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We describe the outcomes of oral mucous membrane grafting as a surgical technique for unilateral lid margin keratinization following radiotherapy. A 47-year- old woman presented with an 8-month history of a white spot in her right eye. She had a history of adenoid cystic carcinoma of the right spheno-ethmoid sinus, for which she underwent radiotherapy. Slit-lamp evaluation revealed lid margin keratinization of the right upper and lower eyelids and a keratin plaque on the corneal surface. We performed excision of the keratin plaque and lid margin keratinization, followed by oral mucous membrane grafting of the upper and lower eyelid margins. Histopathological evaluation of the excised lid margin revealed keratinized stratified squamous epithelium, consistent with lid margin keratinization. The corneal surface and lid margins showed no recurrent keratin deposition at the final follow-up, 11 months postoperatively. [ABSTRACT FROM AUTHOR]

The posterior lid margin, where the mucocutaneous junction (MCJ) between the eyelid skin and tarsal conjunctiva is located, plays a critical role in maintaining the homeostasis of the ocular surface. Posterior migration of the MCJ leads to lid-margin keratinization (LMK), which has a domino effect on the delicate balance of the ocular surface microenvironment. This occurs most commonly following Stevens-Johnson syndrome/toxic epidermal necrolysis and is not known to regress spontaneously or with medical therapy. Over time, LMK causes blink-related chronic inflammatory damage to the corneal surface which may have blinding consequences. Lid-margin mucous membrane grafting (MMG) is the only definitive therapy for LMK. Timely MMG can significantly alter the natural course of the disease and not only preserve but even improve vision in affected eyes. Literature searches were conducted on PubMed, using the keywords “mucous membrane grafts,” “lid margin keratinization,” “Stevens-Johnson syndrome,” “toxic epidermal necrolysis,” “lid related keratopathy,” and “lid wiper epitheliopathy”. This review, which is a blend of evidence and experience, attempts to describe the indications, timing, surgical technique, postoperative regimen, and clinical outcomes of MMG for LMK. The review also covers the possible complications and pearls on how they can be effectively managed, including how suboptimal cosmetic outcomes can be avoided. The authors hope that this review will aid ophthalmologists, including cornea and oculoplasty specialists, to learn and perform this vision-saving surgery better, with the aim of helping their patients with chronic ocular surface disorders, relieving their suffering, and improving their quality of life.

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a spectrum of rare, severe immunological blistering skin reactions which are triggered by medication intake or infections. The acute phase is characterized by necrolysis of the skin and desquamation of mucosa, primarily oral and ocular, with significant mortality rates. The chronic phase is characterized by multi-organ sequelae with increased rates of morbidity and reduced quality of life for patients who have survived the acute phase. Since the primary goal in the acute phase is saving the life of the patient, ocular involvement is often missed and a significant proportion of patients present to an ophthalmologist with the chronic ocular sequelae. In India, chronic ocular sequelae and low vision are observed in two-thirds of patients who present in the chronic phase of SJS/TEN. In the chronic phase of ocular involvement, there are definite windows of opportunity which if targeted with specific interventions such as scleral lenses and mucous membrane grafts can help reduce the incidence of corneal blindness and improve the quality of life for patients with SJS/TEN. Over the last decade, several studies from India have advanced the understanding of the natural course of ocular involvement in SJS/TEN and the outcomes of timely interventions in the chronic phase of the disease. We present an overview of the epidemiology of ocular complications of SJS/TEN in India, the specific challenges faced in the management of ocular complications in the acute stage and recent advances in management of the chronic ocular complications of the disease.

The posterior lid margin, where the mucocutaneous junction (MCJ) between the eyelid skin and tarsal conjunctiva is located, plays a critical role in maintaining the homeostasis of the ocular surface. Posterior migration of the MCJ leads to lid-margin keratinization (LMK), which has a domino effect on the delicate balance of the ocular surface microenvironment. This occurs most commonly following Stevens-Johnson syndrome/toxic epidermal necrolysis and is not known to regress spontaneously or with medical therapy. Over time, LMK causes blink-related chronic inflammatory damage to the corneal surface which may have blinding consequences. Lid-margin mucous membrane grafting (MMG) is the only definitive therapy for LMK. Timely MMG can significantly alter the natural course of the disease and not only preserve but even improve vision in affected eyes. Literature searches were conducted on PubMed, using the keywords "mucous membrane grafts," "lid margin keratinization," "Stevens-Johnson syndrome," "toxic epidermal necrolysis," "lid related keratopathy," and "lid wiper epitheliopathy". This review, which is a blend of evidence and experience, attempts to describe the indications, timing, surgical technique, postoperative regimen, and clinical outcomes of MMG for LMK. The review also covers the possible complications and pearls on how they can be effectively managed, including how suboptimal cosmetic outcomes can be avoided. The authors hope that this review will aid ophthalmologists, including cornea and oculoplasty specialists, to learn and perform this vision-saving surgery better, with the aim of helping their patients with chronic ocular surface disorders, relieving their suffering, and improving their quality of life. [ABSTRACT FROM AUTHOR]

