Comprehensive approach to ocular consequences of Stevens Johnson Syndrome - the aftermath of a systemic condition

Background

Stevens Johnson Syndrome (SJS) can lead to end stage corneal blindness. This study describes the comprehensive treatment measures and their outcomes in the management of ocular sequelae and complications of SJS.

Methods

Four hundred sixty-four eyes of 232 patients of SJS who underwent surgical intervention (punctal cautery, mucus membrane grafting for lid margin keratinisation, fornix reconstructive procedures, tectonic procedures, keratoplasty and keratoprosthesis) were studied. It was a non-comparative, retrospective, interventional case series. The primary outcome was the change in the best corrected visual acuity (BCVA). Secondary outcome measures included an improvement in the ocular surface status as indicated by corneal epithelial fluorescein staining and Schirmer’s I strip wetting.

Results

The BCVA and the ocular surface status improved and/or stabilized in?>?70 % of eyes following punctal cautery (n?=?160) and > 80 % of eyes following lid margin mucus membrane grafting (n?=?238). BCVA improved in 50 % of eyes following fornix reconstructive procedures (n?=?24) with COMET (n?=?6), in 63.9 % eyes with the Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) lens (n?=?36), in 81.8 % of eyes after cataract surgery (n?=?22). A BCVA of ≥20/200 was achieved in 72.34 % of eyes following keratoprostheses procedures (n?=?47). The mean duration of follow up was 53.3?±?15.2 months.

Conclusion

The ocular sequelae of Stevens Johnson Syndrome can be blinding. They need to be identified and addressed early to retard the continued deterioration of the ocular surface. Our study aims to highlight the problem as well as the importance of comprehensive measures in the management of this potentially blinding disorder.     

累及眼部的Stevens-Johnson综合征(附22例分析)

目的 :对 2 2例累及眼部的Stevens Johnson综合征 (SJS)作一初步临床分析。方法 :收集 1990～ 2 0 0 0住院的SJS病例 10 5例。回顾性分析其中眼部受累的患者 2 2例 (占 2 0 95 % )。根据致敏药物、发病前所患疾病、临床表现、实验室检查总结分析其可能的病因、眼部表现规律及治疗。结果 :累及眼部的 2 2例SJS病人中 ,肯定或可能是药物过敏引起的占 86 3 6% ,其中 ,抗生素类占 45 45 %。眼部充血是最常见的体征占 10 0 % ,有 5 0 %的病人结膜和 /或角膜形成假膜 ,视力下降占 3 1 82 %。眼部治疗主要是应用抗感染和人工泪液治疗。结论 :药物过敏是SJS最常见的原因。以结膜充血和假膜形成为特征的卡他性结膜炎是SJS眼部受累的最常见表现 ,重者可导致视力下降 ,早期合理的眼部治疗对预防晚期并发症非常重要。     

The natural history of untreated ocular hypertension and glaucoma

Glaucoma is a chronic, progressive disease leading to irreversible blindness if left untreated; however, since reducing intraocular pressure has proven to be successful in slowing disease progression, little is known about the natural history of untreated glaucoma. This knowledge can be valuable in guiding management decisions in the era of personalized medicine. A systematic search was performed in Medline (PubMed), Embase, and Web of Science in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRIMSA) guidelines. The rate of structural and/or functional progression and conversion to glaucoma or to a more advanced stage of glaucoma are discussed for ocular hypertension and different types of open-angle glaucoma. Forty-three studies were included. Different rates of progression were found both among and within the different diagnostic groups that belong to the open-angle glaucoma spectrum. The highest rate was found in pseudoexfoliation glaucoma, followed by high tension glaucoma, normal tension glaucoma, and ocular hypertension, in decreasing order. The lowest rate was observed in glaucoma suspects. The known rates of progression provide valuable prognostic information for ophthalmologists and patients. Nonetheless, due to high variability among patients, individual progression cannot be accurately predicted and repeated follow-up examinations are required to estimate individual progression.     

