Cicatricial Entropion in Chronic Cicatrizing Conjunctivitis: Potential Pathophysiologic Mechanisms and Long-Term Outcomes of a Modified Technique.

PURPOSE: The purpose of this study was to assess the long-term outcomes of severe cicatricial entropion repair with mucous membrane grafting in patients with chronic cicatrizing conjunctivitis and report histopathological changes in the eyelid margin area. METHODS: Prospective interventional study included 19 patients with severe cicatricial entropion with trichiasis (N = 20 eyelids; 19 upper and 1 lower eyelid) who underwent anterior lamellar recession (with back cuts) and mucous membrane grafting cover for bare anterior tarsus, lid margin, and 2 mm of marginal tarsus, and had a minimum 6 months of follow-up. The anterior lamella and metaplastic eyelid margins were sent for routine Haematoxylin and Eosin and special staining with Masson trichrome stain. RESULTS: The etiologies were chronic Stevens-Johnson syndrome (N = 6), chemical injury (N = 11), and drug-induced pseudopemphigoid (N = 2). Five eyes had undergone entropion correction in the past, and 9 had electroepilation for trichiasis. Entropion was well corrected (without residual trichiasis) in 85% of eyelids with primary surgery. The etiology-wise success rates were 100% for Stevens-Johnson syndrome, 72.7% for chemical injury, and 100% for drug-induced pseudopemphigoid. Three eyelids with failure belonged to chemical injury, and trichiasis in these eyes could be managed with subsequent interventions except in 1 case. All eyelids had no entropion at a mean follow-up of 10.8 months (range, 6-18). Histopathological evaluation of anterior lamella (N = 10) and eyelid margins revealed significant fibrosis in subepithelial, perimysium (muscle of Riolan), and perifollicular areas. CONCLUSION: Anterior lamellar recession combined with mucous membrane grafting achieves good cicatricial entropion correction except in eyes with chemical injury. The eyelid margins in these eyes have persistent inflammation, and fibrosis involving lash follicles.

Preparing the Ocular Surface for a Boston Keratoprosthesis Type 1 Through En Bloc Minor Salivary Gland Transplantation and Mucous Membrane Grafting in End-Stage Stevens-Johnson Syndrome.

PURPOSE: This case describes the successful visual restoration of a patient with end-stage Stevens-Johnson syndrome (SJS) with a severely keratinized ocular surface. METHODS: This study is a case report. RESULTS: A 67-year-old man with SJS secondary to allopurinol sought visual rehabilitation options. His ocular surface was severely compromised from sequelae of chronic SJS, leaving him with light perception vision bilaterally. The left eye was completely keratinized with severe ankyloblepharon. The right eye had failed penetrating keratoplasty, limbal stem cell deficiency, and a keratinized ocular surface. The patient declined both a Boston type 2 keratoprosthesis and a modified osteo-odonto keratoprosthesis. Therefore, a staged approach was pursued with (1) systemic methotrexate to control ocular surface inflammation, (2) minor salivary gland transplant to increase ocular surface lubrication, (3) lid margin mucous membrane graft to reduce keratinization, and finally, (4) Boston type 1 keratoprosthesis for visual restoration. After minor salivary gland transplant and mucous membrane graft, the Schirmer score improved from 0 mm to 3 mm with improvement in ocular surface keratinization. This approach successfully restored the vision to 20/60, and the patient has retained the keratoprosthesis for over 2 years. CONCLUSIONS: Sight restoration options are limited in patients with end-stage SJS with a keratinized ocular surface, aqueous and mucin deficiency, corneal opacification, and limbal stem cell deficiency. This case demonstrates successful ocular surface rehabilitation and vision restoration in such a patient through a multifaceted approach that resulted in successful implantation and retention of a Boston type 1 keratoprosthesis.

Sutureless Amniotic Membrane Transplantation Using Pediatric Nasogastric Tube for Patients With Acute Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.

