Ultra-widefield autofluorescence imaging in the evaluation of scleral buckling surgery for retinal detachment.

PURPOSE: To investigate the use of ultra-widefield fundus autofluorescence imaging in the early postoperative evaluation of scleral buckling surgery for retinal detachment. METHODS: Forty-five eyes from 44 patients with rhegmatogenous retinal detachment were included. Ultra-widefield fundus autofluorescence imaging (Optomap P200Tx) was performed from both eyes preoperatively and early (1-2 days) postoperatively. All patients were operated with 2.5-mm encircling band, 6-mm to 9-mm segmental buckle, transscleral cryopexy, and the choice of drainage and air/gas endotamponade. RESULTS: The mean age of the patients was 58 ± 12 years, and the ratio of macula on-off detachments was 19/26. Light cryopexy induced hyperfluorescence of the treated area (in 11% of cases). Moderate cryopexy resulted in central hypofluorescence with a hyperfluorescent halo (in 51% of cases), whereas extensive cryopexy and disruption of the retinal pigment epithelium resulted in a broad hypofluorescent area (in 36% of cases). Tightening of the indenting elements induced peripheral hyperfluorescent radial streaks in 47% of cases and distinct areas of hyperfluorescence in 58% of cases. Demarcation lines and residual subretinal fluid were observed as hyperfluorescent areas. Central autofluorescence changes were observed in 96% of macula-off surgeries, whereas only 27% of these cases showed distinct hyper- and hypo-autofluorescent streaks. CONCLUSION: Ultra-widefield fundus autofluorescence imaging is a useful adjuvant tool for evaluating early outcome and retinal pigment epithelium function after scleral buckling surgery for retinal detachment.

Autofluorescence Imaging in the Long-Term Follow-Up of Scleral Buckling Surgery for Retinal Detachment.

Purpose: To analyse fundus autofluorescence (AF) changes in retinal reattachment following primary scleral buckling (SB) surgery for rhegmatogenous retinal detachment (RRD). Methods: Prospective noninterventional chart review study. AF images were reviewed for peripheral and central changes and compared to clinical and OCT findings. Results: A total of 73 eyes from 69 patients were included, four presenting with bilateral RRD. Mean age was 55 ± 12 years, male/female ratio 40/29, fovea-on/-off RRD 43/30, and mean follow-up time 376 ± 270 days, with a mean of 5 ± 3 postoperative visits. Preoperatively, RRD was seen as a hypofluorescent area with a hyperfluorescent leading edge. Immediately postoperatively, three types of cryopexy could be differentiated, gradually transforming to scleral hyperfluorescence. Buckle tightening produced alternating hyper-/hypofluorescent streaks, and demarcation lines showed a persistent rugged hyperfluorescent signal. Choroidal detachment led to transient hypofluorescence, whereas vortex vein compression induced persistent hypofluorescence. Peripheral retinal folds were hyperfluorescent and the drainage site was hypofluorescent. AF was highly sensitive in detecting even small amounts of hyperfluorescent persistent subretinal fluid (SRF) that showed a slow resolution during follow-up. A granular "salt-and-pepper-" like pattern in the central macula was seen in 80% of eyes with fovea-off RRD and alternating streaks in 10%. Findings from OCT imaging correlated well with AF regarding SRF, macular oedema, retinal pigment epithelial detachment, and presence of a subretinal scar, but only moderately in epiretinal membrane formation and choroidal folds. Conclusions: AF is a useful, noninvasive, adjuvant tool in the long-term follow-up after SB surgery.

Ultra-widefield autofluorescence imaging findings in retinoschisis, rhegmatogenous retinal detachment and combined retinoschisis retinal detachment.

PURPOSE: Retinoschisis (RS), rhegmatogenous retinal detachment (RRD) and combined RS retinal detachment (RSRD) may resemble clinically and pose a diagnostic challenge. This study investigates the role of the fundus autofluorescence (AF) in differentiating RS, RRD and RSRD. METHODS: Fundus AF changes of 34 eyes diagnosed with RRD, 30 eyes with RS and 12 eyes with RSRD were retrospectively analysed. Ultra-widefield AF (UW-AF) image intensities obtained with the Optomap 200Tx were interpreted as hypo-, hyper- and isoautofluorescent or a mixed pattern with hypo- and hyperautofluorescence over and at the posterior margin (PM) of RRD, RS and RSRD. RESULTS: All RS eyes revealed isoautofluorescence over the area of RS, and nine eyes (30%) showed hypoautofluorescent PM. Among RRD, acute (≤2 weeks) and chronic (>2 weeks) RRD demonstrated distinct AF characteristics. Sixty-two per cent of RRD eyes had acute RRD. From those, 16 eyes (76%) demonstrated hypoautofluorescence over the detached area and 19 (90%) eyes with hyperautofluorescent PM. Sixty-two per cent of chronic RRD eyes demonstrated isoautofluorecence over the detached area. Eight RSRD eyes (67%) revealed hyperautofluorescence in the detached area. The positive predictive value (PPV) for hypoautofluorescence over the area of subretinal fluid (SRF) in RRD was 95%. The PPV for hyperautofluorescence over the area of SRF in RSRD was 100% and for isoautofluorescence for schitic area in RSRD and RS was 76%. CONCLUSION: The UW-AF can be a useful non-invasive adjuvant tool to distinguish between RRD, RS and RSRD. Hypo- or hyperautofluorescence over the area of interest and hyperautofluorescent PM indicates the presence of SRF.

