Acute and Chronic Ophthalmic Involvement,Severity, and Sequelae in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

PurposeTo study the ocular manifestations, its severity and sequelae in patients with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN).MethodsProspective study of 44 consecutive patients (30 SJS and 14 TEN) presenting in the acute phase of the disease. Patients were evaluated by dermatologist as well as physician for systemic status, skin lesions and mucosal involvement. Detailed history taking, visual acuity, ophthalmic evaluation (lid margin, corneal, conjunctival changes, tear film and ocular surface). Ocular severity score (OSS) was assessed at baseline (acute) and at 6 months (chronic / OSS6), graded as mild, moderate and severe.ResultsMean age was 28.15 ± 15.78 years. Sixty-five eyes of 33 patients were included for final analysis. Thirty-eight patients (86.4%) had ocular manifestations. Drugs were the most common causative factor (95.4%). At base line mild, moderate, and severe OSS was seen in 43.1%, 44.6%, and 12.3% eyes. At 6 months mild, moderate, and severe OSS was seen in 44.6%, 7.7%, and 6.2% of eyes. There was a significant correlation between age of the patient and OSS at 6 months (p = 0.02). Younger age had higher chronic OSS. Patients with TEN had higher acute (p = 0.001) and chronic (p = 0.001) OSS than SJS. Three mucosal surface involvement associated with higher acute and chronic OSS (p = 0.001). No long-term ocular complications observed in 27 / 65 (41.5%) eyes. Acute OSS correlated significantly with chronic OSS, at 1 and 6 months (p = 0.001).ConclusionsGreater severity of the disease, more number of mucosal surfaces involved and shorter symptom lag correlated with more severe acute and chronic ocular manifestations. The severity of lid margin involvement and corneal involvement in acute stage were good predictors of severity of chronic ocular findings. Initial severity of ocular involvement correlated with severity of ocular sequelae.     

Ocular manifestation of rickettsial disease in South Indian population

Purpose:The aim of this work was to study the ocular manifestations and its management in spotted fever and typhus group of rickettsial disease.Methods:A retrospective analysis of 50 patients with serologically confirmed Rickettsial disease. In all patients, relevant history, investigations and treatment details were collected and they underwent complete ophthalmic evaluation including measurement of best-corrected visual acuity, anterior segment examination and dilated fundus examination.Results:Mean age was 12.5 ± 8.99 years. Of the 50 patients, 40 patients were ≤18 years of age and 27 (54%) had ocular involvement. Out of 27 patients, bilateral involvement was seen in 10 patients. Most of the patients had no ocular symptoms. Ocular findings included, Retinal vasculitis 6 (22.22%); macular edema 4 (14.81%); vasculitis with macular edema 1 (3.7%); Retinitis 7 (25.92%); Papilloedema 6 (22.22%); Papilloedema with 6^th cranial nerve palsy 1 (3.7%); Isolated 6^th cranial nerve palsy 1 (3.7%) and optic neuritis 1 (3.7%). Ocular involvement was more common in double antigen group (68%) than spotted fever group (50%) or Scrub typhus group (21%) (P = 0.01). Ocular involvement was seen in 94% of the patients with CNS involvement. Cases with bilateral involvement (P = 0.01), pediatric age group (P = 0.01) and CNS involvement (P = 0.02) had poor visual outcome.Conclusion:Rickettsioses patients can have ocular manifestations with predominant posterior segment involvement during acute phase of illness. Ocular involvement was more common in the double antigen group. For any patient who presents with fever and rash living in endemic area, ophthalmic evaluation should be part of routine checkup during the acute phase of illness associated with less frequent ocular symptoms.     

Ocular Complications in Myelodysplastic Syndromes as Preleukemic Disorders

Purpose

To identify ocular complications in patients with myelodysplastic syndromes (MDS), who have a propensity to progress to acute myeloid leukemia (AML).

Methods

Forty-one patients with MDS were the subjects in this retrospective study, and 21 patients with AML were selected as controls. Reviewing their clinical records, we verified that corneal ulcer, iridocyclitis, vitreous hemorrhage, retinal hemorrhage, and optic neuritis had been evaluated using slit-lamp assessment and opthalmoscopy in all the patients. In this study, the MDS patients were classified into those with refractory anemia (RA) and those with refractory anemia with excess blasts (RAEB).

