Early and sustained treatment modifies the phenotype of birdshot retinochoroiditis.

In this single-centre retrospective case review, we investigate the long-term follow-up of birdshot retinochoroiditis (BRC) patients, analysing the impact of early, vigorous, and prolonged treatment on the evolution of indocyanine green angiography (ICGA) signs and fundus appearance. Treatment delay was calculated for each BRC patient, and patients were classified into two groups--treatment delay of <10 months (early-treatment group) and treatment delay of >10 months (delayed-treatment group). Fundus photographs and ICGA frames from the initial visit and from the last follow-up visit were assessed. Fundus photographs were evaluated for the presence of at least three circumpapillary, typical, rice-shaped birdshot lesions in one eye, inferior or nasal to the optic disc. ICGA pictures were evaluated for the presence of lesions (hypofluorescent dark dots, fuzziness). Differences were compared between the two groups and between the first visit and the last follow-up visit. In the early-treatment group, 5/6 patients had no characteristic BRC fundus lesions, but 7/7 patients in the delayed-treatment group displayed typical lesions. At last follow-up, 5/6 early-treatment patients showed no fundus lesions, and 6/7 delayed-treatment patients retained their fundus lesions. At presentation, all 13 patients exhibited lesions on ICGA. At last follow-up, ICGA lesions had completely disappeared in 4/6 early-treatment patients and 3/7 delayed-treatment patients. Thus, early and sufficiently dosed inflammation-suppressive treatment can prevent the appearance of typical BRC fundus lesions. It is therefore crucial to perform ICGA to detect otherwise occult stromal choroiditis in suspected BRC cases and to initiate adequate therapy immediately.

Secondary choriocapillaritis in infectious chorioretinitis.

PURPOSE: To analyse the indocyanine green angiography (ICGA) patterns of hypofluorescence that are compatible with choriocapillaritis that occur secondarily to toxoplasmic retinochoroiditis (ToRC), ocular tuberculosis (including tuberculous choroiditis, TuCR and multifocal serpiginoid choroiditis, TMSC) and syphilitic chorioretinitis (SyCR). METHODS: This was a single centre, retrospective case review study. Patients with a diagnosis of ToRC, TuCR, TMSC or SyCR were identified, their charts were reviewed and fundus photographs, fluorescein angiography (FA) and ICGA pictures were assessed. RESULTS: Indocyanine green angiography was performed at the initial presentation in 63 of the 105 patients with ToRC, in 37 of the 38 patients with TuCR, in six of six patients with TMSC and in two of four patients with SyCR. The following four ICGA patterns indicated choriocapillaritis: extension of hypofluorescence beyond the hypofluorescence of the actual infectious focus as seen on fundus photography or FA (seen only in ToRC and TuCR); small dark dots around the infectious focus (seen only in ToRC); multiple 'confetti-like' hypofluorescent areas or hypofluorescent geographical confluent areas (seen only in TMSC); and widespread areas of nonperfusion visible only in ICGA (seen only in SyCR). CONCLUSIONS: Patients with secondary choriocapillaritis have distinct typical ICGA findings. ICGA is thus an important diagnostic tool that can provide an explanation for otherwise obscure visual loss and that might have diagnostic value for specific conditions like ToRC and SyCR.

Indocyanine green angiography-guided management of Vogt-Koyanagi-Harada disease: differentiation between choroidal scars and active lesions.