PURPOSE: Evaluation of the results of sutureless amniotic membrane transplantation (AMT) using a pediatric nasogastric tube (NGT) for patients with acute Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) was aimed. METHODS: Twenty-six eyes of 13 patients with SJS/TEN with ocular involvement at a single tertiary care hospital between May 2020 and January 2022 were included in the study. An amniotic membrane was implanted to the ocular surface and conjunctival fornix using a modified symblepharon ring (MR) created by inserting NGT end-to-end. Patients were followed for at least 6 months. The mean duration of the surgery was recorded. The presence of corneal epithelial defect and vascularization, meibomian gland dysfunction (MGD) grade, conjunctival scarring, eyelid margin keratinization, symblepharon, and trichiasis were recorded. RESULTS: The mean age of the patients was 38.4±17.6 years. Amniotic membrane transplantation was performed only once for eight patients and twice for five patients. The mean duration of the surgery was 3.2±0.4 min. The modified symblepharon ring was removed 28 days after its application. At the first examination, 20 eyes (76.9%) had a corneal epithelial defect and 6 (23.1%) had early symblepharon. At the postoperative 6 months, although seven eyes (26.9%) had conjunctival scarring and 5 (19.2%) had eyelid margin keratinization, all patients were without any corneal defect, corneal vascularization, or symblepharon. Only 11 eyes (42.3%) showed MGD, and 1 eye (3.8%) showed trichiasis. CONCLUSIONS: This study showed that sutureless AMT using pediatric NGT could be a potentially fast and inexpensive treatment option for the treatment of SJS/TEN with ocular involvement at the bedside without the need for general anesthesia.

Single-Staged Surgical Correction of Eyelid Sequelae Along With Lid Margin Mucous Membrane Grafting in Stevens-Johnson Syndrome and Other Cicatricial Ocular Surface Diseases.

PURPOSE: The purpose of this study was to study the outcome of single-staged entropion surgery along with lid margin mucous membrane grafting for cicatrizing ocular surface disease. METHODS: Retrospective review of medical records of patients who underwent single-staged surgical correction of cicatricial entropion along with lid margin mucous membrane grafting for lid margin keratinization. RESULTS: Twenty-six eyes of 19 patients were studied. The mean age of patients was 42.5 years (standard deviation, SD-17.67), of which 7 patients were male and 12 were female. The most common disorder was Stevens-Johnson syndrome (SJS) sequelae (83.33%, n = 20), followed by mucous membrane pemphigoid (n = 4, 16.67%). The most common eyelid changes observed were cicatricial entropion in all 26 eyes (100%, n = 26), followed by trichiasis in 13 eyes (50%, n = 13). Lid margin keratinization was noted in all eyes. Postoperative improvement in corneal surface staining was noted in 70% of the patients (n = 13), no change in 20% of the patients (n = 4), and worsening of corneal surface staining in 10% of the patients (n = 2). Postoperative visual acuity improvement was noted in 50% of the eyes (n = 13), no improvement in 39% of the eyes (n = 10), and vision worsened in 12% of the eyes (n = 3). An entropion recurrence rate of 25% (n = 6) was observed over an average 10-month follow-up, whereas 75% (n = 20) reported no recurrence. CONCLUSIONS: Single-staged correction of eyelid cicatricial entropion with a lid margin mucous membrane graft (MMG) has promising outcomes in ocular surface diseases. It can decrease the need for multiple surgeries and provide symptomatic relief in patients with chronic cicatricial surface changes.

Severe cicatricial entropion repair using mucous membrane graft in Stevens-Johnson syndrome.

