Prevalence and Morbidity of Undiagnosed Celiac Disease From a Community-Based Study.

BACKGROUND & AIMS: Little is known about the prevalence and burden of undiagnosed celiac disease in individuals younger than age 50. We determined the prevalence and morbidity of undiagnosed celiac disease in individuals younger than age 50 in a community. METHODS: We tested sera from 31,255 residents of Olmsted County, Minnesota (<50 y), without a prior diagnosis of celiac disease assay using an assay for IgA against tissue transglutaminase; in subjects with positive test results, celiac disease was confirmed using an assay for endomysial IgA. We performed a nested case-control study to compare the proportion of comorbidities between undiagnosed cases of celiac disease and age- and sex-matched seronegative controls (1:2). Medical records were abstracted to identify potential comorbidities. RESULTS: We identified 338 of 30,425 adults with positive results from both serologic tests. Based on this finding, we estimated the prevalence of celiac disease to be 1.1% (95% confidence interval, 1.0%-1.2%); 8 of 830 children tested positive for IgA against tissue transglutaminase (1.0%; 95% confidence interval, 0.4%-1.9%). No typical symptoms or classic consequences of diagnosed celiac disease (diarrhea, anemia, or fracture) were associated with undiagnosed celiac disease. Undiagnosed celiac disease was associated with increased rates of hypothyroidism (odds ratio, 2.2; P < .01) and a lower than average cholesterol level (P = .03) and ferritin level (P = .01). During a median follow-up period of 6.3 years, the cumulative incidence of a subsequent diagnosis with celiac disease at 5 years after testing was 10.8% in persons with undiagnosed celiac disease vs 0.1% in seronegative persons (P < .01). Celiac disease status was not associated with overall survival. CONCLUSIONS: Based on serologic tests of a community population for celiac disease, we estimated the prevalence of undiagnosed celiac disease to be 1.1%. Undiagnosed celiac disease appeared to be clinically silent and remained undetected, but long-term outcomes have not been determined.

MicroRNA 29 targets nuclear factor-κB-repressing factor and Claudin 1 to increase intestinal permeability.

BACKGROUND & AIMS: Some patients with irritable bowel syndrome with diarrhea (IBS-D) have intestinal hyperpermeability, which contributes to their diarrhea and abdominal pain. MicroRNA 29 (MIR29) regulates intestinal permeability in patients with IBS-D. We investigated and searched for targets of MIR29 and investigated the effects of disrupting Mir29 in mice. METHODS: We investigated expression MIR29A and B in intestinal biopsies collected during endoscopy from patients with IBS (n = 183) and without IBS (controls) (n = 36). Levels were correlated with disease phenotype. We also generated and studied Mir29(-/-) mice, in which expression of Mir29a and b, but not c, is lost. Colitis was induced by administration of 2,4,6-trinitrobenzenesulfonic acid; intestinal tissues were collected and permeability was assessed. Microarray analysis was performed using tissues from Mir29(-/-) mice. Changes in levels of target genes were measured in human colonic epithelial cells and small intestinal epithelial cells after knockdown of MIR29 with anti-MIRs. RESULTS: Intestinal tissues from patients with IBS-D (but not IBS with constipation or controls) had increased levels of MIR29A and B, but reduced levels of Claudin-1 (CLDN1) and nuclear factor-κB-repressing factor (NKRF). Induction of colitis and water avoidance stress increased levels of Mir29a and Mir29b and intestinal permeability in wild-type mice; these increased intestinal permeability in colons of far fewer Mir29(-/-) mice. In microarray and knockdown experiments, MIR29A and B were found to reduce levels of NKRF and CLDN1 messenger RNA, and alter levels of other messenger RNAs that regulate intestinal permeability. CONCLUSIONS: Based on experiments in knockout mice and analyses of intestinal tissue samples from patients with IBS-D, MIR29 targets and reduces expression of CLDN1 and NKRF to increase intestinal permeability. Strategies to block MIR29 might be developed to restore intestinal permeability in patients with IBS-D.

Resident and fellow participation in strabismus surgery: effect of level of training and number of assistants on operative time and cost.

