Professional Quality of Life and Associated Factors Among Pediatric Glaucoma Providers.

PURPOSE: Even though physician burnout can negatively impact physical/mental health, ophthalmologists' quality of life (QOL) is understudied. Although managing high-risk diseases like pediatric glaucoma may lead to compassion satisfaction (CS), the responsibility of caring for very sick, demanding patients has been linked to higher burnout. Burnout and secondary traumatic stress (STS) constitute compassion fatigue, the negative effects resulting from helping those who experience stress. We assessed professional QOL, including CS, burnout, and STS, along with associated factors among pediatric glaucoma providers managing potentially blinding disease in children. DESIGN: Cross-sectional survey study. PARTICIPANTS: Pediatric glaucoma providers in the Childhood Glaucoma Research Network, American Glaucoma Society, and Indian Paediatric Glaucoma Society. METHODS: Participants were sent an electronic survey of a validated instrument (Professional Quality of Life-Version 5) designed to measure burnout, STS, and CS, as well as questions related to demographics, lifestyle, training, and practice. Burnout, STS, and CS scores range from 10 to 50 and are classified into low (≤ 22), moderate (23-41), and high (≥ 42) subgroups. MAIN OUTCOME MEASURES: Burnout, STS, and CS. RESULTS: Seventy-six pediatric glaucoma providers completed the survey with resulting burnout (22.6 ± 6.3), STS (22.7 ± 6.1), and CS (42.3 ± 6.7) scores. Most respondents had low burnout (43, 56.6%), low STS (44, 57.9%) and high CS (48, 63.2%), though more than a third reported moderate burnout (33, 43.4%), STS (31, 40.8%), and CS (27, 35.6%). Older age and more years in practice correlated positively with CS, and age correlated negatively with STS (P < 0.05 for all). Frequent workday fatigue correlated positively with burnout and STS and negatively with CS, and frequent caffeine consumption correlated positively with burnout and negatively with CS (P < 0.05 for all). Members of a married or unmarried couple had significantly lower CS scores than single, divorced, or separated respondents (P = 0.022). CONCLUSIONS: Pediatric glaucoma providers derive a high level of professional satisfaction from their work, though many report moderate burnout and STS. To comprehensively address provider QOL, CS, and both components of compassion fatigue must be considered. Initial efforts may be focused on younger, early-career providers as this group had lower professional QOL scores than their older, late-career counterparts. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Factors Associated With Gonioscopy-Assisted Transluminal Trabeculotomy (GATT) Complications and Failure in Children.

PURPOSE: To identify the factors associated with the complications and failure of gonioscopy-assisted transluminal trabeculotomy (GATT) in children. DESIGN: Retrospective case series. METHODS: This study was conducted in an institutional setting in a pediatric population (aged <18 years) who had undergone GATT. Records were reviewed, and pre- and postoperative intraocular pressures (IOP), extent of angle treated, medications, complications, and failure were recorded. Failure was defined as IOP >21 mm Hg or <5 mm Hg, absence of at least 20% IOP reduction, performance of additional IOP-lowering surgery, or loss of light perception vision. RESULTS: A total of 74 eyes of 57 patients were included (mean age, 7.1 years). Over a median follow-up period of 28.5 months, 36 eyes (48.6%) failed. IOP spikes occurred in 25 eyes (33.8%) and were a significant risk factor for failure (hazard ratio [HR] = 2.17; P = .0207). Postoperative hyphema was a significant risk factor for IOP spike (HR = 4.13, P = .003) but not for failure (HR = 0.7, P = .2977). The risk of IOP spike was lowest in eyes treated with nonsteroidal anti-inflammatory drugs (NSAIDs; HR = 0.27, P = .0016). The risk of failure increased significantly in eyes that received topical corticosteroids (compared to NSAIDs; HR = 5.72, P = .0005), in eyes with <360○ incisions (HR = 4.69, P < .0001), and in younger children. CONCLUSIONS: GATT is a reasonably effective procedure in childhood glaucoma. Postoperatively, the use of topical NSAIDs (without corticosteroid) may decrease the risk of failure. Eyes with IOP spikes without hyphema are at the highest risk for failure.