Background: Severe cicatricial entropion in Stevens-Johnson syndrome (SJS) patients is difficult to treat and is associated with a higher recurrence rate. Also, entropion in the presence of lid margin mucous membrane graft (MMG) further complicates the surgical anatomy and approach. Purpose: To report a modified surgical technique of repairing severe upper eyelid cicatricial entropion in an SJS patient with history of lid margin MMG. Synopsis: Cicatricial entropion in patients with SJS is entirely different from trachomatous cicatricial entropion. The involvement of the lid margin with keratinization, tarsal scarring, persistent conjunctival inflammation, and unhealthy ocular surface affects the surgical approach and outcomes. Conjunctiva-sparing surgery with reconstruction of the lid margin using MMG, flattening and repositioning the anterior lamella, and covering the bare tarsus with MMG rather than leaving it raw are the necessary modifications in this technique from conventional anterior lamellar recession. The video demonstrates the surgical technique for harvesting and preparation of a labial MMG, the splitting of the anterior and posterior lamella of the lid margin, scar tissue release between the lash line and the tarsus, repositioning of the anterior lamella, and anchoring of the labial MMG. Highlights: Anterior lamellar recession combined with MMG wrapping the lid margin and bare tarsus offers good cicatricial entropion repair outcomes. Removal of fat and submucosa from the mucosal graft should be done for better cosmesis. Adequate separation of the scar tissues from the lash line and the tarsus is essential. Video link: https://youtu.be/6HsKgeZQCyY.

Osteo-odonto-keratoprosthesis According to Strampelli Original Technique: A Retrospective Study With Up to 30 Years of Follow-up.

PURPOSE: To describe clinical, anatomical, and visual outcomes obtained from a long-term follow-up of 59 patients who underwent osteo-odonto-keratoprosthesis (OOKP) using the Strampelli original technique. DESIGN: Retrospective clinical cohort study. METHODS: The study included 82 eyes of 59 patients who underwent OOKP surgery between 1969 and 2011. Patients' clinical characteristics before surgery as well as complications and further surgeries until the end of follow-up were recorded. Best-corrected visual acuity (BCVA) was revised before surgery and at 1 month, 1 year, and every 5 years until the 30th year of follow-up. RESULTS: Mean follow-up post-OOKP was 27.4 ± 11.2 years (range, 2.4-52). The most frequent cause of blindness was chemical injuries (71%). OOKP integrity was maintained in 77 of 82 eyes (94%) until the end of follow-up. Excluding cataract, acquired glaucoma was the most frequent complication, with a prevalence at 10 years of 36%. Mean BCVA improved from 2.60 ± 0.32 at presentation to 0.40 ± 0.65 at 1 year and 1.21 ± 1.19 logMAR at 30 years. Overall, 51% of the included eyes attained a BCVA better than 0.05 logMAR, and stabilization of BCVA was observed for the first 10 years of follow-up post-OOKP. Better BCVA outcomes were observed in the Stevens-Johnson syndrome or toxic epidermal necrolysis (SJS/TEN) group, whereas glaucoma was found not to significantly affect visual acuity. CONCLUSIONS: The original OOKP still represents a valid surgical choice, which is durable over time, for restoring vision in end-stage corneal blindness patients who are not eligible for a corneal transplant.

Chronic Ocular Sequelae and Subsequent Surgical Interventions in Stevens-Johnson Syndrome After Amniotic Membrane Transplantation.

PURPOSE: The purpose of this study was to describe the chronic ocular sequelae and subsequent surgical interventions after amniotic membrane transplantation (AMT) in a patient with Stevens-Johnson syndrome (SJS). METHODS: A 30-year-old woman was diagnosed with SJS after taking phenytoin prescribed for generalized tonic-clonic seizures. Bedside AMT covering the eyelid margins, the conjunctiva, and the cornea was performed in both eyes during the acute phase during hospitalization. A repeat AMT was necessary after 5 days. After AMT, she was prescribed topical steroids, antibiotics, and artificial tears. She was asked to follow up every 2 weeks in the first 3 months after AMT. RESULTS: Eyelid-related complications such as trichiasis, distichiasis, eyelid margin keratinization (LMK), and cicatricial entropion developed during the follow-up period within the first year after AMT. Eyelid margin mucous membrane graft (MMG) of all eyelids was performed at the earliest clinical sign of lid-related keratopathy due to LMK. The right eye developed upper eyelid and lower eyelid cicatricial entropion, for which anterior lamellar repositioning with eyelid margin MMG was performed. Electroepilation of the trichiatic eyelashes in all 4 eyelids was performed more than once to maintain a healthy ocular surface. CONCLUSIONS: Successful AMT was performed twice within the first week of ocular involvement in the acute phase of SJS. Chronic ocular sequelae affecting the eyelid margin were identified during the chronic phase and were successfully treated with eyelid margin MMG. Necessity for the close follow-up and the need for subsequent interventions to maintain visual acuity should be explained to patients after acute SJS with ocular involvement.

