Severity Of Ocular Involvement Research Articles

Visual Acuity in Aniridia and WAGR Syndrome.

Our purpose was to evaluate visual acuity in aniridia subjects and the more severely affected phenotype in WAGR syndrome subjects, and to assess potential impact on visual function. This was a retrospective comparative study of 25 aniridia subjects with nonsense mutations of PAX6 (50 eyes) and 25 WAGR syndrome subjects with large deletion mutations involving PAX6 (50 eyes). Aniridia subjects were age- and gender-matched with WAGR syndrome subjects in the Coordination of Rare Diseases at Sanford (CoRDS) database. Best-corrected ETDRS visual acuity measurements were converted to LogMAR visual acuity values, which were used to perform statistical analyses. The age and gender distribution of the subjects was not statistically significantly different. The mean LogMAR values in aniridia and WAGR syndrome subjects were 0.95±0.53 and 1.51±0.99, respectively (P<0.001). In the better-seeing eye, mean LogMAR values were 0.78±0.15 in aniridia subjects and 1.40±0.88 in WAGR syndrome subjects (P=0.001). The mean LogMAR values for the better-seeing eye corresponded to Snellen visual acuity of 20/125 in aniridia subjects and 20/500 in WAGR syndrome subjects. This average visual acuity was worse than the threshold for profound visual impairment (WHO criteria) and legal blindness (AAO criteria) in WAGR syndrome but not in aniridia subjects. In analysis of both eyes, the visual efficiency was 34% in aniridia subjects and 2% in WAGR syndrome subjects. Visual acuity was significantly worse in WAGR subjects with multi-gene deletion mutations compared with aniridia subjects with nonsense mutations, which corresponded to differences in standard visual function thresholds. Our results suggest that visual acuity may indicate severity of ocular involvement and variability of phenotype in aniridia and WAGR syndrome.

Ocular involvement in STEC-associated hemolytic uremic syndrome.

Hemolytic uremic syndrome (HUS) is a systemic thrombotic microangiopathy characterized by hemolytic anemia, thrombocytopenia, and variable kidney involvement. Extrarenal thrombotic microangiopathy occurs in central nervous system (CNS), colon, and other organ systems, but ocular involvement is rarely recognized. This study aimed to analyze frequency and severity of ocular involvement in STEC-HUS, and the relationship between ocular involvement and disease severity, with emphasis on CNS, kidney, and colonic disease. Prospective, longitudinal, observational study. STEC-HUS patients September 2014-January 2019. Funduscopic examination (FE) was performed within 48h of admission. We evaluated severity of CNS disease, kidney involvement, and presence of hemorrhagic colitis (HC). Ninety-nine patients were included (female 52), mean age 39.4months (DE: 29.8; range 9-132). Thirteen patients (13.1%) had abnormal FE, 10 showing variable degrees of hemorrhagic exudates and 2 with typical Purtscher-like retinopathy. Other findings included tortuous vascularity, cotton wool spots, and transient retinal edema. CNS involvement was present in 16/99 patients, severe in 12 (75%). Abnormal FE occurred in 5/12 (31%) patients with severe CNS involvement vs. 8/87 (9.2%) with mild, moderate, or no CNS disease (p = 0.0191). Abnormal FE was present in 2/33 (6%) patients without dialysis vs. 11/66 (16.6%) requiring dialysis (p = 0.20). Finally, there were FE abnormalities in 6/20 patients with HC vs. 7/79 without HC (p = 0.012). FE abnormalities were present in 13% of HUS patients. Abnormal FE significantly associated with more severe disease, including severe CNS involvement and HC. We suggest FE should be performed in severe HUS, especially in cases with severe CNS disease. A higher resolution version of the Graphical abstract is available as Supplementary information.

Association Between Skin Findings and Ocular Signs in Rosacea.