The Effect of Changing Fellowship Interview Format on Candidate Ranking Variabilities: The COVID-19 Experience.

Purpose: This study investigated the effect of interview format changes (in-person to virtual, one-to-one to multiple-to-one) necessitated by the COVID-19 travel restrictions on preliminary fellowship candidate ranking variabilities. Design: Cross-sectional observational study. Method: In 2018 and 2019, the glaucoma fellowship interviews were conducted in-person in a one-to-one format, whereas in 2020, interviews were virtual and in a multiple (interviewers)-to-one (candidate) format. We compared ranking ranges of interviewers within the same virtual room (WSR) and not within the same virtual room (NWSR) to assess the effect of WSR versus NWSR on ranking variabilities. We also compared ranking categories ("accept," "alternate," and "pass") agreements between 2018, 2019, and 2020 to assess the effect of virtual versus in-person interviews on ranking variabilities. Results: NWSR and WSR mean rankings differed by 1.33 (95% confidence interval difference 0.61 to 2.04, p = 0.0003), with WSR interviewers having less variability than NWSR pairs. The variability between 2018/2019 (in-person interviews) and 2020 (virtual interviews) showed no differences between in-person and virtual interviews (weighted Kappa statistic 0.086 for 2018, 0.158 for 2019, and 0.101 for 2020; p < 0.05 for all years). The overall least attractive candidate has the lowest variability; the most attractive candidate has the second lowest variability. Conclusion: Grouping interviewers WSR during the interview decreased ranking variabilities compared to NWSR, while a change from in-person to virtual interview format did not increase the ranking variabilities. This suggests that the decreased nonverbal interactions in virtual interviews do not decrease interviewers' perceptions as applied to preliminary rankings.

Glaucoma Cascade Screening in a High Risk Afro-Caribbean Haitian Population: A Pilot Study.

PRCIS: Glaucoma cascade screening in first-degree relatives (FDRs) of young Haitian glaucoma patients had high yield for diagnosing manifest and suspected glaucoma in 30.8% of those screened despite modest participation. PURPOSE: To evaluate the outcomes of glaucoma cascade screening in FDRs (parents, siblings, and offspring) of Haitian juvenile open-angle glaucoma (JOAG) patients. PATIENTS AND METHODS: Consecutive index patients (Haitians with JOAG) were identified, and the number/type of FDRs residing in South Florida were recorded. These FDRs were invited for free glaucoma screening, which included a comprehensive ophthalmic exam, gonioscopy, automated visual field testing and optical coherence tomographic analysis of the retinal nerve fiber layers. FDR characteristics and clinical findings from screening are reported. RESULTS: A total of 77 FDRs were invited, 26 (33.8%) agreed to undergo screening (18 females, 9 males), which revealed 2 (7.7%) with manifest glaucoma (mean age 77.5 y; one of whom was previously unaware of his glaucoma diagnosis), 6 (23.1%) with suspected glaucoma (mean age 29.8±18.3 y), and 18 (69.2%) without manifest or suspected glaucoma (mean age 37.2±21.8 y). Siblings of index patients were least likely to participate in cascade glaucoma screening when compared with index patients' parents or offspring. FDR eyes with manifest glaucoma had significantly worse best-corrected visual acuities, higher intraocular pressures, thinner central corneal thicknesses, and thinner circumferential papillary retinal nerve fiber layer thicknesses than those without glaucoma. CONCLUSION: Glaucoma cascade screening of Haitian JOAG patients' FDRs revealed that 30.8% had suspected or manifest glaucoma. Future efforts centered on provider-initiated recruitment and improving public glaucoma awareness and education may increase screening participation.

Virtual Versus In-Person Surgical Fellowship Interviews and Ranking Variability: the COVID-19 Experience.