Results

Ocular complications were found in 19 (46.3%) of the 41 patients with MDS, comprising corneal ulcer (two cases), iridocyclitis (five), vitreous hemorrhage (one), retinal hemorrhage (ten), cotton wool spots (one), and optic neuritis (two). (Some patients had more than one ocular complication.) Ocular complications were identified in 12 of the 21 (57.1%) patients with AML. There was no significant difference in frequency of ocular complications between MDS and AML (P = 0.4892). In MDS, retinal hemorrhage was associated with significantly reduced platelet counts (P = 0.0063). The frequency of ocular complications was significantly higher in MDS-RAEB than in MDS-RA (P = 0.0478). Retinal hemorrhage was significantly more frequent in patients with MDS-RAEB than in patients with MDS-RA (P = 0.0433).

Conclusion

Ocular complications in MDS patients should be carefully examined as prognostic factors for progression to acute leukemia. Jpn J Ophthalmol 2005;49:377–383 © Japanese Ophthalmological Society 2005     

Ocular involvement in brucellosis

Objective: To report the ocular findings associated with brucellosis.Design: Cohort study.Participants: One hundred thirty-two patients with the diagnosis of brucellosis.Methods: Ocular manifestations of 132 patients with brucellosis admitted to the Uveitis-Behçet Service of Ophthalmology Department at the Ankara Education and Research Hospital in Turkey between May 1992 and May 2006 were evaluated prospectively. The diagnosis of brucellosis was based on clinical signs, Brucella agglutination tests, and blood cultures.Results: Ocular involvement was detected in 21% of brucellosis patients. The most frequent manifestations were anterior uveitis (41%) and choroiditis (32%), followed by panuveitis (9%), papilledema (9%), and retinal hemorrhages (9%). Forty-one percent of these patients were in the acute stage and 59% were in the chronic stage of brucellosis. Interestingly, all the patients with anterior uveitis were in the acute stage and all the other patients with choroiditis, papilledema, and retinal hemorrhages were in the chronic stage. All patients responded well to systemic antibiotic treatment along with topical or systemic corticosteroid treatment. No recurrence of ocular manifestations was detected during the follow-up period, after completion of a 2-month systemic antibiotic treatment.Conclusions: Because ocular involvement of brucellosis is frequent in endemic regions, detailed ophthalmic examination of all patients with brucellosis should be done routinely. Ocular brucellosis should be considered in the differential diagnosis of all forms of uveitis or choroiditis in endemic regions, and these patients should undergo serologic screening for brucellosis.     

Ocular manifestations of Behçet’s disease in Jordanian patients

PurposeTo study the prevalence, manifestations and severity of ocular involvement of Behçet’s disease in Jordanian patients.MethodsThe study population consisted of 43 patients diagnosed to have Behçet’s disease through Rheumatologist’s examinations conducted at Jordan University Hospital between January 2002 and July 2009. The sample involved patients who displayed ocular manifestations. This included 18 patients; 12 males and 6 females with a mean age of 35 years (SD = 17.26). Ophthalmological examinations and retrospective analysis of medical files were carried on.ResultsOcular manifestations were seen in 41.9% of patients. The most common manifestation for Behçet’s disease was vitritis with a prevalence of 55.6%, followed by anterior uveitis and retinal vasculitis (50% for each). On the other hand, the most frequent complications involved were cataract, cystoid macular edema (CMO), posterior synechiae and glaucoma with a prevalence of (44.4%), (33.3%), (11.1%) and (5.6%), respectively.ConclusionThe prevalence and severity of ocular lesions in Behçet’s disease is relatively low in Jordanian patients. This result indicates that early diagnoses and intervention might delay or even prevent vision loss for those patients.     

Ocular complications in leukemias and malignant lymphomas in children

PURPOSE: The aim of the study was to determine the prevalence and types of ocular abnormalities in children with leukemias or lymphomas. MATERIAL AND METHODS: We have studied 39 children (age 0.5-17 years) with leukemia or lymphoma. Ocular manifestations were present in 54% of study group (21 children). The most frequent ocular findings were seen in conjunctiva (33.4%). 15.4% patients presented with posterior segment findings, without loss of ocular acuity. Other symptoms were dry eye syndrome and proptosis. CONCLUSIONS: Children treated for leukemia or lymphoma should be systematically examined by ophthalmologist. Ophthalmic complications can be results of basic disease, treatment or manifestations of relapse.     

