Examination of Subbasal Nerve Plexus and Central Corneal Stromal Microstructure in Subjects With Congenital Aniridia, Using in Vivo Confocal Laser Scanning Microscopy.

PURPOSE: During life up to 70% of aniridia subjects develop aniridia-associated keratopathy (AAK). AAK is characterized by limbal stem cell insufficiency, impaired corneal epithelial cell differentiation and abnormal cell adhesion, which leads to centripetal spreading vascularization, conjunctivalization, and thickening of the cornea. Our aim was to examine the subbasal nerve plexus and central corneal stromal microstructure in subjects with congenital aniridia, using in vivo confocal laser scanning microscopy CLSM. METHODS: 31 eyes of 18 patients (55.6% males, mean age: 25.22 ± 16.35 years) with congenital aniridia and 46 eyes of 29 healthy subjects (41.4% males, mean age 30 ± 14.82 years) were examined using the Rostock Cornea Module of Heidelberg Retina Tomograph-III. At the subbasal nerve plexus, corneal nerve fiber density (CNFD), corneal nerve fiber length (CNFL), corneal total branch density (CTBD), and corneal nerve fiber width (CNFW) were analyzed using ACCMetrics software. Keratocyte density in the anterior, middle and posterior stroma was assessed manually. RESULTS: The CNFD (2.02 ± 4.08 vs 13.99 ± 6.34/mm2), CNFL (5.78 ± 2.68 vs 10.56 ± 2.82 mm/mm2) and CTBD (15.08 ± 15.62 vs 27.44 ± 15.05/mm2) were significantly lower in congenital aniridia subjects than in controls (p < 0.001 for all). CNFW was significantly higher in aniridia subjects than in controls (0.03 ± 0.004 vs 0.02 ± 0.003 mm/mm2) (p = 0.003). Keratocyte density was significantly lower in all stromal layers of aniridia subjects than in controls (p < 0.001 for all). Stromal alterations included confluent keratocytes, keratocytes with long extensions and hyperreflective dots between keratocytes in aniridia. CONCLUSIONS: Decrease in CNFD, CNFL, and CTBD, as well as increase in CNFW well refer to the congenital aniridia-associated neuropathy. The decreased keratocyte density and the stromal alterations may be related to an increased cell death in congenital aniridia, nevertheless, stromal changes in different stages of AAK have to be further analyzed in detail.

[Congenital aniridia patients' experience on their visual impairment in Hungary.] / Veleszületett aniridiás betegek látáskárosodással kapcsolatos tapasztalatai Magyarországon.

INTRODUCTION: Aniridia is a rare congenital panocular disease associated with varying degrees of visual acuity impairment. OBJECTIVE: To assess the experiences of congenital aniridia patients in Hungary, with visual impairment using a questionnaire developed by the ANIRIDIA-NET. PATIENTS AND METHOD: Patients completed the Hungarian version of the 20-item ANIRIDIA-NET questionnaire with our assistance. The questionnaire covered demographic data, the most common complaints caused by the disease, the difficulties caused by low vision in different life situations and the frequency of low vision aids used in daily life. RESULTS: 33 subjects (17 female [51.51%] and 16 male [48.48%]), 16 (48.5%) children and 17 (51.5%) adults completed the questionnaire, with an age of 25.69 ± 17.49 years (5-59 years). Daily photosensitivity was reported by 27 (81.8%), dry eyes by 5 (15.2%), tearing by 4 (12.1%), fluctuating vision by 3 (9.1%), and eye pain by 2 (6.1%) subjects. The majority of respondents said that personal communication with schoolmates (16 [48.5%]) or colleagues at work (11 [33.3%]) never caused difficulties because of their visual impairment. 29 people (87.9%) never needed help with daily routines at home, 24 (72.7%) with getting to school/work and 17 (51.5%) with various activities. 29 people (87.8%) never used low vision aids for communication, 23 (69.7%) for travelling, 20 (60.6%) for participating in social activities, 18 (54.5%) for studying/work. CONCLUSION: Although aniridia is associated with reduced visual acuity, the majority of people with congenital aniridia, especially in childhood, manage to cope with personal communication and various life situations without difficulty, despite their eye complaints. Low vision aids can be an important aid for them as they grow into adulthood and as they age. Orv Hetil. 2023; 164(34): 1342-1349.