Objectives:To report the most frequent signs in ocular rosacea and evaluate their association with skin findings.Materials and Methods:Fifty-one patients diagnosed with rosacea by a trained dermatologist were evaluated by an ocular surface specialist. A complete ophthalmological examination was performed.Results:In our study, the prevalence of ocular signs in patients with rosacea was 74.5%. The average age at presentation was 50 years and women were more affected than men. The most common findings were lid margin erythema, meibomian gland dysfunction, and blepharitis. Fifteen patients had decreased visual acuity due to complications related to rosacea such as leukoma and corneal neovascularization. Interestingly, patients that had the lowest visual acuity presented with dermatological signs of papules and pustules (p=0.001) and rhinophyma (p=0.023). Two patients who showed subepithelial fibrosis and fornix foreshortening were diagnosed as having ocular cicatricial pemphigoid (OCP) by immunohistopathological analysis of conjunctival specimens.Conclusion:Ocular compromise is common in rosacea. Our study shows that there might be a relationship between the severity of ocular involvement and certain subtypes of cutaneous disease. Rosacea and OCP may coexist. In cases that present with conjunctival fibrotic changes, a diagnostic biopsy is mandatory.

Prevalence and severity of ocular involvement in Graves' disease according to sex and age: A clinical study from Babol, Iran.

Background:Thyroid-associated eye disease (TED), previously known as Graves’ ophthalmopathy is a cosmetically and functionally debilitating disease that is seen worldwide. The aim of this study was to evaluate the prevalence and clinical severity of ocular manifestations of Graves’ disease according to sex, age and duration in northern Iran.Methods: Between April 2011 and March 2012, 105 patients with Graves’ disease, underwent ophthalmic examination, including ocular motility, exophthalmometry, intraocular pressure (IOP), slit lamp and fundoscopy. Patients received scores according to modified Werner’s NO SPECS classification.Results:Ocular involvement was found in 70 patients with established Graves’s disease. The mean age was 35.0 years, (SD 13.0, range 15 to 69). The most common ocular findings were exophthalmometric proptosis of more than 20 mm (63.8%), lid lag (55.7%), lid retraction (52.8%) and tearing (38.6%). Almost 70% of patients had bilateral involvement. Elevated IOP was seen in 15 (25.4%) patients, and was significantly related to proptosis (P=0.007). More than half of the patients (n=36, 52.2%) had a modified Werner’s NO SPECS score of 3.00. Clinical severity as shown by the increasing number of signs and symptoms per patient was correlated to increasing age (r=0.31, P=0.01) but not to gender (P=0.17).Conclusions:Both functional (ocular motility disorders, increased IOP) and cosmetic (proptosis, periorbital edema) sequels are common ocular presentations in patients with Graves' disease. Proptosis was the most common finding in this study and was associated with elevated IOP. Clinical severity was found to correlate to increasing age.

A Review of Ocular Graft-Versus-Host Disease.

: Graft-versus-host disease (GVHD) is a major complication that occurs following allogeneic hematopoietic stem cell transplantation, which is a potential curative therapy used in a variety of malignant or benign hematological diseases. Graft-versus-host disease primarily occurs in many organs, but most notably in the skin, lungs, gastrointestinal tract, liver, eyes, mucosa, and musculoskeletal system. Ocular manifestations of GVHD may precede other systemic GVHD findings, and it may be a poor prognosis for mortality. While all parts of the eye may be affected, ocular GVHD occurs primarily in the ocular surface. Dry eye disease or keratoconjunctivitis sicca is the most common presenting manifestation of chronic ocular GVHD. Dry eye disease in ocular GVHD is a multifactorial process, which involves destruction and fibrosis of lacrimal glands and conjunctiva, leading to tear film deficiency and instability. Depending on the severity of ocular involvement and response to treatment, ocular GVHD may cause decreased quality of life. Management of GVHD begins with prevention by understanding risk factors and by implementing prophylactic treatment after allogeneic hematopoietic stem cell transplantation. A multidisciplinary approach to the prevention and treatment of GVHD is important, and there are currently no preventive therapies available for ocular GVHD. Once diagnosed, ocular GVHD treatment strategies target ocular surface lubrication and support, tear film stabilization, inflammation reduction, and surgical intervention. The goal of this review is to define ocular GVHD and its categorical manifestations, as well as to describe the importance of comprehensive assessment, diagnosis, and ophthalmologic treatment and management of ocular GVHD with a multidisciplinary approach.

Manifestation and grading of ocular involvement in patients with Tessier number 10 clefts.

