Long-term functional and structural outcomes in patients with primary congenital glaucoma-A Danish nationwide study.

PURPOSE: Evaluation of long-term functional and structural outcomes in patients with primary congenital glaucoma (PCG) based on visual acuity (VA), visual field (VF) using standard automated perimetry, and peripapillary retinal nerve fibre layer thickness (pRNFL). METHODS: We retrospectively reviewed medical records of all patients diagnosed with PCG in Denmark from 1977 to 2016. Severe vision loss was defined as VA <6/60 and/or VF >20 decibels (dB). Prognostic factors were evaluated in a correlation matrix. RESULTS: The median age of the 94 patients (153 PCG eyes) was 12 years (IQR 9-16). In PCG eyes 62% had VA ≥6/18 but 22% had <6/60. VA in the better seeing eye was ≥6/18 in 90% and <6/60 in 5%. VF was measured in 59 PCG eyes and the median mean defect was 5.1 dB (IQR 2.1-9.6) with 52% better than 6 dB and 9% worse than 20 dB. Generalized pRNFL was reduced below the age-expected 1st percentile in 29% of the 58 PCG eyes where pRNFL was measured. Poor VA, poor VF and reduced pRNFL were all correlated (p = 0.0001). More surgeries (p < 0.0001) and longer diagnostic delay (p = 0.004) were associated with poorer vision and to a lesser degree with poor VF pRNFL. CONCLUSION: In Denmark, most patients with bilateral PCG retain VA ≥6/18 in the better seeing eye. Poor VA was associated with poor VF. Longer diagnostic delay and more surgeries were associated with a poorer prognosis.

Long-term success after trabeculotomy in primary congenital glaucoma - a study with up to 35 years follow-up.

PURPOSE: To evaluate the surgical success after trabeculotomy for primary congenital glaucoma (PCG) in Denmark in the last 40 years. METHODS: Retrospective review of medical files on children who all underwent trabeculotomy as the first surgical intervention between January 1rst, 1977 and December 31, 2016. Information on diagnosis and surgical procedures were extracted. Primary outcome was surgical success was defined as intraocular pressure (IOP) < 21 mmHg without medications one year after surgery. Secondary outcome was the number of reoperations needed. RESULTS: Out of 118 children with PCG, 96 children (144 eyes) had an ab externo trabeculotomy as primary IOP lowering procedure opening Schlemm's channel 4 clock hours into the anterior chamber. Complete surgical success at one year was achieved in 73% (106 of 144 eyes). On the long term, IOP could be controlled by one surgical procedure in 2/3 of children, eight eyes required >5 surgical procedures to control IOP. In 12.1% of children the second glaucoma procedure was performed >5 years after the initial trabeculotomy. There was a tendency towards greater risk of needing a second procedure in patients < 3 months of age at primary trabeculotomy (hazard ratio, HR, 2.01, 95% CI 0.96-4.22) and in boys (HR 2.02, 95% CI 0.97-4.18) and a lower risk of requiring a third surgery if the second surgery was trabeculectomy with MMC. CONCLUSION: Dedicated follow-up of patients with PCG is essential as the disease may continue to progress even after years of quiescence and some children need multiple surgeries to control IOP. Additional glaucoma procedures are required in 1/3 of children and boys and younger patients seem to be at greater risk.

Identical Horner Syndrome in Homozygotic Twins Caused by Non-Traumatic Internal Carotid Artery Dissection.

Internal carotid artery dissection is an uncommon entity and the result of a spontaneous or traumatic tear in the vessel lining. We present two almost identical cases of Horner syndrome caused by spontaneous, non-traumatic internal carotid artery dissection within 4 months in two 52-year-old homozygotic twin brothers.

Primary congenital glaucoma in Denmark, 1977-2016.

PURPOSE: To perform an epidemiological survey of all patients in Denmark diagnosed with primary congenital glaucoma (PCG) from 1977 to 2016. METHODS: A retrospective, nationwide study based on a review of medical files of all children with PCG born in Denmark from 1977 to 2016. PCG was defined as glaucoma due to isolated angle dysgenesis affecting children from birth and including late onset/late recognized PCG, excluding glaucoma associated with other congenital abnormalities. Incidence and risk factors of PCG including gender, consanguinity, family history, ethnicity, comorbidity and prematurity were evaluated and stratified by decade (1977-1986, 1987-1996, 1997-2006 and 2007-2016). Age at first symptoms, age at diagnosis, age at time of first surgery and possible diagnostic delay were assessed. RESULTS: Of 118 patients were identified, annual incidence of PCG was 4.8 per 100 000 live born. 62.3% of cases were bilateral. The relative risk (RR) of PCG was significantly higher in boys (62.7% of patients, RR 1.60 (95% CI 1.10-2.32)). 13.6% had comorbidity. Median age at time of first symptoms was 75 days, median age at time of diagnosis was 150 days and median diagnostic delay was 75 days with no significant difference throughout the decades. Unilateral cases presented symptoms and were diagnosed significantly later than bilateral cases. CONCLUSION: We provide unique nation-based information on the incidence of PCG from a single country covering 40 years. Male gender was a risk factor. Diagnostic delay was unchanged throughout the 4 decades and a high percentage of comorbidity was revealed. Introducing a standardized paediatric screening of all PCG children should be considered.