[Staging of aniridia-associated keratopathy]. / Az aniridiához társult keratopathia stádiumbeosztása.

INTRODUCTION: Congenital aniridia is a rare panocular disease that affects almost all eye structures leading in most patients to reduced visual acuity. Ophthalmological signs include aniridia-associated keratopathy, secondary glaucoma, cataract, macular and optic nerve head hypoplasia, nystagmus. Although the term aniridia-associated keratopathy has long been used in the literature, various staging proposals have been described. OBJECTIVE: To analyze aniridia-associated keratopathy stages, using available literature classifications, in patients with aniridia in Hungary. PATIENTS AND METHODS: We examined 65 eyes of 33 patients with congenital aniridia (age: 25.69 ± 17.49 [5-59] years, 17 females [51.51%]). We recorded the corneal status by slit-lamp examination and classified the corneal abnormalities according to the Mackman, Mayer, López-García and Lagali staging. RESULTS: According to Mackman's classification, 8 eyes (12.3%) were in stage 0, 0 eye in stage 1A, 38 eyes (58.46%) in stage 1B and 19 eyes (29.23%) in stage 2. According to Mayer, stage I included 8 eyes (12.3%), stage II 38 eyes (58.46%), stage III 5 eyes (7.7%), stage IV 7 eyes (10.77%) and stage V 7 eyes (10.77%). In López-García's classification, 8 eyes (12.3%) could not be grouped, 20 eyes (30.77%) were in stage 1, 18 eyes (27.7%) in stage 2 and 19 eyes (29.3%) in stage 3. Lagali's classification included 8 eyes (12.3%) in stage 0, 20 eyes (30.77%) in stage 1, 18 eyes (27.7%) in stage 2, 5 eyes (7.7%) in stage 3 and 14 eyes (21.54%) in stage 4. CONCLUSION: We recommend using Lagali's staging scheme for aniridia-associated keratoptahy due to its ease of use, detailed progression assessment, and treatment planning. In stage 1 according to Lagali, blood vessels cross the limbus by up to 1 mm, in stage 2 the central 2-3 mm of the corneal area is spared of blood vessels. When the blood vessels reach the center of the cornea, it is stage 3, followed by opaque, uneven corneal pannus in stage 4. Orv Hetil. 2023; 164(27): 1063-1069.

[The impact of district nurses in screening visual impairments]. / A védonok szerepe a gyermekkori látásproblémák felismerésében.

INTRODUCTION: The children's physical and mental health is determinative for the whole society. Parents, teachers, district nurses, and doctors share the duty to ensure their uninterrupted visual development. District nurses compose key elements in connecting families and health care providers, since they have the chance to detect any visual anomalies, and to refer the children to a pediatrician, ophthalmologist, or a neurologist. Their team work yields advantages to the whole community as beyond preserving good vision, children get a real chance of right physical and mental development. OBJECTIVE: Our aim was to recollect district nurses' areas of activity and to collect data about their present status in visual screening programs. Furthermore, to assess their screening methods and circumstances, and to reshape their continuous learning programs linked to the universities. METHOD: Questionnaires were sent online anonymously to district nurses in practice, in order to collect data referring to their focuses during the visual screening methods. Administration of online parental tutorials was assessed, and measures of professional support in nurses' education was debated. RESULTS: Visual screening among infants looks for potential strabismus, and among school-age children looks for potential refractive errors. Vision tests and stereo tests are usually available as screening tools, but pen lights are underused. Only a few nurses suggest online tutorials to enhance patient education, but nurses are highly motivated in postgraduate courses about vision screening. CONCLUSION: Our results reflect good adherence to the screening protocols but there is some uncertainty in some fields. In harmony with the results, adequate technical and educational support and specific training were compiled for postgraduate district nurses. Coaching and training in visual screening (in the form of seminars or e-learning) should be available in spring 2023. Orv Hetil. 2023; 164(3): 88-95.

