Acute Ocular Complications Research Articles

Correlations between histopathologic findings, serum biomarker levels, and clinical outcomes in Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe dermatological emergencies. The role of cytokines and chemokines in the pathogenesis, progression of the disease, and histopathologic features is not fully elucidated. To address this gap, we conducted a retrospective study examining the associations between 42 serum biomarkers, histopathologic findings, and clinical outcomes in SJS/TEN patients. We reviewed the medical records of 23 patients diagnosed with SJS/TEN. Regarding histopathology, our study did not reveal any significant associations between the degree of epidermal necrosis, dermal mononuclear cell infiltration, and clinical outcomes. However, an intriguing observation was made regarding the degree of dermal infiltration of CD8 + cells, which showed a negative correlation with the severity of acute ocular complications. Notably, serum levels of IFN-γ positively correlated with the number of CD8 + cells in dermal infiltration. Additionally, higher serum levels of myeloperoxidase were associated with greater degrees of epidermal necrosis, while serum Fas ligand and stem cell factor levels were elevated in individuals with increased dermal mononuclear cell infiltration. Furthermore, the levels of S100A8/A9 were significantly correlated with the SCORTEN and mortality rate. These findings provide insights into the intricate pathogenesis of the disease.

Acute Ocular Complications after Recently Diagnosed Goodpasture's Syndrome - An Unusual Case of Hypertensive Retinopathy.

Ernest Goodpasture first identified Goodpastureʼs syndrome (GS) in 1919. It is a rare disease with an estimated incidence of about 0.5 – 1 case per million per year. GS most frequently occurs in two age ranges, 20 – 30 years and 60 – 70 years, and typically includes the following triad: acute renal failure, pulmonary hemorrhage, and presence of anti-glomerular basement membrane (anti-GBM) antibodies. Although the overall incidence is relatively low, GS is the cause of approximately one-fifth of all cases of acute renal failure with rapidly progressive glomerulonephritis. If accompanied by pulmonary hemorrhage or secondary malignant hypertension, the disease may be life-threatening [1].

Characteristics of meibomian gland dysfunction in patients with Stevens-Johnson syndrome.

To evaluate the characteristics of meibomian gland dysfunction (MGD) in patients with Stevens–Johnson Syndrome (SJS) and investigate the risk factors for severe MGD.Sixteen patients with a history of SJS were evaluated for MGD. To assess the SJS severity acute ocular involvement score (AOS), acute systemic involvement score (ASS), and chronic ocular manifestation score (COMS) were measured. Meibomian gland parameters were evaluated using meibomian gland dropout score (meiboscore - using a Keratograph 5 M), meibum expression score (MES), meibum quality score (MQS), and lid margin abnormality score (LMAS). Correlations between severity of meibomian gland parameters and degree of ocular and systemic involvement of SJS were analyzed. Risk factors for development of severe MGD were identified.The patients’ mean age was 32.0 ± 14.3 years. Four patients were men and 12 were women. MGD had developed in 14 patients (87.5%). The meibomian gland parameters were significantly correlated with ocular and systemic degree of SJS as evaluated using AOS (P < .01), ASS (P < .01), and COMS (P < .01). Patients with severe MGD had a higher AOS (P < .01) and COMS (P = .02) values than those without severe MGD. On multivariate analysis, AOS higher than 2 was a significant risk factor for developing severe MGD (P = .03).MGD was a common ocular manifestation with SJS patients. Severity of meibomian gland parameters was correlated with AOS, ASS, and COMS, and the presence of acute ocular complications was a risk factor for severe MGD in patients with SJS.

Plasmapheresis, Intravenous Immunoglobulins, and Autologous Serum Eyedrops in the Acute Eye Complications of Toxic Epidermal Necrolysis.

Toxic epidermal necrolysis (TEN) is a rare, life-threatening, drug-induced, mucocutaneous disease, which can severely affect the ocular surface. The purpose of this study was to investigate the efficacy of plasmapheresis, human IV immunoglobulins (IVIg), and autologous serum (AS) eyedrops in the treatment of the severe acute ocular complications of TEN. A retrospective chart review of all patients admitted to the Burn Unit, Azienda Ospedaliero-Universitaria-Sassari, Sassari, Italy, from 2009 to 2015, identified 9 patients (2 men, 7 women; mean age 63.8 ± 24.7 years) with TEN. Bilateral, acute ocular surface complications were observed in 7 (78%) patients; 3 showed catarrhal conjunctivitis, whereas 4 had severe pseudomembranous conjunctivitis and corneal ulcers. All patients with TEN were immediately treated with plasmapheresis and human IVIg, which produced a marked improvement in the patients' general condition. In the 3 with catarrhal conjunctivitis, preservative-free artificial tears and topical antibiotics were beneficial. In the 4 with severe pseudomembranous conjunctivitis and corneal ulcers, treatment with AS eyedrops resulted in corneal and conjunctival epithelium healing over 3-6 weeks. After a minimum follow-up of at least 12 months, there were minimal/mild residual signs and symptoms of dry eye. Plasmapheresis and IVIg may be life-saving and contribute to reduce ocular surface inflammation in TEN. Autologous serum eyedrops, prepared after plasmapheresis completion and IVIg infusion, may be helpful in the management of the severe acute ocular complications of TEN.

