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Purpose: To provide the long-term outcome of patients with end-stage severe ocular surface disease (OSD) consecutively treated with cultivated oral mucosal epithelial transplantation (COMET) followed by limbal-rigid contact lens (CL)-wear therapy., Design: Retrospective cohort., Methods: In 23 eyes of 18 patients with severe OSD who underwent COMET surgery between 2002 and 2019 and who were followed with limbal-rigid CL-wear therapy for at least 1 year postoperative, patient demographics, best-corrected visual acuity (BCVA, logMAR), Ocular Surface Grading Scores (OSGS), surgical indication and adverse events were reviewed. Primary and secondary outcomes were BCVA and OSGS changes at baseline and final examination, respectively., Results: This study involved 16 patients with Stevens-Johnson syndrome and 2 patients with mucous membrane pemphigoid (mean age: 59±15 years). The indications for COMET were as follows: corneal reconstruction for vision improvement (10 eyes (43.5%)), corneal reconstruction for persistent epithelial defect (4 eyes (17.4%)) and conjunctival (fornix) reconstruction for symblepharon release (9 eyes (39.1%)). The mean duration of CL-wear postsurgery was 6.4±3.9 years (range: 1.4 to 13.3 years). The mean BCVA at baseline and at final follow-up was logMAR 1.9±0.5 and 1.3±0.7, respectively (p<0.05). Compared with those at baseline, the OSGSs for symblepharon and upper and lower fornix shortening showed significant improvement at each follow-up time point post treatment initiation. No serious intraoperative or postoperative adverse events were observed., Conclusion: In patients afflicted with severe OSD, COMET combined with limbal-rigid CL-wear therapy postsurgery was found effective for vision improvement and ocular surface stabilisation., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To investigate the long-term outcomes of fornix reconstruction and cicatricial entropion repair in patients with ocular mucous membrane pemphigoid (MMP) and secondary MMP., Methods: Retrospective chart review of patients with MMP undergoing either fornix reconstruction (with amniotic membrane or buccal mucosal graft) or Wies cicatricial entropion repair between January 1, 2000, and September 1, 2020. Patients had a positive mucosal biopsy and (or) clinical features of MMP or secondary MMP. The primary outcome was overall success of fornix reconstruction based on fornix depth maintenance at latest follow-up. Secondary outcomes included resolution of trichiasis, visual acuity, and improvement of subjective symptoms., Results: Eight patients (10 eyes) with a diagnosis of MMP (3 males and 5 females; median age, 71 years) and 4 patients (4 eyes) with a diagnosis of secondary MMP (2 females and 2 male; median age, 87 years) were enrolled. Mean follow-up was 22.7 months (range, 0.3-87.5 months) for MMP patients and 15.4 months (range, 3.0-43.9 months) for secondary MMP patients. For MMP eyes, 30.0% underwent fornix reconstruction, 60.0% underwent entropion repair, and 10.0% received both. Re-formation of symblepharon and loss of fornix depth occurred in all MMP eyes at an average of 6.4 ± 7.0 months postoperatively, and trichiasis recurred in all patients at the last follow-up visit. In secondary MMP patients, 75.0% of the eyes showed recurrence of symblepharon, and 66.7% re-formed trichiasis. Both MMP and secondary MMP patients had short-term symptom improvements., Conclusions: Fornix reconstruction and cicatricial entropion repair in our cohort of MMP and secondary MMP patients resulted in short-term symptomatic improvement, but recurrence was seen, on average, at 6 months postoperatively., (Copyright © 2023 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Background/aims: To investigate the long-term outcomes of cultivated oral mucosal epithelial transplantation (COMET) for fornix reconstruction in eyes with chronic cicatrising disease., Methods: This retrospective cohort study involved 16 eyes of 15 patients who underwent COMET for symblepharon release and fornix reconstruction between June 2002 and December 2008. The mean postoperative follow-up period was 102.1±46.0 months (range: 32-183 months). The treated cicatrising disorders included ocular cicatricial pemphigoid (OCP, five eyes), thermal/chemical injury (three eyes) and other chronic diseases (seven eyes; including recurrent pterygium (two eyes), Stevens-Johnson syndrome (one eye) and graft-versus-host disease (one eye)). Ocular-surface appearance was evaluated before surgery, at 1, 4, 12 and 24 weeks postoperative, and then annually based on the previously reported scoring system. Main outcome measures included overall and disease-specific fornix-reconstruction success probabilities analysed by the Kaplan-Meier survival curve. Symblepharon/fornix-shortening recurrence at 24 weeks postoperative, and its relationship to long-term surgical success was also examined., Results: At 5 years postoperative, the mean±SD overall fornix-reconstruction success probability was 79.6%±10.7%, and success probability for thermal/chemical injury and OCP was 100% and 53.3%±24.8%, respectively (p=0.53, log-rank test). The 3-year success probability was significantly higher in the no-disease-recurrence group at 24 weeks postoperative (13 eyes) than in the disease-recurrence group (three eyes) (100% and 33.3%±27.2%, respectively) (p=0.0073, log-rank test)., Conclusion: COMET was found to be safe and effective for symblepharon release and long-term fornix reconstruction in eyes with chronic cicatrisation. Although the 5-year success probability differed depend on the underlying disease, ocular-surface appearance at 24 weeks postoperative is a factor for predicting long-term outcome., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To describe a novel surgical technique using the Boston Keratoprosthesis (KPro) type I in a patient with advanced ocular cicatricial pemphigoid (OCP) using oral mucosa for covering the prosthesis., Methods: We present the case of an 85-year-old man previously diagnosed with type 2 diabetes and advanced OCP nonresponsive to immunosuppressive treatment, whose best-corrected visual acuity was light perception and projection in both eyes. After examination, Boston KPro type I in the right eye was contemplated because osteo-odonto KPro and Tibial bone KPro were not feasible because of the patient's osteoporosis and edentulism. Reconstruction of the ocular surface was first performed using oral mucosa to release the symblepharon and try to deep the fornices. Three months later, the oral mucosa was lifted, and the Boston KPro type I was implanted using the patient's own cornea. Then, a modification of the standard surgical technique was carried out, replacing the use of contact lens for covering the prosthesis with an oral mucosa graft with a central trephination as an alternative option in fornix foreshortening cases., Results: After 11 months, visual acuity was stable to 0.2 decimal. No postoperative complications have been encountered, and prosthesis was in place., Conclusions: The surgical technique of transmucosal Boston KPro type I may be considered a surgical alternative in patients with advanced OCP who present with severe fornix foreshortening, where osteo-odonto KPro or Tibial bone KPro cannot be performed due to osteoporosis or edentulism or when the Boston KPro type II is not readily available.

