First Case Report of Developmental Bilateral Cataract with a Novel Mutation in the ZEB2 Gene Observed in Mowat-Wilson Syndrome.

Background: Mowat-Wilson syndrome (MWS) is extremely rare multisystemic autosomal dominant disorder caused by mutations in the Zinc Finger E-Box Binding Homeobox 2 (ZEB2) gene. Ocular pathologies are one of the symptoms that appear in the clinical picture of MWS individuals, but not many have been described so far. Pathologies such as optic nerve or retinal epithelium atrophy, iris or optic disc coloboma as well as congenital cataracts have been most frequently described until now. Therefore, we would like to report the first case of bilateral developmental cataract in a 9-year-old girl with MWS who underwent successful cataract surgery with intraocular lens implantation. Case Presentation: A 9-year-old girl, diagnosed with p.Gln694Ter mutation in ZEB2 gene and suspicion of MWS was referred to the Children's Outpatient Ophthalmology Clinic for ophthalmological evaluation. Her previous assessments revealed abnormalities of the optic nerve discs. The patient was diagnosed with atrophy of the optic nerves, convergent strabismus, and with-the-rule astigmatism. One year later, during the follow-up visit, the patient was presented with decreased visual acuity (VA), developmental total cataract in the right eye and a developmental partial cataract in the left eye. This resulted in decreased VA confirmed by deteriorated responses in visual evoked potential (VEP) test. The girl underwent a two-stage procedure of cataract removal, first of one eye and then of the other eye with artificial lens implants. In the 2 years following the operation, no complications were observed and, most remarkably, VA improved significantly. Conclusions: The ZEB2 gene is primarily responsible for encoding the Smad interaction protein 1 (SIP1), which is involved in the proper development of various eye components. When mutated, it results in multilevel abnormalities, also in the proper lens formation, that prevent the child from normal vision development. This typically results in the formation of congenital cataracts in children with MWS syndrome, however, our case shows that it also leads to the formation of developmental cataracts. This is presumably due to the effect of the lack of SIP1 on other genes, altering their downstream expression and is a novel insight into the importance of the SIP1 in the occurrence of ocular pathologies. To the best of our knowledge, this is the first case of bilateral developmental cataract in the context of MWS. Moreover, a novel mutation (p.Gln694Ter) in the ZEB2 gene was found corresponding to this syndrome entity. This report allows us to gain a more comprehensive insight into the genetic spectrum and the corresponding phenotypic features in MWS syndrome patients.

Evaluation of Macular Ganglion Cell-Inner Plexiform Layer in Children with Deprivational Amblyopia Who Underwent Unilateral Cataract Surgery.

Background and Objectives: The aim of the study is to assess macular ganglion cell and inner plexiform layer (mGCIPL) thickness in deprivational amblyopic eyes (AE), fellow non-amblyopic eyes (FE) and normal eyes (NE) using spectral. domain optical coherence tomography (SD-OCT). Materials and Methods: Twenty two children (64% boys) who underwent surgical removal of unilateral congenital or developmental cataracts and exhibited visual impairment despite postoperative visual rehabilitation were included in the study. Cataract surgery was performed in patients aged 55.82 ± 35.85 months (range 6 to 114 months). The mean age of the study group was 9.73 ± 2.85 years (range 5 to 15 years). The comparison group consisted of 22 healthy age- and gender-matched children. The best corrected visual acuity (BCVA) after surgery was: 0.75 ± 0.27 (range 0.3 to 1.3) in AE, 0.1 ± 0.13 (range 0 to 0.5) in FE and 0.04 ± 0.07 (range 0 to 0.2) in NE. OCT scans were performed in all patients and subsequently corrected for axial length related magnification errors. Results: The average thickness of mGCIPL was 70.6 ± 11.28 µm in AE; 77.50 ± 6.72 µm in FE and 81.73 ± 5.18 µm in NE. We found that mGCIPL was statistically significantly thinner in deprivation AE compared to FE (p = 0.038) and NE (p = 0.0005). The minimum thickness of mGCIPL was respectively: 62.68 ± 13.2 µm, 70.3 ± 7.61 µm, and 74.5 ± 5.47, and also differed between AE and FE (p = 0.023) and AE and NE (p = 0.0004). Also, measurements in the inferior, inferotemporal, and superotemporal sectors showed thinning of mGCIPL in AE compared to NE. Conclusions: This analysis may suggest that deprivational amblyopia caused by unilateral congenital or developmental cataract in children may be associated with mGCIPL thinning.

