Advanced Corneal Imaging in Keratoconus: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the published literature on the diagnostic capabilities of the newest generation of corneal imaging devices for the identification of keratoconus. METHODS: Corneal imaging devices studied included tomographic platforms (Scheimpflug photography, OCT) and functional biomechanical devices (imaging an air impulse on the cornea). A literature search in the PubMed database for English language studies was last conducted in February 2023. The search yielded 469 citations, which were reviewed in abstract form. Of these, 147 were relevant to the assessment objectives and underwent full-text review. Forty-five articles met the criteria for inclusion and were assigned a level of evidence rating by the panel methodologist. Twenty-six articles were rated level II, and 19 articles were rated level III. There were no level I evidence studies of corneal imaging for the diagnosis of keratoconus found in the literature. To provide a common cross-study outcome measure, diagnostic sensitivity, specificity, and area under the receiver operating characteristic curve (AUC) were extracted. (A perfect diagnostic test that identifies all cases properly has an AUC of 1.0.) RESULTS: For the detection of keratoconus, sensitivities for all devices and parameters (e.g., anterior or posterior corneal curvature, corneal thickness) ranged from 65% to 100%. The majority of studies and parameters had sensitivities greater than 90%. The AUCs ranged from 0.82 to 1.00, with the majority greater than 0.90. Combined indices that integrated multiple parameters had an AUC in the mid-0.90 range. Keratoconus suspect detection performance was lower with AUCs ranging from 0.66 to 0.99, but most devices and parameters had sensitivities less than 90%. CONCLUSIONS: Modern corneal imaging devices provide improved characterization of the cornea and are accurate in detecting keratoconus with high AUCs ranging from 0.82 to 1.00. The detection of keratoconus suspects is less accurate with AUCs ranging from 0.66 to 0.99. Parameters based on single anatomic locations had a wide range of AUCs. Studies with combined indices using more data and parameters consistently reported high AUCs. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Outcomes and Complications of Limbal Stem Cell Allograft Transplantation: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the published literature on the safety and outcomes of keratolimbal allograft (KLAL) transplantation and living-related conjunctival limbal allograft (lr-CLAL) transplantation for bilateral severe/total limbal stem cell deficiency (LSCD). METHODS: Literature searches were last conducted in the PubMed database in February 2023 and were limited to the English language. They yielded 523 citations; 76 were reviewed in full text, and 21 met the inclusion criteria. Two studies were rated level II, and the remaining 19 studies were rated level III. There were no level I studies. RESULTS: After KLAL surgery, best-corrected visual acuity (BCVA) improved in 42% to 92% of eyes at final follow-up (range, 12-95 months). The BCVA was unchanged in 17% to 39% of eyes and decreased in 8% to 29% of eyes. Two of 14 studies that evaluated the results of KLAL reported a notable decline in visual acuity over time postoperatively. Survival of KLAL was variable, ranging from 21% to 90% at last follow-up (range, 12-95 months) and decreased over time. For patients undergoing lr-CLAL surgery, BCVA improved in 31% to 100% of eyes at final follow-up (range, 16-49 months). Of the 9 studies evaluating lr-CLAL, 4 reported BCVA unchanged in 30% to 39% of patients, and 3 reported a decline in BCVA in 8% to 10% of patients. The survival rate of lr-CLAL ranged from 50% to 100% at final follow-up (range, 16-49 months). The most common complications were postoperative elevation of intraocular pressure, persistent epithelial defects, and acute allograft immune rejections. CONCLUSIONS: Given limited options for patients with bilateral LSCD, both KLAL and lr-CLAL are viable choices that may provide improvement of vision and ocular surface findings. The studies trend toward a lower rejection rate and graft failure with lr-CLAL. However, the level and duration of immunosuppression vary widely between the studies and may impact allograft rejections and long-term graft survival. Complications related to immunosuppression are minimal. Repeat surgery may be needed to maintain a viable ocular surface. Reasonable long-term success can be achieved with both KLAL and lr-CLAL with appropriate systemic immunosuppression. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Safety and Efficacy of Epithelium-Off Corneal Collagen Cross-Linking for the Treatment of Corneal Ectasia: A Report by the American Academy of Ophthalmology.

