"Coming up short" with an illuminated microcatheter for circumferential trabeculotomy externo in the buphthalmic infant eye: frequency and options.

An illuminated microcatheter is frequently used to perform trabeculotomy ab externo in infants with primary congenital glaucoma (PCG). Enlarged corneas in these children can make it challenging to traverse 360o of Schlemm's canal (SC) before reaching the passable limit of the microcatheter. Using basic geometry, we predicted that the iTrack microcatheter would fail to cannulate SC completely in eyes with a horizontal corneal diameter (HCD) >14.8 mm. A retrospective chart review of patients with PCG undergoing angle surgery identified the frequency of corneas with HCD >14.8 mm and confirmed that this calculated cut-off predicts which eyes can-or cannot-be cannulated successfully (ie, 360o) by the iTrack microcatheter.

Intraoperative Mounted Optical Coherence Tomography Findings Following Reversal of Optic Nerve Head Cupping in Childhood Glaucoma.

PURPOSE: To examine the structural changes occurring in the optic nerve head (ONH) and macula in infants with childhood glaucoma and clinically observed ONH cupping reversal following intraocular pressure (IOP)-reducing glaucoma surgery, as captured by intra-operative spectral-domain optical coherence tomography (SD-OCT). DESIGN: Retrospective observational case series from an ongoing prospective cohort study. METHODS: Included were 18 eyes of 14 patients with childhood glaucoma. All eyes had SD-OCT imaging pre- and post-glaucoma intervention and clinically identified ONH cupping reversal. Patients with poor quality images or persistent optic nerve swelling following IOP reduction were excluded. Outcome measurements included IOP, cup-to-disc ratio, axial length and SD-OCT measurements of the peripapillary retinal nerve fiber layer (pRNFL), transverse horizontal diameter of Bruch membrane opening (BMO-D), cup depth, and macula. RESULTS: Mean age at surgery was 1.14±0.93 years and mean interval between pre- and post-operative imaging was 127 days (range 35-595). Following intervention, mean IOP reduction was 45%, accompanied by significant reductions in the cup-to-disc ratio (0.30±0.12, p<0.001), axial length (0.43±0.28mm, p<0.001) and cup depth (46%, p<0.001). Mean global pRNFL thickness pre- vs. post-treatment was 93.1±14.7µm vs. 93.1±17.1µm, respectively, p=1.0. There was no significant difference in pre- and post-treatment global or sectoral pRNFL, 3mm macular total and segmented retinal layer volumes, or the BMO-D. CONCLUSIONS: Clinical ONH cupping reversal after IOP-lowering surgery was associated with axial length reduction and decrease in cup depth, but no significant change in the pRNFL or macular volume measures. ONH cupping reversal likely marks stabilization but any pre-intervention ONH damage persists.

Rebound tonometry by iCare 200 (IC200): comparison with Tono-Pen in the operating room and Goldmann applanation in the clinic.

BACKGROUND: Tonometry guides decision making in children with known or suspected glaucoma. The iCare IC200 is a recently FDA-approved rebound tonometer that can measure intraocular pressure (IOP) in both supine and upright patients. The purpose of this study was to evaluate this new tonometer against more commonly used standard instruments: Tono-Pen in the operating room (OR arm) and Goldmann applanation in the clinic (clinic arm). METHODS: The OR arm included sequential children undergoing general anesthesia. The clinic arm included cooperative children seen in clinic. IOP was measured twice (right eye then left eye, repeat) with IC200 and either Tono-Pen (OR arm) or Goldmann (clinic arm). RESULTS: A total of 99 children were included: 75 for the OR arm and 24 for the clinic arm. There was a strong correlation between the IOP measured by IC200 and each device (R2 = 0.81 for Tono-Pen; R2 = 0.78 for Goldmann). In the OR arm, the overall mean IOP difference (ΔIOP [Tono-Pen - IC200]) was -1.6 mm Hg. For eyes with corneal edema (n = 12), ΔIOP ranged from -8 to 15 mm Hg. At IOP >20 mm Hg, mean ΔIOP was -3.7 mm Hg. In the clinic arm, the mean ΔIOP (ΔIOP [Goldmann - IC200]) was -1.2 mm Hg. CONCLUSIONS: In eyes of children under general anesthesia with IOP <20 mm Hg without corneal edema, IC200 and Tono-Pen correlated well. In cooperative clinic patients, Goldmann and IC200 correlated well over the range of IOP studied.

Outcomes and lessons learned from two decades' experience with glaucoma drainage device implantation for refractory Sturge Weber-associated childhood glaucoma.

