Simple oral mucosal epithelial transplantation (SOMET) for ocular surface reconstruction in Stevens-Johnson Syndrome: A case report.

INTRODUCTION: We report the clinical outcome of a novel surgical technique called simple oral mucosal epithelial transplantation (SOMET) for the treatment of limbal stem cell deficiency (LSCD) in a patient with Stevens-Johnson Syndrome (SJS). PRESENTATION OF CASE: An eighteen-year-old girl was diagnosed as having chronic bilateral ocular sequelae of SJS. She initially underwent mucous membrane grafting (MMG) in both eyes for lid margin keratinization. Over the course of the next decade, the ocular surface cicatrization worsened in her left eye, leading to progressive symblepharon formation with total corneal conjunctivalization. She then underwent ocular surface reconstruction using bulbar MMG and SOMET. Following SOMET, the ocular surface epithelialized within 3 weeks and remained stable throughout the follow-up period. At one-year postoperatively, the visual acuity had improved from light perception to 20/250 unaided, and to 20/100 with scleral contact lens correction in the left eye. DISCUSSION: Simple limbal epithelial transplantation (SLET) has been a boon for the treatment of unilateral LSCD. Allogeneic SLET and kerato-limbal allografts can be useful for patients with bilateral disease, however this exposes the patients to the risks of long-term systemic immunosuppression. SOMET combines the benefits of cultivated oral mucosal epithelial transplantation (COMET) and SLET, and is an autologous and single-staged surgical alternative for patients with bilateral LSCD. CONCLUSION: This case demonstrates that SOMET is a viable surgical option in cases with bilateral LSCD, eliminating the need for an allogeneic limbal graft, systemic immunosuppression, or laboratory cell culture.

Aqueous-deficient dry eye disease: Preferred practice pattern guidelines on clinical approach, diagnosis, and management.

Dry eye disease (DED) is a broad term that includes a diverse group of clinical disorders. Aqueous-deficient dry eye (ADDE), a subtype of DED, is characterized by decreased tear production by the lacrimal gland. It can be seen in up to one-third of individuals with DED and can be comorbid with a systemic autoimmune process or occur secondary to an environmental insult. Since ADDE can be a source of long-term suffering and severe visual impairment, early identification and adequate treatment are imperative. Multiple etiologies can underlie ADDE, and it is critical to identify the underlying cause to not only improve the ocular health but also to improve the overall quality of life and well-being of affected individuals. This review discusses the various etiologies of ADDE, highlights a pathophysiology-based approach for evaluating underlying contributors, outlines various diagnostic tests, and reviews treatment options. We present the current standards and discuss ongoing research in this field. Through this review, we propose a treatment algorithm that would be useful for an ophthalmologist in diagnosing and managing individuals with ADDE.

Management of an unresponsive case of HSV keratouveitis with trabeculectomy and DSAEK: A case report.

INTRODUCTION AND IMPORTANCE: This case demonstrates an unusual presentation of Herpes simplex virus (HSV) ocular infection and the challenges faced during the management of its complications. PRESENTATION OF THE CASE: A thirty year-old lady, a steroid responder with HSV keratouveitis, was referred for non-response to treatment with the prophylactic dose of oral acyclovir and acetazolamide. She presented with large epithelial bullae, anterior chamber reaction, and raised intraocular pressure in her right eye. Initially, she responded to the therapeutic dose of oral acyclovir, but on follow-up visits, she developed high intraocular pressures of up to 45 mmHg on maximum medical therapy. Hence, trabeculectomy with mitomycin-C was performed. One year later, she developed corneal endothelial decompensation, for which a Descemet's stripping automated endothelial keratoplasty (DSAEK) was done. Eight months post-operatively, she had a best corrected visual acuity of 20/20, clear corneal graft, quiet anterior chamber, and well-controlled intraocular pressures. DISCUSSION: HSV trabeculitis is associated with inflammation of the anterior chamber, endothelitis and raised intra-ocular pressure. A combination of anti-viral, anti-inflammatory, and anti-glaucoma medications helps in the management. However, glaucoma filtration surgery is often needed to the control intra-ocular pressure. Chronic recurrent episodes eventually lead to endothelial failure and demand endothelial keratoplasty (EK). It is prudent to adopt certain measures to perform EK in these phakic eyes without causing any iatrogenic damage to the filtration bleb as well as to the clear crystalline lens. CONCLUSION: This case highlights the difficulties of treating HSV-related keratouveitis with uncontrolled glaucoma, problems of associated steroid response, and complexities in performing corneal endothelial procedures in young phakic patients especially post-trabeculectomy.

A rare ophthalmic complication: Expulsive suprachoroidal hemorrhage.

Background: Expulsive suprachoroidal hemorrhage is a rare but dreadful complication of any ophthalmic surgery. Hence an ophthalmologist should know about the various risk factors, methods for preventing such a situation, and be aware of the various options for timely management to tackle the situation. Purpose: To discuss the risk factors, intraoperative signs, and ways of managing expulsive choroidal hemorrhage. Synopsis: We discuss two cases having multiple risk factors, where the patients underwent tectonic penetrating keratoplasty. In view of extensive involvement of ocular structures, the patients were clearly explained about the guarded visual prognosis and the risk of auto-evisceration. Following trephination of host cornea, rise in intraocular pressure was identified by the increasing size of the vitreous seen prolapsing through the wound and markedly visible pulsations, along with subconjunctival bleeding. Suprachoroidal hemorrhage was suspected and immediate tamponade was given. However, bleeding was not controlled, and eventually, expulsion of all the intraocular contents occurred. Highlights: A surgeon must be aware of the risk factors, be prompt to identify the signs, and must take immediate actions for the management of expulsive choroidal hemorrhage, a rare but dreadful complication of intraocular procedures. Online Video Link: https://youtu.be/UnCH-lWGzwU.