PURPOSE: To review the evidence on the safety and effectiveness of epithelium-off corneal collagen cross-linking (CXL) for the treatment of progressive corneal ectasia. METHODS: A literature search of the PubMed database was most recently conducted in March 2024 with no date restrictions and limited to studies published in English. The search identified 359 citations that were reviewed in abstract form, and 43 of these were reviewed in full text. High-quality randomized clinical trials comparing epithelium-off CXL with conservative treatment in patients who have keratoconus (KCN) and post-refractive surgery ectasia were included. The panel deemed 6 articles to be of sufficient relevance for inclusion, and these were assessed for quality by the panel methodologist; 5 were rated level I, and 1 was rated level II. There were no level III studies. RESULTS: This analysis includes 6 prospective, randomized controlled trials that evaluated the use of epithelium-off CXL to treat progressive KCN (5 studies) and post-laser refractive surgery ectasia (1 study), with a mean postoperative follow-up of 2.4 years (range, 1-5 years). All studies showed a decreased progression rate in treated patients compared with controls. Improvement in the maximum keratometry (Kmax) value, corrected distance visual acuity (CDVA), and uncorrected distance visual acuity (UDVA) was observed in the treatment groups compared with control groups. A decrease in corneal thickness was observed in both groups but was greater in the CXL group. Complications were rare. CONCLUSIONS: Epithelium-off CXL is effective in reducing the progression of KCN and post-laser refractive surgery ectasia in most treated patients with an acceptable safety profile. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Long-term outcomes of permanent keratoprosthesis.

PURPOSE OF REVIEW: To provide an update on recent advances in keratoprosthesis surgery with a focus on long-term outcomes. RECENT FINDINGS: Reports over the past year have focused on long-term outcomes showing good anatomical and functional results. In addition, preoperative diagnosis and surgical modifications that can impact postoperative outcomes are discussed. Outcomes of primary keratoprosthesis as an option for patients with poor prognosis for traditional keratoplasty are also presented in this review. SUMMARY: Keratoprosthesis is an option for visual rehabilitation in select patients. Current 5-year outcomes are promising but improvements in glaucoma prevention and management are needed to enhance long-term results.

Biofilm Formation on Bandage Contact Lenses Worn by Patients with the Boston Type 1 Keratoprosthesis: A Pilot Comparison Study of Prophylactic Topical Vancomycin 15 mg/mL and Linezolid 0.2.

OBJECTIVES: To determine the rate of biofilm formation on bandage contact lenses worn by patients with the Boston type 1 keratoprosthesis (K-Pro) while on prophylactic topical vancomycin versus linezolid. METHODS: Patients wearing a bandage contact lens (BCL) with a K-Pro were eligible for enrollment. After irrigation of the ocular surface with 5% povidone-iodine solution, each patient was placed on either topical vancomycin 15 mg/mL or linezolid 0.2% BID for one month. At the one-month visit, the BCL was collected and stored in fixative solution. Standard photographs were taken of each lens at high magnification using scanning electron microscopy (SEM), which were subsequently analyzed for evidence of biofilm. RESULTS: Nineteen contact lenses were obtained from 12 K-Pro patients at the Illinois Eye and Ear Infirmary. Zero of eight (0%; 95% CI=0 to 37%) contact lenses from patients treated with topical vancomycin, and 1 of 11 (9%; 95% CI=0 to 41%; P-value=1.00) contact lenses from patients treated with topical linezolid were found to have biofilm formation at one month as detected by SEM. None of the patients developed a clinically significant infection while on either prophylactic vancomycin or linezolid during the study period. CONCLUSIONS: Overall, the rate of biofilm formation as detected by SEM on the surface of bandage contact lenses was low. These results suggest that vancomycin and linezolid are both relatively effective in reducing biofilm-forming bacterial growth at one month. Accordingly, linezolid may be an effective alternative to vancomycin in patients with allergy or intolerance. However, further investigation is required to develop evidence-based antibiotic prophylaxis regimens.