OBJECTIVE: To investigate the effect of the level of training and number of assistants on operative time for uncomplicated, 2-muscle, horizontal strabismus surgery at an academic institution. DESIGN: Comparative case series. PARTICIPANTS: A total of 993 children and adults between the ages of 6 months and 75 years. METHODS: Retrospective chart review of strabismus surgeries performed between July 1, 2008, and December 31, 2012, by any of 3 attending surgeons assisted by a resident in the postgraduate year 3 (PGY3), fellow in the postgraduate year 5 (PGY5), or both. MAIN OUTCOME MEASURES: Operative time (minutes) and associated operative cost (dollars). RESULTS: There were 373 cases with 1 assistant and 44 cases with 2 assistants. Of all cases with 1 assistant, there were 200 cases with a PGY3 assistant an average operative time of 62.5 minutes (standard deviation [SD], 15.1) and 173 cases with a PGY5 assistant an average operative time of 59.0 minutes (SD, 14.7); the difference of 3.5 minutes was statistically significant (P = 0.02). The average operative time for all cases with 2 assistants (both PGY3 and PGY5) was 10.6 minutes longer than all cases with 1 assistant (P = 0.0002). No statistically significant variation in operative times was demonstrated when comparing cases with a PGY3 (P = 0.29) and PGY5 (P = 0.44) assistant in their respective first and last halves of the academic year, but operative times within individual quarters of the academic year were significant for PGY3 (P = 0.03) but not for PGY5 (P = 0.24) assistant cases. Operative times were significantly different for individual PGY3 (P = 0.03) but not PGY5 (P = 0.22) assistant cases. Cost per PGY3 assistant per year for additional operative time is $3141.95. CONCLUSIONS: Operative time in strabismus surgery increased with PGY3 participation and further increased with both assistants over either assistant alone. Operative times earlier in the year did not vary from those later in the year for PGY3 or PGY5 assistants. The difference in quarterly and individual PGY3 but not PGY5 assistant operative times suggests that efficiency in strabismus surgery varies by assistants with less experience or interest.

Exposure to persistent hemodynamically significant patent ductus arteriosus is associated with retinopathy of prematurity.

BACKGROUND: Hemodynamically significant patent ductus arteriosus (hsPDA) shunt may predispose infants to retinopathy of prematurity (ROP) because of its higher preductal cardiac output and blood oxygen content, which may augment ocular oxygen delivery. METHODS: A retrospective cohort study of preterm infants, born at <27 weeks' gestation and admitted at <24h postnatal age to a large quaternary referral was conducted. The primary composite outcome was death at <32 weeks or moderate-to-severe ROP (≥stage 2 or requiring treatment) in either eye. Secondary outcomes included ROP requiring treatment, and any ROP. Univariate analysis of patient characteristics and outcomes was performed as well as logistic regression. A receiver operating characteristics curve was generated for the outcome of ROP ≥stage 2 or requiring treatment. RESULTS: A total of 91 patients were screened, of whom 86 (54 hsPDA, 32 controls) were eligible for inclusion. hsPDA patients were younger and lighter at birth and had a higher burden of hyperglycemia and respiratory illness. The rates of the composite outcome (death <32 weeks or moderate-to-severe ROP) and of any ROP were more frequent in the hsPDA group. hsPDA shunt exposure was independently associated with development of any ROP among survivors to assessment (P = 0.006). PDA cumulative exposure score of 78 (clinical equivalent = 7 days high-volume shunt exposure) predicts moderate-to-severe ROP with 80% sensitivity and 78% specificity. CONCLUSIONS: Among infants <27 weeks, hsPDA shunt is associated with increased risks of a composite outcome of death or moderate-to-severe ROP, as well as ROP of any stage. Shunt modulation as a strategy to reduce ROP represents a biologically plausible avenue for investigation.

Xp11.3 microdeletion causing Norrie disease and X-linked Kabuki syndrome.

Purpose: To describe a novel case of Norrie disease and X-linked Kabuki syndrome caused by a microdeletion encompassing multiple genes on the X chromosome. Observations: A 3-day-old boy born at full term had bilateral retrolental fibrovascular plaques. Surgery with lensectomy and vitrectomy revealed bilateral, closed funnel retinal detachments consistent with a clinical diagnosis of Norrie disease. In addition, the baby had congenital heart defects, hearing loss, and dysmorphic facies. His mother carried a clinical diagnosis of Kabuki syndrome. Genetic testing of the baby revealed an Xp11.3 microdeletion that included the NDP and KDM6A genes, confirming the baby had both Norrie disease and X-linked Kabuki syndrome. The mother was found via ultrawide-field fluorescein angiography to have asymptomatic peripheral retinal vascular anomalies, consistent with NDP-associated familial exudative vitreoretinopathy (FEVR). Conclusions and importance: This is the first reported case of Norrie disease together with X-linked Kabuki syndrome. Contiguous gene deletions may explain some of the variable systemic involvement in Norrie disease.