Proliferative vitreoretinopathy in a child with visceral leishmaniasis.

PURPOSE: We present a rare case of ocular leishmaniasis complicated by proliferative vitreoretinopathy in a child with active visceral leishmaniasis. METHODS: A 10-year-old boy with active visceral leishmaniasis presented with a 5- day history of redness, photophobia, and blurred vision in his left eye. Visual acuity was measured and the child had a complete ocular examination. RESULTS: Snellen best-corrected visual acuity (BCVA) was 10/10 in the right eye and 7/10 in the left eye at presentation. Ophthalmic examination of the right eye was normal but the left eye showed clinical signs of panuveitis. Laboratory investigations were negative. Treatment with systemic and local steroids was initiated and clinical improvement achieved. Eight months later, the patient had a relapse of systemic and ocular disease with severe panuveitis in both eyes. A combined tractional-rhegmatogenous retinal detachment was present in the left eye. Pars plana vitrectomy was undertaken in the left eye and the patient was started on systemic and local steroid treatment. Retinal reattachment was achieved postoperatively but visual acuity in the left eye remained poor. CONCLUSIONS: Early diagnosis, prompt systemic and ocular treatment, as well as close ophthalmic examination are essential in such cases.

Fever-related ataxia: a case report of CAPOS syndrome.

BACKGROUND: CAPOS (Cerebellar ataxia, Areflexia, Pes cavus, Optic atrophy and Sensorineural hearing loss) syndrome is caused by the heterozygous mutation, c.2452G > A, in the ATP1A3 gene. Other mutations in this gene can cause a spectrum of overlapping phenotypes including alternating hemiplegia of childhood, rapid onset dystonia parkinsonism, early infantile epileptic encephalopathy and fever induced paroxysmal weakness and encephalopathy. The phenotype is still mistaken for mitochondrial/metabolic disorders and follow up studies are scare. CASE PRESENTATION: We report a 20 year old Norwegian male with ataxia, sensorineural deafness and visual loss. Before the age of five he experienced three fever related episodes of acute neurological deterioration when he temporarily lost his acquired motor skills and developed persistent gait and limb ataxia. In childhood, he developed bilateral optic atrophy and bilateral sensorineural hearing loss. Motor skills improved and at age 20 the patient showed a mild ataxia, hearing loss and reduced vision. A c.2452G > A mutation in the ATP1A3 gene was identified and CAPOS syndrome was confirmed. CONCLUSIONS: This is the first Norwegian patient reported with CAPOS syndrome. Our patient had a de novo, previously identified ATP1A3 mutation. The combination of recurrent episodes of fever related ataxia, loss of motor skills in early childhood, and early onset hearing and vision loss is typical of CAPOS syndrome. Previous reports suggest a gradual progression of the disease after the initial episodes, while this patient showed a good outcome with improvement of motor skills from adolescence long after the last deterioration episode.

ULTRA-WIDE-FIELD FUNDUS IMAGING IN THE DIAGNOSIS AND FOLLOW-UP OF SUSAC SYNDROME.

PURPOSE: To present the use of ultra-wide-field (UW) fundus imaging in the diagnosis and follow-up of a patient with Susac syndrome. METHODS: Case report of a myopic patient presenting initially with rhegmatogenous retinal detachment. A significant portion of the retina was found to be avascular bilaterally at presentation. Surgery was performed with scleral buckle. Then, UW color and autofluorescent imaging and UW fluorescein angiography were obtained. RESULTS: Successful retinal reattachment was obtained. Enlargement of the avascular area with neovascularization was observed at eight-month follow-up. In addition, the patient presented severe neurosensory hearing loss and clinical depression postoperatively. The results of UW fluorescein angiography revealed hyperfluorescent macular spots, arteriolar wall hyperfluorescence, leakage from retinal neovascularization, and confirmed the avascularity of two thirds of the retina, whereas the results of UW autofluorescence showed absence of the normal hypofluorescent retinal vessels outside the posterior pole. Findings of UW imaging in combination with systemic involvement led to the diagnosis of Susac syndrome. Appropriate treatment stopped the disease progress, ameliorated symptoms, and some of the occluded retinal vessels were reperfused. CONCLUSION: In conclusion, UW fundus imaging is a valuable modality in the diagnosis and follow-up of patients with Susac syndrome. Early diagnosis and treatment is critical, particularly as it can lead to reperfusion of occluded retinal vessels.

