Evolution of an Astrocytic Hamartoma of the Optic Nerve Head in a Patient with Retinitis Pigmentosa - Photographic Documentation over 2 Years of Follow-Up

Aim

To report photographically the evolution of an astrocytic hamartoma of the left optic nerve head over a 2-year follow-up in a patient with retinitis pigmentosa.

Methods

A 14-year-old boy was seen in the medical retina clinic with a 3-year history of night blindness. Best corrected visual acuity was 6/18 in both eyes. Colour vision was normal in both eyes and confrontation fields showed peripheral constriction. Fundus examination revealed bone spicule pigmentary changes at the retinal mid periphery typical of retinitis pigmentosa and superficial globules at the margins of both optic nerve heads. Electrodiagnostic tests confirmed moderately severe rod cone dystrophy with macular involvement bilaterally.

Results

Two years later, the ocular examination was unchanged except for the appearance of the optic nerve head lesion in the left eye. There was an increase in the size of the lesion which was diagnosed as an astrocytic hamartoma. Further investigations were recommended to exclude neurofibromatosis and tuberous sclerosis.

Conclusion

Astrocytic hamartomas of the optic nerve head and optic nerve head drusen have both been described in patients with retinitis pigmentosa. They can be a diagnostic dilemma although drusen are more common (10%). To differentiate these two entities it is very important to document any growth during the follow-up period which is suggestive of astrocytic hamartoma rather than optic disc drusen.Key Words: Astrocytic hamartoma, Optic disc drusen, Retinitis pigmentosa     

Vitreous Hemorrhage Caused by Ruptured Retinal Macroaneurysm

Purpose

To report the clinical findings of 7 patients with a vitreous hemorrhage (VH) caused by a ruptured retinal macroaneurysm.

Methods

Interventional case series. The medical records of 7 patients with a VH caused by a ruptured macroaneurysm and treated by either medication or vitrectomy were reviewed. The main outcome measures were the visual acuity, appearance of the fundus and optical coherence tomographic findings.

Results

Two patients, aged 49 and 58 years, had retinal macroaneurysms at the optic disc. The retinal macroaneurysm in the other 5 eyes (mean age 79.0 years) was not at the optic disc. The VH was completely resolved in all 7 patients. The visual acuity improved in all eyes and remained stable for at least 6 months after the treatments (p = 0.0478).

Conclusions

The improvement of the visual acuity in all eyes indicates that the prognosis of eyes with a VH caused by a ruptured retinal macroaneurysm is good.Key words: Vitreous hemorrhage, Ruptured retinal macroaneurysm, Optic disc, Prognosis     

Retinal Angiomatous Proliferation in an Eye with Cuticular Drusen

Purpose

To report the presence of retinal angiomatous proliferation (RAP) in an eye with cuticular drusen detected by fluorescein and indocyanine green angiography by confocal scanning laser ophthalmoscopy and by spectral-domain optical coherence tomography (SD-OCT).

Methods

Case report of a 65-year-old Japanese woman with cuticular drusen.

Results

At her first ophthalmic examination, her visual acuity was 20/20 in both eyes. An ophthalmoscopy showed many small subretinal pigment epithelial deposits in both eyes. These deposits had a ‘saw-tooth pattern’ in the SD-OCT images. During the follow-up examination, retinal hemorrhages were observed, and fluorescein angiography showed a ‘stars-in-the-sky’ appearance and intraretinal neovascularization. The patient was diagnosed with cuticular drusen associated with RAP.

Conclusion

We suggest that the cuticular drusen were associated with RAP, so periodic follow-up examinations are needed for patients with cuticular drusen for the early detection and treatment of RAP.Key Words: Cuticular drusen, Basal laminar drusen, Retinal angiomatous proliferation, Spectral domain optical coherence tomography     

Surgical Management of Optic Disc Pit Maculopathy with Outer Retinal Hole

Objective

To report the successful outcome of a rare optic pit-associated maculopathy with an outer retinal hole following 23 G vitrectomy, internal limiting membrane (ILM) peeling and fluid-gas exchange without additional endolaser.

Method

Interventional case report.

Results

This case report documents a 56-year-old male patient with complaints of progressive diminution of vision in the right eye more than in the left eye due to an optic disc pit with an outer retinal hole and a cataract. Optical coherence tomography confirmed the presence of an outer retinal hole. The case report shows the successful outcome of a rare optic pit-associated maculopathy with an outer retinal hole and a cataract following phacoemulsification with 23 G vitrectomy, ILM peeling and fluid-gas exchange without additional endolaser.Key Words: Optic pit, Outer retinal hole, Retinoschisis, Vitrectomy     

Subretinal fluid is common in experimental non-arteritic anterior ischemic optic neuropathy

Purpose

Anterior ischemic optic neuropathy (AION) is an important cause of acute vision loss for which several animal models exist. It has been associated with subretinal fluid in a previous study on patients but not yet so in animal models.

Patients and Methods

A patient presented with acute non-arteritic AION (NAION) and underwent ophthalmic evaluation and testing including fluorescein angiography and spectral-domain optical coherence tomography (SD-OCT). On the basis of the patient''s findings, we used SD-OCT circular and volume scans to analyze retinal changes in a murine model of NAION.

Results

One week after left eye vision loss, the patient had clinical and imaging findings consistent with NAION. On SD-OCT, there was prominent peripapillary retinal thickening consistent with intra-retinal edema and sub-foveolar fluid. Inspired by the findings in human AION, we looked for similar changes in murine NAION using SD-OCT. The circular scan did not adequately detect the presence of subretinal fluid. Using the 25-line scan, which covered a larger part of the posterior pole, we found that 100% of murine AION resulted in subretinal fluid at day 1. The subretinal fluid resolved by week 1.