Mucous membrane pemphigoid (MMP) with ocular involvement, also referred to as ocular cicatricial pemphigoid, is a rare, autoimmune blistering disease that can have devastating effects for patients. The resulting corneal neovascularization, recurrent corneal abrasions, and ulceration can ultimately result in severe vision loss. We present a 64-year-old man with recalcitrant ocular MMP and consequently advanced corneal involvement that received bilateral keratoprostheses. This case highlights an aggressive but successful placement of bilateral keratoprostheses in recalcitrant ocular MMP, which restored his vision and ultimately his quality of life.

Purpose: To investigate the efficacy and safety of transplantation with biomaterial-free cultured oral mucosal epithelial cell sheets (COMECs) for ocular reconstruction in subjects with total limbal stem cell deficiency., Methods: A prospective clinical trial (NCT02149732) was conducted in 8 subjects with total limbal stem cell deficiency after approval from the institutional review board of Seoul National University Hospital (H-0707-043-213) and the Ministry of Food and Drug Safety of Korea. COMECs were prepared in a culture system without the use of any temperature-sensitive polymers or carriers. The COMECs were transplanted without suture fixation. Four subjects underwent penetrating keratoplasty after stabilization of the COMEC transplant. Stable epithelialization, changes in visual acuity, and postoperative complications were evaluated for 6 months. Corneal cytokeratins (K) of 4 subjects who underwent penetrating keratoplasty were stained with an immunofluorescent agent., Results: The ocular surface was successfully reconstructed in 6 eyes. Complete stable epithelialization was achieved within a mean of 53.6 days. Visual improvement (≥2 lines) was achieved in 62.5% of the eyes. K12 (corneal phenotype), K4, and K13 (mucosal phenotype) were well expressed in grafts after keratoplasty, whereas K1, K8, and K19 were barely expressed. No ocular infections, local tumor formation, or remarkable systemic complications were observed. Ocular reconstruction using COMECs failed in 2 eyes, which had full symblepharon in 4 quadrants., Conclusions: Transplanting biomaterial-free COMECs seems to be an efficient and safe procedure to reconstruct the ocular surface in patients who are completely limbal stem cell deficient without a full symblepharon.

Mucous membrane pemphigoid (MMP) is a rare variant of the skin disease pemphigoid, which predominantly involves the mucous membranes. This rare autoimmune disease that infrequently affects the respiratory tract is characterized by subepithelial blister formation that may result in scarring. Immunopathologic examination of mucous membranes reveals the deposition of immunoglobulins and complement within the subepithelial basement membrane. We describe a patient with undiagnosed MMP, with a near-fatal presentation of central airway obstruction causing acute respiratory distress. The patient was successfully treated with emergent rigid bronchoscopic resection of a ball valve-like endotracheal mass, and diagnosed with a rare variant of pemphigoid disease, MMP. The patient was treated with mycophenolate and was clinically in remission, with bronchoscopically stable lesions at 1 year of follow-up.

This article describes our surgical technique for subperiosteal midface elevation in patients with paralytic lower eyelid malposition or cicatricial inferior eyelid retraction. Nineteen patients with paralytic lower eyelid malposition and 15 patients with cicatricial inferior eyelid retraction underwent a subperiosteal midface lift (n = 34). The procedure was performed under local anesthesia through three nonvisible incisions. A transconjunctival incision allows preseptal dissection to the orbital rim, followed by a subperiosteal dissection of the midface. An oral incision is useful to achieve complete subperiosteal dissection and to perform the distal periostomy, which allows complete release of the midface soft tissues. A temporal incision provides access to the temporal fascia for fixation of the elevated tissues and gives the surgeon the possibility of removing redundant skin from the scalp instead of the lower eyelid. Additional posterior lamellar grafting was performed in 24 patients. There was a statistically significant reduction in the distance from the pupil centre to the lower eyelid (margin reflex distance, MRD2) 1 month after surgery (preoperative MRD2 9.62 ± 4.52 mm, postoperative MRD2 5.28 ± 2.62 mm). The ectropion and lower eyelid retraction was resolved in all patients, except for one recurrence. Midfacial elevation is a safe and effective surgical technique in the treatment of static lower eyelid malposition after chronic facial nerve palsy or posttraumatic or iatrogenic lower eyelid retraction.

We describe the surgical technique of endoscopic cyclophotocoagulation in a Boston keratoprosthesis type II patient. This patient with ocular cicatricial pemphigoid had pars plana endoscopic cyclophotocoagula through wounds created in the eyelids.

