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To derive a mathematical expression for the healthy upper eyelid (UE) contour and to use this expression to differentiate the normal UE curve from its abnormal configuration in the setting of blepharoptosis.The study was designed as a cross-sectional study. Fifty healthy subjects (26M/24F) and 50 patients with blepharoptosis (28M/22F) with a margin-reflex distance (MRD1) of ≤2.5 mm were recruited. A polynomial interpolation was used to approximate UE curve. The polynomial coefficients were calculated from digital eyelid images of all participants using a set of operator defined points along the UE curve. Coefficients up to the fourth-order polynomial, iris area covered by the UE, iris area covered by the lower eyelid and total iris area covered by both the upper and the lower eyelids were defined using the polynomial function and used in statistical comparisons. The t-test, Mann-Whitney U test and the Spearman's correlation test were used for statistical comparisons.The mathematical expression derived from the data of 50 healthy subjects aged 24.1 ± 2.6 years was defined as y = 22.0915 + (-1.3213)x + 0.0318x(2 )+ (-0.0005x)(3). The fifth and the consecutive coefficients were0.00001 in all cases and were not included in the polynomial function. None of the first fourth-order coefficients of the equation were found to be significantly different in male versus female subjects. In normal subjects, the percentage of the iris area covered by upper and lower lids was 6.46 ± 5.17% and 0.66% ± 1.62%, respectively. All coefficients and mean iris area covered by the UE were significantly different between healthy and ptotic eyelids.The healthy and abnormal eyelid contour can be defined and differentiated using a polynomial mathematical function.

AIM: To investigate and compare the cytopathological and clinical effects of amniotic membrane transplantation (AMT) and oral mucosal membrane transplantation (OMMT) in socket contraction. METHODS:Twelve patients who could not be fitted with ocular prosthesis due to socket contracture were included in this study. Seven patients underwent AMT and 5 patients underwent OMMT. Thirteen patients who had healthy sockets were included as control group. Depth of inferior fornix, degree of inflammation, extent of the socket contracture and socket volume were measured in the preoperative period and at sixth and twelfth weeks postoperatively. Impression cytology of conjunctival fornices and tear transforming growth factor beta-1 (TGFβ1) levels were determined. RESULTS:In the AMT group,socket volume and lower fornix depth values were significantly higher (P=0.030 and P=0.004 respectively) and inflammation levels and impression cytology stages (P=0.037 and P=0.022 respectively) were significantly lower in postoperative period compared to preoperative period. In the OMMT group, no statistical differences were found in terms of clinical parameters, inflammation levels and impression cytology stages of preoperative versus postoperative values. Preoperative tear TGFβ1 levels were higher in AMT and OMMT groups compared to the control group (25.5 ng/mL, 26.3 ng/mL and 21.7 ng/mL respectively). Decreased tear TGFβ1 levels were observed in both the AMT and OMMT groups postoperatively (median decrease value=2.1 ng/mL and 2.7 ng/mL respectively). CONCLUSION: AMT is associated with postoperative improvement in inferior fornix depth, socket volume, inflammation and impression cytology levels and may be a more proper alternative method than OMMT in the management of socket contracture.

Purpose: The aim of this study was to compare the RNFL thickness in eyes with myelinated retinal nerve fibers (MRNF) and age, sex-matched controls using scanning laser polarimetry. Methods: Seventeen patients with MRNF underwent a comprehensive ophthalmological examination including automated visual field testing, and scanning laser polarimetry with Nerve Fiber Analyser (NFA II). Twenty eyes with MRNF from 17 patients were available for the study. Sixteen healthy, age and sex-matched subjects were taken as controls. Retinal nerve fiber thickness measurements of the four sectors (superior, inferior, nasal, temporal) and along the whole circumference of the optic disc were obtained for affected and control eyes, and compared. Results: Inferior average and inferior integral parameters of the NFA revealed significantly thinner RNFL thickness measurements in eyes with inferiorly located MRNF compared to controls. Conclusion: Retinal myelination alters the birefringent property of the RNFL, and results in diminished thickness measurements. Hence, NFA measurements in patients with MRNF may not be reliable.

Ligneous conjunctivitis (LC) is a rare disorder characterized by a chronic course of recurrent membranous lesions at conjunctivae. Pseudomembranes of other mucous membranes have been reported in patients with LC, but to the best of our knowledge, no case with alveolar involvement has been described. Here, we report a 2.5-year-old girl with LC who had tracheoaveolar involvement and homozygous type I plasminogen deficiency. Tracheal involvement was diagnosed by bronchoscopic biopsy and alveolar involvement with postmortem biopsy. She was shown to be homozygous for frameshift mutation in plasminogen exon 14 (Gly565ins-G) with molecular genetic examination of DNA which was obtained from parafin embedded postmortem lung tissue. Ligneous inflammation of the upper and lower respiratory tract must be considered in children with LC and recurrent respiratory tract problems.

PURPOSE. To compare topographic indices of surface regularity in dry eye patients and in normal subjects (controls) and to investigate the short-term effect of lacrimal punctal plugs on these indices in dry eye patients. METHODS. The surface regularity index (SRI) and surface asymmetry index (SAI) of the TMS-2 corneal topographic modelling system were used to evaluate corneal surface regularity in 20 eyes of 10 dry eye patients before and after the insertion of Herrick silicon lacrimal plugs (Lacrimedics, Rialto, CA, USA) and in 24 eyes of 12 normal subjects as controls. RESULTS. SRI and SAI were significantly lower in controls than dry eye patients (p=0.00). Median SRI was 1.72 in dry eye patients before punctal occlusion and 0.525 in the control group. Median SAI was 1.305 in dry eye patients and 0.240 in controls. After lacrimal punctal occlusion, Schirmer test results and fluorescein breakup time increased in nine patients and remained the same in one patient. After occlusion, the SRI decreased in 9 eyes, and increased in 11 (p=0.970); SAI decreased in 13 eyes and increased in 7 (p=0.135). CONCLUSIONS. No significant change in topographic indices of corneal surface irregularity could be detected in severe dry eye patients with Iacrimal punctal plugs in the short-term follow-up. (Eur J Ophthalmol 2001; 11: 116-9)

This study was conducted to determine abnormalities of the GDx Glaucoma Scanning System parameters in patients with tilted discs, in order to set guidelines for the evaluation of glaucomatous damage in this situation. The objective was to determine which GDx parameters displayed the highest level of variation, and which remained unchanged in tilted disc syndrome. RNFA was polarimetrically conducted on 45 eyes from 26 subjects with tilted discs, and 43 normal eyes withNerve Fiber Analyzer II (Laser Diagnostic Technologies). All parameters except inferior maximum, average thickness, ellipse average, and inferior average displayed a significant difference between the two groups (p < 0.05). Although NFA is useful in glaucoma diagnosis, the majority of the GDx parameters, as shown in our study, are unreliable in tilted disc syndrome in this respect.