Design and Biocompatibility of a Novel, Flexible Artificial Cornea.

Purpose: We sought to introduce the materials, design, and biocompatibility of a flexible and suturable artificial corneal device. Methods: Single-piece, fully synthetic, optic-skirt design devices were made from compact perfluoroalkoxy alkane. The skirt and the optic wall surfaces were lined with a porous tissue ingrowth material using expanded polytetrafluoroethylene. Full-thickness macroapertures around the skirt perimeter were placed to facilitate nutrition of the recipient cornea. Material properties including the skirt's modulus of elasticity and bending stiffness, optic light transmission, wetting behavior, topical drug penetrance, and degradation profile were evaluated. Results: The final prototype suitable for human use has a transparent optic with a diameter of 4.60 mm anteriorly, 4.28 mm posteriorly, and a skirt outer diameter of 6.8 mm. The biomechanical and optical properties of the device closely align with the native human cornea with an average normalized device skirt-bending stiffness of 4.7 kPa·mm4 and light transmission in the visible spectrum ranging between 92% and 96%. No optical damage was seen in the 36 devices tested in fouling experiments. No significant difference was observed in topical drug penetrance into the anterior chamber of the device implanted eye compared with the naïve rabbit eye. Conclusions: The flexibility and biocompatibility of our artificial cornea device may offer enhanced tissue integration and decreased inflammation, leading to improved retention compared with rigid keratoprosthesis designs. Translational Relevance: We have developed a fully synthetic, flexible, suturable, optic-skirt design prototype artificial cornea that is ready to be tested in early human feasibility studies.

Contact lens surveillance cultures in Boston type 1 keratoprosthesis patients.

OBJECTIVES: To describe surveillance cultures of bandage contact lenses (BCL) in patients with a history of Boston type 1 keratoprosthesis (KPro) surgery. METHODS: An institutional review board-approved retrospective review of patients with a history of Boston type 1 KPro surgery and BCL cultures between July 2008 and June 2010. Data on demographics, preoperative diagnosis, topical corticosteroid and antibiotic use, duration of BCL wear, clinical diagnosis of microbial keratitis or endophthalmitis, and culture results were recorded. RESULTS: There were 14 patients (15 eyes) who underwent Boston type 1 KPro between July 2008 and June 2010 with BCL culture data. Ten eyes showed positive growth and one eye developed an infection. Thirty-four cultures were performed and 12 were positive. The most common organism cultured was coagulase-negative staphylococcus (CoNS). Positive cultures, colonization by non-CoNS pathogens, and infection occurred more frequently in patients with an ocular history of autoimmune disease or chemical burn. The growth of 15 or more CoNS colonies occurred only in patients not on vancomycin. An antibiotic was added to the regimen in response to positive cultures for three patients. CONCLUSIONS: Contact lens surveillance cultures may provide valuable information on the characteristics of microbial colonization, particularly in highlighting inflammatory disease and disuse of vancomycin as possible risk factors. Bandage contact lenses cultures also have the potential to be useful adjuncts in the clinical management of antibiotic therapy.

Twelve-Month Clinical and Histopathological Performance of a Novel Synthetic Cornea Device in Rabbit Model.