A detailed medical history and a careful clinical examination are the basis for developing a list of possible differential diagnoses in lesions of the oral mucosa. On this basis, it can be decided whether a lesion can be observed for 14 days after removal of possible causes or the start of a trial treatment, or whether a biopsy for histological examination must be taken immediately. An excisional biopsy is performed for small and presumably benign lesions, an incisional biopsy for large and presumably malignant lesions. If an autoimmune blistering disease is suspected, a second sample for examination by immunofluorescence is taken. Depending on the results of the histological examination further treatment steps are planned or regular appointments for follow-up are arranged.

A 87-year-old woman presented with a three-year history of partially erosive, partially bullous skin and mucosal lesions, symblepharon of both eyelids as well as dysphagia. To date, multiple excisions of the skin lesions, which had been described as "skin tumors" by surgeons, had been performed. The synopsis of histology, direct and indirect immunofluorescence established the diagnosis of anti-p200/anti-laminin γ1 pemphigoid and BP180 NC16A/4575- positive mucous membrane pemphigoid with an unusual "epitope-spreading" phenomenon. Due to the late initiation of therapy, the disease-related loss of vision unfortunately was irreversible.

Purpose: To assess the clinical efficacy of cultivated oral mucosal epithelial transplantation (COMET) for the treatment of persistent epithelial defect (PED)., Methods: We treated 10 eyes of nine patients with PED (Stevens-Johnson syndrome: three eyes; thermal/chemical injury: five eyes; ocular cicatricial pemphigoid: two eyes) with COMET at Kyoto Prefectural University of Medicine, Kyoto, Japan from 2002 to 2008., Results: Preoperatively, PED existed on over more than 50% of the corneal surface in seven eyes. Severe ocular surface inflammation with fibrovascular tissue surrounded the PED in all 10 eyes. At 24-weeks postoperative, PED had improved in all cases except 1 in which the patient was unable to return to the hospital (95% CI, 55.5-99.7; Wilcoxon signed-rank test, p = 0.0078). The preoperative median of logarithmic minimum angle of resolution was 1.85 (range 0.15-2.70), and 1.85, 1.85, and 1.52 at the 4th, 12th, and 24th postoperative week, respectively. The mean total preoperative ocular surface grading score was 7.0 (range 4-17). At 4 and 12 weeks postoperative, the total ocular surface grading score had improved significantly (p = 0.0020, p = 0.0078), and at 24 weeks postoperative, it was 3.0 (range 2-12, p = 0.0234). During the follow-up period (median 23.3 months, range 5.6-39.7 months), no recurrence of PED was observed in any eye, and long-term ocular surface stability was obtained., Conclusion: COMET enabled complete epithelialization of PED and stabilization of the ocular surface in patients with severe ocular surface disease, thus preventing end-stage cicatrization and vision loss at a later stage., (© 2014 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To report the outcomes of cataract surgery in ocular cicatricial pemphigoid (OCP)., Setting: L. V. Prasad Eye Institute, Hyderabad, India., Design: Retrospective, interventional case series., Methods: Patients diagnosed with OCP who had undergone cataract surgery were included. Staging of disease, type of surgery, pre- and postoperative best-corrected visual acuity (BCVA), and number and duration of topical and systemic medications were recorded. Complications and any exacerbation or worsening of disease were noted., Results: Nine eyes of 7 patients (3 male, 4 female) were included in the study, with mean age of 60.44 ± 2.6 years (range 56-64 years). Follow-up ranged from 6 months to 10 years (mean 52.9 ± 46.25 months). Surgery performed was extracapsular cataract extraction with posterior chamber intraocular lens implantation (n = 5) or phacoemulsification with posterior chamber intraocular lens implantation (n = 4). Best-corrected visual acuity improved by more than 2 lines in 6 of 9 (66.67%) eyes, which remained stable till the last follow-up. Three eyes had no visual improvement due to corneal scar in 2 patients and preexisting posterior staphyloma in 1 eye. Disease progression was noted in 2 of 9 operated eyes by one stage at the end of 1 year., Conclusion: In this series, cataract surgery could be safely performed with no major intra- or postoperative complications. While the surgical intervention itself was not associated with acute exacerbations of inflammation, progression of disease was noted in some cases over time. In spite of ongoing disease, cataract surgery in OCP was associated with stable visual outcomes.

Purpose: To evaluate the use of the Boston keratoprosthesis type I implantation in patients with mucous membrane pemphigoid (MMP)., Methods: Retrospective review of 8 eyes of 8 patients with severe ocular surface disease and corneal blindness as a result of MMP who underwent Boston keratoprosthesis type I implantation at the Massachusetts Eye and Ear Infirmary from January 1, 2000, through December 31, 2009. The main outcome measures were best-corrected visual acuity, keratoprosthesis retention, and postoperative complications., Results: The mean age of patients was 71.3 years (range, 55-94 years), and the mean duration of their disease preoperatively was 6.1 years (range, 1.7-11.4 years). Visual acuity after the surgery improved to 20/200 or better in 6 eyes (75%) and to 20/40 or better in 3 eyes (37.5%). Only 1 of 6 eyes (16.7%) was able to maintain visual acuity of 20/200 or better over a mean follow-up period of 3.2 years. Five of the 8 Boston keratoprosthesis type I devices (62.5%) extruded or had to be replaced during a mean follow-up time of 1.7 ± 1.7 years. Loss of vision to worse than 20/200 during the follow-up period occurred because of keratoprosthesis type I extrusion, end-stage glaucoma, and retinal or choroidal detachment., Conclusions: The clinical outcomes of Boston keratoprosthesis type I implantation in MMP are guarded and, as judged from the literature, less favorable than those with the Boston keratoprosthesis type II for the same disease.