Purpose An oil in water emulsion of 0.01% all-trans-retinoic acid (tretinoin) was prepared and clinically evaluated in dry eye patients. Methods The ophthalmic emulsion consisted of 10% of arachis oil and 90% of the hydro-gel of Carbopol 940. To evaluate retinoic acid emulsion clinically, a placebo-controlled, open-labeled, randomized study was performed with 22 dry-eye patients. Symptoms were recorded before and after the treatments. The Schirmer 112067210001000205l test, measurement of tear film break-up time (BUT), rose Bengal and fluorescein staining of cornea and conjunctiva, and mucus fern test were done. Results Retinoic acid did not improve the dryness, photophobia and foreign body sensation more than placebo. Schirmer test and BUT were significantly improved by retinoic acid treatment. Corneal and conjunctival epithelium maintained their characteristics during the use of retinoic acid, as indicated by rose Bengal and fluorescein staining. Conclusions Ophthalmic emulsion of retinoic acid can be suggested as a promising approach for the treatment of dry eye.

To present ocular findings in a measles epidemic outbreak among young adults.Ophthalmic lesions in an unselected group of 61 young military personnel with measles were followed. Eighteen of the patients had symmetric keratitis in both eyes. They were treated with diclofenac sodium eyedrops qid in the right eye, while the left eye was observed untreated. The disappearance of the corneal lesions in both eyes was compared.Forty patients (65.6%) had measles conjunctivitis with bulbar and tarsal conjunctival hyperemia. Five (8.2%) had increased mucous secretion. Thirty-five (57.4%) had superficial punctate corneal epithelial and subepithelial lesions which stained with fluorescein. Ten patients had corneal lesions without evident conjunctival pathology. Fourteen patients had subconjunctival hemorrhages, 12 of whom had bilateral lesions mostly in the superonasal quadrant. The time to disappearance of corneal lesions was 4.5+/-3.2 days in the diclophenac-treated right eyes, and 4.1+/-3.8 days in the left eyes. We did not observe a significant difference in the healing time between the two eyes (p = 0.75).Measles did not cause major ocular complications in healthy patients. Keratitis was unresponsive to diclophenac sodium eyedrops with respect to healing time and end result.

Purpose To assess the efficacy of trabeculotomy and guarded filtration procedure in the management of glaucoma associated with the Sturge-Weber syndrome. Methods Six patients (seven eyes) with Sturge-Weber syndrome who underwent glaucoma surgery were retrospectively reviewed. One patient had bilateral glaucoma. Age at the time of operation ranged between 23 days and 9 years. The mean preoperative intraocular pressure was 30.6 mmHg (range 25–38 mmHg). Trabeculotomy and guarded filtration procedure were performed in three eyes each and both techniques were used in one eye. Results The mean follow-up was 6.3 (range 2–11) years. A single procedure lowered the intraocular pressure and arrested the progression of the disease in five eyes. Two patients required topical antiglaucoma medication. No patient needed reoperation. Two eyes suffered intra- and post-operative temporary choroidal effusions. Conclusions Trabeculotomy in infancy and guarded filtration procedure in older children effectively stabilized the eyes and prevented further glaucomatous damage in our patients with Sturge-Weber syndrome.

Conjunctival amyloidosis is a rare clinical entity. We herein reported in vivo confocal microscopic features of conjunctival amyloidosis and correlated the results with the histopathological findings. Histopathological examination of the biopsy specimen revealed acellular, amorphous, eosinophilic material in the substantia propria of the conjunctiva and positive staining with Congo red confirmed that the material was amyloid. Confocal microscopy with Heidelberg Retina Tomograph II Rostock Cornea Module showed hyporeflective material deposited in a lobular pattern in the substantia propria and around the blood vessels. The material seemed acellular and no inflammatory or dendriform cell accumulation was noted within or around the lesion. Further studies should be done to understand the confocal characteristics of the conjunctival diseases and find specific clues for the diagnosis.

Purpose To compare the outcomes of 2 techniques of permanent tarsorrhaphy. Methods The clinical records of 30 patients who underwent tarsorrhaphy at a single university-based hospital clinic were reviewed. Patients underwent permanent tarsorrhaphy because they had a nonresolving facial palsy, neurotrophic keratopathy unresponsive to medical treatment, or both. Permanent tarsorrhaphies were carried out by either internal fixation tarsorrhaphy or rubber bolster suture tarsorrhaphy. Results The overall success (100%) of internal fixation tarsorrhaphy on 12 patients (ages, 42.0 +/- 17.6 years) was significantly better than that of rubber bolster suture tarsorrhaphy (44.4%) on 18 patients (ages, 40.6 +/- 25.5 years) (Mann-Whitney U test, p = 0.01). Of the 10 patients who had unsuccessful outcomes, 8 (80%) had their eyelids separated within the first 3 postoperative weeks. Patients whose surgical outcomes were deemed successful were followed for a period of 21.4 +/- 10.7 weeks (range, 11-52 weeks). Conclusions Internal fixation tarsorrhaphy is associated with a more favorable outcome than rubber bolster suture tarsorrhaphy in patients who require permanent eyelid closure.

Purpose In the etiology of pterygium abnormalities in tear functions have also been emphasized. In this study, tear function tests are evaluated in patients with pterygium. Methods Schirmer's test 1, tear film break-up time and mucus fern patterns were evaluated in 70 eyes with pterygium and in 70 eyes of the age matched control group. Marginal tear films were also assessed. Results Tear film break-up time was significantly reduced in the pterygium group. Mucus fern patterns and marginal tear films were found to be markedly abnormal in the eyes with pterygium, however, there was no significant difference in Schirmer's test 1. Conclusion Tear function tests disclosed disrupted tear film stability which is more likely to be due to the altered mucin. This change may either be the primary factor inducing pterygium formation or reflect an existing pathology in the cells lining the ocular surface.