Success rates of probing for congenital nasolacrimal duct obstruction at various ages.

BACKGROUND: Although nasolacrimal duct probing is the standard treatment for congenital nasolacrimal duct obstruction (CNLDO) among children, the optimal timing of this procedure has been a topic of debate. The aim of the study was to analyze the clinical efficacy of nasolacrimal duct probing among patients with CNLDO symptoms at various ages. METHODS: An 8-year retrospective study involved 2434 patients (3009 eyes), who underwent nasolacrimal duct probing conducted under topical anesthesia in the operating theatre. The study group consisted of 1148 girls (47.2%) and 1286 boys (52.8%) from 2 weeks to 41 months (average age was 8 ± 5.6 months). The participants were divided into nine age groups: 0-2 months, 3-6 months, 7-9 months, 10-12 months, 13-15 months, 16-18 months, 19-21 months, 22-24 months and over 24 months. RESULTS: Bilateral obstruction was present among 575 (23.6%) children and was associated with a higher percentage of unsuccessful procedures compared to patients with unilateral obstruction (16.9% vs 10.2%, p < 0.001 Chi-square test). The success rate of the initial probing was 87.2% for all children and it was shown that it decreased with age. In the above age groups, it was 87.9%; 91.4%; 89.6%; 86%; 76.3%; 71.3%; 70.3%; 70.2%; 65.4%, respectively. CONCLUSIONS: Probing is a safe and effective procedure. However, age at the time of the initial intervention and bilateral surgery constitute significant risk factors for failed probing. Probing between 7 and 9 months appears to be reasonable treatment strategy for children without recurrent infections. Early surgical intervention may be considered for patients with additional signs.

Contribution of a Novel B3GLCT Variant to Peters Plus Syndrome Discovered by a Combination of Next-Generation Sequencing and Automated Text Mining.

Anterior segment dysgenesis (ASD) encompasses a spectrum of ocular disorders affecting the structures of the anterior eye chamber. Mutations in several genes, involved in eye development, are implicated in this disorder. ASD is often accompanied by diverse multisystemic symptoms and another genetic cause, such as variants in genes encoding collagen type IV. Thus, a wide spectrum of phenotypes and underlying genetic diversity make fast and proper diagnosis challenging. Here, we used AMELIE, an automatic text mining tool that enriches data with the most up-to-date information from literature, and wANNOVAR, which is based on well-documented databases and incorporates variant filtering strategy to identify genetic variants responsible for severely-manifested ASD in a newborn child. This strategy, applied to trio sequencing data in compliance with ACMG 2015 guidelines, helped us find two compound heterozygous variants of the B3GLCT gene, of which c.660+1G>A (rs80338851) was previously associated with the phenotype of Peters plus syndrome (PPS), while the second, NM_194318.3:c.755delC (p.T252fs), in exon 9 of the same gene was noted for the first time. PPS, a very rare subtype of ASD, is a glycosylation disorder, where the dysfunctional B3GLCT gene product, O-fucose-specific ß-1,3-glucosyltransferase, is ineffective in providing a noncanonical quality control system for proper protein folding in cells. Our study expands the mutation spectrum of the B3GLCT gene related to PPS. We suggest that the implementation of automatic text mining tools in combination with careful variant filtering could help translate sequencing results into diagnosis, thus, considerably accelerating the diagnostic process and, thereby, improving patient management.

Analysis of Peripapillary Retinal Nerve Fiber Layer Thickness in Acute Anterior Uveitis among Children with HLA-B27-Positive Juvenile Idiopathic Arthritis.

