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.

Geographic Information System Mapping of Social Risk Factors and Patient Outcomes of Pediatric Glaucoma.

PURPOSE: This study aimed to use Geographic Information System (GIS) mapping to present the geospatial distribution of visual outcomes and sociodemographic risk factors of a cohort of pediatric glaucoma patients. DESIGN: Retrospective cohort study. SUBJECTS: 233 eyes of 177 pediatric glaucoma patients treated at UC Davis Medical Center. METHODS: We reviewed the medical records of pediatric patients (aged less than 18 years) with the diagnosis of pediatric glaucoma or any adult with a prior history of pediatric glaucoma at UC Davis Medical Center from 2001 to 2019. Patient sociodemographic information and ocular health data were recorded. Patients were mapped to their residential home 3-digit zip code prefix using ArcGIS software to generate geographic representations of the pediatric glaucoma database. Statistical analyses were performed to identify significant risk factors to poor visual outcome. MAIN OUTCOME MEASURES: The primary outcome was the patient's final visual acuity (VA), defined as a binary variable based on the World Health Organization's criteria: good VA (better than 20/200) or poor VA (worse than 20/200). The secondary outcome was final intraocular pressure (IOP) at patients' final follow-ups. Risk factors for poor vision and higher IOP were assessed. RESULTS: At final follow-up, 65 eyes (27.9%) had poor vision and 168 eyes (72.1%) had good vision. In the multivariate analysis, the odds ratio of good VA decreased by 4% for every 1 mmHg increase in initial IOP (P = 0.03), and the odds of good VA decreased by 6% for every year increase in age (P = 0.04). Patients with private insurance had a 3.5 mmHg lower final IOP than those with Medicaid (P = 0.004). Travel distance was not associated with a poorer visual outcome. CONCLUSIONS: Private insurance patients had lower final IOP than Medicaid patients. Age and initial IOP were significant negative predictors of VA. Despite travel distance appearing to be associated with poorer visual outcomes by GIS mapping, it was not statistically significant. Geographic information system mapping of patient outcomes is an innovative way to visualize patient demographics and risk factors. Geographic information system may prove particularly useful in larger nationwide disease and surgical registries, especially for rare disorders like pediatric glaucoma. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

The Wholesome Foods Approach to Trabeculectomy Wet Lab Training.

Purpose In an era when trabeculectomy surgeries in the United States are on the decline, ophthalmology residents may have limited opportunities to practice surgical techniques critical to success. However, key steps of trabeculectomy surgery can be introduced in a wet laboratory using a simple surgical model based on food items. Methods A fresh lime and chicken parts with skin, purchased from a grocery store, were utilized to practice trabeculectomy surgery. The white rind of a lime was used as a surrogate for human sclera and was incised to create a trabeculectomy flap. The flap was then successfully sewn down with 10-0 nylon suture using an operating microscope. The skin of the chicken part was used to re-create a fornix-based and limbus-based conjunctival incision, which was then sutured closed using 6-0 Vicryl suture. A survey of wet laboratory participants was conducted to assess the feasibility and efficacy of this technique. Results Survey respondents were divided into two groups, those who had performed ≥40 incisional glaucoma surgeries and those who had performed <40. Both groups rated the simulation a 4 (mode) out of 5 in terms of how well it prepared them for glaucoma surgery on a human eye and how well the materials replicated human tissue, with 1 being not at all and 5 being very well. Similarly, both groups rated ease of setup and material acquisition a 1 out of 5, 1 being not difficult at all and 5 being extremely difficult. Also, 93.5% of the survey respondents recommended implementing this training model at other teaching hospitals, and none of the respondents recommended against it. Conclusion This trabeculectomy teaching model is inexpensive, clean, and safe, and it provides a reasonably realistic substrate for surgical practice. It does not require cadaver or animal eyes, and no fixatives are needed, thus minimizing the risk of contact with biohazardous materials. Wet laboratory materials are easy to obtain, making this a practical model for practicing glaucoma surgery in both westernized and developing countries.