Aims: To evaluate the clinical outcomes following Moscow Eye Microsurgery Complex in Russia keratoprosthesis (MICOF KPro) implantation in end-stage autoimmune dry eyes., Methods: A retrospective observational case series. Fourteen patients with end-stage autoimmune dry eyes underwent MICOF KPro surgery by one surgeon (YH) in the Chinese PLA General Hospital between 2000 and 2009. Retention of the prosthesis and vision were recorded and postoperative complications were analysed., Results: Preoperative diagnosis included Stevens-Johnson syndrome (n=7), ocular cicatricial pemphigoid (n=4) and Sjogren's syndrome (n=3). The mean follow-up period was 3.9 years (range 10 months-7.8 years). After surgery, 13 eyes (93%) showed a visual acuity of 20/200 or better and 6 eyes (43%) achieved excellent vision of 20/40 or better. At last visit, 69% of the eyes (9/13) maintained a visual acuity of at least 20/200. The first case retained a visual acuity of 20/80 7.8 years after the operation. The most common complication was stromal melting that threatened anatomical success. Seven of the first eight patients showed tissue melting, and four of them had leakage. All of these were repaired successfully. The latter six cases received prophylactic autologous auricular cartilage implantation to reinforce the anterior surface of recipient's cornea at either stage 1 or 2. Other causes of vision loss included pre-existing glaucoma (n=6), sterile vitritis (n=5), cylinder loosening (n=1) and retroprosthetic membrane (n=4)., Conclusion: MICOF KPro provided useful vision for the end stage of autoimmune dry eyes in our study. Anatomical stability of KPro was achieved in all cases using repair and reinforcing surgery.

The basic principles of conjunctival reconstruction are similar in all pathologies that inflict conjunctival tissue loss. Large conjunctival defects are difficult to treat, with little conjunctival reserve that can be used to close the defect. The study had the objective to find alternatives to conjunctival autograft. Frozen amniotic membrane was investigated. From 27 cases, 22 cases were reconstructed using amniotic membrane alone, but in 5 cases conjunctival autograft was also needed. Bulbar conjunctiva was reconstructed in 23 cases, of which 19 with pterygium, 3 with conjunctival tumors, one operatory plague. Fornix reconstruction was carried out in 4 cases, one tumor and 3 symblepharons (2 after conjunctival burns and one pemfigus). The rate of pterygium recurrence was 7,14%. Amniotic membrane can heal large defects of the conjunctiva, either bulbar or forniceal.

Purpose: To evaluate the efficacy of oral mucosal graft to correct not only lid margin keratinization and trichiasis or distichiasis, but also incomplete closure in severe cicatricial ocular surface diseases., Design: Retrospective, noncomparative, interventional case series., Methods: Twenty-two eyes (39 eyelids) of 19 patients received an oral mucosal graft during lid margin reconstruction from September 2007 through February 2010. Relief of symptoms, conjunctival inflammation, corneal epithelial abnormalities, and visual acuity were compared before and after surgeries as outcome measures., Results: Among 22 eyes, 10 eyes (45.5%) had lid margin keratinization, trichiasis or distichiasis, or both, resulting in blink-related microtrauma. The oral mucosal graft resulted in successful correction in 6 eyes; in the remaining eyes, trichiasis in 3 eyes and distichiasis in 1 eye were away from the corneal surface. Incomplete closure present in 12 (54.5%) eyes was completely corrected in 9 eyes and was much improved in the remaining 3 eyes. During a mean follow-up of 16.2 months, reports of foreign body sensation, photophobia, pain, burning, tearing, and discharge were relieved significantly in 17 (77.3%) of 22 eyes. The visual acuity was improved in 13 eyes (59.1%) and was maintained in 8 eyes. Conjunctival inflammation was reduced markedly in 19 eyes (86.4%). Corneal epithelial defect and superficial punctate keratopathy were healed rapidly or improved in 14 eyes, and regression of superficial vascularization was noted in 1 eye., Conclusions: Oral mucosal graft can reduce not only blink-related microtrauma caused by lid margin keratinization and trichiasis or distichiasis, but also exposure caused by cicatricially induced incomplete closure, thus preventing further deterioration of the ocular surface., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objectives/hypothesis: To perform a systematic pooled-data analysis of literature data involving laryngeal mucous membrane pemphigoid (MMP)., Study Design: A systematic review and pooled-data analysis., Methods: We conducted a systematic literature search of MEDLINE, EMBASE, Cochrane Central Register of Clinical Trials, Cochrane Database of Systematic Reviews, clinicaltrials.gov, and the National Guideline Clearinghouse databases without language restriction for studies including combinations of relevant terms. All authors independently screened the abstracts of the search results, identified articles eligible for review, and critically appraised the full-text studies. Pooled-data analyses and Kaplan-Meier survival analyses were conducted using SPSS version 16.0 software (SPSS Inc., Chicago, IL)., Results: Of the 2,524 citations reviewed, the included articles consisted of 63 case reports and 10 case series reporting on 141 patients with laryngeal MMP. No clinical trials or comparative trials were found. The overall calculated prevalence of laryngeal MMP was 12.2% (95% confidence interval [CI], 11.5-12.9%) of cases of MMP or one in 10 million persons in the general population. Mean age at laryngeal MMP onset was 59.7 years (95% CI, 57.9-61.1 years), and the supraglottis was the most commonly affected site (84.8%, 95% CI, 82.5-87.2%). Distribution among genders was equivalent (P = .655). The presence of antiepiligrin autoantibodies was associated with increased laryngeal involvement (Odds ratio 7.9, 95% CI, 3.9-16.0). The overall 5-year relative survival rate was 92.4% (standard error, 8.4) with a follow-up range of 1 to 221 months. Standard medical therapy alone occasionally improved the condition; however, relapses were frequent, and 10.5% eventually required tracheostomy. Laryngeal surgical interventions seemed to be effective in severe cases., Conclusions: Laryngeal MMP is a rare condition that can be life threatening without proper treatment and frequent follow-up.