A beginner's guide to mucous membrane grafting for lid margin keratinization: Review of indications, surgical technique and clinical outcomes.

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.

Complete Fusion of Both Eyelids in Stevens-Johnson Syndrome: Case Report.

An 18-year-old woman developed Stevens-Johnson syndrome (SJS) with ocular involvement after taking ibuprofen. She was admitted to another hospital, received saline flushes and bacitracin ophthalmic ointment to the eyes, and became unable to open them. Upon transfer to this burn center 3 weeks after symptom onset, there was complete fusion of both eyelids with no visible cornea or sclera. She underwent bilateral operative scar release. After opening the lids, meticulous debridement of cicatricial membranes and release of symblephara were performed with subsequent placement of amniotic membrane grafts. Her vision slowly improved, though her long-term visual prognosis remains guarded. Early recognition and treatment of SJS or toxic epidermal necrolysis (TEN) with ocular involvement is imperative. Even mild cases may require intensive topical lubrication, steroids, and antibiotics, with early placement of amniotic membrane grafts in severe cases. Prompt intervention and daily evaluation are paramount in preventing lifelong visual disability.

Mucous membrane grafting (fibrin glue vs. suture) for lid margin pathologies in Stevens-Johnson syndrome: randomized comparative study.

OBJECTIVE: To compare fibrin glue (with three cardinal sutures) (FG) and polygalactin suture (PS) for mucous membrane grafting (MMG) in terms of graft apposition and recurrence of lid margin keratinization (LMK) and metaplastic lashes (ML) in patients with Stevens-Johnson syndrome (SJS). DESIGN: Prospective randomized comparative interventional study. METHODS: Twenty patients diagnosed with SJS and lid margin abnormalities including LMK with or without ML were randomized to undergo either fibrin glue (FG)-assisted MMG (n = 10) or continuous 8-0 polygalactin suture (PS)-assisted MMG (n = 10). They were evaluated preoperatively and during follow-up at 1 week and 1, 2, 3, and 6 months. The parameters assessed were best-corrected visual acuity (BCVA), tear break-up time (TBUT), Schirmer-1 test, corneal and conjunctival complications, graft apposition and width (GW), LMK, ML, impression cytology, and operative time. The primary outcome measures are incidence of graft displacement and recurrence of LMK and ML. RESULTS: None of the eyelids in FG group (0/40) and 1 eyelid in PS group (1/40) had graft displacement. Recurrence of LMK occurred in 7.5% of eyelids (3/40) in both the study groups. Recurrence of ML occurred in 2.5% (1/40) in FG group and 5% (2/40) in PS group. The mean operative time for MMG in FG group was 39.5 ± 2.40 min and in PS group was 56 ± 1.63 min (p = 0.001). CONCLUSIONS: As graft apposition with suture involves significantly longer intraoperative time, if cost is not a limiting factor then fibrin glue is a viable option for the MMG for lid margin pathologies.

Outcomes of Cultivated Oral Mucosal Epithelial Transplantation in Eyes With Chronic Stevens-Johnson Syndrome Sequelae.

