two families with the AR/XL mode of inheritance)., Results: Based on this combined strategy, we managed to identify potentially causative variants in seven out of 18 families with CRD. Five of these variants are novel: c.3142_3143dupAA, p.(Glu1049Argfs*41) in the RPGR-ORF15 gene, two variants: c.1612delT, p.(Trp538Glyfs*15) and c.2389dupG, p.(Ile798Hisfs*20) in the PROM1 gene in one family, c.592A>C, p.(Ser198Arg) in the PRPH2 gene and the variant c.1691A>G, p.(Asp564Gly) in the ATF6 gene that we have already reported to be pathogenic. NGS on the IRD panel allowed the molecular basis of CRD to be identified in four out of 14 families with a total detection rate of 38%. WES allowed identification of the molecular genetic basis of CRD in one family., Conclusions: This is the first report on the spectrum of disease genes and pathogenic variants causing CRD in the Polish population. The study presents five novel variants identified in four genes and therefore, broadens the spectrum of probable pathogenic variants associated with CRD.

Purpose: To collect comprehensive data on choroidal and ciliary body melanoma (CCBM) in children and to validate hypotheses regarding pediatric CCBM: children younger than 18 years, males, and those without ciliary body involvement (CBI) have more favorable survival prognosis than young adults 18 to 24 years of age, females, and those with CBI., Design: Retrospective, multicenter observational study., Participants: Two hundred ninety-nine patients from 24 ocular oncology centers, of whom 114 were children (median age, 15.1 years; range, 2.7-17.9 years) and 185 were young adults., Methods: Data were entered through a secure website and were reviewed centrally. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards regression., Main Outcome Measures: Proportion of females, tumor-node-metastasis (TNM) stage, cell type, and melanoma-related mortality., Results: Cumulative frequency of having CCBM diagnosed increased steadily by 0.8% per year of age between 5 and 10 years of age and, after a 6-year transition period, by 8.8% per year from age 17 years onward. Of children and young adults, 57% and 63% were female, respectively, which exceeded the expected 51% among young adults. Cell type, known for 35% of tumors, and TNM stage (I in 22% and 21%, II in 49% and 52%, III in 30% and 28%, respectively) were comparable for children and young adults. Melanoma-related survival was 97% and 90% at 5 years and 92% and 80% at 10 years for children compared with young adults, respectively (P = 0.013). Males tended to have a more favorable survival than females among children (100% vs. 85% at 10 years; P = 0.058). Increasing TNM stage was associated with poorer survival (stages I, II, and III: 100% vs. 86% vs. 76%, respectively; P = 0.0011). By multivariate analysis, being a young adult (adjusted hazard rate [HR], 2.57), a higher TNM stage (HR, 2.88 and 8.38 for stages II and III, respectively), and female gender (HR, 2.38) independently predicted less favorable survival. Ciliary body involvement and cell type were not associated with survival., Conclusions: This study confirms that children with CCBM have a more favorable survival than young adults 18 to 25 years of age, adjusting for TNM stage and gender. The association between gender and survival varies between age groups., (Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: We investigated characteristics and treatment outcomes of pediatric vs adult retinal detachment managed by retinotomy and retinectomy.
, Methods: This was a retrospective, nonrandomized, interventional study involving 20 pediatric patients and 25 adult patients operated with vitrectomy and retinectomy due to retinal detachment. Outcome measures included preoperative and postoperative visual acuity at 6 months and at final visit, postoperative proliferative vitreoretinopathy, silicone oil removal, final intraocular pressure (IOP), and final anatomic success, defined as complete retinal reattachment.
, Results: Reattachment was observed in 60% (12/20) of pediatric patients and 88% (22/25) of adults at final visit (p=0.041). Statistically significant improvement in visual acuity was observed in adults (p<0.001) but not in children (p=0.360) due to large proportion of anatomic failure. Children required a higher number of further reoperations (p=0.008). Postoperative proliferative vitreoretinopathy was significantly more frequent in pediatric eyes (p=0.003). 
, Conclusions: The clinical features and prognosis for pediatric retinotomies and retinectomies are different from those for adults. When a decision to perform retinotomy in children is made, we can anticipate significantly lower anatomic success than in adults. Even though the overall visual acuity was worse in children than in adults, 25% of pediatric eyes achieved 5/50 or better vision.