When following Vogt-Koyanagi-Harada disease (VKH), indocyanine green angiography (ICGA) is crucial in the subacute and convalescent stages of the disease in order to detect subclinical choroiditis and prevent the development of 'sunset glow' fundus. Hypofluorescent dark dots (HDDs) indicate persisting granulomas in the choroid. However, probably as a result of the healing process of choroidal granulomas, stromal choroidal fibrosis can also be shown by HDDs. We present two cases where intravenous corticosteroid administration because of persistent HDDs led to resolution of lesions in one case while they persisted in the other case. We reviewed the medical history of two VKH patients. Complete routine work-up for patients with posterior uveitis was performed. The charts were screened for the presence of HDDs by ICGA in the subacute and convalescent stages of the disease before and after administration of body weight-adapted pulse intravenous methylprednisolone (PIM). The evolution of HDDs was studied and compared in both patients. A female patient presented with a persistent bilateral granulomatous panuveitis compatible with VKH. Cerebrospinal fluid analysis had shown lymphocytic pleocytosis. At presentation, therapy consisted of oral prednisone 80 mg/day. Prednisone was tapered down to 22 mg/day over 3 months, when a recurrence occurred with the presence of disseminated HDDs. PIM was administered, followed by oral corticosteroids. After 8 days of therapy, ICGA showed an almost complete disappearance of HDDs. A girl presented with bilateral panuveitis and widespread depigmented areas of her fundus. Cerebrospinal fluid analysis showed monocytic pleocytosis. Because of relative resistance to oral inflammation suppressive therapy (IST), PIM was administered for 3 days. Nevertheless, ICGA showed persistence of HDDs. Therapy was continued, and 3 months later, a follow-up ICGA still depicted numerous HDDs. Another PIM course was given, which had no effect on ICGA signs. HDDs in this case were interpreted as stromal choroidal scars. ICGA-guided therapy (mainly HDD evolution monitoring) helps to eradicate occult stromal disease in VKH and avoids 'sunset glow' fundus, by allowing precise adjustment of therapy. In some cases, HDDs do not represent active lesions but presumed intrastromal scars which need to be identified. A limited course of maximal IST including PIM can unmask such cases and avoid overtreatment of these patients.

Inter-observer agreement for spectral- and time-domain optical coherence tomography image grading: a prospective study.

The purpose of this study was to compare inter-observer agreement of Stratus™ OCT versus Spectralis™ OCT image grading in patients with neovascular age-related macular degeneration (AMD). Thirty eyes with neovascular AMD were examined with Stratus™ OCT and Spectralis™ OCT. Four different scan protocols were used for imaging. Three observers graded the images for the presence of various pathologies. Inter-observer agreement between OCT models was assessed by calculating intra-class correlation coefficients (ICC). In Stratus™ OCT highest interobserver agreement was found for subretinal fluid (ICC: 0.79), and in Spectralis™ OCT for intraretinal cysts (IRC) (ICC: 0.93). Spectralis™ OCT showed superior interobserver agreement for IRC and epiretinal membranes (ERM) (ICC(Stratus™): for IRC 0.61; for ERM 0.56; ICC(Spectralis™): for IRC 0.93; for ERM 0.84). Increased image resolution of Spectralis™ OCT did improve the inter-observer agreement for grading intraretinal cysts and epiretinal membranes but not for other retinal changes.

Granulomatous keratic precipitates in birdshot retinochoroiditis.

With the purpose of facilitating clinical studies of this infrequent disease, an expert panel published research criteria for birdshot retinochoroiditis (RCBRC). The aim of our study was to investigate the sensitivity of the exclusion criteria of the RCBRC as applied to all patients seen in our center with a diagnosis of BRC. This was a single center retrospective study involving all patients with an ocular inflammatory disease seen at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland, between 1995 and 2012. The percentage of patients with a diagnosis of BRC was identified. The exclusion criteria of the RCBRC were applied to all patients and the percentage of patients with absence of keratic precipitates (KPs) and absence of posterior synechiae were calculated. Out of 1,504 new patients, 25 patients (1.66 %; 19 female, 6 male) were diagnosed with BRC and 19 patients had sufficient data to be included in the study (1.26 %, 13 female). All patients were positive for HLA-A29 testing. The sensitivity of the RCBRC with respect to the exclusion criteria applied to our patient cohort was 84.2 % due to the exclusion of three patients showing KPs. Our study supports the motion to re-evaluate the RCBRC, since granulomatous KPs can be present and are more frequent than previously estimated and stringent application of the criteria would lead to a substantial loss of study patients.

The ocular pulse amplitude as a noninvasive parameter for carotid artery stenosis screening: a test accuracy study.