PurposeThe objective of this study is to evaluate and grade the extent and severity of ocular involvement in Tessier number 10 cleft.Patients and methodsA retrospective, noncomparative, interventional case series was conducted between January 2006 and December 2015. Clinical data were reviewed from 59 patients (85 eyes) with Tessier number 10 clefts. Detailed medical history and ophthalmic examination of patients with a confirmed diagnosis of Tessier number 10 cleft were recorded on an itemized data collection form. Ocular manifestations were categorized as upper eyelid defect, symblepharon with cutaneous pterygium (skin growing onto the globe), corneal complications, and lower eyelid ectropion; components were evaluated and graded on a scale from 0 to 3, according to their severity.ResultsMore than half of the cases (43 eyes, 53.8%) had severe upper eyelid defect, and severe symblepharon with cutaneous pterygium were observed in 38 eyes (47.5%). Nearly half of the cases (40 eyes, 50.0%) have severe corneal complications, and lower eyelid ectropion was found in 34 eyes (42.5%). The severity of symblepharon, corneal complications, and lower eyelid ectropion were significantly correlated with the upper eyelid defect; the correlation coefficient (r) ranged from 0.844 to 0.629 (P<0.0001).ConclusionThis study presents the ocular manifestation of Tessier number 10 clefts with large-series cases, and establishes an effective grading system to evaluate Tessier number 10 clefts, which is useful for the diagnosis, treatment, and prediction of outcomes in patients with a Tessier number 10 cleft.

Inpatient Ophthalmology Consultation for Fungemia: Prevalence of Ocular Involvement and Necessity of Funduscopic Screening.

To determine the generalizability of recent data assessing the necessity of ophthalmic consultation for fungemic patients, we examined the prevalence, microbial profile, and treatment of fungal chorioretinitis and endophthalmitis among patients with positive fungal cultures referred for ophthalmologic consultation at a tertiary care medical center. Retrospective cross-sectional study. All inpatient ophthalmology consultations from Wills Eye Hospital at Thomas Jefferson University between January 1, 2006 and December 31, 2012 were retrospectively reviewed and cross-referenced to a microbiologic database of positive fungal blood cultures. This included 227 adult consecutive inpatients with positive fungal blood cultures (n= 215) or suspected fungemia (n= 12). Clinical data were extracted from records held by the microbiology laboratory and inpatient records. Patients were deemed to have ocular fungal involvement if dilated fundus examination demonstrated evidence of chorioretinitis or endophthalmitis. Two hundred and twenty-seven consultations were requested to evaluate patients for ocular manifestations of fungemia. Eleven patients (4.8%, 95% CI 2.4%-8.5%) were diagnosed with fungal chorioretinitis or endophthalmitis. Of these 11 patients, 5 had visual symptoms, 2 were asymptomatic, and 4 were unable to communicate. A total of 5 patients (2.2%) received intravitreal injections following funduscopic screening. An additional 11 patients (4.8%) had nonspecific fundus lesions considered to be inconsistent with ocular fungal involvement. The most common fungal species identified were Candida albicans (n= 85), Candida glabrata (n=63), and Candida parapsilosis (n= 44). The current study found a low rate of disseminated ocular involvement in patients with positive fungal cultures referred for ophthalmologic consultation. However, 2 patients with ocular fungal involvement denied visual symptoms and over half of affected patients were asymptomatic or unable to communicate. As the presence and severity of ocular involvement in fungemic patients may dictate the mode and duration of antifungal treatment, funduscopic screenings may still have an important role.

Intraocular cytokines imbalance in retinal vein occlusion and its impact on the efficacy of anti-angiogenic therapy

To study the concentrations of intraocular cytokines in patients with retinal vein occlusion (RVO) before and after intravitreal ranibizumab injection and to compare the results with clinical activity of the disease and treatment efficacy. A comprehensive ophthalmological examination of 44 patients with RVO and macular edema was performed. Intraocular fluid was first collected before the intravitreal injection. Cytokines concentrations were measured using Bio-Plex Pro Human Cytokine 27-plex Panel (Bio-Rad Laboratories, USA) for flow cytometry. The test was repeated 1 month after the injection. A total of 11 cytokines were reliably detected. After ranibizumab injections certain angiogenic (VEGF) and proinflammatory (IL-6, IL-8, IL-13, IL-15, MCP-1) factors appeared to be significantly suppressed. Clinical efficacy of the therapy correlated with the degree of cytokines suppression, which in turn depended on the severity of ocular involvement at baseline. Retinal vein occlusion pathogenesis involves a cascade of immune and inflammatory processes, including activation of not only VEGF but also quite a few inflammatory and chemotactic factors, whose activity depends on the extent of ischemic damage in the retina.