Aim: The aim of our research was to describe the effect of an off-label intravitreal ranibizumab injection for treatment of unilateral extrafoveal choroidal neovascularization a with bilateral optic nerve drusen., Material and Methods: 13-year-old girl presented with decreased visual acuity of her left eye and optic nerve drusen confirmed by B-scan ultrasound examination in both eyes. Fluorescein angiography and optical coherence tomography revealed the presence of choroidal neovascularization in the left eye. The patient was treated with a single injection of ranibizumab and monitored by clinical examination, optical coherence tomography and fluorescein angiography., Results: Choroidal neovascularization was successfully treated and the best corrected visual acuity (Snellen) fully recovered from 20/50 to 20/20 over a period of 2 months. After this time at the 30 months follow-up, visual acuity and fundus were stable without the recurrence of choroidal neovascularization., Conclusions: Optic nerve drusen should be taken into account and carefully observed as a possible cause of peripapillary choroidal neovascularization in children. Ranibizumab can be a successful off-label treatment in children suffering from choroidal neovascularization associated with optic nerve drusen.

The aim of the study is to present a case of unilateral orbital tumor (lymphoma) in a 7 years old boy. The edema of the temporal area of the right orbit with considerable narrowing of the palpebral fissure increased quickly during about 3 weeks. The skin redness without increased skin warmness and hardening by palpation above the lesion were observed. The orbit ultrasound and nuclear magnetic resonance were performed and the biopsy for histopathologic evaluation and immunohistochemical staining was taken. The last examination reveled a very rare kind type of the lymphoma in this age and location--lymphoblastic lymphoma beginning from B cells. In case of lymphoma the biopsy for histopathologic and immunohistochemical evaluation is essential, allowing for diagnosis and appropriate chemotherapy, as far as there is different outline for the treatment of different lymphomas.

Purpose: To investigate the anatomy and function of the macula in patients after retinectomy for retinal detachment complicated by proliferative vitreoretinopathy (PVR) grade C., Methods: A prospective study involving 25 patients operated with vitrectomy and retinectomy due to retinal detachment complicated by PVR grade C. Outcome measures included preoperative and postoperative best-corrected visual acuity (BCVA) at 6 months and the difference between preoperative and postoperative BCVA. Furthermore, the macular status was analyzed on postoperative optical coherence tomography (OCT) scans., Results: Reattachment was observed in 96% (24/25) of patients. Median BCVA improved from 2.30 logMAR (range 1.00 to 2.69 logMAR) to 1.00 logMAR (range 0.38 to 2.90 logMAR). The change from preoperative visual acuity was statistically significant (p=0.0003, Wilcoxon signed rank test). Macular status with pathologies was observed in 75% of eyes on OCT (retinal pigment epithelium irregularities 37.5%, cystoid macular edema 33%, epiretinal membrane 8.3%, subretinal fluid 4%, and subretinal perfluorocarbon liquid 4%). These eyes had significantly worse final visual acuity (p=0.01, Wilcoxon rank sum test) compared to those with a clinically normal macula. The retinectomy size influenced neither visual acuity (p=0.06, Wilcoxon rank sum test) nor incidence of normal macular status (p=0.14, Fisher exact test)., Conclusions: Even though retinectomy is an effective technique to reattach the retina in complicated cases of retinal detachment, the functional outcome was found to be influenced by abnormal macular status, observed in 75% of eyes. Moreover, 360-degree retinectomies did not show different anatomic or functional results vs subtotal ones.

Purpose: To report choroidal melanoma treatment by endoresection., Material and Methods: A retrospective review of a series of 2 patients with choroidal melanoma that did not meet the criteria for brachytherapy. Both patients were treated by tumor endoresection supported by brachytherapy. Outcome measures included preoperative and postoperative visual acuity at 1, 3, 6 months. Preoperative evaluation included tumor measurements in ultrasound. Systemic evaluation (liver function test, chest x ray, abdominal ultrasound), was carried out to monitor possible metastases in the observation period., Results: The two operated patients were followed-up for 6 months. Visual acuity deteriorated from 5/16 (0.51 logMAR) preoperatively to 5/25 (0.7 logMAR) postoperatively in patient 1, and improved from 3/50 (1.22 logMAR) do 5/25 (0.7 logMAR) in patient 2. This corresponded to deterioration by 0.19 logMAR and improvement by 0.52 logMAR respectively. Neither tumor recurrence nor metastases were detected in the follow-up period., Conclusions: Endoresection is a treatment option, alternative to enucleation, that can conserve the eye It sometimes can be performed in eyes that are not eligible for trans-scleral resection. Further larger studies are needed to establish the efficacy of this still controversial surgery.