PURPOSE: To investigate a potential correlation between the ocular pulse amplitude (OPA; i.e., the intraocular pressure difference between the systolic and diastolic phases of the heartbeat) and the severity of carotid artery stenosis (CAS) and to test its role as a screening parameter for CAS during routine ophthalmic examination. DESIGN: Test accuracy study. PARTICIPANTS: Patients referred for color duplex ultrasound examination of the extra- and intracranial cerebral arteries were enrolled consecutively. METHODS: We measured OPA on both eyes by dynamic contour tonometry. Multivariate analyses were performed with risk factors for CAS (age, total cholesterol, low-density lipoprotein, and triglycerides) to compare the diagnostic value of OPA measurements with other non- or minimally invasive screening parameters. MAIN OUTCOME MEASURES: The difference between OPA measurements in patients with no (<50%) and patients with severe CAS (>70%) as well as the value of OPA measurements to predict the severity of CAS taking further risk factors of CAS into consideration. RESULTS: One hundred thirty-four eyes of 67 patients (25 women, 42 men) with a mean age of 67±13 years (range, 25-87) were included. The means of the OPA values of those patients showing no CAS (<50%) differed significantly (P = 0.036) from those with a stenosis of ≥70%. The multivariate model produced a statistically significant odds ratio (0.46; P = 0.007) for CAS of ≥70%. CONCLUSIONS: The results of the present study provide proof of principle that the OPA is reduced in patients with CAS and may be used as a noninvasive, inexpensive, readily available, and unconfounded screening parameter to detect CAS and possibly to reduce the incidence of stroke. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any of the materials discussed in this article.

Repeatability of nerve fiber layer thickness measurements in patients with glaucoma and without glaucoma using spectral-domain and time-domain OCT.

BACKGROUND: The aim of this work is to assess the repeatability of spectral-domain-OCT (SD-OCT) retinal nerve fiber layer thickness (RNFL) thickness measurements in a non-glaucoma group and patients with glaucoma and to compare these results to conventional time-domain-OCT (TD-OCT). METHODS: In a prospective, comparative, observational case-control study, 50 eyes of 25 non-glaucoma and 22 eyes of 11 patients with primary open angle glaucoma (POAG) were included. SD-OCT and TD-OCT circle scans were centered on the optic disc. In each eye, OCT scans were performed three times by two independent observers. RNFL thickness was measured in four quadrants around the optic disc. In addition, the overall mean RNFL thickness was assessed. Intraclass correlation coefficients (ICC) and coefficients of variation (COV) were calculated. Inter-observer and inter-OCT repeatability was visualized by using Bland-Altman analysis. RESULTS: Intra-observer repeatability for TD- OCT was good with an ICC(mean RNFL thickness) of 0.939 in non-glaucomas and 0.980 in glaucomatous eyes. For SD-OCT, intra-observer repeatability was higher with an ICC of 0.989 for non-glaucomas and 0.997 for glaucomatous eyes. COVs for TD-OCT ranged from 2.9-7.7% in non-glaucomas and from 6.0-13.3% in glaucoma patients. COVs for SD-OCT ranged from 0.3-1% in non-glaucomas and from 0.9-2.3% in glaucomatous eyes. COVs were influenced by various factors. In the glaucoma group, COVs were significantly higher (p < 0.001) compared to the non-glaucoma group. COVs increased by a mean of 5.1% when TD-OCT was used instead of SD-OCT (p < 0.001). CONCLUSIONS: SD-OCT RNFL thickness measurements in healthy volunteers and glaucoma patients showed good intra- and inter-observer repeatability. Especially in glaucomatous eyes, repeatability of SD-OCT was superior to TD-OCT.

Long-term follow-up of children with acute acquired concomitant esotropia.