Objective: To investigate the effect of interview format changes (in-person to virtual, one-to-one to multiple-to-one) necessitated by the COVID-19 travel restrictions on candidate ranking variabilities. Method: In 2018/2019, the glaucoma fellowship interviews were conducted in-person and one-to-one, whereas in 2020, interviews were virtual and multiple (interviewers)-to-one (candidate). We compared ranking ranges of interviewers within the same virtual room (WSR) and not within the same virtual room (NWSR) to assess the effect of this change on ranking variabilities. We also compared ranking categories ("accept," "alternate," and "pass") agreements between in-person and virtual interviews to assess the effect of this change on ranking variabilities. Results: NWSR and WSR mean rankings differed by 1.33 (95% confidence interval difference 0.61 to 2.04, p = 0.0003), with WSR interviewers having less variability than NWSR pairs. The variability of in-person interviews and later virtual interviews showed no differences (weighted Kappa statistic 0.086 for 2018, 0.158 for 2019, and 0.101 for 2020; p < 0.05 for all years). The overall least attractive candidate has the lowest variability; the most attractive candidate has the second lowest variability. Conclusion: Grouping interviewers decreased ranking variabilities, while a change from in-person to virtual interview format did not increase the ranking variabilities.

Non-invasive Assessment of Central Retinal Artery Pressure: Age and Posture-dependent Changes.

Purpose: Assessment of Ocular Perfusion Pressure (OPP) requires estimation of the Mean Central Retinal Artery Pressure (MCRAP) [OPP = MCRAP-IOP]. In a seated position, MCRAP is currently estimated as 2/3 of the Mean Arterial Pressure (MAP) to account for the hydrostatic reduction of MAP at eye level. We tested a surrogate method for direct MCRAP assessment by measuring MAP with Arm-Up and cuff at eye level (AUMAP) at different postures and ages. Methods: MAP and AUMAP were assessed in a mixed population of 136 subjects (mean age 44 ± 17.39 years) including healthy participants (N = 30) and patients with optic neuropathies (Glaucoma suspects, N = 14; Open-Angle Glaucoma, N = 26, LHON, N = 19; MS, N = 47) not expected to alter systemic blood pressure. None of the subjects had history of carotid stenosis or pharmacological treatment to regulate blood pressure. AUMAP was also tested in two subgroups in supine (N = 42) and -10° Head Down body Tilt position (HDT, N = 46). Results: In the seated position, both 2/3MAP and AUMAP increased with increasing age, however with steeper (2x) slope for AUMAP (P < .0001). With decreasing angle of body tilt, AUMAP increased while MAP decreased. The mean AUMAP/MAP ratio (posture coefficient) was, seated, 0.73 (SE 0.003); supine, 0.90 (SE 0.005); HDT, 0.97 (SE 0.005). In the seated position only, the AUMAP/MAP ratio significantly increased with age (P < .0001). Mean posture coefficients obtained with AUMAP were in the range of those based on either direct ophthalmodynamometric measurements or hydrostatic estimations. Conclusions: Surrogate measurement of MCRAP in individual subjects is feasible using the simple AUMAP approach that provides a straightforward estimation of OPP (OPP = AUMAP - IOP) at different body postures. The standard method OPP = 2/3*MAP-IOP in the seated posture underestimates OPP at older ages. Clinical estimation of OPP would benefit from the use of AUMAP, in particular for head-down postures.

Risk Factors for Complications and Failure after Gonioscopy-Assisted Transluminal Trabeculotomy in a Young Cohort.