PURPOSE: To evaluate glaucoma drainage device (GDD) implantation for refractory pediatric glaucoma associated with Sturge-Weber syndrome (SWS). METHODS: The medical records of consecutive children with SWS-associated glaucoma at a single center who were treated by a single surgeon using GDDs over 20 years were reviewed retrospectively. The main outcome measure was GDD treatment success, defined as absence of any of the following indications of treatment failure: (1) intraocular pressure (IOP) of >21 mm Hg on two consecutive visits despite maximal medical therapy, (2) additional IOP-lowering surgery, and (3) sight-threatening complications. RESULTS: A total of 22 eyes of 22 children were included. The median age at glaucoma diagnosis was 0.73 years (range, 0.06-13.2), and the median age at GDD surgery was 4.8 years (range, 0.6-13.3). Most eyes (14 [68%]) had prior glaucoma surgery. Mean follow-up was 2.8 ± 1.5 years. Success (95% confidence interval) by Kaplan-Meier analysis for GDD surgery at 1, 3, and 5 years was 91% (68-98), 75% (50-89), and 52% (24-73), respectively. Failure occurred in 8 eyes (36%). Complications were common, occurring in 50% of eyes, with 23% of eyes having more than one complication. Severe vision-threatening complications (n = 3) included one case each of possible infection requiring GDD removal, persistent hypotony, and cilioretinal artery occlusion. CONCLUSIONS: GDDs are an effective treatment for SWS-associated glaucoma but have a high rate of complications. We report several severe complications that prompted surgical modifications for increased safety when implanting GDDs in eyes with SWS-associated glaucoma.

Endoscopic cyclophotocoagulation (ECP) for childhood glaucoma: a large single-center cohort experience.

PURPOSE: To assess the factors associated with successful outcomes in the management of childhood glaucoma treated with endoscopic cyclophotocoagulation (ECP) as both primary and adjunctive surgery. METHODS: The medical records of consecutive children with glaucoma treated by a single surgeon at a single center over a 17-year period using ECP procedures were reviewed retrospectively. Treatment failure was defined as (1) intraocular pressure (IOP) >24 mm Hg at two consecutive examinations despite maximal medical treatment, (2) any additional glaucoma surgery, (3) sight-threatening complications, or (4) progression to no light perception visual acuity. Success was defined as the absence of treatment failure. RESULTS: A total of 107 ECP procedures on 80 eyes of 70 children were included. Glaucoma diagnoses included: following-cataract-surgery (60%), anterior segment dysgenesis (13%), primary congenital (9%), and other (19%). Most eyes (67 [84%]) had prior glaucoma surgery, and 73 (91%) were aphakic or pseudophakic at first ECP. Median follow-up was 2.2 years (IQR, 1.1-3.5) after initial ECP; mean number of ECP treatments per eye was 1.3 (range, 1-3). Success for a single ECP treatment at 1, 3, and 5 years (Kaplan-Meier analysis) was 64% (95% CI, 54-76), 36% (26-50), and 16% (7-37), respectively. Cumulative success (≥1 ECP) at 5 years was 34% (23-50). In multivariable analysis, of many risk factors considered, only a preoperative IOP of <32 mm Hg was significantly associated with treatment success. CONCLUSIONS: ECP represents a modestly effective long-term therapy for childhood glaucoma and may be most successful in patients with preoperative IOP of <32 mm Hg.

The Association of Dietary Lutein plus Zeaxanthin and B Vitamins with Cataracts in the Age-Related Eye Disease Study: AREDS Report No. 37.

PURPOSE: To evaluate whether dietary intake of luteiin/zeaxanthin and B vitamins is associated with cataract prevalence and incidence. DESIGN: Clinic-based, baseline cross-sectional and prospective cohort study designs. PARTICIPANTS: Three thousand one hundred fifteen patients (6129 eyes) enrolled in the Age-Related Eye Disease Study 55 to 80 years of age followed up for mean of 9.6 years. METHODS: Participants completed baseline food frequency questionnaires. Baseline and annual lens photographs were graded centrally. Multivariate models controlling for previously identified risk factors for cataracts tested for the association of cataracts with reported dietary intake, using the lowest quintile as reference. MAIN OUTCOME MEASURES: Cataract surgery, cataract status (type and severity) at baseline, and development of cataracts. RESULTS: At baseline, increased dietary riboflavin and B12 were associated inversely with nuclear and cortical lens opacities. In comparisons of persons with and without cataract, persons with the highest riboflavin intake versus those with the lowest intake had the following associations: mild nuclear cataract: odds ratio (OR), 0.78; 95% confidence interval (CI), 0.63-0.97; moderate nuclear cataract: OR, 0.62; 95% CI, 0.43-0.90; and mild cortical cataract: OR, 0.80; 95% CI, 0.65-0.99. For B12, the results were: mild nuclear cataract: OR, 0.78; 95% CI, 0.63-0.96; moderate nuclear cataract: OR, 0.62; 95% CI, 0.43-0.88; and mild cortical cataract: OR, 0.77; 95% CI, 0.63-0.95. Highest dietary B6 intake was associated with a decreased risk of moderate nuclear lens opacity developing compared with the lowest quintile (OR, 0.67; 95% CI, 0.45-0.99). Highest dietary intake levels of niacin and B12 were associated with a decreased risk of development of mild nuclear or mild cortical cataracts in participants not taking Centrum (Pfizer, New York, NY) multivitamins. For participants taking multivitamins during the study, the highest intake of dietary folate was associated with an increased risk of mild posterior subcapsular lens opacity development. No statistically significant associations were found between lutein plus zeaxanthin intake and presence at baseline or development of nuclear or cortical lens opacity outcomes. CONCLUSIONS: These findings are consistent with earlier studies suggesting that dietary intake of B vitamins may affect the occurrence of age-related lens opacities. Further investigations are warranted.