Purpose: To report a highly unusual case of corneal calcification after brief intraoperative use of topical thrombin. Methods: A 44-year-old man underwent sclerouvectomy for ciliochoroidal leiomyoma, during which 35 UNIH/mL lyophilized bovine thrombin mixed with 9 mL of diluent containing 1500 mmol/mL calcium chloride was used. From the first postoperative day, comeal and anterior lenticular capsule calcifications developed, and comeal involvement slightly enlarged thereafter. Results: A year later, 2 corneal punch biopsies confirmed calcification mainly in the Bowman layer. Topical treatment with 1.5% ethylenediaminetetraacetic acid significantly restored corneal clarity. Six months later, a standard extracapsular cataract extraction with intraocular lens placement improved visual acuity to 20/60. Conclusion: This case suggests that topical thrombin drops with elevated calcium concentrations may cause acute corneal calcification in Bowman layer and on the anterior lens capsule.

Glaucoma is a multifactorial optic neuropathy involving progressive optic nerve damage associated with loss of visual function and often with elevated intraocular pressure (IOP).1 ,2 It is the second most important cause of world blindness after cataracts. It has been estimated that 79.6 million people will suffer from glaucoma in 2020, and of these, 74% will have open-angle glaucoma.3 Elevated IOP has been recognised as the major risk factor for the development of glaucoma and consistently demonstrated to be associated with its progression.4 Among the treatment goals of glaucoma therapy, IOP control is the mainstay. In established open-angle glaucoma, lowering IOP is effective and always advised, regardless of whether IOP is abnormal.5 Topical medical treatment, laser procedures and surgery are various options available to treat glaucoma. All of these modalities act by reducing IOP and, in this way, protect the optic nerve from direct mechanical and/or indirect vascular insult.2 Progression of glaucoma can be stopped if IOP is lowered to a normative value, which may be set as a ‘target-IOP’.6 ,7 However, optimal glaucoma therapy should involve IOP control throughout the 24-h period; in the face of this fact, not all therapies are equally efficacious at all times of the day and night. Initial treatment for glaucoma typically involves lowering and subsequent control of IOP with pharmacological therapy.8 The first line of glaucoma treatment consists of topical IOP-lowering medications, usually initiated as monotherapy.9 Drug therapies effective for IOP reduction include both agents that decrease aqueous humour production (β-blockers and carbonic anhydrase inhibitors) and agents that increase the trabecular and uveo-scleral outflow facility (prostaglandin analogues and cholinergic agents). β-Blockers, once the gold standard of care, are now being replaced by prostaglandin analogues, which have revolutionised the medical treatment of glaucoma as …

Introduction: Glaucoma is a multifactorial optic neuropathy that can lead to progressive and irreversible loss of vision. Today there are > 60 million glaucoma patients worldwide, and this figure is rising due to aging. The aim of glaucoma therapy is to maintain the patient's visual function and quality of life by means of intraocular pressure (IOP) reduction, which currently constitutes the only evidence-based approach. Areas covered: Briefly discussed are selected, recent evidence on antiglaucoma fixed combinations. Then the efficacy and safety of the latanoprost/timolol fixed combination is comprehensively reviewed. Expert opinion: The latanoprost/timolol fixed combination can be a helpful stepwise therapeutic option in patients whose IOP is insufficiently controlled with monotherapy options. Future research is needed to better delineate the role and value of this medication in glaucoma therapy.

To compare serum and tear inflammatory and anti-inflammatory cytokine levels of rosacea patients with the healthy controls and evaluate the correlation of tear cytokine levels with tear function parameters.Tear and serum interleukin (IL)-1α, IL-6, IL-8, IL-10, monocyte chemotactic protein-1 (MCP-1), macrophage inflammatory protein-1α (MIP-1α), epidermal growth factor (EGF), and vascular endothelial growth factor (VEGF) levels were measured using multiplex bead (Luminex) technology in 12 rosacea patients without ocular involvement (group 1), 20 rosacea patients with ocular involvement (group 2), and 22 healthy subjects (group 3). The correlation of the cytokines with tear function parameters was analyzed using Spearman correlation test.Tear IL-10 and VEGF levels were significantly lower in group 1 (median: 35.78 pg/mL and 427.29, respectively) and group 2 (median: 26.25 pg/mL and 348.31, respectively) than in group 3 (median: 75.96 pg/mL and 480.12, respectively) (p 0.05). Mean serum IL-8 level was significantly lower in group 2 (median = 0) compared to group 3 (median = 3.98) (p = 0.02). Tear breakup time was found to be positively correlated with IL-10 (r = 0.46, p = 0.013) and inversely correlated with MCP-1 (r = -0.52, p = 0.004).Tear and serum levels of cytokines and growth factors measured with Luminex technology showed a large variation in rosacea and healthy subjects. Decreased levels of tear IL-10, an anti-inflammatory cytokine, may lead to an inflammatory ocular surface environment, exacerbate ocular surface inflammation, and deteriorate tear function tests. A bigger sample size, including rosacea patients with corneal involvement, is needed to confirm the role of cytokines in the pathogenesis of rosacea-associated ocular inflammation.

Purpose: To determine the prevalence of systemic atopy and immunoglobulin (Ig) deficiencies in vernal keratoconjunctivitis (VKC).Methods: Sixty-seven VKC subjects (79.1% boys) with a mean age of 11.3 ± 4.3 years were included. Serum Ig levels and specific IgE levels were measured using the nephelometric method and reversed enzyme immunoassay with sandwich ELISA technique, respectively. The patients underwent epidermal skin tests with commercial extracts.Results: Family history of atopy and associated systemic allergies were detected in 32.8 and 40.3% of the subjects, respectively. Blood eosinophilia, elevated total, and specific IgE and positive skin tests were detected in 33.8, 42.2, 50, and 35% of the subjects, respectively. Out of 62 subjects, low levels of IgA, IgG, IgM, and IgG3 were detected in 12.9, 8, 6.5, and 1.6% of the patients, respectively.Conclusion: IgE-mediated mechanisms are involved in approximately 40% of VKC patients. A new finding was the higher incidence of Ig deficiency.