Ocular involvement in leukemia--a study of 288 cases

Two hundred and eighty-eight newly diagnosed cases of leukemia (164 males and 124 females, 167 adults and 121 children, 245 acute and 43 chronic, 151 myeloid and 137 lymphoid), aged between 6 weeks and 78 years, were examined for eye changes in the oncology wards within 2 days of diagnosis before starting chemotherapy. Ocular lesions were present in 102 patients (35.4%)--retinal vascular changes in 91 (31.6%); infiltration of ocular tissues in 5 (1.7%), and neuro-ophthalmic signs in 6 (2.1%) cases. Some of the patients had more than one ocular lesion in one or both eyes. The eye changes were seen more often in adults (49.1%) than in children (16.5%), and in myeloid leukemia (41.0%) than in lymphoid leukemia (29.2%). Eye symptoms were present in 29 patients (10%) at initial diagnosis. Since ocular lesions were detected in many asymptomatic leukemia patients, eye examination should be included as a part of routine evaluation at initial diagnosis in these patients.     

Prevalence of Ocular Manifestations of HIV/AIDS in the Highly Active Antiretroviral Therapy (HAART) Era: A Different Spectrum in Central South China

Abstract

Purpose: To investigate ocular manifestations of human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) in a population in central south China during a time of highly active antiretroviral therapy (HAART).

Methods: A cross-sectional study in central south China was performed between June 2009 and April 2010. Ocular examinations were performed on recruited patients with HIV/AIDS. Systemic information (including CD4+ T cell count) was also collected where possible.

Results: Among 1041 patients (2082 eyes) with HIV/AIDS enrolled in our study, we found a broad spectrum of ocular manifestations related to HIV/AIDS. The prevalence of HIV-associated ocular disease was 23.73% (247 patients). Of those with ocular complications, 87.85% had CD4 counts <200?cells/µL. HIV retinopathy (12.68%) was the most common HIV-associated ocular finding, followed by cytomegalovirus retinitis (6.72%). Prevalences of visual impairment and blindness were 7.59% and 0.77%, respectively.

Conclusions: This epidemiologic study shows the spectrum of ocular lesions associated with HIV/AIDS in central south China. Our findings highlight the need for routine ophthalmic examinations in this population, even in patients who are asymptomatic, especially those at high risk, in the era of HAART.     

Ocular Manifestations of Acquired Immunodeficiency Syndrome

Purpose

To investigate the patterns and risk factors of the ocular manifestations of acquired immunodeficiency syndrome (AIDS) and their correlation with CD4+ count in the era of highly active antiretroviral therapy (HAART).

Methods

This retrospective study examined 127 AIDS patients who presented to Soonchunhyang University Hospital. Data were collected from patient interviews, clinical examinations, and laboratory investigations. Ophthalmologic examinations included the best-corrected visual acuity, intraocular pressure, anterior segment and adnexal examination, and dilated fundus examination.

Results

Of the 127 patients with AIDS, 118 were on HAART and 9 were not. The mean CD4+ count was 266.7 ± 209.1 cells/µL. There were ocular manifestations in 61 patients (48.0%). The incidence of anterior segment manifestations was higher than posterior segment manifestations at 28.3% and 19.7%, respectively. The mean CD4+ count was significantly (p < 0.05) lower in the patients with posterior versus anterior segment ocular manifestations. The most common ocular manifestation was retinal microvasculopathy (15.0%), followed by keratoconjunctivitis sicca (14.2%), conjunctival microvasculopathy (9.4%), cytomegalovirus retinitis (3.1%), herpes zoster ophthalmicus (2.4%), and blepharitis (1.6%). Retinal microvasculopathy and cytomegalovirus retinitis were common in patients with CD4+ counts <200 cells/µL, while keratoconjunctivitis sicca and conjunctival microvasculopathy were common in patients with CD4+ counts of 200 to 499 cells/µL. There was a significant (p < 0.05) association between ocular manifestation and CD4+ count or age.