The long-term outcomes of the Anderson-Kestenbaum procedure.

Introduction: Nystagmus is an involuntary, conjugated, rhythmic movement of the eye that can be idiopathic or secondary to ocular or neurologic pathologies. Patients with nystagmus often have a position of gaze in which their symptoms are dampened or absent, referred to as the "null zone." The Anderson-Kestenbaum procedure is a bilateral recess-resect procedure of the four horizontal rectus muscles which aims to bring the null position into the primary gaze. This study aims to further elucidate long-term outcomes and factors associated with optimal postoperative outcomes. Methods: Patients with a diagnosis of nystagmus and a surgical code for strabismus between June 1990 and August 2017 were considered for inclusion in the study. Patients were included if they had undergone the Anderson-Kestenbaum procedure and had follow-up lasting at least 24 months post-operatively. Data collected included demographic information, characteristics of the nystagmus, underlying etiology of nystagmus, and pre-and post-operative measurements. Results: 25 patients were included. At their last recorded follow-up, 44% of patients achieved an optimal surgical outcome -an abnormal head position of 10 degrees or less. 88% of patients showed an overall improvement in their head posture at the last follow-up. The absence of an abnormal head position at the visit closest to 24 months post-operatively was found to be significantly associated with the lack of a significant head position at the last follow-up visit. Optimal surgical outcomes were not significantly associated with the underlying diagnosis, the direction of the abnormal head position, or the type of nystagmus. Discussion: The relatively long follow-up of this cohort allows this study to further elucidate the long-term outcomes of the Anderson-Kestenbaum procedure. Overall, our results suggest that although improvement in head position post-operatively is likely, it is still expected that many patients will have a residual abnormal head position after the procedure. The results of this study are helpful in counseling patients, especially knowing that if they do not have a significant head position at 24 months follow-up, they are unlikely to develop one. However, due to the small sample size, larger cohorts and more standardized follow-up may provide further insight into the procedure's outcomes.

The Ocular Trauma Score Underestimates Visual Recovery for the Most Severe Open-Globe Injuries.

PURPOSE: To compare visual outcomes after open-globe injury (OGI) with those predicted by the Ocular Trauma Score (OTS), and to investigate the effect of treatment with pars plana vitrectomy (PPV). DESIGN: Retrospective cohort study. SUBJECTS: Patients presenting with OGI to an academic United States ophthalmology department from 2017 to 2020. METHODS: Best-corrected visual acuity (VA) measurements at the most recent follow-up were compared with final VA predicted by the OTS, based on preoperative injury characteristics. The most recently measured VA of patients treated with PPV during initial OGI repair (primary PPV group) was compared with patients treated with PPV after initial OGI repair (secondary PPV group) and patients never treated with PPV (No PPV group). MAIN OUTCOME MEASURES: Best-corrected VA in the injured eye at last follow-up; secondary outcome measures included the occurrence of vitreous hemorrhage at any time, occurrence of retinal detachment at any time, rates of additional surgery, and rates of enucleation. RESULTS: One-hundred and thirty-three subjects with OGI were identified and analyzed. The overall rate of PPV was 32%. Predictors of worse VA at last follow-up included older age (P = 0.047) and worse presenting VA (P < 0.001). Visual acuity outcomes for eyes in OTS categories 2 to 5 did not significantly differ from OTS predictions. However, eyes in OTS category 1 had a higher likelihood of last follow-up VA of light perception (LP) to hand motion (46% in the study cohort vs. 15% predicted by the OTS, P = 0.004) and a lower likelihood of no LP (33% vs. 74%, P < 0.001). The secondary PPV group had the worst VA at presentation among the 3 groups (P = 0.016), but VA at last follow-up did not significantly differ between the study groups (P = 0.338). CONCLUSIONS: The most severe OGIs (i.e., OTS category 1) had better visual outcomes than predicted by the published OTS expectations, and secondary PPV was associated with significant visual improvement despite poor prognostic predictions. Evaluation by a vitreoretinal surgeon should be considered for all patients with severe OGI, especially those in OTS category 1. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Respirator selection for clandestine methamphetamine laboratory investigation.