Purpose: To report the biological stability and postoperative outcomes of a second-generation, single-piece, flexible synthetic cornea in a rabbit model. Methods: Device materials and design were amended to enhance biointegration. Optic skirt design devices were made from compact perfluoroalkoxy alkane with porous expanded polytetrafluoroethylene ingrowth surface overlying the skirt and optic wall. Sixteen devices were implanted into intrastromal pocket in rabbit eyes. Rabbits were randomly assigned to 6- and 12-month follow-up cohorts (n = 8 in each) postoperatively. Monthly examinations and optical coherence tomography assessed cornea-device integration, iridocorneal angle, optic nerve, and retina. Results: There were no intraoperative complications. All devices were in situ at exit, with clear optics. No retroprosthetic membrane, glaucoma, cataract formation, or retinal detachment was observed. Two rabbits in the 6-month group had mild, focal anterior lamella thinning without retraction adjacent to the optic near tight sutures. Three postoperative complications occurred in the 12-month group. One rabbit diagnosed with endophthalmitis was euthanized on day 228. Mild sterile focal retraction of anterior lamella occurred in two rabbits, which were terminated on days 225 and 315. Light microscopic examination of enucleated globes demonstrated fibroplasia with new collagen deposition into the porous scaffold without significant inflammation, encapsulation, or granuloma formation. Conclusions: Clinical evaluations, imaging, and histopathological findings indicate favorable outcomes of this synthetic corneal device in a rabbit model. Early feasibility studies in humans are being planned. Translational Relevance: Favorable 12-month results of the device in rabbits demonstrate vision-restoring potential in corneally blind individuals at high risk of failure with donor keratoplasty.

Eyelid procedures in patients who have undergone Boston keratoprosthesis surgery.

PURPOSE: Artificial corneas or keratoprostheses such as the Boston keratoprosthesis (KPro) are being used more frequently to provide a clear corneal window in patients with severe corneal disease. A significant percentage of patients who undergo Boston KPro implantation require subsequent eyelid surgery. However, few articles in peer-reviewed literature evaluate the indication and outcome of eyelid procedures after Boston KPro implantation. This study examines the frequency, nature, and outcomes of oculoplastic procedures in patients with Boston KPro implantation. METHODS: A retrospective chart review was conducted of all KPro-1 procedures performed at the University of Illinois at Chicago between December 2006 and September 2010 and all KPro-1 and KPro-2 procedures performed at the University of Iowa between December 2008 and October 2010. RESULTS: One hundred and twenty eyes underwent Boston KPro-1 procedures, and 2 eyes underwent Boston KPro-2 procedures. Twenty-one (17.2%) of the 122 eyes required subsequent eyelid alterations. Chemical burn was the most common preoperative corneal diagnosis (8 of 21; 38.1%). A variety of oculoplastic procedures were performed; the most common procedure was a permanent lateral tarsorrhaphy. Seventeen (81.0%) of 21 KPro eyes that underwent oculoplastic procedures maintained the KPro at an average of 12.4 months of follow up. CONCLUSIONS: A significant number of patients with Boston KPros require subsequent eyelid surgery. With limited existing literature and increasing popularity for using Boston KPros to treat severe corneal disease, it is essential for oculoplastic and corneal surgeons to understand the need for eyelid alterations in these patients and the surgical intricacies surrounding these cases.

Open globe injuries from projectile impact: Initial presentation and outcomes.

PURPOSE: To describe the characteristics and correlates of open globe injuries secondary to projectile injury and outcomes following surgical open globe repair at an urban tertiary referral center. METHODS: Records of all patients with a history of open globe injury secondary to projectile injury and surgical open globe at a tertiary referral hospital between January 1, 2010 and December 31, 2016 were reviewed. Demographics, type of trauma, wound extent, presence of foreign body, and presenting clinical findings are reported. Outcomes for patients with greater than 6 months of follow-up included additional surgeries, final visual acuity, and clinical findings. RESULTS: Of 214 patients who underwent open globe repair, 73 (34.1%) were due to projectile impact. Mean age was 37.9 years and patients were primarily male (n = 66, P < 0.001). Most injuries resulted in globe laceration (68.5%, P < 0.001), and wound extent was zone 1 (45.2%), zone 2 (20.5%), zone 3 (27.4%), or unknown (6.8%). Associated findings included foreign body (35.6%) and orbital fracture (15%). Of 41 patients with at least 6 months of follow-up, 70% had additional surgeries following their initial surgical repair. Laceration injuries tended to be more anterior (P = 0.002) with better visual outcomes (P = 0.045) than those with globe rupture, and concomitant orbital fracture associated with poor visual outcomes. Overall, 58.5% of patients had 20/40 or better final best-corrected visual acuity. CONCLUSION: This is the largest report of open globe injury due to projectile impact. Visual prognosis in this population is very good, with most patients achieving better than 20/40 vision in our study.