Conclusions

The introduction of HAART has changed the landscape of ocular presentations in patients with AIDS. In this study, anterior segment and external ocular manifestations occurred more frequently than posterior segment manifestations. Also, the mean CD4+ count was significantly lower in patients with posterior segment ocular manifestations versus anterior segment ocular manifestations. We found that CD4+ count and age >35 years were independent risk factors for developing ocular manifestations.     

Ocular abnormalities in multi-transfused beta-thalassemia patients

Aims:The aim of this study was to assess ocular changes in thalassemia patients who have received multiple transfusions and chelate binding therapy in order to avoid iron accumulation.Results:All the thalassemic patients were asymptomatic, but abnormal ocular findings (dry eye (33.3%), cataract (10.2%), retinal pigment epithelium degeneration (16.7%), color vision deficiency (3.7%), and visual field defects (33.7%)) were seen in 68.5% of thalassemic group. The prevalence of ocular abnormalities in normal group was 19.4%, which was significantly lower than that in thalassemia patients (P = 0.000). No significant correlation was found between ocular abnormalities and mean serum ferritin level (P = 0.627) and mean hemoglobin concentration (P = 0.143). Correlation of number of blood transfusion with the presence of ocular abnormalities was found to be statistically significant (P = 0.005).Conclusions:As life expectancy for beta-thalassemia patients extends, regular ophthalmological evaluation to detect early changes in their ocular system is recommended.     

Ocular manifestations of familial amyloidotic polyneuropathy type I: long term follow up

AIMS—To obtain precise information on ocular manifestations of familial amyloidotic polyneuropathy (FAP) type I, the incidence of five main ocular manifestations—abnormal conjunctival vessels (ACV), keratoconjunctivitis sicca (KCS), pupillary abnormality, vitreous opacity, and glaucoma, were compared through long term follow up.
METHODS—Ocular examinations were performed in 37 FAP type I patients (Met30) from once to 12 times over a period of 1 to 12 years and 7 months.
RESULTS—The following incidences were observed on initial examination of each patient with FAP: ACV in 75.5%, pupillary abnormalities in 43.2%, KCS in 40.5%, glaucoma in 5.4%, and vitreous opacity in 5.4%. All ocular manifestations increased with the progression of FAP, and the incidence of ACV reached 100% during follow up: this may be helpful in the diagnosis of FAP.
CONCLUSION—Since no precise statistical ocular study on FAP with long term follow up has been performed, this report may provide important information to help elucidate the mechanism of the amyloid distributing process in the amyloid targeted organs of FAP and to provide the natural course of ocular manifestations of FAP.

     

Characterization of the Spectrum of Ophthalmic Changes in Patients With Alagille Syndrome

PurposeThe purpose of this study was to characterize the phenotypic spectrum of ophthalmic findings in patients with Alagille syndrome.MethodsWe conducted a retrospective, observational, multicenter, study on 46 eyes of 23 subjects with Alagille syndrome. We reviewed systemic and ophthalmologic data extracted from medical records, color fundus photography, fundus autofluorescence, optical coherence tomography, visual fields, electrophysiological assessments, and molecular genetic findings.ResultsCardiovascular abnormalities were found in 83% of all cases (of those, 74% had cardiac murmur), whereas 61% had a positive history of hepatobiliary issues, and musculoskeletal anomalies were present in 61% of all patients. Dysmorphic facies were present in 16 patients, with a broad forehead being the most frequent feature. Ocular symptoms were found in 91%, with peripheral vision loss being the most frequent complaint. Median (range) Snellen visual acuity of all eyes was 20/25 (20/20 to hand motion [HM]). Anterior segment abnormalities were present in 74% of the patients; of those, posterior embryotoxon was the most frequent finding. Abnormalities of the optic disc were found in 52%, and peripheral retinal abnormalities were the most frequent ocular finding in this series, found in 96% of all patients. Fifteen JAG1 mutations were identified in 16 individuals; of those, 6 were novel.ConclusionsThis study reports a cohort of patients with Alagille syndrome in which peripheral chorioretinal changes were more frequent than posterior embryotoxon, the most frequent ocular finding according to a number of previous studies. We propose that these peripheral chorioretinal changes are a new hallmark to help diagnose this syndrome.     