Retinitis pigmentosa in Laurence-Moon-Bardet-Biedl syndrome in India: Electronic medical records driven big data analytics: Report II.

Purpose: To describe the clinical presentation and demographic distribution of retinitis pigmentosa (RP) in Laurence-Moon-Bardet-Biedl (LMBB) syndrome patients. Methods: This is a cross-sectional observational hospital-based study wherein 244 patients with RP in LMBB syndrome presenting to our hospital network between March 2012 and October 2020 were included. An electronic medical record database was used for data retrieval. Results: There were 244 patients in total, with a hospital-based prevalence rate of 0.010% or 1000/100,000 population. The mean and median age of patients was 15.22 ± 7.56 and 14 (IQR: 10-18.5) years, respectively, with the majority being in the age group of 11-20 years (133/244 patients; 54.50%). Males were more commonly affected (164 patients; 67.21%), and the majority (182 patients; 74.59%) were students. All 244 patients (100%) complained of defective central vision at presentation. More than one-fourth of the patients had severe visual impairment to blindness at presentation. Prominent retinal feature at presentation was diffuse or widespread retinal pigment epithelial degeneration in all patients. Conclusion: Patients with RP in LMBB syndrome present mainly in the first to second decade of life with severe visual acuity impairment to blindness early in life. It is important to rule out LMBB syndrome in early-onset RP with central visual acuity impairment. On the contrary, all patients diagnosed or suspected with LMBB syndrome systemic features at physician clinic should also be referred for ophthalmic evaluation, low vision assessment, rehabilitation, and vice versa.

Management of Symblepharon Prior to Keratoprosthesis in Chronic Ocular Burns: A Sequential Approach.

This report describes two cases with stepwise management of chronic ocular burn sequelae with concurrent total limbal stem cell deficiency (LSCD) and advanced symblepharon. Both were mono-ocular patients with the other eye being phthisical. In both cases, a sequential approach was planned, and at the first stage, the symblepharon was released to stabilize the ocular surface and form the fornices. This was followed by a type 1 keratoprosthesis (KPro) after four months of symblepharon release in the first case, and after nine months in the second case. In the first case, after symblepharon release, the resultant bare sclera was addressed with an oral mucous membrane graft (MMG). Over two years of follow-up, there was no evidence of recurrence of the symblepharon. No further surgical interventions were required. In the second case, after symblepharon release, the resultant bare sclera was addressed with a cryopreserved amniotic membrane (AM). Over eight years of follow-up, six episodes of recurrence of the symblepharon were noted over the optic of the KPro, necessitating trimming of the conjunctiva from over the optic. Thus, with these two cases, we would like to emphasize that addressing adnexal pathologies such as a symblepharon with an oral MMG before implanting a KPro, may help prevent further recurrences of symblephara and the need for multiple surgical interventions. The oral mucosa is a better alternative to the conjunctiva as compared to the AM in a mono-ocular patient where conjunctiva cannot be harvested from the contralateral eye.

Lacrimal Gland Insufficiency in Aqueous Deficiency Dry Eye Disease: Recent Advances in Pathogenesis, Diagnosis, and Treatment.

BACKGROUND: Aqueous deficiency dry eye disease is a chronic and potentially sight-threatening condition, that occurs due to the dysfunction of the lacrimal glands. The aim of this review was to describe the various recent developments in the understanding, diagnosis and treatment of lacrimal gland insufficiency in aqueous deficiency dry eye disease. METHODS: A MEDLINE database search using PubMed was performed using the keywords: "dry eye disease/syndrome", "aqueous deficient/deficiency dry eye disease", "lacrimal gland" and "Sjogren's syndrome". After scanning through 750 relevant abstracts, 73 eligible articles published in the English language from 2016 to 2021 were included in the review. RESULTS: Histopathological and ultrastructural studies have revealed new insights into the pathogenesis of cicatrising conjunctivitis-induced aqueous deficiency, where the lacrimal gland acini remain uninvolved and retain their secretory property, while significant ultrastructural changes in the gland have been observed. Recent advances in diagnosis include the techniques of direct clinical assessment of the lacrimal gland morphology and secretion, tear film osmolarity, tear film lysozyme and lactoferrin levels, tear film interferometry and lacrimal gland confocal microscopy. Developments in the treatment of aqueous deficiency dry eye disease, apart from the nanoparticle-based tear substitutes, include secretagogues like diquafosol tetrasodium and rebamipide, anti-inflammatory topical agents like nanomicellar form of cyclosporine and lifitegrast, scleral contact lenses, neurostimulation, and acupuncture for increasing the amount of tear production, minor salivary gland transplantation, faecal microbial transplantation, lacrimal gland regeneration and mesenchymal stem cell therapy. CONCLUSIONS: Significant advances in the understanding, diagnosis and management of lacrimal gland insufficiency and its role in aqueous deficiency dry eye disease have taken place within the second half of the last decade. Of which, translational breakthroughs in terms of newer drug formulations and regenerative medicine are most promising.