PURPOSE: The aim of this study was to evaluate changes in the thickness of the peripapillary retinal nerve fiber layer (pRNFL) in children with a diagnosis of juvenile idiopathic arthritis (JIA) who were positive for human leukocyte antigen (HLA)-B27, treated for the first episode of unilateral acute anterior uveitis (AAU). MATERIALS AND METHODS: This retrospective study included 41 children (aged 5 to 14 years; mean age 8.32 ± 2.4 years) with HLA-B27 positivity and unilateral JIA-AAU, and 40 healthy children. Optical coherence tomography (OCT) imaging was performed during active inflammation and subsequent noninflammatory phases (6 months after the resolution of inflammatory symptoms in the anterior segment of the eye). RESULTS: There was a marked difference in mean pRNFL thickness between eyes with AU in the active phase, unaffected fellow eyes and the control group (110.22 ± 5.95 µm, 102.39 ± 4.39 µm and 95.83 ± 8.84 µm, respectively; p < 0.001). The thickness of pRNFL in eyes with AU in the active phase in all sectors was greater compared to unaffected fellow eyes (p < 0.001) and normal eyes (p < 0.001). In addition, it was demonstrated that pRNFL thickness was significantly increased in the superior and temporal sectors in the unaffected fellow eyes compared to the control group (128.73 ± 13.16 µm vs. 121.48 ± 13.35 µm and 71.37 ± 4.02 µm vs. 64.98 ± 9.12 µm, respectively). Even during the inactive phase, eyes with AU, compared to the healthy control group, had significantly greater pRNFL thickness in the inferior sector (129.78 ± 11.98 µm vs. 122.3 ± 14.59 µm; p = 0.018), along with the temporal sector (70.88 ± 5.48 µm vs. 64.98 ± 9.12 µm; p = 0.001). CONCLUSIONS: An increase in pRNFL thickness in children with unilateral JIA-AAU who were positive for HLA-B27 antigen can be observed in both eyes compared to healthy controls, and this change may persist even after the inflammatory symptoms have resolved. Measurements of pRNFL thickness resulting from JIA-AU-associated glaucoma should be performed during quiescent periods to avoid subclinical changes in pRNFL thickness caused by inflammation. However, when reviewing the results, it should be noted that changes in pRNFL parameters may be present despite evidence of a resolution of inflammation.

Aicardi-Goutières Syndrome with Congenital Glaucoma Caused by Novel TREX1 Mutation.

BACKGROUND: Aicardi-Goutières syndrome (AGS) is a rare genetic disorder characterized by microcephaly, white matter lesions, numerous intracranial calcifications, chilblain skin lesions and high levels of interferon-α (IFN-α) in the cerebrospinal fluid (CSF). However, ocular involvement is reported significantly less frequently. CASE PRESENTATION: We present a case of a neonate with hypotrophy, microcephaly, frostbite-like skin lesions, thrombocytopenia, elevated liver enzymes and hepatosplenomegaly. Magnetic resonance imaging (MRI) of the brain showed multiple foci of calcification, white matter changes, cerebral atrophy, and atrophic dilatation of the ventricular system. The inflammatory parameters were not elevated, and the infectious etiology was excluded. Instead, elevated levels of IFN-α in the serum were detected. Based on the related clinical symptoms, imaging and test findings, the diagnosis of AGS was suspected. Genetic testing revealed two pathogenic mutations, c.490C>T and c.222del (novel mutation), in the three prime repair exonuclease 1 (TREX1) gene, confirming AGS type 1 (AGS1). An ophthalmologic examination of the child at 10 months of age revealed an impaired pupillary response to light, a corneal haze with Haab lines in the right eye (RE), pale optic nerve discs and neuropathy in both eyes (OU). The intraocular pressure (IOP) was 51 mmHg in the RE and 49 in the left eye (LE). The flash visual evoked potential (FVEP) showed prolonged P2 latencies of up to 125% in the LE and reduced amplitudes of up to approximately 10% OU. This girl was diagnosed with congenital glaucoma, and it was managed with a trabeculectomy with a basal iridectomy of OU, resulting in a reduction and stabilization in the IOP to 12 mmHg in the RE and 10 mmHg in the LE without any hypotensive eyedrops. CONCLUSIONS: We present the clinical characteristics, electrophysiological and imaging findings, as well as the genetic test results of a patient with AGS1. Our case contributes to the extended ophthalmic involvement of the pathogenic c.490C>T and c.222del mutations in TREX1.