Stevens–Johnson Syndrome, a life-threatening disorder, can lead to severe ocular morbidity and corneal blindness. The discomfort and visual loss caused leads to a considerable impact on the quality of life. The goals of treatment include measures to reduce inflammation, thereby improving comfort and results for visual rehabilitation. The issues to be addressed include dry eye, lid margin keratinization, adnexal disorders, and fornix obliteration. Measures to address these like punctal cautery and mucous membrane graft have been discussed along with their surgical technique. Visual rehabilitation, following stabilization of the ocular surface, can be achieved with optical iridectomy, cataract surgery or in end-stage disease by performing the modified osteo-odonto keratoprosthesis or Type 2 keratoprosthesis. The procedures to stabilize the ocular surface help in preventing further deterioration of the ocular surface and contribute to improved vision and comfort.

Purpose: To report a rare presentation of bilateral, coexisting ocular surface disease in a case of Xeroderma pigmentosum and its successful management., Methods: Case report., Results: A 21-year-old male with Xeroderma pigmentosum presented with bilateral ocular surface squamous neoplasia (OSSN) along with central guttae in the right eye and corneal decompensation of the left eye. Subsequently, the patient developed dry eyes and lid margin keratinization in both eyes followed by perforation in the left eye. Sequential procedures both medical and surgical, including excision of the tumour, corneal transplantation and mucous membrane grafting addressing each of these ocular surface issues resulted in a successful outcome. There was no recurrence of the tumour over 3 years. Corneal transplantation is preferably done after a minimum of 6 months following excision. Mucous membrane grafting performed for progressive lid margin keratinization resulted in surface stabilization., Conclusions: In Xeroderma Pigmentosum, multiple ocular surface features can rarely coexist and be bilateral. Periodic evaluation of the surface for tumours, progressive dry eyes and endothelial function is recommended as a part of routine evaluation in Xeroderma pigmentosum. Surface procedures should precede intraocular intervention. Sequential management can result in successful outcomes.

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. [ABSTRACT FROM AUTHOR]

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a spectrum of rare, severe immunological blistering skin reactions which are triggered by medication intake or infections. The acute phase is characterized by necrolysis of the skin and desquamation of mucosa, primarily oral and ocular, with significant mortality rates. The chronic phase is characterized by multi-organ sequelae with increased rates of morbidity and reduced quality of life for patients who have survived the acute phase. Since the primary goal in the acute phase is saving the life of the patient, ocular involvement is often missed and a significant proportion of patients present to an ophthalmologist with the chronic ocular sequelae. In India, chronic ocular sequelae and low vision are observed in two-thirds of patients who present in the chronic phase of SJS/TEN. In the chronic phase of ocular involvement, there are definite windows of opportunity which if targeted with specific interventions such as scleral lenses and mucous membrane grafts can help reduce the incidence of corneal blindness and improve the quality of life for patients with SJS/TEN. Over the last decade, several studies from India have advanced the understanding of the natural course of ocular involvement in SJS/TEN and the outcomes of timely interventions in the chronic phase of the disease. We present an overview of the epidemiology of ocular complications of SJS/TEN in India, the specific challenges faced in the management of ocular complications in the acute stage and recent advances in management of the chronic ocular complications of the disease., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Shanbhag, Sangwan, Singh, Donthineni, Basu, Srinivasan, Agarwal and Iyer.)

Lid margin keratinization (LMK) is a chronic ocular sequela of Stevens-Johnson syndrome (SJS), which causes lid wiper epitheliopathy and progressive ocular surface damage. The exact etiopathogenesis of LMK, however, remains elusive. This review summarizes the potential pathophysiological mechanisms of LMK and describes its histopathological features. A literature search of articles discussing the pathophysiology of LMK in SJS was performed. The possible pathophysiologic mechanisms contributing to LMK, as identified on the literature review, included loss of the muco-cutaneous junction barrier leading to epidermalization, dyskeratosis involving the meibomian gland orifices, altered lid margin microbiome, and de novo squamous metaplasia of the marginal conjunctival epithelium. Based on these mechanisms, the possible sources of keratinized epithelium at the posterior lid margin in SJS could be the adjacent anterior eyelid skin, hyperkeratinized epithelium from the meibomian gland ductal orifices, or the inflamed marginal conjunctiva. The epithelial, sub-epithelial, and stromal changes seen in keratinized posterior lid margins in SJS patients undergoing mucous membrane grafting were also investigated. The findings revealed keratinizing squamous metaplasia of the posterior lid margin accompanied by subepithelial infiltration of helper T cells predominantly on the conjunctival side. The visible meibomian gland orifices had ductal hyperkeratinization and plugging. These findings support a role for inflammation in the pathogenesis of LMK in SJS. Future research can be directed at delineating the pathways that lead to LMK by studying the changes in the lid margin microbiome, and the molecular mechanisms regulating keratinization in the conjunctiva and the meibomian gland orifices in eyes affected by SJS., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Competing Interests: Declaration of competing interest None.