Docosahexaenoic acid, protectins and dry eye.

PURPOSE OF REVIEW: To report recent data on the potential role of omega-3 fatty acids, in particular docosahexaenoic acid and its derivatives, in the treatment of dry eye syndrome. RECENT FINDINGS: Dietary supplementation with polyunsaturated fatty acids yields positive results in the improvement of dry eye signs and symptoms. Although several studies have shown this, evidence is still lacking as to which fatty acid or what combination constitutes the most effective treatment. Studies show that treatment with alpha-linoleic acid reduces dry eye-induced inflammation. Eicosapentaenoic acid and docosahexaenoic acid derivatives, particularly resolvin E1 (RvE1) and neuroprotectin D1, appear to be responsible for docosahexaenoic acid's anti-inflammatory effect. This is supported in a study in which topical RvE1 resulted in decreased inflammation in a mouse dry eye model. Topical administration of pigment epithelium-derived factor in combination with docosahexaenoic acid accelerates the regeneration of corneal nerves after their damage during corneal surgery, promoting the return of sensitivity and reducing the signs of dry eye. This combined treatment also reduces objective signs of dry eye, such as rose bengal staining. SUMMARY: No firm recommendations can be made regarding optimal dietary supplementation of essential fatty acids that benefit dry eye patients. On the basis of animal data and preliminary human studies, docosahexaenoic acid and its derivatives appear to be a safe, effective topical treatment for dry eye patients. This may result from their role in the resolution of inflammation and the regeneration of damaged corneal nerves.

Predictive factors of Boston Type I Keratoprosthesis outcomes: A long-term analysis.

PURPOSE: To study the long-term visual- and device retention-related outcomes and complications of the Boston Type I Keratoprosthesis (KPro). METHODS: Single-center, retrospective cohort study of all patients undergoing KPro implantation from February 2007 to April 2014 with at least 5 years of follow-up. RESULTS: 68 eyes from 65 patients underwent KPro implantation during the study period. At 5 and 10 years, the probability of maintaining or improving visual acuity (VA) was 75.0% and 66.7%, respectively, and the probability of KPro retention was 89.2% and 89.2%, respectively. Initial device retention rate at 10 years was significantly lower in those with underlying ocular surface disease (46.8% [30.6-63.2] vs 75.8% [61.0-90.7], P = 0.03), while other baseline characteristics showed no significant association. Final VA was more likely to be stable or improved in patients with fewer failed grafts (2 [1-6] vs 3 [1-6], P < 0.01), and a final VA of 20/200 or better was more likely in primary KPro eyes (44.8% [26.7-62.9] vs 19.4% [6.5-32.3], P = 0.03). Combined KPro-vitrectomy eyes were more likely to have stable or improved final VA than non-vitrectomy eyes (88.5% [76.2-100.0] vs 64.1% [49.1-79.1], P = 0.04). All complications had increasing incidence beyond 5 years; in particular, corneal melt, surgical glaucoma interventions, and endophthalmitis tended to have late presentations, with 79.0%, 78.6%, and 88.9% of these complications occurring beyond one year, respectively. CONCLUSIONS: KPro devices show favorable long-term visual and retention outcomes in select patients. Careful long-term, multidisciplinary follow-up is warranted to address potential complications.