Isolated retinal astrocytic hamartoma with 7-year follow-up: A case report.

RATIONALE: Retinal astrocytic hamartoma (RAH) is a rare benign tumor originating from astrocytic cells located in the neural cell layer of the retina. It is commonly seen in patients with phakomatoses such as tuberous sclerosis complex or neurofibromatosis, rarely as an isolated retinal mass. This lesion is usually asymptomatic; however, these located in the area of the optic nerve, macula, or exhibiting the features of exudation, neovascularization may present visual disturbances and decreased visual acuity. PATIENT CONCERNS: We present a rare case of a 15-year-old boy, with no significant past medical history, whose cause of visual disturbances turned out to be isolated RAH. DIAGNOSES: Based on the results of color images of the fundus, fluorescein angiography as well as the analysis of magnetic resonance imaging, the patient was diagnosed with RAH. INTERVENTIONS: Additionally an B-scan ultrasonography, static and kinetic perimetry were performed. OUTCOMES: Fundoscopic examination showed a unilateral yellowish, well-circumscribed, mulberry-like lesion with a wide base, located in inferosnasal quadrant, in the vinicity of the optic nerve. The patient underwent neurological, pediatric, and genetic evaluations that excluded other pathological findings or underlying systemic disease. LESSONS: The prognosis for RAH is generally good, however, the lesion requires regular ophthalmologic follow-up to rule out the progression of the tumor mass. The patient 7-year follow-up history is without evidence of tumor growth, local or general deterioration of the condition.

Anterior Uveitis and Coats Disease in a 16-Year-Old Girl with Noonan Syndrome-A Case Report.

Background: Noonan syndrome (NS) represents a fairly common genetic disorder with a highly variable phenotype. Its features include inherited heart defects, characteristic facial features, short stature, and mild retardation of motor skills. Case presentation: A 16-year-old Caucasian girl with NS reported visual deterioration, photophobia, and pain in the right eye (RE). The initial best-corrected visual acuity (BCVA) was 0.3 in the RE. An examination demonstrated conjunctival and ciliary body hyperemia, keratic precipitates, and flare in the anterior chamber. In addition, post-hemorrhagic floaters, tortuous vessels, and an epiretinal membrane in the RE were present. Diagnosis of unilateral anterior uveitis was made, and this resolved after the use of topical steroids and cycloplegic drops. Due to the presence of retinal telangiectasias and extraocular exudates (consistent with Coats' disease (CD) stage 2A) in the RE, laser therapy was performed. The patient remains under constant follow-up, and after one year, the BCVA in the RE was 0.7. Conclusions: Here, we report the clinical characteristics, genetic findings, and retinal imaging results of a patient with NS. To our knowledge, this is, to date, the first report of an association of NS with a PTPN11 mutation with anterior uveitis and CD.

Nonpenetrating very deep sclerectomy with hyaluronic acid implant vs trabeculectomy--a 2-year follow-up.

OBJECTIVE: To study and compare the efficacy and safety of nonpenetrating very deep sclerectomy (NPVDS) with the use of hyaluronic acid implant (SKGEL) to trabeculectomy (TB) in patients with with medically uncontrolled glaucoma. METHODS: Prospective, controlled study of patients with open-angle glaucoma was designed. Seventy-eight eyes of 68 patients with medically uncontrolled glaucoma were assigned either to the NPVDS or to the TB group of trial. Examinations were applied before and 7 days, 1, 3, 6, 12, 18, and 24 months after surgery. MAIN OUTCOME MEASURE: Best-corrected logMAR visual acuity, intraocular pressure, number of additional procedures, antiglaucoma medications, number of complications. RESULTS: At 24 months, success rate defined as IOP ≤ 21 mmHg with medication and additional procedures in NPVDS group was 92.31 %, and 94.88 % in control group (p = 0.64). There was no statistically significant difference between intraocular pressure in NPVDS (14.56 ± 4.07 mmHg ) and control (TB) (15.38 ± 3.38 mmHg) group (p = 0.34). Number of glaucoma mediations decreased from 2.18 ± 0.56 to 0.54 ± 0.56 in NPVDS and from 2.28 ± 1.41 to 0.61 ± 0.03 in TB group (p = 0.71). Early and late postoperative complications included two cases of hyphema, two of choroidal detachment, two of filtering bleb fibrosis, four of cataract progression in the NPVDS group; and five cases of hyphema, four of choroidal detachment, one of filtering bleb fibrosis, one of blebitis, and 12 of cataract progression in the control group. CONCLUSIONS: NPVDS is an effective surgical option for patients with medically uncontrolled glaucoma. NPVDS is associated with a lower risk of complications, and in particular cataract progression, compared to TB. APPLICATION TO CLINICAL PRACTICE: NPVDS is a modification of NPDS devised for patients with medically uncontrolled open-angle glaucoma.