Severity of acute ocular involvement is independently associated with time to resolution of ocular disease in toxic epidermal necrolysis patients

PurposeToxic epidermal necrolysis (TEN) is a severe, life-threatening mucocutaneous disorder that frequently involves the ocular surface. This study aims to investigate the natural history and resolution of acute ocular involvement...

Cicatricial changes in ocular pemphigus

To describe the clinical characteristics of ocular involvement in patients with pemphigus at an ophthalmological referral center. A retrospective review was conducted on patients with the immunopathological diagnosis of pemphigus examined between 1 January 2000 and 1 April 2010. Uncorrected distance visual acuity (UDVA), best corrected distance visual acuity (BCVA), ocular symptoms, and ocular surface inflammatory and scarring changes were assessed. A total of 15 patients were identified, with a mean age of 68.27 ± 14.35 years, and 80% (n=12) were female. Extraocular involvement was reported in one patient. All of the eyes showed cicatricial changes in the conjunctiva. In all, 6 eyes (20%) were classified as stage I; 12 eyes (40%) as stage II; 10 eyes (33%) as stage III; and 2 eyes (7%) as stage IV. A statistically significant association was found between BCVA and the severity of ocular involvement. The mean BCVA logMAR was 1.66 (20/914), with a range from logMAR 0 (20/20) to logMAR 4 (NLP). Other ocular diseases were found in 8 (53.3%), systemic diseases in 10 (66.7%), and the use of pemphigus-inducing drugs in 10 patients (66.7%). The present report represents the largest series of ocular involvement in pemphigus confirmed by immunopathology. The clinical manifestations varied from conjunctival hyperemia to corneal scarring and perforation. There was a strong association between scarring changes and low BCVA. Ocular and systemic diseases as well as the use of pemphigus-inducing drugs may predispose to ocular cicatricial changes observed in this series.

Cutaneous and ocular signs of childhood rosacea

AbstractPurpose To describe the clinical features of cutaneous and ocular manifestations of childhood rosacea, to propose diagnostic criteria, and to emphasize the possible severity of ocular complications in this age group.Methods Children aged 1 to 15 years who had received a diagnosis of cutaneous and/or ocular rosacea and were seen between January 1, 1996, and December 31, 2011.Results Of 20 patients, 11 had ocular and cutaneous rosacea, 6 had isolated cutaneous involvement, and 3 had isolated ocular involvement. Dermatologic examination results were sufficient to diagnose rosacea in 12 of the patients (60%). The most common presentation was a papulopustular eruption on a telangiectatic background. In 11 patients (55%), ocular involvement preceded the skin eruption. Among the ophthalmologic manifestations, chalazions and blepharoconjunctivitis were the main presenting symptoms; keratitis was observed in 4 patients and corneal ulcers in 2. Ten patients were treated with oral metronidazole. Intermittent treatment for at least 3 months was used to avoid neurologic toxic effects and to achieve complete remission.Conclusion Although rare, childhood rosacea should be recognized because of the possible severity of ocular involvement.

Long-term ocular consequences of sulfur mustard in lung-injured war veterans

Purpose: Ocular and pulmonary involvement are the most important complications of sulfur mustard (SM) that may happen many years after exposure. This study aims to evaluate the severity of ocular involvement and the correlation between late ocular and lung complications in patients exposed to SM.Methods: This descriptive cross-sectional study was conducted on SM lung-injured war veterans. Status of pulmonary involvement was categorized into normal, mild, moderate, and severe based on the “Forced Expiratory Volume in first second (FEV1)”.Status of ocular involvement was also categorized into normal, mild, moderate, and severe, based on the slit lamp findings. Correlation between pulmonary and ocular involvements was evaluated by Spearman rank correlation test.Results: Totally, 292 war veterans with clinical pulmonary involvement were included in the study. Status of pulmonary involvement was 3.8% normal, 11.2% mild, 16.1% moderate, and 68.9% severe. Status of the ocular involvement was 68.2% normal, 13.8% mild, 5.4% moderate, and 12.6% severe. Among all patients, 96.3% had pulmonary involvement and 32.5% had ocular involvement. There was a positive correlation between the severity of ocular and pulmonary involvements (p = 0.049 and r = 0.122).Conclusion: The results of this study showed that although there was a positive correlation between the severity of pulmonary and ocular involvement, this correlation was weak. This might be due to the nature of the studied population or differences in the tissue susceptibilities, gas types, or exposure patterns.