Subfoveal neovascularization in the ocular histoplasmosis syndrome. A natural history study

The visual outcome of 74 eyes with the ocular histoplasmosis syndrome and active subfoveal subretinal neovascular membranes was studied retrospectively. Follow-up time ranged from 12 to 109 months (median, 36.5 months). Ten eyes (14%) retained visual acuity of 20/40 or better. Fifty-seven eyes (77%) suffered visual loss of 20/100 or worse and 36 eyes (49%) suffered visual loss of 20/400 or worse. Factors significantly associated with retaining vision of 20/40 or better were: age less than 30 years (P = 0.008); smaller membrane size (P = 0.0002); and absence of visual loss secondary to ocular histoplasmosis in the fellow eye (P = 0.053). Factors significantly associated with vision decreasing by four or more lines were: older patient age (P = 0.006); better initial visual acuity (P = 0.090); and more than 50% involvement of the foveal avascular zone (P = 0.059). These results were confirmed by multiple logistic regression. Final vision of 20/40 or better was associated with better initial visual acuity using univariate analysis, but this result was not confirmed by multiple logistic regression.     

Pfeiffer syndrome: systemic and ocular implications.

BACKGROUND: In 1964, Pfeiffer described a three-generation family in which eight individuals had a syndrome consisting of craniosynostosis, broad thumbs and great toes, and partial syndactyly of the hands and feet. Pfeiffer syndrome affects males and females equally, and is most commonly a result of de novo mutations, but can be inherited in an autosomal dominant fashion. Pfeiffer syndrome is considered Type V of the five acrocephalosyndactly syndromes (ACS), a group of rare genetic diseases that involve premature closure of the cranial sutures. Cohen, in 1993, further described Pfeiffer syndrome and it's various expression patterns by creating three subgroups of the syndrome. CONCLUSIONS: While Pfeiffer syndrome is clearly a rare disorder, affecting 15 of every 1 million births, there has been a series of publications reviewing the difficult differential diagnosis among Pfeiffer types and between the other acrocephalosyndactly syndromes. While these publications individually focus on a variety of specific systemic and ocular implications of the syndrome, together they encompass the scope of the syndrome. Since Pfeiffer syndrome mainly affects the craniofacial regions, the eye care professional plays an essential role in diagnosis and management. What follows are guidelines to aid in the diagnosis, ophthalmic and functional testing, and management of this disorder.     

Cytostatic and immunosuppressive treatment of ocular Beh?et syndrome

Ocular symptoms of Beh?et's syndrome in 16 patients and the results of cytostatic and immunosuppressive therapy with cyclosporin A (6 patients), chlorambucil (6 patients), and a combination of cyclophosphamide and procarbacin (4 patients) are described. The clinical course and therapeutic outcome were compared to the patients' visual acuity and the duration of the disease prior to institution of cytostatic therapy. Average follow-up was 4.6 years, maximum 9 years. The principal ocular symptom in all patients was hemorrhagic, occlusive periphlebitis. Other symptoms, in descending order of frequency, were chorioretinitis, iridocyclitis, complicated cataract, secondary glaucoma and exudative retinal detachment. The patients in whom cytostatic therapy was instituted no later than 6 months after onset of the disease showed an improvement in or stabilization of visual acuity, as well as a clear reduction in signs of intraocular inflammation and frequency of recurrence. No improvement in visual acuity or ocular symptoms was achieved in cases where the disease was very advanced, despite cytostatic-immunosuppressive therapy. The results support early institution of immunosuppressive therapy in cases with Beh?et's syndrome where severe, irreversible damage has not yet occurred and in which vision is threatened in both eyes. However, due consideration must be given to the potential risks of immunosuppressive therapy, in particular the risk of a malignant tumor.     

The effects of systemic cyclosporine in acute Stevens-Johnson syndrome/toxic epidermal necrolysis on ocular disease

PurposeTo evaluate the effect of systemic cyclosporine (CsA) on ocular disease in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) patients.MethodsIn this retrospective, comparative cohort study at a single center, patients with a diagnosis of SJS/TEN and with at least 3 months of follow up were divided into two groups: those who received systemic CsA and those who did not receive systemic CsA. Best-corrected visual acuity (BCVA) and chronic ocular surface complications score (COCS) at final follow-up were compared between the two groups.ResultsThe median age and follow-up period of patients was 29 years (range, 1.5–71 years) and 16.8 months (range, 3.67–91.58 months), respectively. BCVA, COCS, meibomian gland dysfunction, limbal stem cell deficiency, and the need for mucous membrane grafting and scleral lenses were not significantly different between patients who received systemic CsA as compared to patients who did not receive systemic CsA.ConclusionsIn this small cohort of patients with SJS/TEN, we could identify no association between the use of systemic CsA as a component of their initial therapy and chronic ocular complications.     