A Clinical Study of the Impact of Soft Contact Lenses on the Progression of Myopia in Young Patients.

PURPOSE: To assess the impact of soft contact lenses on the progression of myopia in young patients. PATIENTS AND METHODS: The observational study included 102 patients divided into 3 groups: MFCL (multifocal contact lenses) group: 15 girls and 9 boys, aged 8-20 (= 14.12 ± 2.863) with soft multifocal contact lenses with myopia: = -3.12 D ± 1.776 D and mean myopia progression -0.23 ± 0.233D after 2 years; SVCL (single vision contact lenses) group: 30 girls and 5 boys, 11-20 years old (=15.5 ± 2.24) with myopia = -2.88 ± 2.122 D at admission and mean myopia progression -0.54 ± 0.464 D after 2 years; the spectacle (single vision glasses) group: 25 girls and 18 boys, aged 8-18 years ( = 13.65 ± 2.448) with single vision glasses with myopia: = -1.74 ± 1.412 D at admission and mean myopia progression -0.86 ± 0.489D after 2 years. Medical history and physical examination were performed every 6, 12, 18 and 24 months. Refractive error was examined using the autorefractometry after cycloplegia. RESULTS: The analysis of myopia correction after 2 years showed differences between MFCL and spectacle correction. The change in myopia progression after 2 years was statistically significant for MFCL vs SVCL and MFCL vs spectacle correction when the myopia occured before the period of intensive growth. When myopia occurred during the period of intensive growth, difference was noted for MFCL vs spectacle correction and SVCL vs spectacle correction. When myopia occurred after a period of intensive growth, no significant differences between the groups were observed. CONCLUSION: 1) Multifocal contact lenses and some single vision contact lenses (Biofinity) may be useful in the control of myopia in younger patients, slowing the progression of nearsightedness; therefore, they can be a therapeutic option in inhibiting the progression of myopia. 2) The best effects of using multifocal contact lenses occur if myopia is diagnosed before the period of intensive growth.

Ocular features in Aicardi syndrome: A case report.

RATIONALE: Aicardi syndrome is a genetic malformation syndrome with a triad of dysgenesis or agenesis of the corpus callosum, distinctive chorioretinal lacunae and infantile spasms. It is a rare developmental disorder first described in 1965. The disease affects 1 in 100,000 live births. PATIENT CONCERNS: We describe a 34-month-old girl diagnosed with Aicardi Syndrome. DIAGNOSIS: Based on the results of color images of the fundus, medical history as well as the analysis of karyotype and DNA microarrays, the patient was diagnosed with Aicardi's syndrome. INTERVENTIONS: Additionally an B-scan ultrasonography and an electrophysiological test was performed. OUTCOME: Fundoscopic examination revealed optic disc colobomas in both eyes, extensive chorioretinal lacunae at the posterior pole with retinal pigment epithelium regrouping and atrophy. Flash visual evoked potentials (FVEP) P2 amplitude was lower than normal range. B-scan ultrasonography revealed an optic disc lesion consistent with optic disk coloboma. LESSONS: Children with congenital central nervous system malformations should undergo regular ophthalmic checkups to facilitate diagnosis and determine prognosis of visual function development.

Quantitative relationships between transforming growth factor beta mRNA isoforms in congenital and traumatic cataracts.