Purpose: The aim of the study was the evaluation of the frequency and intensity of the disturbances of the eye position and motility in patients after scleral buckling surgery due to retinal detachment, treated in Ophthalmology Clinic in Poznar. Additionally the aim was to diagnose the reason for muscles damage by means of the Hess screen., Material and Methods: Seventy patients were treated with this method between 2007-2009 and the study group was built by 40 patients, treated in average 15 months before the examination, at the age 12-79 years (mean age 55 years and 8 months). The correlation between the location of retinal holes treated with cryotherapy, the type of surgery and the changes in eye position and motility or diplopia in clinical evaluation and on the Hess screen, were evaluated., Results: The disturbances of eye motility in clinical evaluation was diagnosed in 10 eyes (25% cases), and on the Hess screen in 16 eyes (40% cases). We noted, that the damaged muscle (overacting or weakened), was localized always on the site of cryotherapy or in the area at three o'clock from the retinal hole., Conclusions: (1) Patients during visual rehabilitation after scleral buckling surgery should be controlled for eye motility disturbances and diplopia. (2) The percentage of eye motility disturbances in our study group was comparable to this presented in literature, but higher in evaluation by means of Hess screen. (3) Accidental cryotherapy of the muscle, causing its weakening or overaction, seems to be a particular risk factor of extraocular muscles function disturbances.

Purpose: To report a new and atypical pathology of epiretinal membrane formation on the top of the retinoschisis (schisis-membrane)., Methods: We describe a patient with recent visual acuity and visual field deterioration due to a rapid progression of retinoschisis in the macular area resulting from shrinking membrane on the top of the schisis. The patient underwent vitrectomy with membrane removal., Results: Partial regression of the schisis was observed and visual acuity improved from 5/12 to 5/5. Perimetry demonstrated that the corresponding visual field deterioration partially regressed., Conclusions: Release of traction exerted by a shrinking membrane is a surgical option to promote regression of retinoschisis and improvement in visual field and vision in schisis-membrane cases.

Purpose: To report autologous choroidal RPE patch transplantation for treatment of submacular hemorrhage in a patients with age related macular degeneration., Material and Methods: A case report of a patient with sudden vision deterioration due to submacular hemorrhage in AMD. The visual acuity was 5/5 in the right eye and 0.5/50 in the left eye. He was treated with autologous choroidal RPE patch transplantation. Outcome measures included preoperative and postoperative visual acuity at 1, 3, 6 months, duration of hemorrhage, hemorrhage size on preoperative fluorescein angiography and thickness of the subretinal hemorrhagic complex on preoperative and postoperative OCT scans., Results: Visual acuity improved from 0.5/50 to 5/50 in 6 months follow-up. No major intraoperative and postoperative complications were observed. The perfusion of the graft was confirmed in ICG angiography., Conclusions: Autologous choroidal RPE patch transplantation is an effective option for submacular hemorrhage treatment in AMD. Unlike macular translocation, the surgery can be performed even in patients with excellent visual acuity in the fellow eye.

Cataract is a leading course of a treatable blindness and serious visual impairment in children. Treatment, especially in one-sided cataract is a challenge and it is connected with the risk of complications. The decision to perform a surgery in older children with already coexisting amblyopia is not easy to make. The aim of our study was to present the effects of the cataract treatment in children between 3 and 18 years old, in the Ophthalmology Clinic in Poznań. Between 2006 and 2008, the surgery was done in 36 children (42 eyes). The data of those patients, e.g. the type and location of the cataract, accompanying disorders and visual acuity reached, were analyzed. In this group of patient 60% of children reached visual acuity equal to 0.5, and 24% of them full visual acuity. The way to prepare the patient to the surgery, the way to calculate the power of the intraocular lens to be implanted and the surgery technique will be discussed.