Orbital and ocular manifestations of acute childhood leukemia: clinical and statistical analysis of 180 patients

PURPOSE: To investigate the association between presence of orbital or ocular lesions and type and stage of leukemia and to investigate whether orbital and ocular lesions are significant in predicting leukemia prognosis. METHODS: The authors evaluated 180 patients with acute childhood leukemia. Lesions associated with leukemia may be classified as specific (due to leukemic infiltration of various ocular tissues), nonspecific (due to one of the secondary complications), or iatrogenic manifestations caused by chemotherapy. Risk-based treatment assignment is based on clinical and laboratory features at diagnosis. Children with presenting white blood cell count below 50,000 mm3 are considered at standard risk for treatment failure, while all others are considered at high risk for treatment failure. RESULTS: Specific lesions were noted in 66% of patients with acute myeloid leukemia (AML) and 11.5% patients with acute lymphocytic leukemia (ALL) (p<0.05), and were more severe in patients with high risk leukemia than in patients with standard risk leukemia. Orbital or ocular lesions were noted more commonly in patients with AML (66.6%) compared to patients with ALL (15.1%). In both the AML and ALL groups, there was a higher frequency of leukemic relapses in the bone marrow and/or central nervous system in patients with specific lesions (63.1%) compared to patients with nonspecific lesions (42%), and in patients without orbital or ocular lesions (29.2%) (p<0.05). CONCLUSIONS: In both the AML and ALL groups, the presence of specific orbital or ocular lesions was associated with a higher frequency of bone marrow relapses and CNS involvement (p<0.05), leading to a lower survival rate.     

Manifestations of ocular rosacea in females with dark skin types

BackgroundCurrent knowledge about ocular rosacea in dark skin individuals is lacking. The prevalence of ocular rosacea varies considerably among studies and is probably higher than previously presumed.ObjectiveTo estimate the prevalence and pattern of ocular rosacea among dark skinned female patients, compare it with fair skinned, and to correlate the severity of cutaneous disease with ocular findings.MethodFemale patients diagnosed with rosacea between 2011 and 2013 were studied prospectively. They were referred to ophthalmology for clinical observations and slit lamp examination. In all patients Schirmer and Tear break up time tests to diagnose dry eye were performed.ResultFifty six consecutive female patients, joined the study with different skin types ranging from skin type 4 to 6. A total of 43 patients (76.8%) were positive for ophthalmologic findings. The most frequent symptoms were itching, burning sensation and redness, while the most frequent signs were meibomian gland dysfunction, dry eyes, eyelid telangiectasia and irregular margin. Significant correlation was noted between meibomian gland dysfunction and irregular lid margin (P = 0.003). Dry eye and Schirmer test significantly correlated with eye lid telangiectasia (p = 0.004; 0.015) respectively. No significant correlation was found between the severity of cutaneous disease and ocular findings.ConclusionOcular rosacea in dark skinned females is a common presentation and is comparable to that reported for fair skin, with eyelid telangiectasia and meibomian gland dysfunction being early phenomena. Earlier onset and more benign course were seen compared to other studies. Ocular and cutaneous rosacea are independent of each other.     

Ophthalmologic manifestations as the initial presentation of chronic myeloid leukemia: A review

The presentation of chronic myeloid leukemia (CML) can be variable and related to the phase of the disease. It can manifest a wide range of symptoms and signs; ocular involvement is reported in patients with leukemia at the time of diagnosis. We describe ophthalmic manifestations as an initial presentation in patients with CML. We identified 38 publications between 1971 and 2020 describing ocular manifestations in CML. Ophthalmic problems occur either from direct or indirect infiltration of neoplastic cells or from secondary causes. Although nearly all ocular structures may be affected, leukemic retinopathy is the most frequent clinical manifestation. Others include iris infiltration, anterior uveitis, hypopyon, exudative/serous retinal detachment, and optic nerve infiltration.     