[Congenital aniridia - Hungarian data of a spectrum disease]. / Congenitalis aniridia ­ egy spektrumbetegség magyarországi adatai.

INTRODUCTION: Congenital aniridia is a rare disease, characterised by the complete or partial absence of the iris, but lesions may be present in all structures of the eye. OBJECTIVE: To determine the prevalence of ocular diseases in congenital aniridia by analyzing patients from a Hungarian centre. PATIENTS AND METHODS: Patients at the Department of Ophthalmology of Semmelweis University, examined between October 2005 and May 2022, have been included. After taking the patients' medical history, a detailed ophthalmological examination has been performed. RESULTS: Of the 82 patients in the database, 33 (age 25.69 ± 17.49 [5-59] years, 17 females [51.51%]) presented for examination and 65 eyes were examined. Nystagmus was found in 45 eyes of 23 patients (69.23%), and the patients' uncorrected distance visual acuity was 0.14 ± 0.128 (0.9 logMAR; 0.63-0.005). The aniridia-associated keratopathy was Grade 0 in 8 eyes (12.3%), Grade 1 in 10 eyes (15.38%), Grade 2 in 16 eyes (24.62%), Grade 3 in 4 eyes (6.15%) and Grade 4 in 25 eyes (38.46%). 30 eyes (46.15%) of 15 patients had secondary glaucoma, 6 eyes (9.2%) of 3 patients were glaucoma suspect. 8 eyes (12.3%) had a clear lens, 44 eyes (67.69%) had cataract, of which 22 (33.84%) were anterior cortical polar cataracts. 13 eyes (20%) were pseudophakic (PCL) and 7 eyes (10.77%) had lens dislocation or zonular insufficiency. Macular hypoplasia was found in 6 eyes of 3 patients (4.6%) and optic nerve head malformation in 2 eyes of 1 patient (3.03%). CONCLUSION: The ocular signs of congenital aniridia are aniridia-associated keratopathy, secondary glaucoma, cataract, macular and optic nerve head hypoplasia. Systematic collaboration of different ophthalmological specialties is required for the management and care of all these ocular abnormalities. Orv Hetil. 2023; 164(4): 148-155.

Axenfeld­Rieger syndrome: ophthalmological and dental diagnostic and therapeutic options / Axenfeld-Rieger-szindróma: szemészeti és fogászati diagnosztikai és kezelési lehetoségek.