PURPOSE: To determine the risk factors for failures and complications of gonioscopy-assisted transluminal trabeculotomy (GATT) in a young cohort. DESIGN: Retrospective case series. PARTICIPANTS: Eyes that underwent GATT at Bascom Palmer Eye Institute. METHODS: Preoperative and postoperative intraocular pressure (IOP), extent of angle treated, postoperative IOP spike, postoperative drop regimen, and postoperative failure were recorded. Failure was defined as (1) IOP of more than 21 mmHg or less than 5 mmHg after postoperative month 3, (2) absence of at least 20% reduction from the preoperative IOP baseline after postoperative month 3, or (3) performance of additional IOP-lowering surgery before postoperative month 3. Intraocular pressure spike complication was defined as an increase in IOP in the operated eye at any postoperative visit to higher than preoperative IOP within the first 3 months. Kaplan-Meier survival analysis and Cox proportional hazards regression were used to assess the time to events (failure and IOP spike). MAIN OUTCOME MEASURES: Risk of failure associated with any of the preoperative, intraoperative, or postoperative variables. RESULTS: A total of 102 eyes of 88 patients were included. The median follow-up time was 7.4 months, with an interquartile range of 3.3 to 13.1 months (standard deviation, 8.7 months). Patients who used corticosteroids after surgery were more likely to experience IOP spikes than those using nonsteroidal anti-inflammatory drugs (NSAIDs) alone (hazard ratio, 3.34; P = 0.042), and patients who underwent noncircumferential trabeculotomy were 2.56 times more likely to experience treatment failure (P = 0.002) compared with those who underwent circumferential surgery. CONCLUSIONS: Eyes that received postoperative corticosteroids were more likely to experience an IOP spike complication than those that received postoperative NSAIDs alone, which may suggest corticosteroid-mediated outflow obstruction distal to the trabecular meshwork. Achieving a circumferential trabeculotomy and using only NSAIDs may be more likely to result in surgical success when compared with noncircumferential trabeculotomy or using postoperative corticosteroids.

Microcornea and Thickened Lens in Angle Closure following Nonsurgical Treatment of Retinopathy of Prematurity.

PURPOSE: To characterize the clinical features in young patients with angle closure and to determine the characteristics associated with acquired anterior segment abnormality following retinopathy of prematurity (ROP) treatment. METHODS: We performed two retrospective case-control series. In the first series, we identified consecutive young angle closure patients without prior surgeries, with and without a history of ROP treatment; in the second series we identified consecutive patients who underwent ROP treatment, without and without anterior segment changes. RESULTS: In the first series, 25 eyes of 14 consecutive angle closure patients were included: 19 eyes (11 patients, 78.6%) had a history of treated ROP, while 6 eyes (3 patients) belonged to full-term patients. The treated ROP eyes had significantly shallower anterior chambers (1.77 ± 0.17 mm vs 2.72 ± 0.18 mm, P < 0.0001) and thicker lenses (5.20 ± 0.54 mm vs 3.98 ± 0.20 mm, P = 0.0002) compared to the full-term controls. In the second series, 79 eyes of 40 patients were included, with median gestational age of 24.6 weeks. Acquired iridocorneal adhesion was noted in the eight eyes (10.1%) at a mean age of 4.7 years and was associated with prior zone 1 and plus disease (P = 0.0013), a history of initial intravitreal bevacizumab treatment (IVB, P = 0.0477) and a history of requiring additional IVB after initial treatment (P = 0.0337). CONCLUSIONS: Many young angle closure patients may have a history of treated ROP and may present with the triad of increased lens thickness, microcornea, and angle closure.

Spectrum of Genetic Variants Associated with Anterior Segment Dysgenesis in South Florida.

Anterior segment dysgenesis (ASD) comprises a wide spectrum of developmental conditions affecting the cornea, iris, and lens, which may be associated with abnormalities of other organs. To identify disease-causing variants, we performed exome sequencing in 24 South Florida families with ASD. We identified 12 likely causative variants in 10 families (42%), including single nucleotide or small insertion-deletion variants in B3GLCT, BMP4, CYP1B1, FOXC1, FOXE3, GJA1, PXDN, and TP63, and a large copy number variant involving PAX6. Four variants were novel. Each variant was detected only in one family. Likely causative variants were detected in 1 out of 7 black and 9 out of 17 white families. In conclusion, exome sequencing for ASD allows us to identify a wide spectrum of rare DNA variants in South Florida. Further studies will explore missing variants, especially in the black communities.