* BACKGROUND AND OBJECTIVE: The effectiveness of 0.04% mitomycin-C, either postoperatively for 2 weeks or intraoperatively as a single dose, as an adjunct in the surgical treatment of primary pterygium was evaluated. * PATIENTS AND METHODS: Mitomycin-C was used in addition to the bare sclera technique in 43 eyes with pterygia. The mean follow-up time was 18.5 months for the 24 eyes in the postoperative mitomycin-C group, 10.3 months for the 19 eyes in the intraoperative mitomycin-C group, and 17.3 months for the 17 eyes in the control group (eyes that had undergone surgical excision only). * RESULTS: The recurrence rate was 4.2% with postoperative administration, 5.3% with intraoperative application, and 41.2% in the control group. There was a significant reduction in recurrence rates for both the postoperative and the intraoperative mitomycin-C groups compared with the control group (P= .005 and P= .01, respectively). However, recurrence rates were not statistically different according to the type of application. No serious side effect occurred during the follow-up period. * CONCLUSION: Topical mitomycin-C, either intraoperatively or postoperatively, as an adjunct decreases the recurrence rate of primary pterygium. Intraoperative application seems advantageous because it decreases the symptomatic side effects and restricts the inappropriate use by the patient. [Ophthalmic Surg Lasers 1996;27:600-604.]

Objective: To evaluate the complications of extracapsular cataract extraction in patients with pseudoexfoliation (PE) syndrome. Study Design: A retrospective study of patients operated on between 1990 and 1992. Setting: Hacettepe University School of Medicine, Department of Ophthalmology. Patients: Seventy-three patients with PE syndrome. The mean age of the patients was 71.3 ± 8.0 years with ages ranging between 54 and 89. There were 42 male patients with a mean age of 71.8 and 31 female patients with a mean age of 70.6. Results: Pupillary irregularity was the most frequent complication (23.3%) in the study. The other complications were as follows: posterior capsule rupture in 6.8%, posterior capsule rupture with vitreous loss 4.1%, fibrinoid reaction in 4.1%, posterior synechia in 4.1%, intraocular lens (IOL) decentralization in 2.7% and UGH syndrome in 1.4%. Conclusions: Cataract surgery in PE syndrome may be linked to several problems. Eyes with PE may show spontaneous subluxation of the lens. The zonular fibres are known to be weak in PE syndrome and the central posterior lens capsule very thin. However the low rates of complications of ECCE with and without IOL implantation has convinced us that this procedure is relatively safe in patients with PE syndrome.

Purpose To determine the association of serum vitamin D levels with exfoliation syndrome (XFS) and evaluate its impact on co-associated systemic diseases. Methods Forty patients with XFS and 40 control subjects without XFS were recruited for this study. Serum concentrations of 25-hydroxy vitamin D [25(OH) D] were measured by high-performance liquid chromatography. Vitamin D deficiency was defined as a serum 25(OH) D concentration of Results The mean age of patients with XFS and control subjects was 69.6 ± 8.1 years (range 58–90 years) and 67.1 ± 6.3 years (range 60–86 years), respectively (p>0.05). Mean 25(OH) D levels did not differ between XFS (19.8 ± 8.3 ng/mL) and control (19.9 ± 10.3 ng/mL) groups (p = 0.978). Patients with XFS had higher prevalence of cerebrovascular (p = 0.026) and cardiovascular disease (p = 0.001). There was no association between the systemic disease status and serum vitamin D levels of patients with XFS. Conclusions Although vitamin D levels were similar between XFS and control subjects, the levels were found to be decreased in both groups. Patients with XFS had a significantly higher prevalence of cerebrovascular and cardiovascular disease as compared to controls independent of their serum 25(OH) D levels. Low vitamin D level does not appear to be linked to XFS in the studied population.

Successful treatment of allergic eye disease depends on understanding the pathophysiology of ocular allergy. Thus, in this review, recent experimental and clinical research that has provided significant information about the immunopathology of allergic eye disease will be discussed.Recently, role of various cells, cytokines and chemokines has been scrutinized in the immunopathogenesis of ocular allergy. In this respect, current research highlights contribution of special molecules and cells in the development and clinical features of immunoglobulin E (IgE) and T-cell-mediated eye allergies.Recent findings in molecular immunology of ocular allergy, which comprise complex inflammatory conditions of the conjunctiva, have enabled us to better understand the pathophysiology of these diseases and have aided in the potential development of new therapeutic agents.

Objective To evaluate the effects of artificial tears on automated visual field (VF) testing in patients with glaucoma with dry eye syndrome. Design Prospective study. Participants Fifty eyes of 50 patients with medically treated primary open-angle glaucoma (POAG) were recruited for this study. The patients were subdivided into 2 subgroups: those with dry eye syndrome (group I) and those without dry eye syndrome (group II). Methods Tear break-up time, Lissamine green staining, and Schirmer I test with topical anesthesia were performed in the same order in all patients. Ocular Surface Disease Index questionnaire was completed by the patients. All participants underwent automated perimetry using the 24-2 SITA (Swedish interactive threshold algorithm) standard strategy before and after 1-week treatment with a lubricating eye drop 4 times daily. VF results were compared before and after the treatment using Wilcoxon signed rank test, and Mann–Whitney test was performed to compare the results of patients in groups I and II. Results Of the 50 patients, 25 (50%) were diagnosed with dry eye syndrome. No significant differences between both groups were found with respect to age ( P = 0.779) and glaucoma duration ( P = 0.722). In patients with glaucoma with dry eye syndrome, there was a significant improvement in test duration, mean deviation, and the number of depressed points at probability level less than 0.5% and less than 1% in pattern deviation plots after the treatment ( P Conclusions The use of artificial tears in patients with glaucoma with dry eye syndrome decreased VF testing time and improved test results.