Összefoglaló. Az Axenfeld-Rieger-szindróma ritka betegség. A közlemény bemutatja a klinikai megjelenési formáit, a diagnosztikus és terápiás lehetoségeket. A szemgolyó elülso szegmentumát érinto fejlodési rendellenességek vizsgálata a hagyományos biomikroszkópos vizsgálat mellett digitális kamerával is történhet, mely a csarnokzugi képleteket nagy nagyításban, éles képet mutatva tudja megjeleníteni. Az elülso szegmentum leképezését segíto optikaikoherencia-tomográfia és ultrahang-biomikroszkópia a fejlodési rendellenességnek és a csarnokvíz-elvezeto sönt tubusának vizsgálatára, megjelenítésére is alkalmas. A szemnyomást a gyermek kooperációjának függvényében többféle módon mérhetjük. A szabálytalan alakú, sokszor csak résnyi pupilla, valamint a szemnyomás-emelkedés miatt szemészeti beavatkozás lehet szükséges. A korai pupillaképzés az amblyopia megelozését, az antiglaucomás mutétek (trabeculectomia, hosszú tubusú sönt implantációja) pedig a látási funkciók megorzését szolgálják. A szemészeti mutéteket akár már néhány hónapos korban szükséges elvégezni altatásban. A maxillahypoplasia és a fogászati fejlodési anomáliák miatt kétirányú panoráma-röntgenfelvétel szükséges. Többlépcsos fogászati konzervatív és restoratív kezelés hozhat eredményt. A diagnózis felállítása és a kezelés is multidiszciplináris megközelítést igényel. Gyermekgyógyász, genetikus, kardiológus, fogász-szájsebész és gyermekszemész együttes munkája biztosíthat megfelelo eredményt. Orv Hetil. 2021; 162(5): 192-199. Summary. Axenfeld-Rieger syndrome is a rare disease. Our paper presents its clinical manifestations, diagnostic and therapeutic options. Due to maxillary hypoplasia and dental developmental anomalies, bidirectional panoramic radiography is required. Multi-stage dental conservative and restorative treatment can provide better results. In addition to traditional biomicroscopic examination, developmental abnormalities affecting the anterior segment of the eye can also be examined with a digital camera, which can display the angle of the anterior chamber at high magnification, with a sharp image. Anterior segment optical coherence tomography and ultrasound biomicroscopy are also suitable for the examination and display of developmental abnormalities and drainage shunt tubes. Intraocular pressure can be measured in several ways depending on the child's cooperation. Due to the irregular shape of the pupil, often with only a slit aperture, and an increase in intraocular pressure, ophthalmic intervention may be required. The pupilloplasty is important preventing amblyopia prevention and early glaucoma surgery (trabeculectomy, shunt implantation) helps to preserve visual function. Eye surgeries need to be performed under anaesthesia, sometimes at few months of age. Both diagnosis and treatment require a multidisciplinary approach. The joint work of a paediatrician, geneticist, cardiologist, dental-oral surgeon and paediatric ophthalmologist may provide a satisfactory result. Orv Hetil. 2021; 162(5): 192-199.

Preoperative biometry data of eyes with unilateral congenital cataract.

PURPOSE: To investigate the differences in biometry data of eyes with unilateral congenital cataract and the contralateral normal eyes in pediatric patients. SETTING: Pediatric Ophthalmology Division, Ophthalmology Department, Semmelweis University in Budapest, Hungary. DESIGN: Retrospective case series. METHODS: Data of visually significant unilateral congenital cataract patients who had cataract surgery in the ophthalmology department at Semmelweis University between 2013 and 2016 were collected. At the time of the examinations, the mean age of the patients was 36.4 weeks ± 25.3 (SD). Central corneal thickness (CCT), corneal refractive power (keratometry [K]), horizontal corneal diameter, and axial length (AL) measurement data were obtained from both eyes of each patient. The measurements were taken under general anesthesia using a handheld kerato-refractometer (Retinomax K-plus 3) and an ultrasound instrument (Ocuscan RxP) with contact applanation method and Castroviejo straight-tip calipers at the beginning of the cataract surgery. For statistical evaluation, Originlab 7.0 software was used; paired t tests were performed for the difference analysis between the 2 sides. RESULTS: Forty-two infants (50% girls) were included. In the cases of eyes with unilateral congenital cataract, a greater CCT (P = .01330), higher average K (P = .00243), and smaller corneal diameter (P = .00010) were found, although there was no significant difference in AL when compared with the unaffected contralateral eyes. CONCLUSION: The data showed that biometric characteristics of the eyes with unilateral congenital cataract differ from the opposite normal eye before the cataract surgery. It is essential to use this biometric data in intraocular lens power calculation and to take them into account in long-term care when screening for secondary glaucoma.

Starflo glaucoma implant: early experience in Hungary.