Postoperative Complications in the Primary Tube Versus Trabeculectomy Study During 5 Years of Follow-up.

PURPOSE: To describe postoperative complications encountered in the Primary Tube Versus Trabeculectomy (PTVT) Study during 5 years of follow-up. DESIGN: Multicenter randomized clinical trial. PARTICIPANTS: A total of 242 eyes of 242 patients with medically uncontrolled glaucoma and no previous incisional ocular surgery, including 125 patients in the tube group and 117 patients in the trabeculectomy group. METHODS: Patients were enrolled at 16 clinical centers and randomly assigned to treatment with a tube shunt (350-mm2 Baerveldt glaucoma implant) or trabeculectomy with mitomycin C (MMC, 0.4 mg/ml for 2 minutes). MAIN OUTCOME MEASURES: Surgical complications, reoperations for complications, visual acuity, and cataract progression. RESULTS: Early postoperative complications occurred in 24 patients (19%) in the tube group and 40 patients (34%) in the trabeculectomy group (P = 0.013). Late postoperative complications developed in 27 patients (22%) in the tube group and 32 patients (27%) in the trabeculectomy group (P = 0.37). Serious complications producing vision loss and/or requiring a reoperation were observed in 3 patients (2%) in the tube group and 9 patients (8%) in the trabeculectomy group (P = 0.11). Cataract progression was seen in 65 patients (52%) in the tube group and 52 patients (44%) in the trabeculectomy group (P = 0.30). Surgical complications were not associated with a higher rate of treatment failure (P = 0.61), vision loss (P = 1.00), or cataract progression (P = 0.77) CONCLUSIONS: A large number of surgical complications were observed in the PTVT Study, but most were transient and self-limited. The incidence of early postoperative complications was higher following trabeculectomy with MMC than with tube shunt surgery. The rates of late postoperative complications, serious complications, and cataract progression were similar with both surgical procedures after 5 years of follow-up. Surgical complications did not increase the risk of treatment failure, vision loss, or cataract progression.

Treatment Outcomes in the Primary Tube Versus Trabeculectomy Study after 5 Years of Follow-up.

PURPOSE: To report 5-year treatment outcomes in the Primary Tube Versus Trabeculectomy (PTVT) Study. DESIGN: Multicenter randomized clinical trial. PARTICIPANTS: A total of 242 eyes of 242 patients with medically uncontrolled glaucoma and no previous incisional ocular surgery, including 125 patients in the tube group and 117 patients in the trabeculectomy group. METHODS: Patients were enrolled at 16 clinical centers and randomly assigned to treatment with a tube shunt (350-mm2 Baerveldt glaucoma implant) or trabeculectomy with mitomycin C (MMC) (0.4 mg/ml for 2 minutes). MAIN OUTCOME MEASURES: The primary outcome measure was the rate of surgical failure, defined as intraocular pressure (IOP) > 21 mmHg or reduced < 20% from baseline, IOP ≤ 5 mmHg, reoperation for glaucoma, or loss of light perception. Secondary outcome measures included IOP, glaucoma medical therapy, and visual acuity. RESULTS: The cumulative probability of failure after 5 years of follow-up was 42% in the tube group and 35% in the trabeculectomy group (P = 0.21; hazard ratio = 1.31; 95% confidence interval = 0.86-2.01). At 5 years, IOP (mean ± standard deviation) was 13.4 ± 3.5 mmHg in the tube group and 13.0 ± 5.2 mmHg in the trabeculectomy group (P = 0.52), and the number of glaucoma medications (mean ± standard deviation) was 2.2 ± 1.3 in the tube group and 1.3 ± 1.4 in the trabeculectomy group (P < 0.001). CONCLUSIONS: Trabeculectomy with MMC and tube shunt surgery produced similar IOPs after 5 years of follow-up in the PTVT Study, but fewer glaucoma medications were required after trabeculectomy. No significant difference in the rate of surgical failure was observed between the 2 surgical procedures at 5 years.