The natural history of glaucoma and ocular hypertension after pediatric cataract surgery.

INTRODUCTION: We sought to define the prevalence and natural history of ocular hypertension and glaucoma for at least a 10-year period after pediatric cataract surgery. METHODS: We conducted a prospective observational study of patients who received pediatric cataract surgery. Inclusion criteria included 2 directed ophthalmologic examinations performed at a minimum of 5 and 10 years after surgery. RESULTS: A total of 63 patients (22 with bilateral cataracts and 41 with unilateral cataracts) were examined at a median of 15.1 year (range, 10.3-21.3 years) after surgery. A majority of the subjects had glaucoma or ocular hypertension (ie, 59%; 37/63). Nineteen percent (12/63) had glaucoma (5/22 with bilateral cataracts and 7/41 with unilateral cataracts). Approximately half (7/12) had developed glaucoma during the first 5-year observational period and the remainder (5/12) developed it during the following observational period. Forty percent (25/63) of the patients had ocular hypertension in at least one aphakic eye (9/23 with bilateral cataracts and 16/40 with unilateral cataracts). The rate of progression from ocular hypertension to glaucoma over a mean observational period of 7.2 years (range, 6.2-8.1 years) was 23% (5/22). DISCUSSION: Patients who receive surgery for pediatric cataracts are at very high risk of developing ocular hypertension and glaucoma. Patients can develop late-onset glaucoma and ocular hypertension more than 10 years after surgery. Years of ocular hypertension may precede the diagnosis of late-onset glaucoma.     

The role of chemokines and their receptors in ocular disease

The migration and infiltration of cells into the eye whether blood-borne leucocytes, endothelial or epithelial cells occurs in many ocular diseases. Dysregulation of this process is apparent in chronic inflammation, corneal graft rejection, allergic eye disease and other sight-threatening conditions. Under normal and inflammatory conditions, chemokines and their receptors are important contributors to cell migration. To date, 47 chemokines and 19 chemokine receptors have been identified and characterised. In recent years, investigations into the role of chemokines and their receptors in ocular disease have generated an increasing number of publications. In the eye, the best understood action of these molecules has arisen from the study of their ability to control the infiltration of leucocytes in uveitis. However, the involvement of chemokines in angiogenesis in several ocular conditions and in the survival of corneal transplants demonstrates the multifaceted nature of their effects. Interestingly, the constitutive expression of chemokines and their receptors in ocular tissues suggests that certain chemokines have a homeostatic function. In this review, we discuss the nature and function of chemokines in health and disease, and describe the role of chemokines in the pathogenesis of different ocular conditions.     

The prevalence of ocular disease in chronic lymphocytic leukaemia

AIM: Previous surveys of ocular disease in leukaemia patients have shown the retina, choroid, and orbit as the most commonly involved sites. Depending on the type of leukaemia and the study design, the prevalence of ocular disease in leukaemia varies from 30 to 90%. Although chronic lymphocytic leukaemia (CLL) is the most common leukaemia in Western countries, the prevalence of ocular disease in CLL is not known. The aim of this prospective study was to estimate the prevalence of ocular disease in CLL. PATIENTS AND METHODS: All CLL patients attending either a teaching or district general hospital haematology clinic were invited to undergo a full eye examination. The clinical stage of the CLL (Binet) at the time of the eye examination and the most recent full blood count (FBC) indices were recorded for each patient. RESULTS: Over 6 months, 25 patients with an average age of 65 were recruited. A total of 18 patients had Binet stage A disease, three had stage B, and four stage C. The mean FBC indices at the time of examination were haemoglobin 13.5 g/dl, white cell count 37.4 x 10(9)/l, and platelets 172 x 10(9)/l. Only three patients (12%) had ocular disease that was likely to be a secondary complication of CLL. These secondary complications included bilateral posterior subcapsular cataract following radiotherapy, unilateral acute retinal necrosis, and unilateral conjunctival vascular anomalies. The presence of ocular complications was not related to the CLL stage or to the current FBC parameters. DISCUSSION: This study demonstrates that ocular involvement in CLL is uncommon, a reflection of the indolent course of CLL as compared to other leukaemias. Although sight-threatening ocular complications have been documented in this and other studies, the low prevalence of complications and the lack of association with disease parameters make it impossible to identify a 'high-risk' group. Routine screening of CLL patients for ocular complications is not justified.     