The right eye of a 59-year-old man was the subject of extracapsular cataract extraction and posterior chamber lens implantation. He gradually developed superior sectoral scleral and diffuse iris hyperpigmentation within 7 years postoperatively. The iris was also uniformly thickened. He sustained 20/20 visual acuity in the Pseudophakie eye. Despite marked pigmentation of the angle, his right intraocular pressure remained within normal limits. We use the term acquired anterior ocular melanocytosis because the pigmentary changes were confined to the anterior segment and the choroid was not involved. We conclude that surgical injury in the form of cataract extraction can induce episcleral/scleral hyperpigmentation and iris hyperchromia. [Ophthalmic Surg Lasers 2002; 33:71-73]

Anterior segment dysgenesis comprises a spectrum of malformations arising from faulty neural crest cell migration. We report a newborn boy with partial trisomy 16q and partial monosomy 3p who presented with anterior segment dysgenesis with iris hypoplasia on the right and glaucoma on the left in association with systemic anomalies. The anterior segment dysgenesis features observed in this case have not been previously associated with partial trisomy 16q or partial monosomy 3p. Our findings support the hypothesis that an additional anterior segment dysgenesis gene may reside on chromosome 3p or 16q.

Tumour necrosis factor-alpha (TNF-α) is an important proinflammatory cytokine driving axonal degeneration and retinal ganglion cell apoptosis in glaucoma. The aim of the study was to evaluate the association of TNF-α -308 G/A and -238 G/A polymorphisms with primary open-angle glaucoma (POAG).A prospective, case-control study, university hospital setting.Eighty-six POAG patients and 193 healthy unrelated controls.TNF-α polymorphisms were screened by using direct gene sequencing.Frequency of TNF-α -308 G/A and TNF-α -238 G/A promoter polymorphisms in glaucoma and healthy subjects.The frequencies of TNF-α -308 GA genotype and 'A' allele were higher in patients with POAG (22.1% and 12.2%, respectively) in comparison with the control group (10.9% and 6%, respectively) (P = 0.046 and 0.02, respectively), with odds ratios of 2.45 (P = 0.01, 95% CI = 1.23-4.87) and 2.19 (P = 0.013, 95% CI = 1.18-4.08), respectively. Genotype distribution of the TNF-α -238 variants did not yield a statistically significant difference between the two groups (P = 0.87).TNF-α -308 G/A polymorphism seems to be associated with POAG in Turkish population. However, population-based studies with large number of subjects and long-term follow-up are needed to verify the association of TNF-α -308 G/A polymorphism with glaucoma susceptibility.

SummaryInherited severe hypoplasminogenaemia is a multisystemic disorder leading to deficient extravascular fibrinolysis. As a clinical consequence wound healing capacity of mucous membranes is markedly impaired leading to ligneous conjunctivitis and several other manifestations. Here we report the molecular genetic and clinical findings on 23 new cases with severe hypoplasminogenaemia. Homozygous or compound-heterozygous mutations in the plasminogen (PLG) gene were found in 16 of 23 patients (70%), three of which were novel mutations reported here for the first time (C166Y, Y264S, IVS10–7T/G). Compared to 79 previously published cases, clinical manifestations of the current group of patients showed higher percentages of ligneous periodontitis, congenital hydrocephalus, and involvement of the female genital tract. In contrast, involvement of the gastrointestinal or urogenital tract was not observed in any of the cases. Patients originated to a large extent (61%) from Turkey and the Middle East, and showed a comparably frequent occurrence of consanguinity of affected families and a greater female to male ratio than was derived from previous reports in the literature. Individual treatment of ligneous conjunctivitis included topical plasminogen or heparin eye drops, topical or systemic fresh frozen plasma, and surgical removal of ligneous pseudomembranes, mostly with modest or transient efficacy. In conclusion, the present study underscores the broad range of clinical manifestations in PLG-deficient patients with a trend to regional differences. Transmission of genetic and clinical data to the recently established Plasminogen Deficiency Registry should help to determine the prevalence of the disease and to develop more efficient treatment strategies.

Abnormal iris transluminance is a key ocular symptom of extracranial cerebrovascular disease in blue eyes. The dark-colored irides of 24 patients who had experienced transient ischemic attacks (TIA) and 24 healthy control subjects were evaluated for the presence of iris transluminance and pseudoexfoliation syndrome. Abnormal iris transluminance was detected in neither group. Pseudoexfoliation syndrome was found in 8 eyes (16.6%) of patients with TIA and in 1 control eye (2.1%).

Purpose: To evaluate the ocular blood flow velocities and haemorheological parameters in patients with primary open-angle glaucoma (POAG), exfoliative glaucoma (XFG) and exfoliation syndrome (XFS) and to compare their results with those of healthy controls. Methods: Twenty-five patients with POAG (group 1), 25 patients with XFG (group 2), 25 patients with XFS (group 3) and 25 healthy controls (group 4) were included in the study. Ocular blood flow velocities of ophthalmic artery (OA), central retinal artery (CRA) and short posterior ciliary arteries (SPCAs) were measured using colour Doppler imaging (CDI). Haemorheological parameters (erythrocyte elongation and aggregation index, aggregation amplitude, aggregation half-life, plasma viscosity, haematocrit) were measured in venous blood samples of all patients. Results: The peak systolic velocity (PSV) and end-diastolic velocity (EDV) values were lower and resistive indices (RI) were higher for the OA, CRA and SPCA of glaucomatous (groups 1 and 2) patients compared with those of controls (group 4) (PSV: OA, 40.4 ± 11.3 versus 52.6 ± 12.8 cm/second, p