First responders to illicit drug labs may not always have SCBA protection available. Air-purifying respirators using organic vapor cartridges with P-100 filters may not be sufficient. It would be better to use a NIOSH-approved CBRN respirator with its required multi-purpose cartridge system, which includes a P-100 filter. This would remove all the primary drug lab contaminants­organic vapors, acid gases, ammonia, phosphine, iodine, and airborne meth particulates. To assure the proper selection and use of a respirator, it is recommended that the contaminants present be identified and quantified and the OSHA 29 CFR 1910.134 respirator protection program requirements followed.

A comparative study of strabismus surgery with retained or removed scleral explant after retinal detachment surgery.

PURPOSE: To compare, in a larger study population, the outcomes of strabismus surgery in patients who either had the scleral explant (EX) retained or removed after surgery for retinal detachment. METHODS: A comparative retrospective study includes data from 2 centres. Surgical outcome, motor success, sensory success, the number of secondary operations for strabismus and complications were compared between the two groups. Motor success was defined as horizontal deviation of ≤6 prism diopters (PD) and vertical deviation of ≤6PD. Sensory success was defined as no diplopia without use of prism. RESULTS: Forty-seven patients were included in the study; 70% had retained the EX and 74% had vertical strabismus. Horizontally, the final alignment was equal between the 2 groups, 4 PD vs.5 PD in the EX removed group. The patients with retained EX were referred with a significant lower mean vertical deviation and had a significant lower post-operative mean vertical deviation of 2 PD vs. 3 PD in the EX-removed group. The rates of motor success (76% vs. 71%) and sensory success (79% vs. 93%) did not differ significantly between the EX-retained and EX-removed groups. No patients underwent more than 2 operations. Two complications occurred in the EX-retained group: a subconjunctival cyst and an exposed EX. In the EX-removed group, 2 patients with retinal re-detachment were found in the follow-up period. CONCLUSION: Good surgical outcomes including high motor and sensory success were obtained regardless of the presence of the EX. Retinal re-detachment was observed in two patients with previously removed EX.

Is YouTube a Useful Tool for Trainees in Pediatric Cataract Surgery?

PURPOSE: To determine the utility of surgical videos published on YouTube (Google) as resources for trainee education in pediatric ophthalmology, the authors assessed the surgical proficiency, patient care, and video quality displayed in the published YouTube content. METHODS: The 10 most highly viewed pediatric and congenital cataract procedural videos published within the past 10 years were independently reviewed by three separate board-certified, fellowship-trained, practicing pediatric ophthalmologists. Videos were assessed for surgical competency on a 5-point Likert scale in six key areas as outlined in the American Academy of Ophthalmology's congenital cataract surgery guidelines. The teaching quality of the videos was also subjectively assessed based on multiple measures. RESULTS: The mean overall score was 3.93 ± 0.94 (range: 2.67 to 4.67). Only one video failed to receive an overall score of greater than 3, indicating incompetent overall surgical performance. No other video failed to have a mean competent score for any single individual technique. One video demonstrated potential patient safety concerns. Eighty percent of videos had adequate or better picture quality. CONCLUSIONS: Of the 10 most popular pediatric cataract surgical videos published on YouTube, all but one displayed competent overall surgical technique. Although viewers must always be wary because unvetted and potentially harmful videos may be published on the platform at any time, if used correctly, surgical content published on YouTube can be a helpful tool for ophthalmologic trainees. [J Pediatr Ophthalmol Strabismus. 2022;59(3):200-203.].

Vision screening outcomes in children less than 3 years of age compared with children 3 years and older.