AIM: To present the early experience with the implantation technique, safety and efficiency of STARflo device for open angle glaucoma (OAG). METHODS: referring intra- and postoperative clinical experience with a series of seven cases in three glaucoma centers in Hungary. RESULTS: No intraoperative complications were observed. Postoperative inflammatory signs disappeared rapidly. The mean IOP reduction was from 27.6 ± 5.0 mmHg to 18.9 ± 3.4 mmHg (32% reduction/ patient) at six months postoperatively. CONCLUSION: STARflo implant was safe and (except for one case with neovascular glaucoma) effective in our cases. The learning curve for experienced anterior segment surgeons was short.

Comparison of the current and a new RTVue OCT software version for detection of ganglion cell complex changes due to cataract surgery.

The purpose was to compare the current (6.3) and a novel software version (6.12) of the RTVue-100 optical coherence tomograph (RTVue OCT) for ganglion cell complex (GCC) and retinal nerve fibre layer thickness (RNFLT) changes after phacoemulsification in healthy cataract eyes, and to investigate whether version 6.12, in which image segmentation is improved, provides benefits over version 6.3 for RNFLT and GCC imaging via mild cataract. One eye of 22 consecutive healthy cataract patients were imaged before and 1 month after uncomplicated cataract surgery using RTVue-100 OCT software version 6.3. The images were analysed with both software versions. Signal strength index increased significantly after surgery for both RNFLT and the GCC measurements (p ≤ 0.0015). No difference was seen for any RNFLT parameter between the software versions and time points (p ≥ 0.0140). The GCC values did not differ between the versions either before or after surgery (p ≥ 0.4471), but all increased significantly after surgery with software version 6.12 (p < 0.0001). Neither focal loss volume (FLV) nor global loss volume (GLV) differed between the software versions before and after surgery, respectively, but GLV decreased (improved) significantly after surgery (p = 0.010 and <0.001 for versions 6.3 and 6.12, respectively). Cataract surgery induced similar changes with both software versions, but version 6.12 identified the increase of GCC thickness and the decrease of GLV better than the current version. Although no significant difference between software versions was seen before surgery, our results suggest that version 6.12 may be more precise in measuring GCC parameters than the currently available version.

The role of complement activation in the pathogenesis of Fuchs' dystrophy.

PURPOSE: Inflammation can be an etiologic factor of Fuchs' dystrophy according to previous studies. Our aim was to analyse the activation of the complement system in the aqueous humor in this pathological condition. METHODS: 100 µl aqueous humor sample was taken during keratoplasty of 11 Fuchs' dystrophic patients and during phacoemulsification surgery of 18 control patients. The samples were mixed with EDTA and stored at -80 °C. Concentrations of C1rC1sC1Inh and C3bBbP complexes as markers of the activation of the classical and alternative complement pathways, respectively, were measured with ELISA method. The results of the patient group and the control group were compared with statistical analysis (non-parametric Mann Whitney test). RESULTS: Both the concentrations of C1rC1sC1Inh [4.3 (3.2-20.2)AU/ml] and of C3bBbP [15.3 (7.8-22.6)AU/ml] were significantly higher in the Fuchs' dystrophic group than in the control group [C1rC1sC1Inh: 0.0 (0.0-5.6)AU/ml, C3bBbP: 1.4 (0.0-7.8)AU/ml]. The median value is shown along with the (25% and 75% percentiles). CONCLUSIONS: Based on our results, the complement system may be activated both through the classical and alternative pathways in the aqueous humor of the patients with Fuchs' dystrophy.

Complement activation in the aqueous humor of pseudophakic bullous keratopathy patients.