Retinal Vessel Diameter Changes in Relation to Dark Adaptation and Acute Hyperglycemia.

The purpose of this experimental clinical study was to assess the effects of dark adaptation and acute changes in glycemia on retinal vessel diameters in men. The study included 14 patients (mean age 63 years, range 48-74 years) with type 2 diabetes mellitus and minimal or no diabetic retinopathy. Retinal vessel diameters were assessed using infrared photography before and after dark adaptation, first while fasting and then at peak hyperglycemia during an oral glucose tolerance test (OGTT). Dark adaptation was accompanied by retinal vasodilatation, both during fasting (mean glycemia 7.6 ± 1.7 mM) and postprandial hyperglycemia (15.7 ± 4.2 mM). When fasting, the increase in vein diameter during dark adaptation was 2.0% after 20 min (P=0.018) and 2.9% after 40 min (P=0.010). When subjects were hyperglycemic, the increase during dark adaptation was 2.8% for retinal vein diameters (P=0.027) and 2.0% for retinal artery diameters after 20 min (P=0.002) and 1.7% for retinal artery diameters after 40 min (P=0.022). For identical conditions of light/dark adaptation, retinal vessels were dilated when subjects were fasting compared to postprandial hyperglycemia. Thus, darkness and fasting were both associated with retinal vasodilation in this short-term experiment in patients with type 2 diabetes. Future studies should determine whether both the stimuli of vasodilation lead to retinal hyperperfusion, which would support that they may be involved in the aggravation of diabetic retinopathy.

Visual function and retinal vessel diameters during hyperthermia in man.

PURPOSE: To assess the effect of elevated core body temperature on temporal and spatial contrast sensitivity and retinal vessel diameters. METHODS: The study included 13 healthy volunteers aged 20-37 years. Core body temperature elevation (target +1.1°C) was induced by wrapping the participants in cling film, tinfoil and warming blankets. Subsequent cooling was achieved by undressing. Flicker sensitivity (critical flicker fusion frequency) was chosen to assess temporal resolution, while the Freiburg Vision Test was used to determine spatial contrast sensitivity at 1.5 cycles per degree. Scanning laser ophthalmoscopy was used to measure retinal trunk vessel diameters. Assessment was made at baseline, during hyperthermia and after cooling. RESULTS: The induction of a mean increase in core body temperature of 1.02°C was associated with a 7.15-mmHg mean reduction in systolic blood pressure (p < 0.01), a 10.6-mmHg mean reduction in diastolic blood pressure (p < 0.01), a mean increase in pulse rate of 36.3 bpm (p < 0.0001), a 2.66% improvement in flicker sensitivity (CI95 1.37-3.94, p < 0.001), a 2.80% increase in retinal artery diameters (CI95 1.09-4.51, p < 0.01) and a 2.95% increase in retinal vein diameters (CI95 0.96-4.94, p < 0.01). There was no detectable effect of temperature on spatial contrast sensitivity. All ocular test parameters returned to baseline levels after cooling. CONCLUSION: Increased core body temperature was accompanied by improved temporal visual resolution and retinal trunk vessel dilation. The results suggest that hyperthermia is associated with enhanced retinal function and increased retinal metabolism.

Electroretinography in healthy subjects in relation to systemic glucocorticoid intake.

This study examined electroretinographic function in healthy subjects before and after prednisolone intake. To separate the effect of prednisolone on the retina from the potentially confounding hyperglycemia-inducing effect of prednisolone, electroretinography was made while fasting and at a pre-specified level of clamped hyperglycemia. The study included 10 eyes in 10 healthy lean men aged 25 ± 3 years (mean ± SD). The subjects were examined before and after oral intake of prednisolone 37.5 mg/day for 9.1 ± 1.4 days. The diabetogenic potential of prednisolone was reinforced by the intake of a high-caloric diet and by the reduction of physical activity. Full-field electroretinography (ffERG) demonstrated no significant change (P < 0.05) in amplitudes or implicit times in relation to prednisolone intake, neither at fasting glycemia levels, which were 4.9 ± 0.2 mM before and 5.0 ± 0.3 mM (P = 0.467) after the intervention, nor at 10 mM clamped hyperglycemia. Specifically, the fasting b-wave amplitude of the combined rod-cone response was 432 ± 84 mV before and 463 ± 71 mV (P = 0.13) after prednisolone intake. Furthermore, the ffERG could not be shown to be influenced by the doubling of glycemia from fasting to clamped hyperglycemia, neither before, nor after prednisolone (P > 0.05). The stability of ffERG performance in the face of shifting glycemia levels, which differs from what has been found in diabetes, was not influenced by the mild diabetogenic effect of the intervention on insulin resistance (P = 0.011) and post-prandial glycemia (P = 0.023). We conclude that prednisolone had no detectable effect on the ffERG in healthy lean men in this study. Retinal function may be less sensitive to changes in glycemia in healthy subjects than in people with diabetes, a characteristic that was unchanged by a short course of prednisolone.