The effect of clinical severity and eyelid rash on ocular involvement in primary varicella infection

To describe ocular manifestations in primary varicella infection and their relationship to systemic severity and the associated eyelid rash. One hundred consecutive children with primary varicella were examined prospectively. The cases were classified as mild, moderate, and severe according to the severity of clinical presentation. Excluding the presence of eyelid rash, children with ocular findings were assigned to group 1 (G1), and those without ocular findings were assigned to group 2 (G2). Patients in G1 were also evaluated according to the nature of ocular manifestations and the course of uveitis. Twenty-one percent of patients had ocular involvement (G1) and 79% had no ocular involvement (G2). While chickenpox had a mild course in 85.7% of patients in G1 and 88.6% of patients in G2, all others had a moderate course. None of the children had a severe course. A varicella eyelid rash was present in 28.6% of patients in G1 and 13.9% in G2. Among ocular findings, 38.1% of patients had conjunctivitis, 57.1% had anterior uveitis, and 4.8% had disciform keratouveitis. There was no significant association between severity of chickenpox and severity of ocular involvement (p=0.712). There was also no relationship between eyelid rash and ocular involvement (p=0.787). There is neither an association between the severity of chickenpox and the severity of ocular involvement nor an association between the presence of a varicella eyelid rash and the development of uveitis. As the prognosis regarding sequelae of ocular involvement in varicella infection is good, only those patients with ocular signs and symptoms need be referred by pediatricians for an ophthalmologic examination.

Ocular involvement in paediatric haemolytic uraemic syndrome

The aim of this study was to estimate the frequency and severity of ocular involvement in paediatric patients with haemolytic uraemic syndrome (HUS). The study was designed as an institutional, retrospective, observational case series. Charts for all 87 paediatric patients with HUS treated at the University Children's Hospital Zurich between 1995 and 2007 were reviewed. Patients with ocular involvement were identified and clinical findings presented. Three of 69 examined patients with HUS showed ocular involvement. Ophthalmic findings in two children were consistent with bilateral Purtscher retinopathy, showing multiple haemorrhages, exudations and superficial retinal whitening. The third child presented with bilateral isolated central intraretinal haemorrhages as a milder form of ocular involvement. In one of the children with Purtscher retinopathy, laser photocoagulation was required for bilateral rubeosis irides and development of disc neovascularization. Longterm outcomes in the two severely affected children showed decreased visual acuity caused by partial atrophy of the optic nerves. In the milder case visual acuity was not impaired at any time. A minority of paediatric patients with HUS developed ocular involvement. Acute ocular findings varied in severity from isolated intraretinal haemorrhages to Purtscher-like retinopathy with retinal ischaemia. Longterm complications included the development of neovascularizations and consecutive optic nerve atrophy. Although ocular involvement in HUS seems to be rare, physicians should be aware of this complication because of its possible vision-endangering consequences.

Eruption severity and characteristics in herpes zoster ophthalmicus: correlation with visual outcome, ocular complications, and postherpetic neuralgia

Herpes zoster ophthalmicus (HZO) is characterized by a typical vesicular eruption affecting the distribution of the ophthalmic division of the trigeminal nerve which can be of varying severity. The correlation of eruption severity and ocular involvement and subsequent visual loss is still to be established. In this prospective longitudinal study we evaluated the correlation of eruption severity with ocular complications, visual outcome and postherpetic neuralgia. Patients with HZO underwent detailed ophthalmological and dermatological examination at presentation and follow-up on 1(st) , 2(nd) and 4(th) week and 3(rd) and 6(th) month. Eruption severity was graded as mild, moderate and severe based on the number of vesicles. The correlation of eruption severity and distribution with ocular complications, visual outcome and postherpetic neuralgia was statistically evaluated. Severe eruption was seen in 14(28%), moderate eruption in 21(42%) and mild eruption in 15(30%). Severe eruption was significantly associated with increased incidence and severity of ocular involvement (p = 0.04 and p = 0.04 respectively), occurrence of uveitis (p = 0.004), reduced visual outcome (p = 0.002) and the occurrence of PHN (p = 0.05). Lacrimal nerve involvement was also associated with increased incidence (p = 0.03) and severity (p = 0.02) of ocular complications and visual loss (p = 0.01). Increasing eruption severity is a good predictor of occurrence of ocular complications and subsequent visual loss in HZO. Presence of a severe eruption is an indication for early ophthalmic intervention. Nasociliary nerve and lacrimal nerve involvement are also good predictors of ocular complications.

