Reactivation After Teprotumumab Treatment for Active Thyroid Eye Disease.

PURPOSE: To determine the recurrence and reactivation rates after teprotumumab therapy for active thyroid eye disease. DESIGN: Retrospective consecutive case series. METHODS: This was a study of all patients followed for active thyroid eye disease at the Cole Eye Institute, Cleveland Clinic, treated with teprotumumab between May 2020 and May 2021. Patients with less than 6 months follow-up after completion of infusions were excluded. The primary outcome measure was reactivation, defined as a regression in proptosis (increase of ≥2 mm in either eye and to within ≤2 mm of pre-treatment level and Clinical Activity Score [CAS] worsening of 2 points or greater). Secondary outcome was diplopia response. RESULTS: A total of 21 patients were included in the study. The average long-term improvement in proptosis in the eye with more proptosis after teprotumumab was 1.57mm (range, -3 to 4 mm). Of the 17 initial responders, there were 8 reactivations (47%) and 2 isolated proptosis regressions (12%); Overall, 7 of 21 patients (33%) responded throughout the study period. Average time to regression was 12.25 months (range, 2-22.5 months). There was no statistically significant change in diplopia at final visit in any subgroup (P = 0.68 to >.99). CONCLUSIONS: At most, 33% of patients demonstrate continued response 2 years after teprotumumab treatment. The proptosis and CAS regression occurs in the setting of disease reactivation in 80% of regressions. Teprotumumab treatment appears to offer minimal long-term improvement in diplopia.

Conjunctival Müllerectomy With or Without Tarsectomy Using Absorbable Versus Nonabsorbable Suture.

PURPOSE: To compare the outcomes of blepharoptosis repair using conjunctival Müllerectomy with or without tarsectomy (CM±T) using absorbable suture versus nonabsorbable suture. METHODS: Retrospective case-series of all consecutive cases of CM±T ptosis repair between January 1, 2019, and August 31, 2021. Patients were placed in groups based on whether they had absorbable or nonabsorbable suture used during their procedure. Preoperative and postoperative measurements of MRD1 were gathered in both groups, and information on patient satisfaction, symmetry, complications, and reoperations. RESULTS: Ninety-two patients had surgery using nonabsorbable suture and 96 patients had surgery using absorbable suture, with average age of 72.0 and 70.9 years, respectively ( p = 0.488). When comparing nonabsorbable to absorbable suture, the groups did not differ on average preoperative MRD1 (1.11 mm [±0.96] vs. 0.96 mm [±0.86]; p = 0.161), average postoperative MRD1 (3.18 mm [±0.54] vs. 3.20 mm (±0.51); p = 0.736) or average MRD1 improvement (2.09 mm (±0.86) vs. 2.25 mm (±0.79); p = 0.089). Goal MRD1 was achieved in 85.3% of nonabsorbable cases and 82.8% of absorbable cases ( p = 0.562) and the rate of reoperation between groups was not significant ( p = 0.63). Good postoperative symmetry was noted in 91.6% of nonabsorbable and 91.0% absorbable suture cases ( p = 0.83). No difference in the number of complications was noted between groups ( p = 0.88), including need for contact lens placement (2.7% nonabsorbable, 1.3% absorbable; p = 0.37). CONCLUSIONS: The use of absorbable suture was found to have predictable and effective outcomes similar to cases using nonabsorbable suture for CM±T without an increase in complications or rate or reoperation.

iStent Trabecular Microbypass Stent Implantation with Phacoemulsification in Patients with Open-Angle Glaucoma: 6-Year Outcomes.

PURPOSE: To investigate the long-term safety and efficacy of an iStent trabecular microbypass stent in combination with cataract surgery in eyes with primary open-angle glaucoma (POAG). SETTING: Private practice; Sioux Falls, South Dakota. DESIGN: Retrospective, consecutive case series. METHODS: This case series included eyes implanted with a single trabecular microbypass stent in combination with phacoemulsification in patients with mild to severe POAG. Data were collected preoperatively and at day 1, week 1, month 1, and up to 6 years postoperatively. Primary outcome measures included mean intraocular pressure (IOP) and number of glaucoma medications. Safety was noted by assessing the incidence of IOP spikes and need for additional surgery. RESULTS: The study comprised 411 eyes. Mean IOP was reduced to 14.9±4.2 mmHg compared to 18.8±5.6 mmHg at baseline at 6 years postoperative. The mean number of medications was reduced to 1.2±1.0 from 1.4±1.1 at baseline. In eyes with severe stage of disease, there was a mean IOP reduction >6 mmHg at 6 years postoperative. Eyes with baseline IOP ≥18 mmHg achieved a more robust reduction in IOP. Fifteen eyes underwent a secondary glaucoma procedure. There were no intra- or postoperative complications. CONCLUSION: Trabecular microbypass stent implantation in combination with cataract surgery provides a sustained IOP reduction in eyes with mild-to-severe POAG. The degree of IOP reduction was more significant in eyes with higher baseline IOP and severe stage of disease.