PURPOSE: To study the outcomes of cultivated oral mucosal epithelial transplantation (COMET) in eyes with chronic Stevens-Johnson syndrome (SJS) sequelae. DESIGN: Prospective interventional case series. METHODS: Forty-five eyes of 41 patients with chronic SJS sequelae were recruited and evaluated from 2013 to 2017 in an institutional setting. All patients underwent COMET, with an aim of fornix reconstruction and visual rehabilitation. Change in corrected distance visual acuity (CDVA), severity scores of various ocular surface parameters, and the occurrence of complications were documented during a follow up period of 2 years. Attainment and maintenance of a stable ocular surface, as assessed by change in the ocular surface severity scores was the primary outcome measure, while change in CDVA was the secondary outcome measure. RESULTS: The mean preoperative CDVA was 2.7± 0.5 logMAR, which improved to 1.5± 0.7 logMAR and 1.49± 0.98 postoperatively, at 1- and 2-year follow-up visit. Overall, 82.2% eyes (37/45) had improvement in visual acuity, 13.3% (6/45) experienced no change, whereas 2 eyes (4.4%) had worsening of visual acuity. The total ocular surface severity scores improved from a mean preoperative value of 29.1± 9.7 to 18.7± 7.2 postoperatively, at 2-year follow-up. Two eyes developed persistent epithelial defects, with progression to corneal melting requiring keratoplasty. CONCLUSIONS: COMET allows successful and sustained restoration of ocular surface anatomy with functional improvement, in eyes with chronic sequelae of SJS.

Reconstruction of Major Burns With Stevens-Johnson Syndrome: A Case Report and Literature Review.

Stevens-Johnson syndrome (SJS) is a severe type of pleomorphic erythema and a rare disorder of the skin and mucous membranes, which can lead to serious infections, pulmonary embolism, acute respiratory distress syndrome, multiple organ dysfunction syndrome, and other serious consequences. Patients with SJS are usually treated in burn centers. SJS complicated by severe burns is very rare, and this is associated with a high risk of infection and other more serious complications. With SJS, the availability of donor sites is compromised given the lack of healthy epidermis, and this makes it more difficult to treat. The patient was a 52-year-old man with 45% TBSA burns with 40% TBSA full-thickness burns on both lower limbs. During treatment, his condition was complicated by SJS, renal failure, and respiratory failure. After 31 days, he was transferred to our department. On the 22nd day, the patient recovered from SJS, and after undergoing four skin grafting procedures, the burn wounds healed, and the donor site had healed spontaneously. He was discharged after 86 days of treatment in our department. In conclusion, major burns complicated with SJS are rare clinical presentations. The skin affected by the drug eruptions can be used as a donor site for transplantation to the burn wounds, and this donor area can also heal.

Four in one: Four recepients with a single donor tissue - A novel concept for eye transplantation surgery post-COVID-19.

A donor corneo-scleral button was dissected into four parts using a simple manual technique. The anterior corneal lamellae was stripped from the Descemet's Membrane (DM) and Deep Anterior Lamellar Keratoplasty (DALK) was performed in a patient with advanced keratoconus after removing the recipient's stroma using the big bubble technique. Descemet's Membrane Endothelial Keratoplasty (DMEK) was done with the stripped donor DM in a patient with Fuch's endothelial dystroph (FECD). The cadaveric limbal stem cells from the tissue were used for simple limbal epithelial transplantation (SLET) in a Steven- Johnson Syndrome (SJS) with localized limbal stem deficiency and symblepharon. The sclera was used to revise a leaking hypotonus bleb in an advanced single-eyed glaucoma patient. No intraoperative or postoperative complications were observed. At 1 year, all the 4 cases retained healthy transplanted tissues with good visual outcomes. Shortage of donor eyes is a global problem and with the present COVID-19 scenario the situation is bound to worsen. The advent of customized component corneal transplantation using simple cost-effective techniques will be the future trend in the years to come.

Ocular Sequelae of Stevens-Johnson Syndrome: A Comprehensive Approach.

The chronic sequelae of Stevens-Johnson syndrome cause severe ocular morbidity. Among the various manifestations, including dry eye, cicatrization causing forniceal shortening, adnexal disorders, lid margin keratinization, and limbal stem cell deficiency, keratinization of the lid margin leads to progressive ocular surface damage through constant blink-induced microtrauma. This damage induces corneal punctate keratopathy and neovascularization and can produce epithelial defects leading to corneal infection or perforation. Management strategies for chronic ocular sequelae are broadly categorized into ocular surface stabilization procedures, tectonic procedures, and visual rehabilitation procedures. Interventions in the acute stage of the disease include amniotic membrane grafting. Timely intervention helps to prevent deterioration of the ocular surface in these eyes.