Purpose: To evaluate vitrectomy with intravitreal antibiotic administration in exogenous endophthalmitis treatment in eyes with vision better than light perception., Material and Methods: Retrospective review of 9 patients with exogenous endophthalmitis and VA better than light perception. All patients were treated with vitrectomy and intravitreal antibiotic administration. Outcome measures included: preoperative visual acuity and postoperative visual acuity at 6 months, time from the initial ocular surgery and the presentation of endophthalmitis symptoms., Results: Visual acuity at 6 months improved in 88% (8/9), of patients and decreased in 12% (1/9). The mean change in visual acuity was a gain 1.01 +/- 0.80 logMAR that corresponds to improvements of 10 lines on ETDRS chart. The differences in mean preoperative and postoperative visual acuity were statistically significant (p = 0.009, Wilcoxon signed rank test). No statistically significant differences were detected in mean visual acuity improvement between acute and chronic form of endophthalmitis (p = 0.88, Wilcoxon rank sum test)., Conclusions: There is more evidence to consider vitrectomy with intravitreal antibiotic administration in eyes with visual acuity better than light perception i.e. in cases that Endophthalmitis Vitrectomy Study (EVS) recommends for intravitreal antibiotic administration alone.

Purpose: To report macular translocation with 360 retinectomy for treatment of submacular hemorrhage in patients with age related macular degeneration., Material and Methods: Retrospective review of the two patients with age related macular degeneration and submacular hemorrhage, treated with macular translocation with 360 retinectomy. Outcome measures included preoperative and postoperative visual acuity at 1, 3, 6 months and 1 year and a difference between preoperative and postoperative visual acuity at 1 year, as well as the duration of hemorrhage, hemorrhage size on preoperative fluorescein angiography and thickness of the subretinal hemorrhagic complex on preoperative OCT scans., Results: The two operated patients were followed-up for 1 year. Distance visual acuity at 12 months improved in both cases by 0.22 and 0.48 logMAR respectively. No major intraoperative and postoperative complications were observed except for a foveal atrophy on OCT in one patient., Conclusions: MT360 seems to be an effective option for submacular hemorrhage treatment in AMD in the second affected eye. However a further face to face comparison with other treatment methods and with combination of tPA, anti-VEGF and pneumatic displacement in particular, is necessary to fully determine the efficacy of this technique.

Purpose: Evaluation of Acrimed LENTIS UNICO LU-800 RZI discoid lens in the combined cataract and vitrectomy surgery., Material and Methods: ten patients that underwent combined cataract and vitrectomy surgery between January and April 2007 were included in the prospective study. There were 8 female and 2 male patients with the mean age of 58.4 +/- 18.7 years in the study group. Data including age, sex, indications for surgery were collected with a special form. Furthermore, difficulty of lens implantation, retinal periphery visualization during vitrectomy, and retinal periphery visualization in the follow up ophthalmoscopy were evaluated with a special scale ranging from 0 to 5 (0 = impossible, 1 = very difficult, 2 = difficult, 3 = moderate, 4 = easy, 5 = very easy)., Results: The difficulty of the anterior segment surgery was graded 3.1 +/- 0.74. Visualization of the retinal periphery in the vitrectomy was ranked 4.8 +/-0.42. In the postoperative follow up ophthalmoscopy visualization of the peripheral retina was scored as 4.4 +/- 0.7. The most severe complication observed intraoperatively was discoid lens dislocation into the vitreous cavity. Postoperatively there was a case of a malignant glaucoma-like syndrome that required a second surgery., Conclusions: In our opinion the discoid lens yields a very good visibility of the peripheral retina during vitreoretinal procedures and in the follow up ophthalmoscopy. We do not recommend this lens for routine cases, because of the difficult implantation and the relatively high number of complications is observed. It can be considered in some selected eyes when maneuvers in the peripheral retina or laser therapy in the future are planned.

Central serous choroidoretinopathy (CSCR)--recurrent disorder characterized by the detachment of neurosensory retina and/or detachment of the RPE in the posterior pole, caused by fluid leakage from choroidal vessels, coming through retinal pigment epithelium layer. The aim of the following paper was to create a historic revue of examinations of CSCR and modern description of signs and symptoms of this disease. CSCR may be present in various clinical forms with different prognosis, and therefore also all kind of retina and choroidea lesions were described. The role of hypertension, steroid treatment and stress in course of CSCR was shown in many studies during past years and for that reason the correlation between those factors and the incidence and the course of CSCR, was analysed. It is important to stress, that in contrast to current opinion, steroids can evoke CSCR and intensificate its symptoms and are contraindicated in the treatment of this disease. To addition CSCR is not always a benign disease, and may cause significant impairment of the visual acuity and colour vision, therefore, it should be carefully followed-up.