Plasmapheresis and autologous serum eye‐drops in the treatment of acute ocular complications from toxic epidermal necrolysis (Lyell syndrome)

AbstractPurpose Toxic epidermal necrolysis (TEN) is a rare, life‐threatening, drug‐induced, mucocutaneous disease, which can affect the ocular surface. The purpose of this study was to evaluate the acute ocular complications from TEN and investigate on the efficacy of plasmapheresis and serum‐eyedrops in the treatment of this condition.Methods A retrospective chart review of all Burn Unit patients admitted from 2009‐2013 identified 7 TEN patients (2 men, 5 women; mean age: 69.6±20.3 years). TEN was associated with allopurinol use in 4 patients, with gefitinib in 1, and with trimethoprim/sulphamethoxazole in 1. In the remaining patient, the causative agent was not identified. Acute bilateral ocular complications were observed in 5 patients; 3 showed mild ocular surface inflammation, whereas 2 had severe pseudomembranous conjunctivitis with corneal ulcers. All TEN patients were immediately treated with plasmapheresis.Results In the 2‐3 weeks after plasmapheresis, there was a marked improvement of the patients' general condition. In those with mild ocular surface inflammation, the use of preservative‐free artificial tears and steroids was beneficial. In the 2 with pseudomembranous conjunctivitis and corneal ulcers, treatment with autologous serum eye‐drops and preservative‐free artificial tears was effective, resulting in corneal epithelium healing and mild conjunctival scarring.Conclusion Not all TEN patients have ocular complications at onset. Plasmapheresis may be life‐saving and contribute to reduce ocular surface inflammation. Autologous serum eye‐drops prepared after plasmaferesis may be helpful in the management of the acute ocular complications caused by TEN.

Prevalence of chronic ocular complications in Stevens-Johnson syndrome and toxic epidermal necrolysis.

Purpose:The aim of this study is to identify and grade the severity of chronic ocular complications in patients who suffered from Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) treated in Cape Town, South Africa.Methods:A total of 54 patients with SJS or TEN for 6 months or longer were examined. The ocular complications were classified into corneal, eyelid and conjunctival complications. The complications were graded from 0 to 3 depending on the severity.Results:A total of 108 eyes were included in the study. Medications caused SJS or TEN in all cases, and the most common associated drugs were anti-retroviral medications. 59.3% of patients were HIV-positive, with CD4 counts ranging from 6 to 521. Although only 11% of patients with SJS or TEN had acute ocular complications during the initial illness, 89% developed chronic ocular complications. Loss of the palisades of Vogt was the most common corneal complication. Among the six components of conjunctival and eyelid complications, irregularity of the mucocutaneous junction abnormalities was the most common, followed by mild conjunctival hyperemia. There was no statistically significant difference in the severity of chronic ocular complications between HIV-positive and HIV-negative patients (P = 0.4). In addition, the severity of chronic ocular complications was not statistically significantly associated with visual acuity loss (P = 0.3).Conclusion:We conclude that almost 90% of patients who are diagnosed with SJS or TEN will develop chronic ocular complications. Unless eyelids are severely affected, most chronic complications are mild to moderate ocular surface abnormalities and not necessarily vision-threatening complications.

“Triple-TEN” in the Treatment of Acute Ocular Complications From Toxic Epidermal Necrolysis