PURPOSE. To evaluate the corneal characteristics of patients with epithelial basement membrane dystrophy (EBMD) using an in vivo laser confocal microscope, Heidelberg Retina Tomograph II, Rostock Cornea Module (HRT II RCM). METHODS. Sixteen women and 13 men who were diagnosed with or suspected to have EBMD were included in the study. The mean age of the patients was 56.4±17.2 years within a range of 25 to 81 years. Nine patients (31%) had a history of recurrent corneal erosions and 3 patients had been misdiagnosed with herpetic epithelial keratitis. The remaining 17 patients were asymptomatic and EBMD was diagnosed incidentally during routine eye examinations. RESULTS. Confocal microscopy revealed highly reflective tissue in various configurations within the intermediate and basal epithelial cell layers corresponding to the abnormal basement membrane extending into the epithelium. There were thin, parallel hyperreflective lines and high contrast round lesions in sizes ranging between 10 and 250 µm within the epithelium. Basal epithelial cells around the abnormal basement membrane and cysts seemed to be highly distorted. In two subjects with bleb-like disorder, the authors observed circular or oval hyporeflective areas with a diameter ranging between 40 and 100 µm at the level of basal epithelium and the Bowman layer, accompanied by hyperreflective, linear structures extending into the epithelium. CONCLUSIONS. EBMD is most commonly asymptomatic and undiagnosed; however, it might be associated with recurrent corneal erosions and lead to severe complications after LASIK surgery. The confocal images are highly characteristic for EBMD; therefore, confocal microscopy seems to be a valuable tool in the diagnosis of EBMD. (Eur J Ophthalmol 2009; 19: 348-54)

To identify the morphologic appearance of keratic precipitates (KPs) with in vivo confocal microscopy (IVCM) in uveitic syndromes.A total of 75 eyes of 72 patients with a mean age of 38.6+/-15.1 years who had active intraocular inflammation and whose corneas had KP on slit-lamp examination were included in this study. IVCM (Confoscan 3.0, Vigonza, Italy) was used to image the part of the corneal endothelium in which KP were most densely deposited. KP were classified into five groups: type I (small, round), type II (stippled), type III (dendritiform), type IV (large, smooth-rounded), and type V (globular). When more than one type of KP was observed with IVCM, a distinction between the predominant and the less frequent KP was made as 'primary' and 'secondary' KP.In 50 (66.7%) eyes more than one type of KP was imaged. The size of the KP ranged between 5 and 150 microm. The most frequently observed primary KP type in Behçet's disease was type I (100%), in ankylosing spondylitis type II (57.1%), in Fuchs' uveitis syndrome type III (85.7%), in granulomatous uveitis type V (42.9%), in infectious uveitis type III (66.7%), and in juvenile idiopathic arthritis associated uveitis type I (66.7%). The KP types showed a statistically significant difference between different uveitic syndromes (Fisher's exact test, P0.001).Certain KP types appear to be characteristic of various uveitic syndromes. IVCM may have a potential role in the diagnostic work-up of uveitic patients.

PURPOSE. To study the frequency of exfoliation syndrome (XFS) and its association with intraocular pressure (IOP) and systemic diseases in patients with age-related cataract scheduled for surgery. METHODS. All 1480 cataract patients had a comprehensive systemic and eye examination, including slit-lamp biomicroscopy before and after mydriasis, IOP measurement, gonioscopy, and fundus examination. RESULTS. The percentage of XFS among patients scheduled for cataract surgery was 16.4, with no gender difference (p=0.833). The mean age of XFS patients (74.3±7.0 years) was significantly higher when compared to the ones without XFS (66.5±10.9 years) (p

Purpose:To determine whether there is an asymmetry in optic nerve head morphometry in subjects with hyperopic anisometropia.Methods:Seventeen men and 20 women with a mean age of 31.43 ± 2.73 (standard error of mean) years underwent a complete eye examination and optic nerve head topography with the Heidelberg Retina Tomograph II (Heidelberg Engineering, GmBH, Heidelberg, Germany). Differences in optic nerve head parameters between anisometropic eyes and fellow eyes were evaluated using the paired-sample t test or Wilcoxon signed rank test and the correlations between refraction, visual acuity, and disc parameters were evaluated with Spearman’s correlation coefficient.Results:The median refractive errors of hyperopic anisometropic and fellow eyes were +4 D (range: +2.0 to +7.50 D) and 0 D (range: 0 to +4 D), respectively. The mean best-corrected visual acuity of the amblyopic eyes was 0.43 ± 0.25. The mean disc area of the anisometropic eyes (1.69 ± 0.35 mm2) was significantly smaller than the fellow eyes (2.01 ± 0.42 mm2) (P < .001). The differences between eyes in mean cup area, rim area, rim volume, and cup shape were statistically significant (P < .05). In aniosmetropic eyes, refractive error (r = −0.406; P = .013) and visual acuity (r = 0.347; P = .035) showed significant correlations with rim area.Conclusion:Hyperopic anisometropia is associated with a remarkable interocular difference in optic disc size and other topographic parameters.

To report the efficacy of topical cyclosporine A (CsA) 0.05% drops (Restasis; Allergan, Irvine, CA) in the treatment of superior limbic keratoconjunctivitis (SLK).Seven patients with SLK refractory to corticosteroid drops and topical silver nitrate application were treated with 0.05% topical CsA 4 times a day, preservative-free artificial tears, and a lubricant ointment. The patients were evaluated for symptomatic and clinical improvement after 2 weeks, 1 month, 3 months, 6 months, and in 6 patients,12 months.After 2 weeks of topical CsA treatment, symptoms of foreign-body sensation, irritation, and photophobia and signs of conjunctival hyperemia, tarsal papillary reaction, and filamentous keratopathy improved remarkably in all patients. The patients had some burning of short duration after administration of topical CsA. However, none of them suffered from systemic or serious local side effects. After cessation of the topical CsA treatment, 6 patients were followed up for at least 6 months. In this period, the patients continued using lubricant agents. Three patients (50%) showed signs of recurrence after 3 months, and topical CsA was restarted. The clinical findings improved again within a 2-week period, and none of these patients needed further use of topical steroids.Topical CsA 0.05% can be effective in patients with SLK who do not respond to other treatment modalities. It can also be used in the long term to prevent recurrences during treatment and to avoid steroid dependency and the side effects of steroids.

Primary Sjögren's syndrome (SS) is an autoimmune disease resulting from lymphocyte infiltration of lacrimal and salivary glands (SG). This study was designed to investigate the peripheral blood (PBL) and SG lymphocytes in 14 patients with primary SS and control subjects. With the use of monoclonal antibodies, cells were stained to identify T-cells and T-cell subsets (T-helper and T-suppressor) and cells positive for HLA-DR antigen, whereas B cells were determined by the Smlg (surface membrane immunoglobulin) method. Lymphocytes in SG biopsy specimens were characterized by means of monoclonal antibodies and the immunoperoxidase technique. In the peripheral blood lymphocytes, there was a significant reduction in T cells and suppressor T cells. T lymphocytes and mostly helper T cells were predominant around the ducts and within the lymphocytic infiltrates in the minor SG biopsy samples of patients with SS. Suppressor T cells and B cells were found in fewer numbers, HLA-DR(+) cell populations had increased, and IgG- and IgA-bearing plasma cells were also present within the infiltrates. These results may contribute to our understanding of the immunopathogenesis of primary SS.