There are many clinical forms of central serous choroidoretinopathy (CSCR) with different prognosis. Although fluoresceine angiography still plays the most important role in the diagnosis of CSCR, it should be pointed out, that examination with retinal thickness analyser and optical coherent tomography is a modern and non-invasive method of retinal examination, which can provide with some significant and reliable observations helping in objective monitoring of CSCR. The aim of this paper was the description of diagnostic methods and analysis of optimal methods used for monitoring of CSCR. Because the research of the literature suggests, that local: nonsteroid anti-inflammatory drugs, beta-blockers and systemic or local carbonic anhydrase inhibitors may be helpful in therapeutic choice, the treatment options of CSCR were also analysed.

In this review short biological characteristics of human cytomegalovirus (CMV), the sources and its modes of transmission to human organisms, are presented. The authors described clinical pathology mainly of ocular organ and central nervous system in children with congenital cytomegalovirus infection and in acquired form of cytomegaly in adults with immunosupression (especially with HIV infection and AIDS). In review there are included contemporary diagnostic laboratory methods used in detection of CMV infection and in evaluation of clinical pathology.

The Lowe syndrome is a rare genetic disorder. It is inherited in an X-linked recessive manner. The clinical symptoms occur in eyes, central nervous system and kidneys. We describe the case of 16 years old boy with Lowe syndrome who underwent the bilateral congenital cataract surgery at the age of 14 months. The clinical symptoms of patient's mother are also described.

Purpose: To appoint the most adequate follow-up parameter and to find the most reliable scheme of intravitreal TA injections in CME treatment in the course of CRVO and BRVO., Material and Methods: 18 eyes treated with intravitreal injection of 4 mg (in 0.1 ml) of crystalline TA., Results: There was improvement of BCVA for distance noticed in 38.9% of eyes and for near in 44.4% of cases. There was statistically significant difference in retinal thickness outcomes measured at baseline and at the end of the follow-up by both OCT and RTA. No correlation of BCVA changes and retinal thickness changes measured in OCT and RTA was found., Conclusions: The intravitreal TA injections should be considered as an effective method. Foveal morphology and thickness analysis performed by OCT is the most adequate method in follow-up monitoring of intravitreal TA injections in CME treatment in non-ischemic CRVO or BRVO. Central retinal thickness measured by RTA and FA changes analysis are alternative monitoring methods. Dosage of 4mg TA in 0.1 ml seems to be sufficient. In cases of CME recurrence of subsequent intravitreal injection in the first 6 month after initial injection seems to be unnecessary.

We report case of acute retinal pigment epithelitis (ARPE), a very rare ophthalmic disorder, which has a very good final visual prognosis. The 18 years old woman was examined by ophthalmologist for sudden visual decrease of vision and dark spot in visual field of her left eye. The best corrected visual acuity (BCVA) of the left eye at the initial diagnosis was 0.1 and after 4 months improved to 0.9. The diagnosis or ARPE was made on the basis of fundoscopy, fluorescein angiography, electroretinography and optical coherence tomography (OCT) examination.

Purpose: To appoint the most adequate follow-up parameter of intravitreal TA injections in CME treatment due to diabetic maculopathy and to find the most reliable treatment scheme., Material and Method: 4 mg (in 0.1 ml) of crystalline TA were injected into the vitreous of 17 eyes., Results: There was a statistically significant improvement of BCVA for distance and near vision. There was statistically significant difference in retinal thickness outcomes measured at baseline and at the end of thefollow-up by both OCT and RTA No correlation of BCVA changes and retinal thickness changes measured in OCT and RTA was found., Conclusions: Intravitreal TA administration seems to be an effective method of treatment in diabetic CME. OCT is the most adequate method of follow-up of intravitreal TA injections in treatment of CME due to diabetic maculopathy. RTA and FA changes analysis are alternative monitoring methods. Dosage of 4mg TA in 0.1 ml seems to be sufficient. In cases of CME recurrence subsequent intravitreal injection in the first 6 month after initial injection seems to unnecessary.