A Novel Surgical Approach in the Management of Peters Anomaly With Glaucoma.

Treatment options for Peters anomaly vary depending on the degree of corneal and lenticular involvement. The authors report a novel surgical approach for patients with type I Peters anomaly and glaucoma. It involves ab externo circumferential trabeculotomy, simultaneous lysis of iridocorneal adhesions at the time of trabecular cleavage, and optical iridectomy. [J Pediatr Ophthalmol Strabismus. 2020;57:e25-e29.].

Early Predictors of Long-term Outcomes in Childhood Glaucoma.

PURPOSE: A data-based staging system for childhood glaucoma is likely to improve the prediction of visual acuity and intraocular pressure (IOP) control outcomes. We investigated early clinical factors associated with poor long-term visual, and IOP control outcomes in early-onset glaucoma as the initial steps to constructing a severity staging system. DESIGN: Statistical modeling of retrospective case series data. PARTICIPANTS: Glaucoma patients younger than 3 years of age who presented to Bascom Palmer Eye Institute between 1990 and 2010 with at least 5 years of follow up. METHODS: Statistical modeling of retrospective case series data from first, second, third, 3-year, 5-year, and final visits. MAIN OUTCOME MEASURES: Association of early clinical characteristics to final IOP-control outcomes and visual acuities. RESULTS: A total of 26 eyes of 15 children were included. Nine of 15 (60%) of patients were male. Mean age at initial presentation: 9.98±10.55 months. Mean duration between initial and final visits: 11.13±3.55 years. By the third visit (mean 6.69 mo after presentation), presence of nystagmus, anterior segment dysgenesis (ASD) or having failed angle surgery increased the final LogMAR visual acuity by 0.76 (P=0.0516), 0.64 (P=0.0618), and 0.58 (P=0.0159), respectively. At year 3, failed amblyopia therapy, failed angle surgery, nystagmus, media opacity (MO), or ASD increased the final LogMAR by 1.30, 1.34, 1.21, 0.85, 0.64, respectively (all P<0.02). Failed angle surgery or MO increased the chance of uncontrolled IOP at the final visit (proportional odds ratio of 6.77 and 12.88, respectively). CONCLUSIONS: In this pilot study of a modest size cohort, the presence of nystagmus, ASD or failed angle surgery early in the course of infantile-onset glaucoma management predicted poor final visual outcome, whereas failed angle surgery and presence of MO predicted poor final IOP control. These predictors of poor outcomes will serve as the initial steps in constructing a severity staging system.

Retinal pigment epithelium changes in pediatric patients with glaucoma drainage devices.

PURPOSE: Retinal changes secondary to hypotony are usually described as wrinkling or folding of the inner portion of the choroid, the retinal pigment epithelium (RPE), and the outer retinal layers in the macular area due to scleral wall collapse. We describe a new retinal finding in children with suspected hypotony after implantation of Baerveldt Glaucoma Implant (BGI). OBSERVATIONS: Four patients in our series developed significant RPE defects after BGI implant. The RPE defects appeared as elongated white lines observed solely in the posterior pole, in no particular pattern, and seemed to be worse in infants with anterior segment dysgenesis and with collagen disorders. CONCLUSION AND IMPORTANCE: Children have thinner and more elastic scleral walls than adults. This characteristic may cause the inward scleral wall to collapse when the eye is hypotonic. The resulting redundancy of the retina leads to wrinkling and RPE defects characterized by hypopigmented lines predominantly in the macular area. Such findings, to our knowledge, have not been previously reported in pediatric patients.

A retrospective survey of childhood glaucoma prevalence according to Childhood Glaucoma Research Network classification.