促红细胞生成素在眼部新生血管疾病发病中的作用

眼部新生血管是一组难治性致盲性眼病的共同临床表现.多种促新生血管因子是其发病的主要机制.促红细胞生成素(erythropoietin,EPO)不仅具有调节红细胞生成的作用,也是一种促新生血管因子,在生理和病理性血管形成过程中起重要作用.EPO在角膜新生血管、视网膜新生血管等形成过程中表达增加,在氧诱导的视网膜新生血管动物模型中,阻断EPO信号可以抑制新生血管形成.这些研究提示EPO在眼部新生血管疾病中起重要作用,有望成为治疗眼部新生血管的新靶点.     

Effect of highly active antiretroviral therapy (HAART) on the natural history of ocular manifestations in HIV-infected children

PURPOSE: To determine the effect of highly active antiretroviral therapy (HAART) on the natural history of ocular manifestations in HIV-infected children. METHODS: All of the HIV-infected children attending the Institute of Pediatrics, University of Milan, Milan, Italy, between 1982 and 2004 were studied. Every 3 months, they were physically examined and underwent indirect fundoscopy conducted by an experienced ophthalmologist; if diagnosed as having eye disease, they were evaluated by the ophthalmologist every week while on specific therapy and monthly thereafter. The clinical and laboratory findings before and after the introduction of HAART were compared. RESULTS: The cohort consisted of 117 HIV-infected children (61 males), with a follow-up ranging from 0.09 to 22.31 years (median, 16.33 years). A total of nine cases of ocular involvement (7.7%) were diagnosed between 1983 and 1994, before the introduction of HAART. All nine children died 4-24 months (median, 15 months) after the diagnosis of ophthalmic disease. No case of ocular involvement was observed after the introduction of HAART (P=0.011 vs. before HAART). CONCLUSION: The introduction of HAART has had a significant impact on the natural history of ocular manifestations in HIV-infected children, thus suggesting that a reduction in the frequency of ophthalmologic follow-up should be considered for HAART-treated HIV-infected children with immune reconstitution and no visual symptom.     

A case of bilateral silent sinus syndrome presenting with chronic ocular surface disease

The silent sinus syndrome is characterized by atelectasis of the maxillary sinus in the setting of subclinical maxillary sinusitis and obstruction of the osteomeatal unit. The resultant expansion of orbital volume causes enophthalmos and hypoglobus. A review of the literature reveals only unilateral cases. The authors present a case of bilateral silent sinus syndrome presenting with chronic ocular surface disease. A 56-year-old man was referred for tarsorrhaphy to treat a chronic recurrent corneal ulcer. Exam revealed an area of corneal thinning in the left eye. The patient appeared cachectic and there was enophthalmos, hypoglobus, and lagophthalmos on both sides, left worse than right. Due to the patient's appearance and a history of sinonasal malignancy, recurrent malignancy was suspected. A systemic workup was unrevealing, and radiographic studies showed the features of silent sinus syndrome bilaterally. The patient underwent sinus surgery and placement of a left orbital floor implant.     

Blinking Sam: the ocular afflictions of Dr. Samuel Johnson

The poor health of Samuel Johnson (1709-1784) has fascinated the public for more than 200 years. The illnesses of few famous men, with the possible exception of Napoleon, have attracted more speculation. Johnson was an outstanding 18th-century literary figure, an essayist, novelist, and poet, and is particularly famous as the creator of the first important dictionary of the English language. His writings and those of his physicians and friends, particularly his biographer, James Boswell, provide an intimate account of a cultural icon.