Use of a Novel Microshunt in Refractory Childhood Glaucoma: Initial Experience in a Compassionate Use/Early Access Cohort.

PURPOSE: In patients with refractory childhood glaucoma, treatment options include trabeculectomy or large glaucoma drainage devices (GDDs) with attendant short- and long-term risks. A novel polymer-based microshunt is under review by the US Food and Drug Administration (FDA) for use in adults. The device is attractive for children given the long-term stability of the polymer and the small conjunctival incision required for implantation. This early clinical series explores the safety and efficacy of this device in patients with refractory childhood glaucoma who would otherwise undergo trabeculectomy or implantation of a GDD. DESIGN: Prospective single-center case series under FDA compassionate use investigational device exemption. METHODS: FDA and institutional review board approvals were obtained to treat ≤20 children using this investigational device under the compassionate use pathway. Single eyes in patients with refractory childhood glaucoma were treated surgically with the microshunt. Patients with ≥1 year of follow-up are reported. RESULTS: Twelve eyes of 12 children (15 months to 14 years if age) with mean preoperative intraocular pressure of 22.72 ± 4.8 mm Hg on 3.3 ± 0.65 medications were treated beginning in December 2019. No intraoperative complications occurred. Among eyes with ≥1 year of follow-up (range 12-23 months), 9 were successfully controlled. In this group, preoperative intraocular pressure 21.6 ± 4.9 mm Hg dropped 45% to 11.9 ± 3.8 mm Hg at 1 year; 7 patients were taking no medications at 12 months, and 2 required 2 medications (fixed-combination dorzolamide-timolol). Three eyes failed, requiring additional surgery. CONCLUSION: These early data suggest that the device is safe and appears effective in patients with refractory childhood glaucoma. A prospective, multicenter pivotal trial is planned.

Consensus Statement for the Management and Treatment of Sturge-Weber Syndrome: Neurology, Neuroimaging, and Ophthalmology Recommendations.

BACKGROUND: Sturge-Weber syndrome (SWS) is a sporadic, neurocutaneous syndrome involving the skin, brain, and eyes. Because of the variability of the clinical manifestations and the lack of prospective studies, consensus recommendations for management and treatment of SWS have not been published. OBJECTIVE: This article consolidates the current literature with expert opinion to make recommendations to guide the neuroimaging evaluation and the management of the neurological and ophthalmologic features of SWS. METHODS: Thirteen national peer-recognized experts in neurology, radiology, and ophthalmology with experience treating patients with SWS were assembled. Key topics and questions were formulated for each group and included (1) risk stratification, (2) indications for referral, and (3) optimum treatment strategies. An extensive PubMed search was performed of English language articles published in 2008 to 2018, as well as recent studies identified by the expert panel. The panel made clinical practice recommendations. CONCLUSIONS: Children with a high-risk facial port-wine birthmark (PWB) should be referred to a pediatric neurologist and a pediatric ophthalmologist for baseline evaluation and periodic follow-up. In newborns and infants with a high-risk PWB and no history of seizures or neurological symptoms, routine screening for brain involvement is not recommended, but brain imaging can be performed in select cases. Routine follow-up neuroimaging is not recommended in children with SWS and stable neurocognitive symptoms. The treatment of ophthalmologic complications, such as glaucoma, differs based on the age and clinical presentation of the patient. These recommendations will help facilitate coordinated care for patients with SWS and may improve patient outcomes.

Assessment of Cumulative Incidence and Severity of Primary Open-Angle Glaucoma Among Participants in the Ocular Hypertension Treatment Study After 20 Years of Follow-up.