PURPOSE: The aim of this study was to determine differences in the expression profiles of transforming growth factor (TGF) ß isoforms in the fragments of anterior lens capsules (ALCs) and peripheral blood mononuclear cells (PBMCs) of pediatric patients with congenital and traumatic cataracts. METHODS: Forty children with congenital cataracts (19 girls and 21 boys) and 22 children with traumatic cataracts (six girls and 16 boys) participated in the study. Fragments of ALCs obtained during cataract surgery and whole blood samples were analyzed. Quantification of TGFß1, TGFß2, and TGFß3 mRNA was performed by real-time quantitative reverse transcription (QRT)-PCR using SYBR Green I chemistry. RESULTS: TGFß1, TGFß2, and TGFß3 mRNA was detected in all the studied samples. Significant differences were found for TGFß1 and TGFß2 expression profiles in PBMCs between the patients with congenital and traumatic cataracts. The expression profiles of TGFß isoforms in ALCs did not differ significantly between the groups. CONCLUSIONS: Overexpression of TGFß1 and TGFß2 in the PBMCs of patients with congenital cataracts might indicate that these cytokines are involved in the development of lens opacity.

Etiopathogenesis and management of high-degree myopia. Part I.

High-degree myopia (HM) is defined as a refractive error above -6 D (-8 diopters). An axial eyeball length above 26 mm and is also connected with excessive myopisation. Mechanical stretching and thinning of the choroids may lead to the development of irreversible degenerative changes in the eyeball and visual impairment. HM prevalence varies significantly by geographical area, ranging from 1.7-3.3%, while in highly urban regions of South-East Asia it affects up to 24% of university students. HM is not a homogenous disease. It is considered to be inherited in different patterns: dominant, autosomal recessive, and X-linked. Many genetic mutations linked to HM development have been described and the different patterns of inheritance may reflect different types of HM. Moreover, the clinical state of the HM eyeball may result from both genetic and environmental risk factors influencing the progression of the refractive error. The development and progression of HM is connected with sustained prolonged near work involving accommodation-convergence imbalance leading to retinal blur, the years spent at school, the amount of near work, especially writing and reading, which is related to slight chronic under-accommodation, and improper light stimuli in early childhood. Contemporary definitions of HM, views on etiopathogenesis, excessive axial elongation, and pathological changes within the eyeball in the course of HM leading to visual impairment are presented here. Genetic and environmental risk factors influencing the progression of HM, its significantly different prevalence in various geographical areas, and therapeutic options for the refractive error are also presented.

Etiopathogenesis and management of high myopia. Part II.

High myopia (HM) is defined as refractive error above -6.0 D (-8.0 diopters) with axial eyeball length above 26 mm, and is connected with the process of excessive myopisation. HM is not a homogenous disease. HM is considered to be inherited in 3 different patterns: dominant, autosomal recessive, and X-linked. Many genetic mutations linked to the development of HM have been described, including high grade myopia, and MYP1-16; different patterns of inheritance may reflect different types of HM. Over 150 genetic syndromes are associated with HM. The clinical state of the HM eyeball may also depend on environmental risk factors influencing the progression of refractive error. The complexity of etiopathogenesis makes it difficult to distinguish to what extent the development of HM is related to genetics versus exposure to environmental factors. HM remains a leading cause of visual loss. HM and its complications are considered to be one of the most significant causes of blindness and visual impairment in young, professionally active people, becoming an important social problem. Contemporary data concerning genetic factors, family aggregation, and epidemiological data of visual impairment connected with HM are presented in this paper. Therapeutic options for this refractive error are presented as well.

[Gaucher's disease--ocular manifestation and treatment]. / Choroba Gauchera--objawy i leczenie.

Gaucher's disease is the most common lysosomal storage disorder due to a deficiency of glukocerebrosidase activity. It leads to an accumulation of glukosylceramide within the cells of the the reticuloendothelial system. Gaucher's disease is divided into three subtypes based on clinical symptoms. Type I--nonneuronopathic--chronically progressive in adulthood, type II--acute neuronopathic--infantile form lead up to the difficult damage nervous system, and type III--juvenile form--subacute neuronopathic. The aim of this paper is to present the typical ocular symptoms which occured in the disease. Gaucher's disease is the lysosomal storage disorder which is treated by enzyme replacement therapy.

[Variability of the eyeballs axial length in children with pseudophakia]. / Zmiennosc dlugosci osiowej pseudofakijnych galek ocznych u dzieci.