The objective of this study was to determine cyclosporine A (Cy A) levels in ocular tissues and fluids after topical administration of poly-epsilon-caprolactone (PCL)/benzalkonium chloride (BKC) nanospheres and hyaluronic acid (HA) coated PCL/BKC nanospheres onto healthy rabbit corneas. Nanospheres were prepared by nanoprecipitation and purified by gradient-rate centrifugation. Cy A (0.1%) in either castor oil solution (group 1), PCL/BKC nanosphere formulation (group 2) or HA coated PCL/BKC nanosphere formulation (group 3) was instilled onto rabbit corneas. Tear samples were adsorbed onto Schirmer tear strips. Cy A concentrations of fluid (blood, aqueous humor, tear) and specimen extracts (cornea, conjunctiva, iris/ciliary body) were determined by high performance liquid chromatography-mass spectrometry (LC-MS). The mean corneal Cy A concentration obtained at 0.5, 1, 2, 4, 8 and 24h following instillation of the formulations ranged between 0.12 and 1.2 ng/mg tissue for group 1, 5.9-15.5 ng/mg tissue for group 2 and 11.4-23.0 ng/mg for group 3 (one-way analysis of variance (ANOVA) and pairwise tests (SNK (Student-Newman-Keuls) and Tukey); p

To determine the incidence of glaucomatous progression at mean intraocular pressure (IOP) levels in patients with ocular hypertension (OHT).A retrospective, multicentre, cohort analysis of 230 OHT patients with 5 years of follow-up evaluated for risk factors associated with progressive optic disc and visual field loss to determine the incidence of glaucomatous progression.Forty percent of patients with IOPsor = 24 mmHg, 18% of patients with IOPs of 21-23 mmHg, 11% of patients with IOPs with 18-20 mmHg, and 3% of patients with IOPs ofor = 17 mmHg progressed to glaucoma. The mean IOP was 19.8+/-2.4 mmHg in the stable group and 21.7+/-2.6 mmHg in the progressed group (P=0.0004). The highest average peak IOP was 23.4+/-4.0 mmHg in the stable group and 25.2+/-3.1 mmHg in the progressed group (P=0.006). Based on the pachymetry values for central corneal thickness, patients with thinner corneas more often progressed to glaucoma (P0.0001). A multivariant regression analysis to determine risk factors for progression was positive primarily for higher peak IOPs, older age, male gender, argon laser trabeculoplasty, visual acuityor = 20/50, and no topical medical therapy or beta-blocker therapy prior to the study.IOP reduction within the normal range over 5 years of follow-up reduces the chance of progression to primary open-angle glaucoma in OHT patients.

We report the case of a 30-year-old woman with recent onset of unilateral iris distortion and glaucoma that was noticed following uneventful laser in situ keratomileusis (LASIK). The corneal endothelium in the right eye appeared normal with the slitlamp microscope, and LASIK-related anterior segment ischemia was initially considered in the differential diagnosis. However, in vivo confocal microscopy revealed abnormal endothelial cells, confirming the diagnosis of iridocorneal endothelial syndrome. In patients having refractive corneal procedures, in vivo confocal microscopy is recommended to diagnose preexisting corneal abnormalities that might otherwise remain undetected.

A 15-month-old girl was referred for evaluation of congenital absence of both eyelids. Clinical evaluation revealed bilateral total cryptophthalmos and enlarged globes in addition to genitourinary anomalies, umbilical hernia, renal agenesis, and facial anomalies. The clinical findings were consistent with Fraser syndrome. Magnetic resonance imaging of the orbits revealed dysplastic globes that were characterized by cystic expansions that extended laterally anterior to the lateral orbital rim. Bilateral orbito-palpebral cysts that communicate with a dysplastic globe and extend beyond the orbital cavity may be a rare presenting feature of Fraser syndrome.

To evaluate the impact of punctal occlusion on ocular tear clearance and occurrence of epiphora in patients with conjunctivochalasis (CCh).Seventy-five eyes with the diagnosis of CCh were included in the study. CCh was graded on the basis of the extent of inferior lid margin involvement as follows: 1 = one location, 2 = 2 locations, and 3 = whole lid. Patients within the 3 groups were also subdivided on the basis of the presence of punctal occlusion. The presence of epiphora, occlusion of the inferior punctum, and fluorescein clearance test (FCT) results were recorded for every patient.Of the 75 patients, 56 (75%) had epiphora, 47 (63%) had punctal occlusion, and 54 (72%) had delayed FCT. Twenty-two patients had grade 1 CCh, 43 had grade 2 CCh, and 10 had grade 3 CCh. Epiphora was present in 10 (45%) patients with grade 1 CCh, 38 (88%) patients with grade 2 CCh, and 8 (80%) patients with grade 3 CCh. Punctal occlusion and epiphora were observed more frequently in patients with grades 2 and 3 CCh compared with those with grade 1 CCh (Pearson chi test, P0.001). There was a strong correlation between delayed FCT and punctal occlusion (Spearman rank correlation analysis, r = 0.808, P0.001). Punctal occlusion was also correlated with the presence of epiphora (Spearman rank correlation analysis, r = 0.691, P0.001).A significant proportion of patients with CCh are symptomatic beginning with early stages of CCh. There seem to be no significant differences in any of the clinical parameters between patients with grade 2 and grade 3 CCh. Punctal occlusion seems to play an important role in the impairment of ocular tear clearance and the occurrence of epiphora.

We present the brain MR imaging findings in a 13-month-old male infant with intracranial extracerebral brain tissue demonstrating the typical laminar organization pattern of fetal cerebral wall accompanied by unilateral anophthalmia and ethmoidal encephalocele supported by CT imaging.