Management of Congenital Aniridia-Associated Keratopathy: Long-Term Outcomes from a Tertiary Referral Center.

PURPOSE: To report the outcomes of medical and surgical management for congenital aniridia-associated keratopathy (AAK) over a long-term follow-up period. DESIGN: Retrospective, comparative case series. METHODS: Medical records of patients diagnosed with congenital aniridia were retrospectively reviewed. Age, sex, ethnicity, follow-up time, AAK stage, noncorneal abnormalities, ocular surgeries, and complications were recorded. The visual acuity equivalent (VAE), approximate Early Treatment Diabetic Retinopathy Study (appETDRS) letter score, was calculated using recorded Snellen visual acuities. RESULTS: A total of 92 eyes of 47 patients (31 females) with mean age of 48.0 ± 18.0 years and mean follow-up of 78.6 ± 42.2 months were included. At the initial visit, 12 eyes (13%) were classified as Stage I AAK, 33 eyes (35.9%) were Stage II, 25 eyes (27.2%) were Stage III, 17 eyes (18.5%) were Stage IV, and 5 eyes (5.4%) were Stage V. Limbal stem cell transplantation (LSCT) and Boston keratoprosthesis (KPro) were frequently performed in eyes with Stages III-V. These advanced corneal surgeries significantly improved the median (95% confidence interval [CI]) of calculated appETDRS scores from 2 (0-20) to 26 (15-41) (Snellen values, 20/20,000 to 20/300; P = 0.0004). Patients with earlier Stages (I-II) of AAK were managed medically and had stable visual acuity through their final visits (appETDRS score of 26 [20-35] to 35 [26-35]; Snellen, 20/300 to 20/200; P > 0.05). The appETDRS VAE was significantly improved from 20 (0-35) to 30 (20-55), Snellen, 20/400 to 20/250, following LSCT (P = 0.021) and from 2 (0-20) to 2 (0-41) after KPro; Snellen, 20/20,000 VAE but with improved 95% CI after follow-up (P = 0.019). CONCLUSIONS: With proper characterization and staging of AAK, individualized medical and advanced surgical interventions preserves and improves visual acuity.

Intraocular Light Scatter in Eyes With the Boston Type 1 Keratoprosthesis.

PURPOSE: The Boston keratoprosthesis (KPro) is the most commonly used artificial cornea. It has been proven to be successful for severe corneal disease not amenable to keratoplasty. We have observed our patients reporting debilitating glare despite attaining good visual acuities. This motivated us to objectively measure light scatter in eyes with a KPro. METHODS: Light scatter was measured in 21 eyes with the Boston type 1 KPro with a VA of 20/150 or better and was compared with 13 healthy control eyes using the Oculus C-Quant device (Arlington, WA). Eyes were also measured using an occluder contact lens. RESULTS: Light scatter (quantified as the logS value) was significantly higher in the KPro group than in the control (2.34 ± 0.15 vs. 1.29 ± 0.17, P < 0.001). The polymethylmethacrylate (PMMA) KPro group (16 eyes, logS 2.49 ± 0.19) but not the titanium group (5 eyes, logS 1.87 ± 0.15) had significantly higher light scattering than the control. The use of an occluder contact lens significantly decreased light scatter in eyes with a PMMA backplate (n = 11, logS 1.71 vs. 2.42, P = 0.028). CONCLUSION: This study confirms that intraocular light scatter is increased in KPro eyes. Possibly because of its opaque nature, the titanium backplate model seems to produce less light scatter than does the PMMA model. Occluder contact lenses reduced light scatter significantly in the PMMA KPro and could be a treatment option for symptomatic patients.

Staged ocular fornix reconstruction for glaucoma drainage device under neoconjunctiva at the time of Boston type 1 Keratoprosthesis implantation.