Importance: Ocular hypertension is an important risk factor for the development of primary open-angle glaucoma (POAG). Data from long-term follow-up can be used to inform the management of patients with ocular hypertension. Objective: To determine the cumulative incidence and severity of POAG after 20 years of follow-up among participants in the Ocular Hypertension Treatment Study. Design, Setting, and Participants: Participants in the Ocular Hypertension Treatment Study were followed up from February 1994 to December 2008 in 22 clinics. Data were collected after 20 years of follow-up (from January 2016 to April 2019) or within 2 years of death. Analyses were performed from July 2019 to December 2020. Interventions: From February 28, 1994, to June 2, 2002 (phase 1), participants were randomized to receive either topical ocular hypotensive medication (medication group) or close observation (observation group). From June 3, 2002, to December 30, 2008 (phase 2), both randomization groups received medication. Beginning in 2009, treatment was no longer determined by study protocol. From January 7, 2016, to April 15, 2019 (phase 3), participants received ophthalmic examinations and visual function assessments. Main Outcomes and Measures: Twenty-year cumulative incidence and severity of POAG in 1 or both eyes after adjustment for exposure time. Results: A total of 1636 individuals (mean [SD] age, 55.4 [9.6] years; 931 women [56.9%]; 1138 White participants [69.6%]; 407 Black/African American participants [24.9%]) were randomized in phase 1 of the clinical trial. Of those, 483 participants (29.5%) developed POAG in 1 or both eyes (unadjusted incidence). After adjusting for exposure time, the 20-year cumulative incidence of POAG in 1 or both eyes was 45.6% (95% CI, 42.3%-48.8%) among all participants, 49.3% (95% CI, 44.5%-53.8%) among participants in the observation group, and 41.9% (95% CI, 37.2%-46.3%) among participants in the medication group. The 20-year cumulative incidence of POAG was 55.2% (95% CI, 47.9%-61.5%) among Black/African American participants and 42.7% (95% CI, 38.9%-46.3%) among participants of other races. The 20-year cumulative incidence for visual field loss was 25.2% (95% CI, 22.5%-27.8%). Using a 5-factor baseline model, the cumulative incidence of POAG among participants in the low-, medium-, and high-risk tertiles was 31.7% (95% CI, 26.4%-36.6%), 47.6% (95% CI, 41.6%-53.0%), and 59.8% (95% CI, 53.1%-65.5%), respectively. Conclusions and Relevance: In this study, only one-fourth of participants in the Ocular Hypertension Treatment Study developed visual field loss in either eye over long-term follow-up. This information, together with a prediction model, may help clinicians and patients make informed personalized decisions about the management of ocular hypertension. Trial Registration: ClinicalTrials.gov Identifier: NCT00000125.

Central Corneal Thickness in the Ocular Hypertension Treatment Study (OHTS).

OBJECTIVE: Central corneal thickness influences intraocular pressure (IOP) measurement. We examined the central corneal thickness of subjects in the Ocular Hypertension Treatment Study (OHTS) and determined if central corneal thickness is related to race. DESIGN: Cross-sectional study. PARTICIPANTS: One thousand three hundred one OHTS subjects with central corneal thickness measurements. INTERVENTION: Central corneal thickness was determined with ultrasonic pachymeters of the same make and model at all clinical sites of the OHTS. MAIN OUTCOME MEASURES: Correlation of mean central corneal thickness with race, baseline IOP, refraction, age, gender, systemic hypertension, and diabetes. RESULTS: Mean central corneal thickness was 573.0 ± 39.0 µm. Twenty-four percent of the OHTS subjects had central corneal thickness > 600 µm. Mean central corneal thickness for African American subjects (555.7 ± 40.0 µm; n = 318) was 23 µm thinner than for white subjects (579.0 ± 37.0 µm; P < 0.0001). Other factors associated with greater mean central corneal thickness were younger age, female gender, and diabetes. CONCLUSIONS: OHTS subjects have thicker corneas than the general population. African American subjects have thinner corneas than white subjects in the study. The effect of central corneal thickness may influence the accuracy of applanation tonometry in the diagnosis, screening, and management of patients with glaucoma and ocular hypertension.