Background: In vivo confocal microscopy is being increasingly used to evaluate corneal disorders. This study aimed to evaluate whether topical fluorescein application prior to in vivo confocal microscopy had any effect on the imaging characteristics of the corneal epithelium. Methods: Confocal microscopy in vivo with Confoscan 3.0 (Vigonza, Italy) was performed on 22 corneas of 22 study patients before and 1 min after application of unpreserved 2% fluorescein solution on the cornea. Ten patients with normal corneal findings served as control. The quality of superficial epithelial (SE) visualization, the SE density, the image intensity and the presence of hyperreflective SE cells were evaluated. Results: Eleven patients had normal corneal epithelium (group 1), six had keratoconus (group 2) and five had overt epitheliopathy (group 3). The visibility of SE layers of all subjects appeared to be enhanced in the post-fluorescein images. There was a significant difference between the mean pre-fluorescein (857.2 + 319.4 cells/mm2) and post-fluorescein (1378.0 ± 292.1 cells/mm2) SE densities of all study subjects (paired t-test, P

PURPOSE To evaluate the subbasal and the stromal nerves of the corneas of patients with type 2 diabetes with in vivo confocal microscopy and to compare them with those of nondiabetic patients. METHODS Thirty-five corneas of patients with type 2 diabetes and 24 corneas of age-matched control subjects were included in the study. Patients with diabetes were further classified with respect to the stage of retinopathy. Subbasal and stromal nerve plexus morphology and thickness were evaluated with in vivo confocal microscopy. Subbasal long nerve fiber (LNF) and total nerve branch (NB) densities were calculated. RESULTS The mean stromal nerve thickness was significantly higher in patients with diabetes (8.99 +/- 2.32 microm) than that of the control subjects (5.69 +/- 1.49 microm; Mann-Whitney U test; P < 0.001). The proportion of curved stromal nerves in patients with diabetes (45.7%) was also higher than that of normal subjects (20.8%; chi(2), P = 0.05). Subbasal LNF and NB densities were found to significantly lower in the corneas of patients with diabetes (28.3 +/- 10.4 and 39.7 +/- 13.2 nerve/mm(2), respectively) than those of the control subjects (34.1 +/- 5.7 and 58.5 +/- 12.4 nerve/mm(2), respectively; Mann-Whitney, P = 0.012 and P < 0.001). In addition, the subbasal nerve plexus of patients with diabetes appeared significantly thicker and more tortuous than those of the control subjects (Mann-Whitney, P = 0.002 and P = 0.001). CONCLUSION Both stromal and subbasal nerves appear abnormal in the corneas of patients with diabetes. Patients with proliferative diabetic retinopathy show more pronounced nerve alterations than patients who do not have diabetic retinopathy.

Ciftci, Ergin/0000-0002-4955-160X; Klebe, Sonja/0000-0002-8976-4714; Hugle, Boris/0000-0003-1145-6719; Klammt, Jurgen/0000-0003-2293-9193 WOS: 000241586100028 PubMed: 16849641 Severe type I plasminogen (PLG) deficiency has been causally linked to a rare chronic inflammatory disease of the mucous membranes that may be life threatening. Here we report clinical manifestations, PLG plasma levels, and molecular genetic status of the PLG gene of 50 patients. The most common clinical manifestations among these patients were ligneous conjunctivitis (80%) and ligneous gingivitis (34%), followed by less common manifestations such as ligneous vaginitis (8%), and involvement of the respiratory tract (116%), the ears (14%), or the gastrointestinal tract (2%). Four patients showed congenital occlusive hydrocephalus, 2 with Dandy-Walker malformation of cerebellum. Venous thrombosis was not observed. In all patients, plasma PLG levels were markedly reduced. In 38 patients, distinct mutations in the PLG gene were identified. The most common genetic alteration was a K19E mutation found in 34% of patients. Transient in vitro expression of PLG mutants R134K, delK212, R216H, P285T P285A, T319_N320insN, and R776H in transfected COS-7 cells revealed significantly impaired secretion and increased degradation of PLG. These results demonstrate impaired secretion of mutant PLG proteins as a common molecular pathomechanism in type I PLG deficiency.

Evidence of Waite–Beetham lines in the corneas of diabetic patients as detected by in vivo confocal microscopy

Purpose: The purpose was to evaluate the diurnal variation (DV) of intraocular pressure (IOP) in patients with exfoliation syndrome (XS), to measure retinal nerve fiber layer (RNFL) thickness by using scanning laser polarimetry, and to compare these measurements with those of normal subjects. Methods: Forty-five subjects with XS and 40 healthy, age/sex matched subjects were recruited into the study. A detailed ophthalmologic examination was performed. IOP measurements were obtained at 08: 00 am, 12: 00 pm, 03: 00 pm, and 06: 00 pm. The XS group was further divided into DV >= 5 mmHg and DV < 5 mmHg groups and also according to the existence of IOP fluctuation. The IOP measurements and RNFL thickness measurements were compared between the groups. Results: The mean IOP value was found to be highest in the morning both in the XS and control groups. IOP showed a gradual decrease from 8.00 am to 6.00 pm in the control group, whereas a second peak at 03: 00 pm was observed in the XS group. There was a fluctuation in 53.3% of the XS group, while none of the healthy subjects showed fluctuation. Superior and inferior ratios were statistically lower in XS patients than those in control subjects (p < 0.05). Moreover, in patients with XS showing a DV >= 5 mmHg and/or a fluctuation, the superior ratio, inferior ratio, the number, superior average and superior integral were significantly different (all p values < 0.05) from those of control subjects. Conclusions: As the XS patients with high diurnal IOP variation and fluctuating pattern of IOP had lower RNFL thickness measurements, it is crucial to follow up these patients by performing scanning laser polarimetry in order to discover any possible glaucomatous damage at an earlier stage than with the use of conventional visual field analysis.

Dacryoliths are uncommon causes of partial or complete obstruction of the nasolacrimal drainage apparatus. We report our findings of a dacryolith that we studied by culture, light microscopy, atomic absorption spectrophotometry, and scanning electron microscopy. Although no fungi were recovered by culturing, hyphae-like structures were observed. No inorganic material was detected by atomic absorption spectrophotometry. Scanning electron microscopy of ultrastructure showed the stone was composed of lobes and lobules built on an amorphous core material.