PURPOSE: To study the clinical features and surgical outcome of type 2 (Burian-Franceschetti) acute acquired concomitant esotropia (AACE). METHODS: Retrospective analysis of children with AACE type 2. All patients underwent strabismus surgery to restore ocular alignment. All children underwent a complete assessment including medical history and pre- and postoperative ophthalmological and orthoptic examinations. Postoperative follow-up was at least 12 months in all cases. RESULTS: A total of 25 consecutive patients were included. All but 2 patients (92%) were aligned within 8(∆) or less of orthotropia. Of the 25, 15 (60%) regained normal stereovision. In 6 additional cases (24%) some level of binocular vision (Titmus test, 200'' to 3000'') was demonstrated. All of the patients who finally achieved normal stereopsis had lower levels of binocularity on the first postoperative day. The mean interval between surgery and first occurrence of full stereovision was 18 months (range, 2 to 58 months). CONCLUSIONS: General features of AACE type 2 are concomitance of strabismus, absence of an accommodative component even in the presence of hyperopic refractive errors, and no neurological pathology. The potential for normal binocular vision plays a key role in defining this entity. The reemergence of full stereopsis may take several years.

Hand-held dynamic contour tonometry.

PURPOSE: We present a prototype of the hand-held dynamic contour tonometer (HH-DCT) and prospectively compare this HH-DCT with the well-established Perkins applanation tonometer (PAT) and the TonoPenXL (TPXL). METHODS: In a prospective, single-centre, randomized study, intraocular pressure (IOP) readings were taken in random order using HH-DCT, PAT and TPXL tonometers. Intra-observer variability was calculated for each observer and compared between three experienced ophthalmologists and an inexperienced medical student. RESULTS: Ninety-two corneas of 92 healthy participants were enrolled. IOP [mean mmHg ± standard deviation (SD)] as measured by HH-DCT was 16.97 ± 2.71, by PAT 13.98 ± 2.52 and by TPXL 13.34 ± 2.68. The range of three consecutive IOP readings differed significantly between the devices [p < 0.001; mean range: 1.45 ± 1.07 (HH-DCT), 1.87 ± 0.97 (PAT) and 2.08 ± 1.77 (TPXL)]. There was no difference of the range in all devices between the ophthalmologists and the medical student (HH-DCT p = 0.68, PAT p = 0.54, TPXL p = 0.48). CONCLUSION: IOP readings measured by HH-DCT are significantly higher than by PAT and TPXL. The differences of IOP measurements are in good accordance with previous studies using the slit-lamp-mounted DCT (SL-DCT) and Goldmann Applanation Tonometry, where SL-DCT readings were 1-3.2 mmHg higher. HH-DCT seems to give more constant results, which can be seen in the lower intra-observer variability compared to PAT and TPXL.

Use of intraoperative fourier-domain anterior segment optical coherence tomography during descemet stripping endothelial keratoplasty.

PURPOSE: To evaluate the intraoperative use of handheld Fourier-domain optical coherence tomography (OCT) during Descemet stripping automated endothelial keratoplasty (DSAEK) to assess the donor-host interface. DESIGN: Prospective, observational case series. METHODS: Six patients undergoing DSAEK surgery were included. OCT scans of the cornea were performed intraoperatively after insertion of the donor disc, after instillation of air in the anterior chamber beneath the disc, after vent incisions in the host cornea in each quadrant, following air-fluid exchange at the end of operation, and on day 1 after surgery. The central 3 mm of each cornea was scanned. The broadest gap between donor and host cornea (interface space) was measured. RESULTS: Adequate readings could be obtained from all patients without any complications. In 2 patients there was a decrease in the width of the interface space after each surgical step documented by the OCT scans. At the end of their operation, no interface space was detectable. In 2 patients, interface space disappeared after the vent incisions and did not reappear during the further course of the surgery. In further 2 patients the separation between the host and donor was still detectable at the end of the operation. All patients had no detectable interface gap on day 1. CONCLUSIONS: Handheld anterior segment OCT can be used to assess the host-donor interface in lamellar corneal transplantation surgery. Donor adherence can occur in spite of residual interface space at the end of surgery. Further studies should be conducted to answer the question of which surgical steps are useful in assisting with donor adhesion.

Intraocular pressure during a very high altitude climb.