PURPOSE: Glaucoma is the leading cause of vision loss in eyes with Boston Keratoprosthesis (KPro). Glaucoma drainage devices (GDDs) have been shown to be effective in controlling glaucoma with KPro. Cicatricial conjunctival disease with forniceal shortening is a major challenge and limitation to the use of GDD. In our series, we report the success of fornix reconstruction in cicatricial ocular surface disease as a staged procedure prior to a combined KPro/GDD surgery. METHODS: Retrospective case series involving 4 eyes with surface cicatricization. Three patients with chemical burns and one patient with Ectrodactyly Ectodermal Dysplasia-Clefting syndrome. Preoperative data, surgical interventions, and clinical outcomes were reviewed. CONCLUSION: This series represents the first report of fornix reconstruction for combined KPro/GDD surgery. Fornix reconstruction with the aid of AMT with or without OMM grafting is a relatively safe and effective way to manage complex cicatricial surface disease associated with corneal scarring and glaucoma, allowing for subsequent successful implantation of GDD and KPro in cases that were otherwise poor surgical candidates.

Management of advanced ocular surface disease in patients with severe atopic keratoconjunctivitis.

AIM & OBJECTIVE: Severe ocular surface disease, including limbal stem cell deficiency (LSCD) can occur as a consequence of severe atopic keratoconjunctivitis (AKC) that has been inadequately treated. Our goal was to describe the management and outcomes of severe ocular surface disease in AKC patients. METHODS: We performed a retrospective analysis of a case series of 13 eyes of 8 patients with advanced ocular surface disease associated with severe AKC. The clinical presentation, medical and surgical management, and visual and anatomic outcomes were analyzed. RESULTS: Five eyes were treated with medical interventions alone, which included topical or systemic immunomodulatory therapy (IMT) for all eyes. These eyes had a decline in mean visual acuity from LogMAR 0.96 to 2.04 between the initial and final visits related to recurrent epithelial defects or corneal ulceration. Eight eyes were treated with surgical approaches in addition to medical treatment. Initial surgical treatments included limbal stem cell transplantation (n = 5), Boston keratoprosthesis (n = 2), and superficial keratectomy (n = 1). Both eyes that underwent primary keratoprosthesis had severe post-operative complications and became no light perception. In the remainder of the surgically treated eyes, there was an improvement visual acuity from LogMAR 1.43 to 0.6 between the pre-operative and final post-operative visit. CONCLUSION: Visual rehabilitation in eyes severe ocular surface disease due to prolonged AKC is challenging. While some patients did experience improved vision, most eyes did not improve or experienced severe complications with vision loss. Early intervention with immunomodulatory therapy may prevent progression of the disease to advanced stages.

A complex choristoma presenting as a salmon patch lesion in the bulbar conjunctiva.

PURPOSE: We describe a rare case of a complex choristoma appearing as a salmon-patch lesion in the nasal conjunctiva. While benign, complex choristomas are grossly indistinguishable from malignant lesions, and an excisional biopsy is warranted to confirm the diagnosis. OBSERVATIONS: A 31-year-old man presented with an elevated glistening pink mass on the conjunctiva. An excisional biopsy with a "no-touch" technique was performed, followed by placement of an amniotic membrane graft without postoperative complications. No subsequent medical treatment was pursued after the biopsy and histopathologic evaluation. Histopathology confirmed a diagnosis of a complex choristoma with sections consisting of fibroadipose tissue with cartilage, smooth muscle, and nerves. Histopathology was negative for malignant cells without morphologic evidence of a lymphoma. CONCLUSIONS AND IMPORTANCE: Conjunctival tumors are difficult to distinguish clinically, and a differential diagnosis often includes the possibility of a malignancy. Histopathologic diagnosis may be required to distinguish between various entities. In our case, a salmon-patch conjunctival tumor was biopsied and confirmed to be a benign complex choristoma.