PURPOSE: The aim of the study is to evaluate the dynamics of axial elongation of pseudophakic eyes and changes in refraction pseudophakic eyes in children after monocular or binocular cataract surgery. MATERIAL AND METHODS: the observations of 79 children (158 eyes) aged from 4 to 18 years (mean 9.7 +/- 0.55) after cataract surgery were conducted. The examined group consisted of 105 pseudophakic eyes, the comparative group consisted of 53 eyes without surgery in the same observed group of children. Moreover, the patients were evaluated in the following group: moncular - binocular cataract, primary or secondary IOL implantation, the age in groups were between 4-7 and between 8-18 years. Analysis statistically: STATGRAPHICS and SIMSTAT programs, p (alpha) = 0.05. RESULTS: In the examined group (105 eyes) the mean follow-up time was 4.2 years (+/- 0.3), the average age of patients was 9.7 years (+/- 0.7). Improvement of visual acuity was achieved mean 0.5 in 52.4% post operative eyes observed, myopic shift was -0.7D (+/- 0.52). The anatomic eyeball length increased up to 0.5mm (+/- 0.27). The average age of patients of the control group was 9.7 years (+/- 0.83), follow-up 4.3 years (+/- 0.44), visual acuity 0.8 (+/- 0.03). Mean elongation of the axial length was 0.56 mm(+/- 0.26), mean refraction of the eyeball was +0.38 D (+/- 0.54). The difference of the changes of refraction between examined and control group was statistically significant. The comparison of final refraction in optical pathway (p = 0.32) and the growth of anatomic eyeball length (p = 0.14), proved no significant differences in the group with monocular and binocular IOL. The comparative analysis of final refraction in optical pathway (p1 = 0.36), and the growth of anatomic eyeball length (p2 = 0.26) in the group with primary or secondary IOL and in the younger or older children (p1 = 0.52; p = 0.98) in the both groups, did not significantly differ. CONCLUSIONS: The dynamics of axial elongation of pseudophakic and phakic eyes in children is similar. The myopic shift of pseufophakic eyes is bigger than in phakic eyes. It should be undertaken in calculation of the refractive power of intraocular lenses.

[Vernal keratoconjunctivitis in children]. / Wiosenne zapalenie rogówek i spojówek u dzieci.

PURPOSE: To evaluate the course of palpebral type of vernal keratoconjunctivitis (VKC) and relation of the number of recurrences per year, to patients age and to estimate the frequency of irreversible corneal transparency changes and visual acuity decrease. MATERIAL AND METHODS: There were 34 eyes of 17 children, 13 boys (76.4%) included 2 brothers and 4 girls. The age of patients was from 5 to 14 years, mean 9.1. All patients were hospitalized because of pathological corneal changes. Follow up from 2 to 13 years (mean 8.4). The number of recurrences ranged from 2 to 5 per year and there was not correlation between age of children and frequency of episodes. RESULTS: We have observed punctate keratopathy (79.14% of cases) and corneal ulcers (26.5% of cases). In 25 eyes of 13 patients cryocoagulation and/or excision of giant palpebral papillae were done. Visual acuity ranged from 0.01 to 1.0 and mean value before treatment was 0.879 +/- 0.09 and after 0.884 +/- 0.10. The difference was not statistically significant (p = 0.878). CONCLUSIONS: (1) The course of VKC is recurrent, the number of recurrences per year does not depend on the age of patients, but is correlated with giant papillae of palpebral conjunctiva presence. (2) Large papillae should be excised, in order to make the healing of cornea quicker. (3) Permanent visual function decreasing is not frequent because partial leucoma is localized at the periphery of cornea.

[Prognosis in juvenile glaucoma after trabeculectomy]. / Rokowanie w jaskrze mlodzienczej po trabekulektomii.