PURPOSE: To evaluate the Childhood Glaucoma Research Network (CGRN) classification system and describe the prevalence of each subtype according to this classification. MATERIALS AND METHODS: Retrospectively, the medical records of 205 consecutive childhood glaucoma and glaucoma suspect patients at an urban tertiary care center were reviewed. The initial diagnosis and new diagnosis according to CGRN classification were recorded. RESULTS: All patients fit one of the seven categories of the new classification. Seventy-one percent of diagnoses were changed upon reclassification. Twenty-three percent of patients had primary glaucoma (juvenile open-angle glaucoma and primary congenital glaucoma [PCG]); 36% had secondary glaucoma (glaucoma associated with nonacquired ocular anomalies; glaucoma associated with nonacquired systemic disease or syndrome; glaucoma associated with acquired condition; and glaucoma following cataract surgery); and 39% were glaucoma suspect. Of the patients diagnosed with glaucoma, PCG was the most common diagnosis, seen in 32% of patients. CONCLUSION: The CGRN classification provides a useful method of classifying childhood glaucoma.

Peripheral retinal vasculopathy in childhood glaucoma.

PURPOSE: To report wide-field fluorescein angiography findings in the peripheral retina of eyes with childhood glaucoma. METHODS: Fluorescein angiograms were examined retrospectively in 12 patients with childhood glaucoma who underwent wide-field fluorescein angiography after surgical or medical treatment of glaucoma. This series included 12 eyes of 6 patients with primary congenital glaucoma, 5 eyes of 3 patients with glaucoma associated with congenital cataract surgery, 4 eyes of 2 patients with glaucoma associated with a systemic condition, and 1 eye of 1 patient with phakomatosis pigmentovascularis Type II. RetCam fundus photography images and digital fluorescein angiography were performed under general anesthesia. RESULTS: The most common finding observed in our series was peripheral retinal nonperfusion, which affected 20 eyes (91%). Other notable features seen were circumferential branching of the retinal vessels parallel to the ora serrata (77%), venous shunts (50%), and abnormal capillary branching patterns, including capillary dilatation (41%) and tortuous capillary tangles (18%). Leakage at the junction of vascular and avascular retina was not commonly seen (13.6%). None of the eyes had evidence of retinal neovascularization or fibrovascular proliferation. CONCLUSION: Retinal vascular abnormalities and peripheral nonperfusion are novel findings in childhood glaucoma. Further studies are needed to understand the significance and possible long-term consequences of these findings.

Fluorescein angiography findings in phacomatosis pigmentovascularis.

The authors report the fluorescein angiography findings in a 3-month-old patient with phacomatosis cesioflammea, which revealed venous-venous anastomoses in addition to previously undescribed features of peripheral retinal vascular nonperfusion. The authors encourage physicians to consider phacomatosis pigmentovascularis in the differential diagnosis of patients presenting with facial port-wine stain and to screen these patients for peripheral retinal avascularity in addition to glaucoma and primary uveal melanoma.

Fluorescein angiography findings in a case of Rubinstein-Taybi syndrome.

The purpose of this report is to describe the fluorescein angiography findings in a case of Rubinstein-Taybi syndrome. Fundus photography and fluorescein angiography were performed on a 6-year-old male with Rubinstein-Taybi syndrome due to CREB binding protein gene mutation. Fundus photography showed glaucomatous cupping and diffusely attenuated retinal vasculature. Choroidal vasculature was prominent due to diffuse retinal atrophy with scattered focal retinal pigment epithelial changes. Fluorescein angiography showed retinal vascular attenuation, prolonged arteriovenous transit time with delayed venous filling, late small vessel leakage, and 360 degrees of peripheral avascularity. Peripheral retinal avascularity and retinal vascular inflammation evidenced by late small vessel leakage can be demonstrated by fluorescein angiography in the retinal dystrophy of Rubinstein-Taybi syndrome.

Prenatal ultrasonographic detection of ophthalmic diseases.

Four patients with prenatal sonographic findings suggestive of ophthalmic pathology were detected in utero. The definitive diagnoses of infantile fibrosarcoma, persistent hyperplastic primary vitreous/persistent fetal vasculature, Fraser syndrome, and microphthalmia with coloboma and retrobulbar cyst were made postnatally. High-resolution intrauterine sonograms expedited ophthalmic referral and influenced prenatal planning.