Amniotic membrane transplantation for ocular surface reconstruction in Stevens-Johnson syndrome

PURPOSE: To evaluate amniotic membrane transplantation (AMT) for ocular surface reconstruction in Stevens-Johnson syndrome (SJS). DESIGN: Prospective interventional case series. PARTICIPANTS: Ten consecutive patients (10 eyes) with SJS that underwent AMT as the first step in staged ocular surface reconstruction were included. METHODS: Amniotic membrane was processed under sterile conditions from a fresh placenta obtained from cesarean section in a seronegative pregnant woman and stored at -700 degrees C. Symblepharon release, excision of epibulbar fibrous tissue, and clearing of the fibrovascular membrane over the cornea was performed in all cases. Amniotic membrane covered the entire bulbar surface up to the fornices in five eyes; cornea and the perilimbal area in two eyes; cornea, the inferior bulbar surface, and the lower fornix in two eyes; and cornea and the superior bulbar surface in one eye. Obliterated fornices were deepened by use of fornix-formation sutures in all eyes. Symblepharon ring was placed postoperatively for 3 weeks to 2 months. Mean postoperative follow-up was 13.5 months (SD, +3.8 months; range, 9-30 months). MAIN OUTCOME MEASURES: Restoration of adequate bulbar surface free of symblepharon and good fornix depth were the main outcome measures. RESULTS: Complete corneal reepithelization occurred in all eyes between 1 and 6 weeks. Adequate bulbar surface and fornix depth were achieved in nine eyes, all of which were free of symblepharon at the final follow-up visit. Cicatricial entropion resolved in four of five lower eyelids and one of two upper eyelids after AMT. One patient had a central corneal melt that required or necessitated a penetrating keratoplasty. CONCLUSIONS: AMT restores adequate bulbar surface and fornix depth and prevents recurrence of symblepharon in severe cases of SJS.     

Stevens-Johnson syndrome in India - risk factors, ocular manifestations and management

We conducted a study to analyse the presentation, risk factors, ocular manifestations and ophthalmic management results of Stevens-Johnson syndrome (SJS) in Indian patients. A total of 20 patients with SJS with ocular involvement were studied. Female predominance (70%) was observed. The age ranged from 10 to 30 years (mean +/- SD: 16.85 +/- 5.96). The commonest precipitating risk factor was oral drug intake (80%), sulphonamide (30%; sulphamethoxazole-trimethoprim) being the commonest. One patient had reaction to ciprofloxacin which has not been reported previously. Four patients (20%) had spontaneous onset with no identifiable risk factor. None of the patients had specific infection per se as a risk factor. Conjunctival involvement and its sequelae were the major ocular manifestations. At presentation, the majority of the eyes (68%) had visual acuity less than 3/60. Despite appropriate medical treatment and surgical interventions (11 eyes; 28%) vision continued to deteriorate. In 1 eye, the vision improved after stem cell transplantation. Therapeutic penetrating keratoplasty could preserve ocular integrity in 1 patient.     

Significant impact of cryopreserved amniotic membrane on acute ocular Stevens-Johnson syndrome and toxic epidermal necrolysis

The ocular surface is covered by an epithelium encompassing an area including the cornea,the limbus and the conjunctiva bordered by the upper and lower lids.The healthy state of the ocular surface epithelium depends on a stable and protective preocular tear film when the eye is open.A stable preocular tear film is governed by sound ocular surface defense that involves effective neuroanatomic integration of compositional and hydrodynamic factors by two neural reflexes (1).     

Management of ocular complications of Vogt-Koyanagi-Harada syndrome

Background Vogt-Koyanagi-Harada syndrome (VKH) is a multisystem disorder, characterized by the T-cell-mediated autoimmune process directed against melanocytic antigens in the ocular, nervous, auditory and integumentary systems. The ocular hallmarks of the disease involve severe bilateral panuveitis associated with exudative retinal detachment. Case report We report a pediatric case of probable VKH Syndrome with isolated ocular findings, in which bilateral vitritis, papillitis and serous retinal detachments involving the macula with intra-retinal edema and choroideal thickening were detected. Intervention Subtenon triamcinolone acetonide injection was performed in addition to systemic corticosteroid and cyclosporine treatments. Evolution Prompt improvement was seen in the ocular manifestations of VKH syndrome, which persisted for at least 4 months. Subtenon corticosteroid injection, together with systemic corticosteroid and cytotoxic treatment, prevented the ocular complications of invasive intraocular treatment modalities, and at the same time reduced the systemic corticosteroid dosage. Conclusion We recommend use of ocular invasive treatment modalities in Vogt-Koyanagi-Harada Syndrome only if the disease progression cannot be controlled. If systemic medications fail to stop the progression of the disease activity, subtenon injections may be considered before intraocular treatment modalities. The authors have full control of all primary data and they will allow International Ophthalmology to review their data upon request.     