Purpose: To clarify the relationship between clinical symptoms and histological status in patients with ocular cicatricial pemphigoid (OCP) and Stevens-Johnson syndrome (SJS)., Methods: Clinical symptoms of four OCP and eight SJS patients in the chronic phase were scored with our recently proposed grading system. The histological status of the pannus tissue removed from the corneal surface during surgery was investigated using immunohistological techniques., Results: All participants showed total loss of the palisades of Vogt and conjunctivalization of the entire corneal surface. All pannus tissues expressed the conjunctival epithelium marker CK4/13. The pannus tissue in clinically keratinized SJS expressed skin epidermal major cytokeratins, but the tissues of nonkeratinized SJS did not., Conclusions: Clinical observation and the use of our recently proposed grading system agreed with the immunohistological status with respect to keratinization, cell proliferation, and corneal/conjunctival cell typing. These findings facilitate our understanding of the pathogenesis of OCP and SJS, and will hopefully contribute to the development of future treatment strategies and improve predictions of the postoperative prognosis of ocular surface reconstruction in patients with OCP and SJS.

The authors present a case of secondary glaucoma associated with silicone oil in a patient with ocular cicatricial pemphigoid successfully managed with transscleral diode laser cyclophotocoagulation. Six months following treatment, the patient remained satisfied and free of pain, with an intraocular pressure of 24 mm Hg without topical drops or oral acetazolamide. There was no evidence of exacerbation of ocular cicatricial pemphigoid and no adjustment was required to the patient's immunosuppressive therapy. This case suggests that transscleral diode laser cyclophotocoagulation may be safely used to control raised intraocular pressure in patients with ocular cicatricial pemphigoid without causing an exacerbation of the condition., (Copyright 2009, SLACK Incorporated.)

Purpose: To determine the influence of topical vancomycin prophylaxis on the incidence of bacterial endophthalmitis in eyes with a Boston Keratoprosthesis (KPro)., Methods: A retrospective chart review was performed for 255 eyes of 231 patients who received a KPro between March 1990 and December 2006. Preoperative diagnoses were burn, ocular cicatricial pemphigoid (OCP), Stevens-Johnson Syndrome (SJS), and graft failure/other. Patients used topical antibiotic prophylaxis for the duration of the KPro: polymyxin-trimethoprim or a quinolone in the 1990s, or a quinolone with or without vancomycin beginning in late 1999. For each KPro eye, the follow-up interval was divided into months on or off vancomycin (vancomycin versus no-vancomycin group). The incidence of endophthalmitis was calculated with Kaplan-Meier survival curves., Results: The 255 eyes were followed for 673.6 patient-years (mean, 2.64 years; range, 1 week to 13 years). There were 18 cases of bacterial endophthalmitis; 17 occurred at least 6 weeks postoperatively (range, 1.5 to 46 months). Gram-positive cocci caused over 80% of cases. Only 1 case, due to an atypical mycobacterium, occurred in a patient using vancomycin.The incidence of bacterial endophthalmitis was lower in the vancomycin group than in the no-vancomycin group: 0.35% versus 4.13% per patient-year (P = 0.001). It was also lower in SJS eyes using vancomycin versus no vancomycin: 1.76% versus 18.39% per patient-year (P = 0.009). In eyes with preoperative diagnoses of burn, OCP, or graft failure/other, the incidence in the vancomycin group was zero., Conclusion: Topical vancomycin plus a quinolone is effective in preventing bacterial endophthalmitis in KPro eyes.

Mucous membrane pemphigoid includes chronic autoimmune sub-epithelial blistering diseases that predominantly affect mucous membranes, with varying combinations of oral, ocular, cutaneous, genital, nasopharyngeal, oesophageal and laryngeal lesions. The case is reported of a man with multiple manifestations of mucous membrane pemphigoid. A 53-year-old male presented at our Department with a 4-year clinical history of diagnosed cicatricial pemphigoid. The patient was affected by ocular and urinary symptoms and presented with nasal obstruction and dysphonia. Nasal endoscopy revealed crusting and synechiae with pale and atrophic mucosa. Computed tomography examination showed hypodense-hyperdense material occupying all paranasal sinuses and nasal fossae. Laryngoscopy showed anterior para-commessural and inter-arytenoidal synechiae. The patient underwent functional endoscopic sinus surgery for incision of synechiae and removal of scars and inflammatory material from all sinuses. Nasal splints were then inserted. A wait-and-see policy was adopted for the laryngeal lesion. One year later, the splints were removed; the upper airways were still free and there were no signs of nasal obstruction. An endoscopic approach appears to be efficacious in the surgical treatment of nasal cicatricial pemphigoid, and long-term stenting may be necessary to avoid recurrence. Although surgery has not a curative role in long-term therapeutic strategies, it may, nonetheless, improve the quality of life and ensure good nasal respiration.

Osteo-odonto-keratoprosthesis (OOKP) is a keratoprosthesis technique in which the patient's own tooth root is used to support an optical cylinder. It was invented by Strampelli in 1963 and modified and established by Falcinelli about 10 years later. This method is particularly useful for restoring sight in end-stage Stevens-Johnson syndrome (SJS) and ocular cicatricial pemphigoid (OCP). We started performing OOKP surgery in June 2003 supported by Dr Christopher Liu of Sussex Eye Hospital, Brighton, UK. Till now, we have performed 4 OOKP surgeries for end-stage SJS and OCP. We modified the original method by using artificial buccal mucous membranes to the bone and buccal mucous membrane defects for better wound healing. Case 1 was a 49-year-old woman (SJS), and the corrected visual acuity was 0.5 in 4 years 8 months. Case 2 was a 68-year-old woman (SJS), and the corrected visual acuity was 0.04 in 3 years 10 months. Case 3 was a 63-year-old man (SJS), and the corrected visual acuity was 0.1 in 3 years 2 months. Case 4 was a 71-year-old woman (OCP), and the corrected visual acuity was 0.04 in 1 year 3 months. Despite some minor optical cylinder troubles such as MRSA colonization, tilting, and buccal mucous coverage, their visual acuities were stable without any serious complications. It was demonstrated that OOKP is useful for visual rehabilitation and durable with minimum eye care for severe ocular surface diseases.