BACKGROUND/AIMS: According to some studies, inflammation is a potential etiological factor in pseudophakic bullous keratopathy (PBK). Our aim was to obtain information on the activation of the complement system in the aqueous humor in this disorder. METHODS: Aqueous humor samples were collected during keratoplasty of 12 PBK patients, as well as during phacoemulsification surgery of 18 control patients. The concentrations of the protein-protein complexes generated during complement activation (C1rC1sC1inh and C3bBbP) through the classical and alternative pathways, respectively, as well as of the C3 cleavage product C3a, were measured with ELISA methods. The correlation among the complement factors and between the duration of the edema, the stage of the disease, and the level of the complement activation products was examined. RESULTS: The concentration of C1rC1sC1inh, C3bBbP complex and C3a was significantly higher in the PBK group (p < 0.001) compared to the control group. In PBK patients, a correlation was found between the levels of the C1rC1sC1inh complex and C3a only. CONCLUSION: Our new findings indicate that in PBK the complement system is activated - via the classical pathway - in the aqueous humor. The activated complement may play a role in increased endothelial cell loss.

Increased tear osmolarity in patients with severe cases of conjunctivochalasis.

PURPOSE: To evaluate and compare tear osmolarity in different grades of conjunctivochalasis. PATIENTS AND METHODS: Forty subjects divided into four groups based on grading of conjunctivochalasis by lid-paralell conjunctival fold (LIPCOF) test and tear osmolarity evaluated by TearLab osmometer. Schirmer test, tear film break up time (BUT) and vital staining were also performed. RESULTS: Patients with mild and moderate conjunctivochalasis showed similar tear osmolarity to normal subjects. A significant increase (P < 0.008) of osmolarity was found in patients with severe conjunctivochalasis. A significant increase of osmolarity was found in patients with abnormal BUT (P = 0.018) and in patients with positive lissamine-green staining (P = 0.023). No association could be elicited when results of osmolarity were compared to those of Schirmer test or when compared those of fluorescein staining to osmolarity, respectively. CONCLUSION: Hyperosmolarity of tear was reported as a fact of ocular surface inflammation. While in mild and moderate conjunctivochalasis tear osmolarity was similar to normal controls, increased osmolarity can refer to the presence of ocular surface inflammation in these patients with severe conjunctivochalasis.

Bacterial contamination in the anterior chamber after povidone-iodine application and the effect of the lens implantation device.

PURPOSE: To assess the incidence of anterior chamber bacterial contamination during cataract surgery, and compare results of injector implantation and forceps implantation of foldable intraocular lenses (IOLs). SETTING: Department of Ophthalmology and Institute of Medical Microbiology, Semmelweis University, Budapest, Hungary. METHODS: This prospective randomized controlled clinical study comprised 97 eyes of 96 patients. Antibiotic eyedrops were not used; however, povidone-iodine 10% solution was used to prepare the eyebrow and eyelids and povidone-iodine 5% to disinfect the ocular surface. A Steri-Drape (3M) was used to surround the eye. Aqueous fluid samples were aspirated from the anterior chamber at the beginning and the end of surgery. The samples were cultured for 14 days under aerobic and anaerobic conditions simultaneously. Cataract surgery was performed using a sutureless, superotemporal, clear corneal phacoemulsification technique. The IOL was implanted with an injector (n = 47) or a forceps (n = 50), with the instrument randomly selected. The frequency of positive bacterial cultures with each implantation method was compared using the Fisher exact test. RESULTS: Bacteria were found in the conjunctival samples in 21 eyes (21.65%) before povidone-iodine application and in 4 eyes (4.12%) after disinfection. The anterior chamber sample before surgery was culture positive for Staphylococcus epidermidis in 2 eyes and for Micrococcus luteus in 1 eye. After surgery, the culture was positive for S epidermidis in 1 eye (2.15%) in the injector group and 1 eye (2.00%) in the forceps group (P = .74). Neither sample came from an eye that had a positive culture preoperatively. There were no intraoperative complications. CONCLUSIONS: In uneventful clear corneal phacoemulsification, meticulous technique can prevent antibiotic use during surgery. No difference in anterior chamber bacterial contamination was found between IOL implantation using an injector or a forceps.