Chronic ocular complications in lamotrigine vs. trimethoprim-sulfamethoxazole induced Stevens-Johnson syndrome/toxic epidermal necrolysis

PurposeThe purpose of this study is to compare the severity of chronic ocular complications of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) induced by lamotrigine (LT) vs. trimethoprim-sulfamethoxazole (TS).MethodsThis retrospective cross-sectional study evaluated all SJS/TEN patients treated within our hospital network from 2008 to 2018. Inclusion criteria included patients with reactions identified as caused by either LT or TS, and patients with at least one ophthalmology follow up in the chronic phase (≥3 months from disease onset). Primary outcome measures included LogMAR best-corrected VA at most recent visit and the presence or absence of severe ocular complications (SOC). Secondary outcome measures included chronic ocular complication severity scores using a modified Sotozono scoring system.ResultsForty-eight eyes of 24 patients were included in the study. The mean duration of follow-up was 39.50 ± 35.62 vs. 48.17 ± 33.09 months, respectively (p = 0.482). The LT group had worse average VA at the most recent visit (LogMAR VA; 0.508 vs. 0.041, p < 0.0001) and had a higher prevalence of SOCs (66.7% vs. 8.3%, p = 0.0038). The LT group scored worse on Sotozono chronic complications scores for the cornea (1.875 vs. 0.5, p = 0.0018), eyelid margin (5.583 vs.3.083, p = 0.0010), and overall condition (8.500 vs. 4.833, p = 0.0015). Sub-analyses showed that a moderate or severe acute ocular severity score was a significant predictor of chronic outcomes.ConclusionsCompared to patients with TS-induced SJS/TEN, patients with LT-induced SJS/TEN developed worse chronic ocular complications on several parameters. Future prospective studies are warranted to provide additional insight into the drug type as a predictor of chronic ocular complications.     

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.     

Dacryocystoceles in the aftermath of Stevens-Johnson syndrome

Although Stevens-Johnson syndrome has been reported to result in canalicular and nasolacrimal duct obstruction, reports of dacryocystocele formation are lacking. We describe the occurrence and management of bilateral dacryocystoceles related to Stevens-Johnson syndrome. A 45-year-old man, recovered from Stevens-Johnson syndrome, presented with bilateral medial canthal masses and intermittent overlying facial cellulitis. Examination revealed severe keratitis sicca with extensive corneal scarring, cicatricial occlusion of all puncta, and palpable medial canthal masses bilaterally. Computed tomography demonstrated bilateral cystic masses consistent with dacryocystoceles. Bilateral lacrimal sac/cyst excision was attempted, but the left cyst recurred within 6 months after surgery. A dacryocystorhinostomy was then performed, effectively marsupializing the cyst. The puncta were not canalized and stents were not placed. The masses have not recurred with 2 years of follow-up. Dacryocystoceles may arise in the setting of Stevens-Johnson syndrome. Successful management may be achieved with marsupialization to the nasal cavity (dacryocystorhinostomy).     

Cataract surgery in Stevens-Johnson syndrome

Cataract surgery can be safely performed in patients with Stevens-Johnson syndrome (SJS) with visually significant cataracts. Maintaining ocular surface integrity improves the prognosis of cataract surgery in this high-risk population. We present the outcome of cataract surgery in a retrospective chart review of 3 eyes in 2 patients with SJS. Uneventful extracapsular cataract extraction with intraocular lens implantation was performed. Follow-up ranged from 3 to 24 months. The visual acuity and ocular surface integrity were reviewed. The preoperative visual acuity was counting fingers at 1 meter in all 3 eyes. The best corrected visual acuity (BCVA) improved to 20/40 in 2 eyes and 20/50 in 1 eye postoperatively. A drop in BCVA ranging from 20/100 to 20/200 was noted during follow-up.