PURPOSE: To compare the rates of amblyopia and treatment outcomes in children 0-2 years to those of children aged 3-5 years referred from a well-established community-based photoscreening program. METHODS: The medical records of children who failed vision photoscreening through Iowa KidSight and were subsequently seen at the University of Iowa for a complete eye examination over a 13-year period were retrospectively reviewed. Outcome measures were the number of children obtaining normal vision, the age at which normal vision was attained, and the elapsed time from screening examination to first documentation of normal vision. RESULTS: Of 319 subjects, 67 (21%) were 0-2 years of age and 252 (79%) were at least 3 years of age at screening. Amblyopia was found in 19% of the younger group and 30% of the older group (P = 0.12). Follow-up time was similar between groups. At final follow-up, 8% of children in the younger group did not attain normal vision, compared with 40% in the older group (OR = 8.92; 95% CI, 1.65-92.95; P = 0.009). Normal vision was attained on average at 35 months of age in the younger group and 69 months in the older group (P < 0.0001). CONCLUSIONS: In our study cohort, children <3 years of age were found to have an equivalent rate of amblyopia compared with children ≥3 of age. Those screened between ages 0-2 years of age attained normal vision at a significantly younger age and were more likely to attain normal vision.

Optical Coherence Tomography-Assisted Limbal Dermoid Removal.

This report and accompanying video show the novel use of intraoperative optical coherence tomography (OCT) in the excision of a limbal dermoid. Examining for residual opaque tissue in the cornea accompanied by OCT imaging is a useful technique to confirm complete excision of the lesion. [J Pediatr Ophthalmol Strabismus. 2017;54:e58-e59.].

Can screening for optic nerve gliomas in patients with neurofibromatosis type I be performed with visual-evoked potential testing?

BACKGROUND: Screening for optic nerve gliomas (ONGs) in children with neurofibromatosis type 1 (NF1) is problematic. Visual acuity (VA) can be unreliable in children. Magnetic resonance imaging is the most sensitive test for ONG, but it is expensive. This study was designed to determine whether visual-evoked potential testing (VEP) is a sensitive and cost-effective screening test for ONG in NF1 in children. METHODS: We undertook a retrospective review of patients with NF1 at a tertiary care eye center that were born between 1983 and 2003. VA was considered abnormal if 20/40 or worse or more than 2 lines difference between eyes. VEP was abnormal if the P100 was >108 ms (ms) or the interocular difference was greater than 5.0 ms. RESULTS: Of 297 patients found with NF1, 144 were children and 30 had VEP and MRI. Of those, 14 had ONG and 16 did not. The average P100 of the VEP was 110.5 ms in patients with ONG compared with 103.1 ms (p = 0.004) in those without ONG. VEP was 86% sensitive and 75% specific in detecting ONG. VA was 50% sensitive and 50% specific. Six patients with ONG had normal vision and abnormal VEP. Two subjects had initial abnormal VEP but normal MRI and showed ONG on follow-up imaging. One subject with ONG had a normal VEP initially but subsequent VEP was abnormal. CONCLUSION: Using serial VEPs, the sensitivity is 93%. Cost of VEP and MRI is $150 and $1750, respectively. VEP is a sensitive and cost-effective screening test for ONG in NF1. VEP may assist in earlier diagnoses of ONG, especially in children with equivocal or difficult ophthalmic examinations.

Description and validation of a structured simulation curriculum for strabismus surgery.

PURPOSE: To describe and validate a Web-based structured simulation curriculum of strabismus surgery for residents in training using noncadaveric eye models. METHODS: A pre- and posttest of cognitive skills, objective wet laboratory structured assessment of technique, and summative global evaluation form were implemented as part of a systematic ophthalmology wet lab (OWL) curriculum. Strabismus techniques were taught using a structured simulation method on noncadaveric models of eyes. Likert scale questionnaires were administered to assess the comfort level with strabismus surgery of residents before and after participation in the wet laboratory. Statistical analysis was performed using the paired t test. RESULTS: Seven residents participated in the curriculum. Average test scores improved from 65% to 91% (P = 0.0002). Resident comfort level improved from an average score (assessed by Likert scale of 1-5) with standard deviation of 2.6 ± 1.0 to 4.3 ± 0.5 for passing scleral sutures (P = 0.0008), of 2.6 ± 0.5 to 4.3 ± 0.5 for isolating and suturing muscles (P = 0.00004), and of 2.7 ± 1.0 to 4.6 ± 0.5 for comfort with naming instruments (P = 0.0007). CONCLUSIONS: In this study of 7 residents, knowledge and comfort level with strabismus surgery statistically improved with a structured simulation curriculum.