Concise Review: Altered Versus Unaltered Amniotic Membrane as a Substrate for Limbal Epithelial Cells.

Limbal stem cell deficiency (LSCD) can result from a variety of corneal disorders, including chemical and thermal burns, infections, and autoimmune diseases. The symptoms of LSCD may include irritation, epiphora, blepharospasms, photophobia, pain, and decreased vision. There are a number of treatment options, ranging from nonsurgical treatments for mild LSCD to various forms of surgery that involve different cell types cultured on various substrates. Ex vivo expansion of limbal epithelial cells (LEC) involves the culture of LEC harvested either from the patient, a living relative, or a cadaver on a substrate in the laboratory. Following the transfer of the cultured cell sheet onto the cornea of patients suffering from LSCD, a successful outcome can be expected in approximately three out of four patients. The phenotype of the cultured cells has proven to be a key predictor of success. The choice of culture substrate is known to affect the phenotype. Several studies have shown that amniotic membrane (AM) can be used as a substrate for expansion of LEC for subsequent transplantation in the treatment of LSCD. There is currently a debate over whether AM should be denuded (i.e., de-epithelialized) prior to LEC culture, or whether this substrate should remain intact. In addition, crosslinking of the AM has been used to increase the thermal and mechanical stability, optical transparency, and resistance to collagenase digestion of AM. In the present review, we discuss the rationale for using altered versus unaltered AM as a culture substrate for LEC. Stem Cells Translational Medicine 2018;7:415-427.

Transcriptome Analysis of Cultured Limbal Epithelial Cells on an Intact Amniotic Membrane following Hypothermic Storage in Optisol-GS.

The aim of the present study was to investigate the molecular mechanisms underlying activation of cell death pathways using genome-wide transcriptional analysis in human limbal epithelial cell (HLEC) cultures following conventional hypothermic storage in Optisol-GS. Three-week HLEC cultures were stored in Optisol-GS for 2, 4, and 7 days at 4 °C. Partek Genomics Suite software v.6.15.0422, (Partec Inc., St. Louis, MO, USA) was used to identify genes that showed significantly different (P < 0.05) levels of expression following hypothermic storage compared to non-stored cell sheets. There were few changes in gene expression after 2 days of storage, but several genes were differently regulated following 4 and 7 days of storage. The histone-coding genes HIST1H3A and HIST4H4 were among the most upregulated genes following 4 and 7 days of hypothermic storage. Bioinformatic analysis suggested that these two genes are involved in a functional network highly associated with cell death, necrosis, and transcription of RNA. HDAC1, encoding histone deacetylase 1, was the most downregulated gene after 7 days of storage. Together with other downregulated genes, it is suggested that HDAC1 is involved in a regulating network significantly associated with cellular function and maintenance, differentiation of cells, and DNA repair. Our data suggest that the upregulated expression of histone-coding genes together with downregulated genes affecting cell differentiation and DNA repair may be responsible for increased cell death following hypothermic storage of cultured HLEC. In summary, our results demonstrated that a higher number of genes changed with increasing storage time. Moreover, in general, larger differences in absolute gene expression values were observed with increasing storage time. Further understanding of these molecular mechanisms is important for optimization of storage technology for limbal epithelial sheets.

Autofluorescence imaging in the differential diagnosis of optic disc melanocytoma.

PURPOSE: Optic disc melanocytoma (ODM) is a benign tumour that usually occurs on or adjacent to the optic nerve head. The aim of the study was to evaluate fundus autofluorescence (FAF) imaging as a diagnostic tool in ODM. METHODS: Retrospective comparative case series study of six patients with ODM and a comparing group of four patients with juxtapapillary choroidal nevus (JCN) and four with juxtapapillary uveal melanoma (JUM). Clinical examination was supplemented with ultrasound B-scan examination and spectral-domain optical coherence tomography. FAF images were obtained with the 532-nm laser (Optomap P200Tx) from all patients. RESULTS: Clinical examination in the ODM group revealed a dome-shaped, darkly pigmented tumour on or adjacent to the optic disc in all patients, with a mean tumour basal dimension 1.4 mm and mean tumour thickness by ultrasonography of 1.0 mm. FAF revealed a totally hypofluorescent mass with sharply demarcated, feathery edges. No hyperfluorescent changes due to orange pigment or subretinal fluid were seen. In contrast, patients with JCN and JUM manifested focal hyperfluorescence as well as larger hyperfluorescent areas at the tumour and its borders. CONCLUSION: Fundus autofluorescence imaging is a non-invasive adjuvant tool in the differential diagnosis of ODM characterized by lack of hyperfluorescence compared to JCN and JUM.