Ocular Dominance in Open-angle Glaucoma: The Shifting Trend Depending on Stage of the Disease

PurposeTo investigate the characteristics and distribution of ocular dominance in primary open-angle glaucoma eyes. In addition, we tried to catch any trend of ocular dominance according to the stage of disease.MethodsTwo hundred participants with bilateral open-angle glaucoma underwent ocular dominant testing by “the hole-in-a-card” test. Using optical coherence tomography, macular ganglion cell-inner plexiform layer, as well as circumpapillary retinal nerve fiber layer thickness were measured and compared according to ocular dominance. Of the two eyes of one subject, the eye with less glaucomatous damage based on mean deviation was considered to be the “better eye” in our study.ResultsOcular dominance was in the right eye in 66% of the population and ocular dominance was positioned in the better eye in 70% of the population (p = 0.001 and p = 0.002, respectively). In conditional logistic regression analyses, right eye and better mean deviation were significantly associated with ocular dominance (p = 0.001 and p = 0.002, respectively). Ocular dominance tends to be present in the better eye and this trend was more apparent as the severity of glaucoma increased. Intereye comparison of visual field indices and retinal nerve fiber layer thickness between dominant versus nondominant eye become apparent in moderate and advanced glaucoma whereas it was not as apparent in early glaucoma.ConclusionsIn glaucomatous eyes, laterality and severity of glaucoma determined ocular dominance. Intereye difference between nondominant and dominant eyes increased with the severity of glaucoma. Our findings suggest the existence of potential reciprocal interactions between ocular dominance and glaucoma.     

The AFIP history of ocular leprosy

PurposeTo investigate the Armed Forces Institute of Pathology (AFIP) experience with Ocular Leprosy.MethodsThe AFIP data banks were screened for cases with diagnosis of ocular leprosy. Files and slides stained with Hematoxylin-eosin and acid-fast staining were reviewed by the Division of Ocular Pathology and by the Infectious Diseases Pathology Branch.ResultsTwenty-five cases were found from 1951 to 1985 and there were 15 males and 7 females and in 3 cases the sex was not given. The disease process ran from 4 months to 50 years in this series. Three patients also had systemic mycobacterium tuberculosis infections. The clinical manifestations of leprosy did not correlate with the histopathological findings. Clinically, corneal manifestations were the most common clinical presentation in 15 cases with only one perforation, iridocyclitis following in 9 patients and eyelid abnormalities ranging from ectropion to trichiasis in 7 patients. Two patients at autopsy had lepromatous cells in the ciliary body as the only ocular manifestation.ConclusionsIn summary, this paper represents a series of ocular leprosy cases from the AFIP which shows the severity of ocular involvement, the predilection of anterior segment involvement and a large number of histopathologic indeterminate cases. The current immigrant and refugee crisis warrants revisting this ancient disease in the differential diagnosis.     

Dupilumab-associated ocular surface disease: presentation,management and long-term sequelae

ObjectivesTo determine the presenting features of ocular surface disease in patients with atopic dermatitis (AD) treated with dupilumab at a tertiary, university hospital. To establish the need for treatment of dupilumab-associated ocular surface disease and report any long-term effects on the ocular surface.MethodsA retrospective analysis of consecutive patients treated with dupilumab for AD between January 2017 and August 2019 was undertaken. Data were collected on demographics, incidence and type of ocular disease features, natural history and treatment.ResultsA total of 50% (14/28) patients developed ocular symptoms with a mean time of onset of 6.75 (±6.1) weeks from starting dupilumab. Of these, 69% (9/13) were diagnosed with conjunctivitis associated with cicatrisation in two patients and periorbital skin changes in four. Of these nine, four had prior history of atopic keratoconjunctivitis. All were treated with topical steroids; two required additional ciclosporin drops. In all, 67% (6/9) patients went on to have on-going ocular inflammation requiring maintenance drops at a mean of 16 (±6.9) months of follow-up. All patients had improvement in their AD severity; only one patient discontinued dupilumab due to ocular side effects.ConclusionThe rate of dupilumab-associated ocular surface disease was 32%. Periorbital skin changes and conjunctival cicatrisation were noted in association with conjunctivitis. Ocular surface disease improved on topical steroids and ciclosporin but 67% of patients needed on-going treatment. Close liaison with an ophthalmologist should be considered in those patients who develop conjunctivitis or have a past history of severe ocular surface disease.Subject terms: Eye manifestations, Conjunctival diseases