A Comparison of Trabeculectomy Surgery Outcomes With Mitomycin-C Applied by Intra-Tenon Injection Versus Sponge.

PURPOSE: To compare the outcomes of mitomycin-C (MMC) delivered by intra-Tenon injection vs sponge application during trabeculectomy surgery. METHODS: We retrospectively reviewed 566 patients with primary and secondary glaucoma diagnoses who received trabeculectomy surgery with MMC in an academic medical center. Exclusion criteria were age less than 18 years, no light perception vision, combined surgery, previous glaucoma incisional surgery, intraoperative 5-fluorouracil, or follow-up <1 month. Subjects were divided into 2 cohorts: MMC delivered by sponge application or by intra-Tenon injection. Main outcome measures were postoperative intraocular pressure (IOP) level and secondary measures were survival rate for IOP control, glaucoma medication use, complication rate, and vision. RESULTS: After inclusion/exclusion criteria, 316 eyes were available for analysis; 131 eyes had MMC delivered via sponge and 185 eyes via injection. Mean postoperative IOP was not significantly different between treatment groups but change in IOP from baseline was lower in the sponge vs the injection group 24 months after surgery (P = .038). The MMC sponge group had significantly more tense, vascularized, or encapsulated blebs as a late complication (P = .046). Time to failure for postoperative IOP control was not significantly different between MMC treatment groups, but older patient age and limbus-based conjunctival incision were associated with significantly longer time to fail. CONCLUSIONS: The application of MMC by injection was similar to application by sponge in lowering IOP in patients with glaucoma and the safety of both techniques appears to be comparable. Limbus-based conjunctival incision had longer time to failure for postoperative IOP control vs fornix-based incision. NOTE: Publication of this article is sponsored by the American Ophthalmological Society.

Combined Dexamethasone Intravitreal Implant and Glaucoma Drainage Device Placement for Uveitic Glaucoma.

PRECIS: At 1-year postoperative follow-up, concurrent placement of a dexamethasone intravitreal implant and glaucoma drainage device effectively controlled intraocular pressure (IOP) and inflammation in eyes with uveitic glaucoma with no changes in systemic immunomodulatory therapy. PURPOSE: The purpose of this study was to assess 1-year postoperative outcomes in eyes with uncontrolled uveitic glaucoma following concurrent placement of a dexamethasone intravitreal implant and glaucoma drainage device. MATERIALS AND METHODS: This is a retrospective, observational case series of patients with chronic, noninfectious uveitis and uveitic glaucoma uncontrolled on maximal tolerated medical therapy with at least 1-year postoperative follow-up. The main outcomes were visual acuity, IOP, number of glaucoma medications, recurrent inflammation, frequency of topical steroids, systemic immunomodulatory therapy, and adverse events. Success was defined as IOP <21 mm Hg and IOP reduced by >20% from baseline on at least 2 consecutive visits after 3 months either with or without glaucoma medications (ie, partial or complete success, respectively). RESULTS: Eight eyes in 6 patients met the inclusion criteria. The average age was 44.1±19.7 years (range: 10 to 68 y) and 50% were female. At 1-year, there was no significant change in visual acuity. No eyes lost ≥3 lines of vision. The majority of eyes (87.5%) achieved complete (n=2) or partial success (n=5) with a decrease in average IOP from 36.5 to 11.8 mm Hg (P=0.002). Glaucoma medication use decreased from 3.0 to 1.3 medications (P=0.04). There was a significant decrease in the number of episodes of recurrent inflammation in the 6 months following surgery compared with the 6 months before surgery (P=0.004). CONCLUSION: In this small case series, dexamethasone intravitreal implant combined with Ahmed glaucoma drainage device appears to be an effective approach for the management of uncontrolled uveitic glaucoma.