Author Correction: Characterization of a recurrent missense mutation in the forkhead DNA-binding domain of FOXP1.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Bilateral Papillitis and Anterior Uveitis in a Pediatric Patient.

We report a case of bilateral papillitis and anterior uveitis in a pediatric patient with preceding viral prodrome. Because this is a rare disease in the pediatric population, work-up in this case was extensive given the wide differential of papilledema in the pediatric setting. The clinical significance of this case is to bring to light the timely recognition, follow-up, and treatment of this disease to minimize the burden to the patient and family.

Characterization of a recurrent missense mutation in the forkhead DNA-binding domain of FOXP1.

Haploinsufficiency of Forkhead box protein P1 (FOXP1), a highly conserved transcription factor, leads to developmental delay, intellectual disability, autism spectrum disorder, speech delay, and dysmorphic features. Most of the reported FOXP1 mutations occur on the C-terminus of the protein and cluster around to the forkhead domain. All reported FOXP1 pathogenic variants result in abnormal cellular localization and loss of transcriptional repression activity of the protein product. Here we present three patients with the same FOXP1 mutation, c.1574G>A (p.R525Q), that results in the characteristic loss of transcription repression activity. This mutation, however, represents the first reported FOXP1 mutation that does not result in cytoplasmic or nuclear aggregation of the protein but maintains normal nuclear localization.

Influences on rebubble rate in Descemet's membrane endothelial keratoplasty.

PURPOSE: Descemet's membrane endothelial keratoplasty (DMEK) is a minimally invasive partial corneal transplant procedure used in patients with failing endothelial membranes. This study aims to identify those factors which influence the need for a rebubble of the corneal graft. METHODS: A total of 94 eyes that received DMEK between March 2014 and January 2016 at Vance Thompson Vision were used in the study. Demographic and graft data were collected from the patients and donors, and perioperative statistics of the procedures. A logistical regression was used to compare eyes that did and did not require a rebubble. RESULTS: Among those characteristics that were included (patient age/sex, donor age/sex, death to processing time, donation to surgery time, death to procurement time, specular cell count density, burping procedure, postoperative day 1 intraocular pressure [IOP], and postoperative week 1 IOP, concurrent phacoemulsification, and how well the Descemet graft was centered), only a lower specular cell count density of the corneal graft, and a graft that was not well-centered correlated with needing a rebubble due to partial graft detachment (p=0.021) and (p=0.023), respectively. CONCLUSION: An increased specular cell count density may allow for better placement of the corneal graft by allowing for better unfolding in DMEK procedures. A well-centered graft may decrease postoperative complications by increasing adherence. Additionally, postoperative management of IOP may not affect the rebubble rate, and therefore should be left to the discretion of the provider to determine whether it is necessary.

Midwest Science Festival: Exploring Students' and Parents' Participation in and Attitudes Toward Science.

INTRODUCTION: Compared to national numbers, South Dakota has a higher proportion of students interested in science, technology, engineering, and mathematics (STEM) fields. Interest in science can be influenced by exposure to science through formal and informal learning. Informal science activities (including exposures and participation) have been found to elicit higher levels of interest in science, likely impacting one's attitude towards science overall. The current study goal is to better understand the levels and relationships of attitude, exposure, and participation in science that were present among students and parents attending a free science festival. METHODS: The project collected survey data from 65 students and 79 parents attending a science festival ranging from age 6 to 65. RESULTS: Informal science participation is significantly related to science attitudes in students and informal science exposure is not. No relationship was found for parents between science attitudes and participation. CONCLUSIONS: Students who indicated high levels of informal science participation (i.e., reading science-themed books) were positively related to their attitudes regarding science. However, informal science exposures, such as attending the zoo or independently visiting a science lab, was not significantly associated with positive attitudes towards science.