PURPOSE: Reports on intraocular pressure (IOP) changes at high altitudes have provided inconsistent and even conflicting RESULTS: The purpose of this study was to investigate the effect of very high altitude and different ascent profiles on IOP in relation to simultaneously occurring ophthalmic and systemic changes in a prospective study. METHODS: This prospective study involved 25 healthy mountaineers who were randomly assigned to two different ascent profiles during a medical research expedition to Mt. Muztagh Ata (7,546 m/24,751 ft). Group 1 was allotted a shorter acclimatization time before ascent than was group 2. Besides IOP, oxygen saturation (SaO(2)), acute mountain sickness symptoms (AMS-c score), and optic disc appearance were assessed. Examinations were performed at 490 m/1,607 ft, 4,497 m/14,750 ft, 5,533 m/18,148 ft, and 6,265 m/20,549 ft above sea level. RESULTS: Intraocular pressure in both groups showed small but statistically significant changes: an increase during ascent from 490 m/1,607 ft to 5,533 m/18,148 ft and then a continuous decrease during further ascent to 6,265 m/20,549 ft and on descent to 4,497 m/14,750 ft and to 490 m. Differences between groups were not significant. Multiple regression analysis (IOP-dependent variable) revealed a significant partial correlation coefficient of beta = -0.25 (P = 0.01) for SaO(2) and beta = -0.23 (P = 0.02) for acclimatization time. DISCUSSION: Hypobaric hypoxia at very high altitude leads to small but statistically significant changes in IOP that are modulated by systemic oxygen saturation. Climbs to very high altitudes seem to be safe with regard to intraocular pressure changes.

Dynamic contour tonometry: handheld versus slit-lamp-mounted.

PURPOSE: To compare the newly developed handheld dynamic contour tonometer (hh-DCT) with the established slit-lamp-mounted DCT (sl-DCT) in terms of agreement for intraocular pressure (IOP) and ocular pulse amplitude (OPA) measurements, intraobserver variability, and tonography effect. DESIGN: Evaluation of diagnostic technology. PARTICIPANTS: Fifty eyes of 50 healthy volunteers were examined with the hh-DCT and sl-DCT. METHODS: A paired t test was performed to analyze differences in IOP and OPA readings. Measurements were compared for bias and agreement according to the method of Bland and Altman. Intraclass correlation coefficients (ICCs) were calculated to compare intraobserver variability. A mixed model analysis was performed to assess a possible tonography effect. MAIN OUTCOME MEASURES: The differences in IOP and OPA measurements, intraobserver variability, and tonography effect between the hh-DCT and the sl-DCT were examined. RESULTS: No statistically significant differences in IOP (sl-DCT-hh-DCT: 0.1+/-1.43 mmHg, 95% confidence interval [CI], -0.31 to 0.5, P = 0.63) or OPA (sl-DCT-hh-DCT: -0.1+/-0.52 mmHg, 95% CI, -0.28 to 0.01, P = 0.08) measurements were detected. Bland-Altman analysis revealed a bias of 0.1 with the limits of agreement of IOP measurement differences ranging from -2.71 to +2.90 mmHg. With regard to IOP readings obtained by sl-DCT, hh-DCT overestimated IOPs less than 15.6 mmHg and underestimated IOPs more than 15.6 mmHg. ICCs calculated for IOP readings were 0.87 (95% CI, 0.8-0.92) for hh-DCT and 0.85 (95% CI, 0.78-0.90) for sl-DCT. ICCs for OPA readings were 0.86 (95% CI, 0.79-0.91) for hh-DCT and 0.87 (95% CI, 0.8-0.91) for sl-DCT. Comparing the ICCs revealed no statistically significant difference between the devices with regard to IOP and OPA measurements (P = 0.53 and P = 0.93, respectively). Mixed model analysis of 3 consecutive IOP measurements revealed a decrease in IOP of 0.5 mmHg after each measurement in both devices, which was not significant between the 2 methods (P = 0.68). CONCLUSIONS: IOP and OPA readings obtained by the novel hh-DCT were shown to be strongly concordant with measurements obtained by the sl-DCT device. The hh-DCT may be a valuable alternative to the sl-DCT in clinical practice.