Risk Factors for the Development of Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

To describe the acute and late ocular manifestations of toxic epidermal necrosis (TEN), Stevens-Johnson syndrome (SJS), and overlap syndrome and to identify predictors for the development of ocular complications. Retrospective cohort study. A single referral unit in a university hospital. The study included 159 patients (mean [SD] age, 49.9 [19.8] years) with TEN and SJS during an 8-year period. Forty-nine patients were contacted at least 15 months after hospital discharge. Records were reviewed for demographics, cause of the condition, and severity of ocular involvement. The patients were contacted to assess late ocular complications. A total of 117 patients (74%) had acute ocular involvement, which was mild in 58%, moderate in 8%, and severe in 8%. Patients with TEN had more frequent (odds ratio [OR], 2.7; 95% confidence interval [CI], 1.06-6.90; P = .05) but not more severe (OR, 0.95; 95% CI, 0.20-4.5; P = .99) acute ocular involvement. Forty-nine patients were contacted at least 15 months after hospital discharge, and 63% had late ocular complications. Dry eye syndrome was the most common. The mean (SD) Ocular Surface Disease Index score was 32.9 (30.3) (range, 0-97.5). The severity of the acute ocular disease was found to be the only significant risk factor of late complications (P = .002), even though 5 patients without acute ocular involvement developed dry eye syndrome. Ocular involvement is common in patients with SJS and TEN. Late complications are more frequent in patients with severe initial eye involvement but may also develop in patients without patent initial ocular symptoms.

Cutaneous and Ocular Signs of Childhood Rosacea

Objectives To describe the clinical features of cutaneous and ocular manifestations of childhood rosacea, to propose diagnostic criteria, and to emphasize the possible severity of ocular complications in this age group. Retrospective study. Tertiary referral center. Patients Children aged 1 to 15 years who had received a diagnosis of cutaneous and/or ocular rosacea and were seen between January 1, 1996, and December 31, 2005. Of 20 patients, 11 had ocular and cutaneous rosacea, 6 had isolated cutaneous involvement, and 3 had isolated ocular involvement. Dermatologic examination results were sufficient to diagnose rosacea in 12 of the patients (60%). The most common presentation was a papulopustular eruption on a telangiectatic background. In 11 patients (55%), ocular involvement preceded the skin eruption. Among the ophthalmologic manifestations, chalazions and blepharoconjunctivitis were the main presenting symptoms; keratitis was observed in 4 patients and corneal ulcers in 2. Ten patients were treated with oral metronidazole. Intermittent treatment for at least 3 months was used to avoid neurologic toxic effects and to achieve complete remission. Conclusion Although rare, childhood rosacea should be recognized because of the possible severity of ocular involvement.

New Grading System for the Evaluation of Chronic Ocular Manifestations in Patients with Stevens–Johnson Syndrome