Purpose: To evaluate the long-term clinical efficacy of the Seoul-type keratoprosthesis (S-KPro)., Methods: S-KPros were implanted into 4 unsighted and 5 sighted eyes in 9 patients: 6 patients were diagnosed with Stevens-Johnson syndrome, 2 had chemical burns, and 1 suffered from ocular pemphigoid. The preoperative visual acuity ranged from light perception to hand motion. The average follow-up period was 62.8 months. We evaluated several clinical factors, including visual acuity, visual field, number of additional grafting procedures, number of capsulotomy procedures, and the interval between retinal detachment and skirt exposure., Results: The S-KPro showed anatomic success for an average of 62.8 months in 66.7% of the eyes. The average visual acuity preservation time was 31.6 months. Localized glaucomatous visual field defect was not found in any of the sighted patients; however, diffuse visual field constriction was observed after long-term follow-up. Average time of skirt exposure and mean number of additional grafting procedures were 12.9 months and 2.44, respectively. Retinal detachments were developed in all the patients at a mean time interval of 2 months after S-KPro exchange., Conclusions: S-KPro achieved visual rehabilitation for an average of 31.6 months with long-term anatomic stability in patients with severe intractable ocular surface disease.

Purpose: To analyze factors influencing clinical outcomes in cultivated limbal epithelial transplantation (CLET)., Design: Retrospective, observational case series., Methods: Twenty-seven eyes of 27 patients that had CLET for severe chronic cicatricial ocular surface disorders were studied. Two different cultivation methods were used to prepare epithelial sheets. Method 1 used the explant technique and neither feeder cells nor air-lifting were used. In Method 2, cell suspension technique and 3T3 feeder cells were used, and air-lifting was applied after cultivated cells became confluent. Clinical outcomes including corneal surface epithelialization and incidence of postoperative complications were studied. The relationship between the clinical outcome and type of cultivation method, original diseases, tear function, or preoperative ocular surface status was also studied., Results: Both cultivation methods produced transplantable epithelial sheets with corneal phenotype. With a mean follow-up period of 127 weeks, corneal epithelialization was achieved in 16 eyes (59.3%). Eyes that had CLET with Method 1 suffered more severe postoperative complications such as infection, ulceration, and perforation (P = .053). Eyes with Stevens-Johnson syndrome (SJS) had poor final corneal epithelialization compared with other diseases (P = .034). CLET was more successful when performed to conjunctivalized corneas compared with eyes with persistent epithelial defects or with dermalized corneas., Conclusions: CLET offers new treatment modalities to chronic cicatricial ocular surface disorders with moderate success rates. The treatment is feasible for eyes with non-immune-mediated disorders with stable ocular surface conditions. Epithelial sheets with better structural integrity seem to be superior to obtain early postoperative epithelialization and to avoid serious postoperative complications.

Severe ocular surface diseases, such as Stevens-Johnson syndrome, ocular cicatricial pemphigoid or severe ocular burns may result in a significant loss of corneal stem cells, eventually leading to vision impairment or even corneal blindness. In case of unilateral involvement, limbal autografting, by means of transplanting limbal stem cells from the healthy fellow eye, has proved to be an effective procedure for restoring the integrity of the ocular surface. Limbal allografts may be performed in patients with bilateral disease, however, systemic immunosuppression is mandatory in these cases, with a long-term outcome that is frequently reduced compared to limbal autografts due to acute or chronic graft rejection. In recent years, amniotic membrane transplantation has been successfully employed as an additional tool in ocular surface reconstruction. The AlphaCor synthetic cornea, which is made of flexible acrylic may be considered as an alternative in patients with repeated corneal graft failures. Both limbal transplantation and the AlphaCor have been shown to be effective in eyes with an adequate tear film, but are most likely to fail in severe dry eyes or in patients with cicatrising diseases. Such conditions are the domain of keratoprostheses (KPros) with rigid optics, which certainly can be considered as the 'last resort' to restore vision in patients with profound corneal blindness not amenable to conventional corneal and limbal grafting. The osteo-odonto-keratoprosthesis according to Strampelli and modified by Falcinelli makes use of a "biological" support consisting of a longitudinal section of one of the patient's teeth that is also supported by the surrounding alveolar bone tissue. Compared to other devices favourable long-term results have been reported. In patients lacking any usable teeth, implantation of a keratoprosthesis with haptics made of Dacron (Pintucci-KPro) or tibial bone (Temprano-KPro) might be considered.