Inhaled amikacin for treatment of refractory pulmonary nontuberculous mycobacterial disease.

RATIONALE: Treatment of pulmonary nontuberculous mycobacteria, especially Mycobacterium abscessus, requires prolonged, multidrug regimens with high toxicity and suboptimal efficacy. Options for refractory disease are limited. OBJECTIVES: We reviewed the efficacy and toxicity of inhaled amikacin in patients with treatment-refractory nontuberculous mycobacterial lung disease. METHODS: Records were queried to identify patients who had inhaled amikacin added to failing regimens. Lower airway microbiology, symptoms, and computed tomography scan changes were assessed together with reported toxicity. MEASUREMENTS AND MAIN RESULTS: The majority (80%) of the 20 patients who met entry criteria were women; all had bronchiectasis, two had cystic fibrosis and one had primary ciliary dyskinesia. At initiation of inhaled amikacin, 15 were culture positive for M. abscessus and 5 for Mycobacterium avium complex and had received a median (range) of 60 (6, 190) months of mycobacterial treatment. Patients were followed for a median of 19 (1, 50) months. Eight (40%) patients had at least one negative culture and 5 (25%) had persistently negative cultures. A decrease in smear quantity was noted in 9 of 20 (45%) and in mycobacterial culture growth for 10 of 19 (53%). Symptom scores improved in nine (45%), were unchanged in seven (35%), and worsened in four (20%). Improvement on computed tomography scans was noted in 6 (30%), unchanged in 3 (15%), and worsened in 11 (55%). Seven (35%) stopped amikacin due to: ototoxicity in two (10%), hemoptysis in two (10%), and nephrotoxicity, persistent dysphonia, and vertigo in one each. CONCLUSIONS: In some patients with treatment-refractory pulmonary nontuberculous mycobacterial disease, the addition of inhaled amikacin was associated with microbiologic and/or symptomatic improvement; however, toxicity was common. Prospective evaluation of inhaled amikacin for mycobacterial disease is warranted.

Lipodermoid in a patient with Emanuel syndrome.

We report an 8-month-old boy with Emanuel syndrome who also had the clinical features of Goldenhar syndrome. At birth, he was observed to have bilateral microtia with multiple auricular pits, retrognathia, and a unilateral lipodermoid. Further testing revealed cardiac defects. The finding of a lipodermoid in Emanuel syndrome demonstrates phenotypic overlap between Goldenhar and Emanuel syndromes and suggests a role for genetic analysis in all patients with clinical features that include ear anomalies and lipodermoids. Correct identification of patients with Emanuel syndrome is important for determining whether there is risk of long-term neurodevelopmental disability, and genetic testing can determine parental carrier status to aid in family planning.

Prior elicitation and Bayesian analysis of the Steroids for Corneal Ulcers Trial.

PURPOSE: To elicit expert opinion on the use of adjunctive corticosteroid therapy in bacterial corneal ulcers. To perform a Bayesian analysis of the Steroids for Corneal Ulcers Trial (SCUT), using expert opinion as a prior probability. METHODS: The SCUT was a placebo-controlled trial assessing visual outcomes in patients receiving topical corticosteroids or placebo as adjunctive therapy for bacterial keratitis. Questionnaires were conducted at scientific meetings in India and North America to gauge expert consensus on the perceived benefit of corticosteroids as adjunct treatment. Bayesian analysis, using the questionnaire data as a prior probability and the primary outcome of SCUT as a likelihood, was performed. For comparison, an additional Bayesian analysis was performed using the results of the SCUT pilot study as a prior distribution. RESULTS: Indian respondents believed there to be a 1.21 Snellen line improvement, and North American respondents believed there to be a 1.24 line improvement with corticosteroid therapy. The SCUT primary outcome found a non-significant 0.09 Snellen line benefit with corticosteroid treatment. The results of the Bayesian analysis estimated a slightly greater benefit than did the SCUT primary analysis (0.19 lines verses 0.09 lines). CONCLUSION: Indian and North American experts had similar expectations on the effectiveness of corticosteroids in bacterial corneal ulcers; that corticosteroids would markedly improve visual outcomes. Bayesian analysis produced results very similar to those produced by the SCUT primary analysis. The similarity in result is likely due to the large sample size of SCUT and helps validate the results of SCUT.