To evaluate and grade the extent and severity of chronic ocular manifestations in Stevens-Johnson syndrome (SJS). Prospective multicenter case series. We enrolled 73 patients (138 eyes) with SJS seen between April 2003 and March 2005 at 3 tertiary referral centers. Patients with a confirmed history of SJS and chronic ocular complications that persisted for at least 1 year from the onset of SJS were included. Their detailed medical history and ophthalmic examination results were recorded on an itemized data collection form. Complications were categorized as corneal, conjunctival, and eyelid complications, and 13 components were evaluated and graded on a scale from 0 to 3 according to their severity. These were broadly classified as corneal (superficial punctate keratopathy, epithelial defect, loss of the palisades of Vogt, conjunctivalization, neovascularization, opacification, keratinization), conjunctival (hyperemia, symblepharon formation), and eyelid (trichiasis, mucocutaneous junction involvement, meibomian gland involvement, punctal damage) complications. The most severely affected complication components were loss of the palisades of Vogt (114 eyes; 82.6%) and meibomian gland involvement (102 eyes; 73.9%). Visual acuity in 74 of the 138 eyes (53.6%) was worse than 20/200. The severity of corneal, conjunctival, and eyelid complications was significantly correlated with visual loss. All 13 complications were correlated significantly with logarithm of the minimum angle of resolution (logMAR) visual acuity; the correlation coefficient (R) ranged from 0.359 to 0.810 (P<0.0001); for corneal epithelial defects, R was 0.169 (P = 0.0473). Eyes with a higher total score for the 3 complication categories had poorer vision (R = 0.806; P<0.0001). Multivariate regression analysis showed that corneal neovascularization, opacification, keratinization, and cataracts significantly affected logMAR visual acuity (P<0.0001, P<0.0001, P = 0.0142, P = 0.0375, respectively). The authors describe a new method for grading the extent and severity of ocular involvement in patients with SJS and demonstrate that the severity of ocular involvement is correlated significantly with the final visual outcome. This new grading system provides a more objective method for evaluating SJS patients and may be adapted for use in other cicatricial ocular surface diseases.

Ocular manifestations and complications of Stevens–Johnson syndrome and toxic epidermal necrolysis: an Asian series*

To describe the acute and late ocular manifestations and complications in toxic epidermal necrosis (TEN) and Stevens-Johnson syndrome (SJS), and identify predictors for development of late complications. Cases of TEN and SJS during a 9-year period were included. Patients with ocular involvement were reviewed for acute ocular complications. Patients with a minimum 6 months follow-up were reviewed for late complications. Records were reviewed for their demographics, etiology, and severity of ocular involvement. There were 117 patients with a mean age of 52.2 +/- 18.6 years. Eighty-one of these (69%) had acute ocular involvement. This was mild in 40%, moderate in 25% and severe in 4%. Adverse drug reactions were the predominant cause. Patients with thrombocytopenia had more severe acute ocular involvement. Forty-four patients had a minimum 6 months of follow-up and half developed late complications. Severe dry eyes and trichiatic lashes were the commonest late complications. Patients treated with topical antibiotic were more likely to have late complications, particularly dry eyes. There was no difference in the severity of acute eye involvement or late complications when SJS and TEN patients were compared. The severity of the acute ocular disease and abnormal laboratory tests were not found to be the significant risk factors of late complications. Ocular involvement is common in SJS and TEN and can be severe and blinding. The severity of acute ocular complications does not predict late complications. The diagnosis of TEN does not imply a more severe ocular involvement or increased frequency of late ocular complications compared with SJS. Care should be taken even in mild cases. Appropriate intervention during acute ocular disease may prevent late complications.

Factor V Leiden and prothrombin gene G20210A mutations in Italian patients with Behçet's disease and deep vein thrombosis

To evaluate the frequency and type of vascular lesions and to study the association of factor V gene G1691A (Leiden) and prothrombin gene G20210A polymorphisms with venous thrombosis in Italian patients with Behçet's disease (BD). Included were 118 consecutive Italian BD patients followed over a 3-year period (1997-1999) who satisfied the International Study Group criteria for BD. The control group consisted of 132 healthy Italian blood donors. All BD patients and controls were genotyped by polymerase chain reaction and allele-specific restriction enzyme techniques for factor V Leiden and prothrombin gene G20210A polymorphisms. Vascular lesions were observed in 37 (31.4%) patients. The 2 most common lesions were subcutaneous thrombophlebitis (10.2%) and deep vein thrombosis (DVT) of the legs (22.8%). No significant demographic and clinical differences between patients with and without DVT were present. The distribution of allele and genotype frequencies of prothrombin gene G20210A and factor V Leiden polymorphisms did not differ significantly between BD patients and healthy controls. The frequencies of carriage rates of prothrombin gene G20210A and factor V Leiden polymorphisms in BD patients with and without DVT were similar. However, the frequency of 20210A allele was significantly higher in BD patients with ocular disease than in those without, particularly in the patients with posterior uveitis/retinal vasculitis. The frequency and types of vascular lesions in Italian BD patients were similar to those reported in studies from other countries. No association between factor V Leiden mutation and G20210A mutation in the 3'-untranslated region of the prothrombin gene with DVT was found. However, a prothrombin gene G20210A mutation may influence the development and severity of ocular involvement in BD.