Background: Ocular trauma or disease may lead to severe corneal opacification and, consequently, severe loss of vision as a result of complete loss of corneal epithelial stem cells. Transplantation of autologous corneal stem-cell sources is an alternative to allograft transplantation and does not require immunosuppression, but it is not possible in many cases in which bilateral disease produces total corneal stem-cell deficiency in both eyes. We studied the use of autologous oral mucosal epithelial cells as a source of cells for the reconstruction of the corneal surface., Methods: We harvested 3-by-3-mm specimens of oral mucosal tissue from four patients with bilateral total corneal stem-cell deficiencies. Tissue-engineered epithelial-cell sheets were fabricated ex vivo by culturing harvested cells for two weeks on temperature-responsive cell-culture surfaces with 3T3 feeder cells that had been treated with mitomycin C. After conjunctival fibrovascular tissue had been surgically removed from the ocular surface, sheets of cultured autologous cells that had been harvested with a simple reduced-temperature treatment were transplanted directly to the denuded corneal surfaces (one eye of each patient) without sutures., Results: Complete reepithelialization of the corneal surfaces occurred within one week in all four treated eyes. Corneal transparency was restored and postoperative visual acuity improved remarkably in all four eyes. During a mean follow-up period of 14 months, all corneal surfaces remained transparent. There were no complications., Conclusions: Sutureless transplantation of carrier-free cell sheets composed of autologous oral mucosal epithelial cells may be used to reconstruct corneal surfaces and can restore vision in patients with bilateral severe disorders of the ocular surface., (Copyright 2004 Massachusetts Medical Society)

Purpose: To report a patient with severe corneal and conjunctival toxicity from long-term, habitual use of hydrogen peroxide as an eye wash., Design: Observational case report., Intervention and Testing: Serial examinations of the cornea, conjunctiva, and ocular adnexa were done. Penetrating keratoplasty with amniotic membrane transplantation was performed., Main Outcome Measures: Ocular inflammation, pain, and visual acuity outcome., Results: Bilateral corneal and conjunctival inflammation and scarring mimicking ocular-cicatricial pemphigoid were noted. Formation of a descemetocele after starting treatment with low-dose topical steroids required emergent penetrating keratoplasty with amniotic membrane transplantation., Conclusions: This is the first reported case of ocular surface toxicity in a patient after deliberate chronic use of high-dose hydrogen peroxide. It highlights the value of obtaining a thorough medical and social history and the importance of direct questioning about the use of any medications or agents on the eyes before making a diagnosis or initiating therapy.

Mucous membrane pemphigoid (MMP) is a sub-epithelial blistering disease that primarily involves mucosal surfaces but may also involve the skin. Clinically, it appears as vesiculobullous lesions of the oral cavity and eyes, but other tissues such as the nasopharyngeal and laryngeal mucosa can also be affected. Ultimately, scarring and airway stenosis may occur. The condition should be managed by a multidisciplinary team led by a dermatologist. Immunosuppressive therapy forms the mainstay of treatment, with surgery having both a diagnostic role and a use in the treatment of complications such as airway obstruction. There must be a low threshold of suspicion for laryngeal involvement in this group of patients, so that prompt action can be taken. Once laryngeal stenosis has occurred repeated endoscopic laser excision of scar tissue can be used to maintain an adequate airway. Adjuvant use of mitomycin-C can be used beneficially in the treatment of laryngeal complications of MMP.

Purpose: To evaluate the role and the effectiveness over time of amniotic membrane transplantation (AMT) as a first-step procedure to treat conjunctival reconstruction in late-stage ocular-cicatricial pemphigoid (OCP)., Design: Prospective interventional noncomparative case series., Participants: Nine eyes (9 patients) with advanced OCP., Methods: Preoperatively, the ocular surface conditions were evaluated by immunohistochemistry of conjunctival biopsy and impression cytology specimens. The amniotic membrane was obtained during cesarean section from women who were 39 weeks pregnant and seronegative for human immunodeficiency virus, hepatitis B and C, and syphilis; it was processed, histologically tested, and stored at -80 degrees C. After scar tissue was removed, the preserved amniotic membrane was placed over the cornea, the bulbar, and tarsal conjunctiva, and was secured with 8-0 Vicryl sutures to the conjunctival edges and the deep fornices with double-armed 6-0 silk sutures. In 2 cases a double layer of amniotic membrane was transplanted. All patients received immunosuppressive systemic therapy and preservative-free tear substitutes and steroids topically for at least 6 months. During follow-up (average, 48 weeks; range, 28-96 weeks), a new standardized method was used to evaluate the fornix depth, and impression cytology testing was performed and conjunctival inflammation recorded and used as parameters for monitoring disease activity., Main Outcome Measures: Symblepharon, increased inferior fornix depth, presence of conjunctival goblet cells, and the degree of conjunctival inflammation., Results: The conjunctival surface was free from symblepharon in all subjects for the first 16 weeks. At the week 28 examination, a small area of symblepharon was present in four eyes (44.4%). The depth of the fornix was significantly (P < 0.0001, analysis of variance) improved at weeks 4, 16, and 28. The normal conjunctival epithelium with goblet cells was restored in 6 of 9 eyes (66.7%) at the week 4 examination and in 4 eyes (44.4%) at the week 28 examination. Conjunctival inflammation was clinically but not statistically reduced. The visual acuity improved in 5 subjects., Conclusions: AMT can be a first-step procedure for ocular surface reconstruction in OCP, but its effectiveness deteriorates slightly over time.

Purpose: The surgical procedures used until now to rebuild the conjunctiva in patients affected by ocular cicatricial pemphigoid (OCP) have not demonstrated appreciable anatomical and functional success. This study reports the postoperative clinical and cytological outcomes of a human amniotic membrane transplantation (AMT) to rebuild the conjunctiva in a case of late stage OCP., Methods: We present a 75-year-old woman with a severe form of pemphigoid with entropion, trichiasis, symblepharon and blunting of the fornices, who underwent excision of the scarred and inflamed tissue covering the ocular surface and AMT., Results: Our data show an improvement of the ocular surface condition, with reacquired fornix depth, reduced inflammation and presence of goblet cells at each follow-up., Conclusion: Amniotic membrane transplantation was successful as a first step procedure to reduce inflammation and to rebuild a physiological conjunctival epithelium in late stage OCP.