Treatment of Oculoplastic and Ocular Surface Disease in Eyes Implanted with a Type I Boston Keratoprosthesis in Southern China: A Retrospective Study.

INTRODUCTION: This study aimed to describe the clinical features, surgical management of the eyelid and ocular surface, and outcomes of 16 patients implanted with a Boston type I keratoprosthesis (KPro). METHODS: A retrospective, single-center, consecutive case series of 16 patients with Stevens-Johnson syndrome (1), ocular chemical burns (12), and ocular thermal burns (3) implanted with KPro was studied. All subjects were men aged 27-51 years. Surgical treatment and outcomes for eyelid malposition, symblepharon, and glaucoma were assessed. RESULTS: From September 2010 to February 2019, 29 patients were admitted to Zhongshan Ophthalmic Center for KPro implantation, of whom 16 (55%) required eyelid or ocular surface surgeries to maintain hydration and protect the corneal tissue, which is vulnerable to epithelial defects. Forty-one adnexal surgical procedures were performed. The most common indication for surgery was symblepharon, and the most frequent procedures were symblepharon lysis with ocular mucous membrane grafts and amniotic membranes (7) and full-thickness skin grafts to the eyelids (7). Preoperative conjunctival injection and corneal staining were documented in 9 (56%) and 8 (50%) eyes, respectively, and at up to 4 months postoperative follow-up (the last adnexal surgery before KPro) were recorded in 3 (19%, p = 0.03) and 2 (12%, p = 0.02) eyes, respectively. Glaucoma drainage devices were inserted in six patients. One patient with Stevens-Johnson syndrome underwent FP7 Ahmed glaucoma valve (AGV) implantation inferotemporally and developed plate exposure 2 months postoperatively. Five patients underwent FP8 AGV implantation with tube insertion into the vitreous cavity due to the scarred conjunctiva and limited subconjunctival space. In the study period, intraocular pressure (IOP) was in the normal range, and no tube or plate exposure was observed. CONCLUSION: The ocular environment is critical for successful KPro surgery. A multidisciplinary approach for any lid and ocular surface abnormality in ocular burns or Stevens-Johnson syndrome is important to improve the quality of the ocular surface and accommodate KPro and AGV, which is vital for maintaining vision after KPro surgery. FP8 AGV may be feasible for IOP control in adult KPro cases with restricted subconjunctival space.

Preparation of placental tissue transplants and their application in skin wound healing and chosen skin bullous diseases - Stevens-Johnson syndrome and toxic epidermal necrolysis treatment.

Unique properties of amniotic membrane make it a promising source for tissue engineering and a clinically useful alternative for patients suffering from chronic wounds including, for example, ulcers, burns, ocular surface damages and wounds occurring in the course of bullous diseases like stevens-johnson syndrome and toxic epidermal necrolysis. Its use has many advantages over standard wound care, as it contains pluripotent cells, nutrients, anti-fibrotic and anti-inflammatory cytokines, growth factors and extracellular matrix (ECM) proteins. Placental tissues can be prepared as a medical component, an advanced therapy medicinal product or a tissue graft. In addition to basic preparation procedures such as washing, rinsing, cutting, drying and sterilisation, there are many optional steps such as perforation, crosslinking and decellularisation. Finally, transplants should be properly stored-in cryopreserved or dehydrated form. In recent years, many studies including basic science and clinical trials have proven the potential to expand the use of amniotic membrane and amnion-derived cells to the fields of orthopaedics, dentistry, surgery, urology, vascular tissue engineering and even oncology. In this review, we discuss the role of placental tissues in skin wound healing and in the treatment of various diseases, with particular emphasis on bullous diseases. We also describe some patented procedures for placental tissue grafts preparation.