PURPOSE: To evaluate the efficacy of trabeculectomy in the patients with juvenile glaucoma in the long-term follow-up. MATERIAL AND METHODS: In 22 of 37 patients--41 trabeculectomies with basal iridectomy were performed. The age of children ranged from 6 to 18 years, mean 12.4 years. In one eye of 15-year-old boy we used Mitomycin C during reoperation. The follow-up period ranged from 2 to 14 years, mean 8 years. Patients were divided into 2 groups depending on the follow-up: I group (19 eyes) was observed 2-6 years, mean 5.1 years, II group (18 eyes) 7-14 years, mean 11.1 years. In the I group 57.9% and in the II group 61% of children were myopic. RESULTS: Intraocular pressure lower or equal to 21 mmHg with no glaucoma medication was obtained in 79% of eyes in the I group and 66.7% of eyes in the II group. The difference was not statistically significant. In other eye local application of B-blockers and dorzolamid decreased intraocular pressure to values safe for the optic nerve, what was monitored by visual acuity and laser scanning tomography. CONCLUSION: Prognosis of juvenile glaucoma patients treated with trabeculectomy is satisfactory but the effectiveness of this procedure decreases together with the elongation of the follow-up period.

[Central corneal thickness and intraocular pressure in the youth]. / Grubosc centralnej czesci rogówki a pomiary cisnienia wewnatrzgalkowego u mlodziezy.

PURPOSE: To estimate the central corneal thickness in young patients and to evaluate the relationship between the intraocular pressure (IOP) and pachometry. PATIENTS AND METHODS: Eighty eyes of 40 children at the age from 9-18 years, mean 13.75 were examined. The applanation tonometry, pachometry, refraction and examination of corneal diameter were done. To statistical purpose t-Student test was used alpha = 0.05. RESULTS: IOP ranged from 12 to 24 mmHg, in 32 eyes was above 17 mmHg, mean 20.2 +/- 1.46 mmHg (I group) and in 48 eyeballs ranged from 12 to 17 mmHg, mean 15.6 +/- 0.96 mmHg (II group), p = 0. In the I group pachometry was from 480 to 591 microm, mean 542 +/- 11.1 and in the II group from 451 to 570 microm mean 521.7 +/- 9.39 p = 0.006. Mean refractive error in the I group was -0.4 D (from -5.0 to +4.0 D) and in the II group mean -0.8 (from -6.25 to +4.75), p = 0.45. In the both groups corneal diameter was from 11.5 to 12.5 mm. Comparing eyeballs of patients at similar age, refraction and corneal diameter, it was shown that central corneal thickness in the group with higher IOP was statistically significantly bigger than in the group II. CONCLUSION: Measuring IOP in children we have to take into account the corneal thickness.

[Optic nerve disc in unilateral myopia in children]. / Obraz tarczy nerwu wzrokowego w krótkowzrocznosci jednostronnej u dzieci.

PURPOSE: The evaluation of biomorphometric parameters of optic nerve head of the eyes in myopic anisometropia, to state if glaucoma similar changes described in adults were present. MATERIAL AND METHODS: 17 children, 14 girls and 3 boys at the age from 7-18 years, mean 12.6 years with unilateral myopia of mean value 8.6 D. The parameters of optic nerve disc: total contour area (TCA), cup/disc ratio (C/D), neuroretinal rim area (Rim), volume of neuroretinal rim (Vol. A), cup volume (Vol. B), maximum slope (MSL) and average slope angle (ASL) were examined using laser scanning ophthalmoscope in the TopSS system of Laser Diagnostic Technologies Inc. The I group consists of myopic eyes, in the II group there were emmetropic eyes of the same children. RESULTS: Refraction in the I group ranged from -4.5 to -12 D, mean -8.6 D, in the II group from 0 to + 1.0 D, mean 0.07 D. The mean axial length of eyeballs was 25.57 SD 1.9 mm in the I and 22.47 SD 1.09 mm in II group--the difference was significant. There was not statistically significant difference between TCA in the I (mean 1.19 SD 0.75 mm2) and II group (mean 2.13 SD 0.49 mm2). Mean C/D ratio and cup volume were significantly smaller in myopic (C/D 0.23 SD 0.16 Vol. B -0.06 SD 0.05) than in emmetropic eyes (C/D 0.33 SD 0.11 mm3, Vol. B -0.18 SD 0.14 mm3). CONCLUSIONS: Optic nerve disc images in myopic and glaucomatous eyes in children are different. Smaller optic disc cup in myopic than in emmetropic eyes can show, that children myopic eyeballs enlarged without nerve fibers atrophy.