A case of antiepiligrin cicatricial pemphigoid that primarily involved the larynx and required a tracheostomy was studied. The diagnosis was based on the direct immunofluorescence findings of a biopsy specimen from the glottis, immunofluorescence using normal and 1M sodium chloride-split normal human skin as substrates, and immunoprecipitation. A dramatic clinical improvement was observed after the combined administration of tetracycline hydrochloride and niacinamide. The tracheal stoma could be shut after the narrow segment was cut by means of carbon dioxide laser therapy. The patient showed no respiratory difficulty during the 2-year follow-up period. The combined therapy of tetracycline and niacinamide is thus considered to be an effective treatment for various types of cicatricial pemphigoid.

Background/aims: The use of "fresh" (hypothermically stored) and frozen amniotic membrane (AM) was compared in a patient with cicatricial pemphigoid with stem cell failure. The viability of both "fresh" and frozen AM epithelial cells was assessed after storage., Methods: AM was stored at either +4 degrees C ("fresh") or at -80 degrees C (frozen). A "fresh" graft was applied to the cornea following superficial keratectomy. Subsequently, a further frozen graft was applied to the same eye. Viability of the stored AM epithelium was assessed by investigating membrane integrity and mitochondrial activity., Results: In both cases the cornea re-epithelialised and visual acuity improved. Improvement, however, was not sustained., Conclusion: Although both procedures led to an improvement in visual acuity, "fresh" tissue performed no better than frozen in promoting re-epithelialisation. The authors suggest that logistical, safety, and cost considerations outweigh any benefits of using "fresh" as opposed to frozen graft material.

Cicatricial pemphigoid is one of a number of mucocutaneous disorders that can present in the oral cavity with desquamation, pain, and bleeding of the gingiva and oral mucosa. This case report describes the management of cicatricial pemphigoid in a patient with multiple sites of gingival recession using connective tissue grafting to alleviate root surface sensitivity and improve esthetics. The treatment rationale is presented and discussed in terms of timing of therapy and implications for wound healing in patients who present with desquamative gingivitis.

Purpose: To determine the efficacy of a one-piece lamellar keratolimbal graft for the treatment of ocular surface disorders involving the limbus and cornea., Methods: A retrospective study of a case series including eight patients with either Stevens-Johnson syndrome (four patients), ocular cicatricial pemphigoid (three patients), or familial subepithelial amyloidosis of the cornea (one case) was performed with a mean follow-up period of 18 months after surgery., Results: Of the eight cases, three patients retained a corneal epithelial appearance upon their latest visit. No statistically significant difference was observed between preoperative visual acuity (0.013) and postoperative acuity (0.082). Stromal neovascularization was observed in all cases. Seven of eight cases experienced persistent epithelial defects (PED), four of which evolved secondary infectious ulcers. Two cases required further surgical intervention to treat PED or infectious ulcers. None of the cases developed glaucoma., Conclusion: A one-piece keratolimbal graft does not seem to offer any advantage over a two-piece limbal and corneal graft in the treatment of severe ocular surface disorders involving the limbus and cornea.

Purpose: To determine the effectiveness of using a radiofrequency instrument to resect a V-shaped tarsal wedge for the correction of cicatricial upper eyelid entropion., Methods: Prospective evaluation of 16 upper eyelids of 11 consecutive patients., Results: The operation was successful in all 16 eyelids, with excellent cosmetic and functional outcomes., Conclusions: Tarsal V-wedge resection is an effective alternative to other complicated techniques in the management of upper eyelid cicatricial entropion.

A 48-year-old woman with anti-epiligrin cicatricial pemphigoid (CP) who showed clinical features resembling epidermolysis bullosa acquisita was found to have adenocarcinoma of the stomach. Histological examination of lesional skin demonstrated a subepidermal blister. Direct immunofluorescence microscopy of perilesional skin revealed linear deposits of IgG and C3 at the basement membrane zone. The patient's serum contained IgG autoantibodies that bound to the dermal side of 1 mol/L NaCl-split normal human skin as determined by indirect immunofluorescence microscopy, and the lamina lucida as determined by indirect immunoelectron microscopy. The patient's serum immunoprecipitated laminin-5 from extracts and media of biosynthetically radiolabelled human keratinocytes. Immunoblot studies showed that the patient's autoantibodies specifically bound the alpha3 subunit of this laminin isoform. Fragility of the skin and bullous lesions disappeared after total gastrectomy, but soon reappeared possibly in association with metastatic disease in a lymph node. The possibility that anti-epiligrin CP may develop paraneoplastically in some patients is discussed.

Four cases of cicatricial pemphigoid complicated by oesophageal involvement are presented. All patients suffered dysphagia but repeated radiological studies were required for confirmation of oesophageal ulceration, webs and strictures. A combination of systemic drug therapy and oesophageal dilatation were necessary for the suppression of symptoms. The clinical and immunopathological features, management and complications of oesophageal involvement in cicatricial pemphigoid are discussed. Dermatologists should be aware of these features and make regular inquiries about swallowing difficulties in patients with cicatricial pemphigoid to guide appropriate investigations and treatment.

We present herein the exceedingly uncommon case of a patient with cicatricial pemphigoid (CP) who gradually developed conjunctival, oral, and esophageal involvement. Despite long-term medical management with dapsone and disulone, the ocular lesions progressed to produce further scarring, which led to ankyloblepharon, symblepharon, and ultimately, blindness of the right eye. After a period of 5 years the patient developed a total esophageal stricture, intractable by dilation, necessitating esophageal reconstruction. The details of an original procedure using a continuous colic loop are described, highlighting the better tolerance of this technique by a high-risk patient. A discussion on the recent advances in diagnosing bullous dermatoses is presented following this case report.