Toxic epidermal necrolysis (TEN) is a devastating form of Stevens-Johnson syndrome (SJS) with acute and chronic ocular complications. We present a novel aggressive combination strategy, termed "Triple-TEN", for the management of acute ocular TEN designed to minimize the risk of chronic, blinding sequelae. Two patients with life-threatening TEN accompanied by severe ocular surface defects and fulminant symblephara formation underwent "Triple-TEN" management of their acute ocular disease under aseptic techniques in the critical care setting, after failed treatment with intensive topical therapy and surgical division of symblephara. The Triple-TEN protocol comprises (1) subconjunctival triamcinolone (Kenalog 20 mg) administered into each of the fornices to curb the local inflammatory response without compromising systemic immunity, (2) placement of amniotic membrane tissue mounted on a polycarbonate skirt (ProKera) over the corneal and limbal regions to facilitate reepithelialization of the ocular surface, and (3) insertion of a steeply curved acrylic scleral shell spacer (Technovent, SC21) to vault the lids away from the globe providing a barrier to symblephara formation. In both cases, ocular surface inflammation resolved within 4 weeks with no progression of conjunctival cicatrization or evidence of limbal epithelial stem cell failure at 1 year follow-up. There were no long-term complications of the Triple-TEN regimen. Aggressive treatment with the Triple-TEN protocol for acute ocular TEN resistant to first-line therapy, may help prevent long-term blinding sequelae.

Flecked retina, choroidopathy, and retinal pigment epithelial tear in familial pulmonary arterial hypertension.

Thanks to advancement in treatment modalities, the medial survival rate of patients with familial pulmonary arterial hypertension (FPAH) has been improved. Unfortunately, ocular complications because of the chronically elevated systemic venous pressure become more frequent. The authors report new FPAH-associated fundus changes in a 50-year-old male patient treated with sildenafil. The anomalies were studied with autofluorescence, fluorescein and indocyanine green angiography, and spectral optical coherence tomography including enhanced depth imaging. Loss of vision and cystoid macular edema were associated with a flecked retina, a thick choroid, central serous choroidopathy-like changes in both eyes and with retinal pigment epithelium detachments and a retinal pigment epithelium tear in the right eye. Treatment of ocular findings associated with FPAH mainly involves optimal control of pulmonary arterial hypertension and ophthalmic supportive treatment toward preventing acute ocular complications. The ocular complications occur as a result of enhanced pressure in the superior vena cava and in the ophthalmic veins, resulting in dilation of the ocular veins and congestion of the choroid. Sildenafil treatment in FPAH may enhance the congestion of the choroid and can induce central serous choroidopathy-like changes. A flecked retina, central serous choroidopathy-like changes, and retinal pigment epithelium tear are rare complications of FPAH.

Ophthalmic Manifestations of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis and Relation to SCORTEN

To evaluate the severity of ocular involvement of patients with Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap, and to investigate the relationship of the SCORTEN (a severity-of-illness score for SJS and TEN based on a minimal set of well-defined variables calculated within 24 hours of admission) with eye disease in this patient population. Retrospective observational case series. Charts of all patients admitted to the Parkland Memorial Hospital Burn Center with a preliminary diagnosis of SJS, SJS/TEN overlap, or TEN between 1998 and 2008 were reviewed. Patients were included for study if they met clinical criteria, had positive diagnostic skin biopsy, and had dermatologic and ophthalmologic consultations. Eighty-two patients with a diagnosis of SJS, SJS/TEN overlap, or TEN met inclusion criteria. Ocular manifestations were classified as mild, moderate, or severe. Admission data were used to calculate the SCORTEN. Main outcome measure was the severity of ocular involvement with respect to diagnosis and SCORTEN. Overall, 84% of patients had ocular involvement (71% SJS, 90% TEN, 100% SJS/TEN overlap). There was no difference in the severity of acute ocular complications among groups. While the SCORTEN value did correlate well with mortality rate (correlation coefficient 0.97, P = .005), there was no correlation between the SCORTEN value and severity of eye involvement in the acute setting. There was also no association of any individual diagnosis of SJS/overlap/TEN with the severity of eye involvement, although eye findings are more common in TEN (P = .03). Ocular damage in the acute setting was more frequent in patients with epidermal detachment >10% of the total body surface area. The SCORTEN value did not correlate with the severity of eye involvement in the acute setting.

Acute ocular complications from self-administered topical Kermes

To report severe ocular complications and their management after self-administered topical kermes dye eye drops. A case report of a 55-year-old man who suffered severe ocular surface damage after application of topical kermes eye drops to his left eye. Active compounds of the kermes eye drops were studied for their composition. Patient reported decreased vision in the affected eye and the external eye examination revealed complete corneal abrasion, cicatrization of the conjunctiva, and symblepharon formation. The patient required immediate cleansing of the ocular surface along with irrigation. He was treated with topical corticosteroids and frequent lubrication. Gas chromatography/mass spectrometry analysis of the retrieved topical material revealed the presence of acid. The patient’s visual acuity improved from 20/200 before treatment to 20/25 after treatment. Topically administered kermes eye drops may cause severe ocular injuries. Public education, early recognition of such injuries, and timely intervention may prevent permanent damage to the ocular adnexae.

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.