Conclusion

This study detailed a case of clinical NAION associated with intra-retinal and subretinal fluid. We also found that subretinal fluid was common in murine photochemical thrombosis model of AION and could be found far away from the optic disc.     

Characteristic Findings of Optical Coherence Tomography in Retinal Angiomatous Proliferation

Purpose

To identify the unique pathologic findings of retinal angiomatous proliferation (RAP) in optical coherence tomography (OCT).

Methods

Retrospectively, 29 eyes of 25 patients with age-related macular degeneration and complicated RAP were analyzed. All 29 eyes had choroidal neovascularization (CNV) in the area of pigment epithelial detachment (PED) or adjacent to it, which was visible with fluorescein angiography or indocyanine green angiography. Cross-sectional images were obtained by OCT scanning through the CNV lesions.

Results

Six distinctive findings of OCT included drusen (100%), inner retinal cyst (80%), outer retinal cyst (68%), fibrovascular PED (84%), serous retinal detachment (40%), and PED (68%).

Conclusions

Through analysis of OCT findings, we revealed six different types of lesions distinctive of RAP which may provide helpful diagnostic information for subsequent treatment and predicting the prognosis of RAP.     

Factors influencing the visual acuity of chronic central serous chorioretinopathy

Purpose

To investigate correlated factors on final visual acuity in conjunction with fluorescein angiography (FA) and optical coherence tomography (OCT) findings of chronic central serous chorioretinopathy (CSCR).

Methods

Twenty-four patients (36 eyes) with typical findings of chronic CSCR based on medical records, FA and OCT results were enrolled in this study. We investigated demographic findings, initial and final visual acuity (VA), and some typical findings of FA including the type of leakage pattern, the existence of a gravitational tract and an abnormal hyperfluorescent area centered on the fovea. We also investigated OCT findings to examine serous retinal detachment, outer photoreceptor layer (OPRL) preservation, continuity of the inner segment (IS) and the outer segment (OS) of the photoreceptor layer in case of macular attachment, and other typical findings. The converted logarithm of the minimum angle of resolution VA was used to investigate the statistical correlation with these FA and OCT findings.

Results

An abnormal hyperfluorescent area within 1 macular photocoagulation study disc area on FA and cystoid degeneration on OCT were correlated with poor final VA of less than 20 / 40. However, the preserved OPRL and the continuity of IS / OS junction were correlated with a good final VA of 0.5 or more.

Conclusions

These specific findings could be associated with recurrent or persistent subretinal fluid and could be important parameters of decision for treatment.     

Multimodal imaging including spectral-domain optical coherence tomography and confocal near-infrared reflectance for characterization of lacquer cracks in highly myopic eyes

Purpose

To compare multimodal imaging in detecting lacquer cracks in highly myopic eyes, and to correlate these findings with those of spectral-domain optical coherence tomography (SD-OCT).

Methods

An observational case series study. Patients with a refractive error worse than −8 diopters and lacquer cracks were recruited. The rates of detection of the lacquer cracks using multimodal imaging including near-infrared reflectance (NIR) imaging, fundus autofluorescence (FAF) imaging, and fluorescence angiography (FA) were compared. The characteristic findings of multimodal imaging were correlated with those of SD-OCT.

Results

NIR imaging was more sensitive (92.9%) in detecting lacquer cracks than either FAF (12.5%) or FA (67.9%). Lacquer cracks showed hyperreflectance on NIR, and they were consistently associated with a continuous retinal pigment epithelium-Bruch''s membrane complex, thinner choroid, and acoustic shadows on SD-OCT.

Conclusions

NIR imaging is superior to blue laser light (FAF and FA) imaging in detecting lacquer cracks. SD-OCT in combination with NIR located primary pathological lacquer cracks in the intact retinal pigment epithelium-Bruch''s membrane complex as well as thinner choroid. These findings indicate that multimodal cSLO and SD-OCT imaging allow for detecting of lacquer cracks in highly myopic eyes.     

Assessment of rates of structural change in glaucoma using imaging technologies

Purpose

To review the ability of current imaging technologies to provide estimates of rates of structural change in glaucoma patients.

Patients and methods

Review of literature.

Results

Imaging technologies, such as confocal scanning laser ophthalmoscopy (CSLO), scanning laser polarimetry (SLP), and optical coherence tomography (OCT), provide quantifiable and reproducible measurements of the optic disc and parapapillary retinal nerve fibre layer (RNFL). Rates of change as quantified by the rim area (RA) (for CSLO) and RNFL thickness (for SLP and OCT) are related to glaucoma progression as detected by conventional methods (eg, visual fields and optic disc photography). Evidence shows that rates of RNFL and RA loss are significantly faster in progressing compared with non-progressing glaucoma patients.

Conclusion

Measurements of rates of optic disc and RNFL change are becoming increasingly precise and individualized. Currently available imaging technologies have the ability to detect and quantify progression in glaucoma, and their measurements may be suitable end points in glaucoma clinical trials.     

Comparison of OCT and HRT Findings Among Normal, Normal Tension Glaucoma, and High Tension Glaucoma

Purpose

To evaluate the relationship between optic disc and retinal nerve fiber layer (RNFL) measurements obtained with the optical coherence tomography (OCT) and the Heidelberg retina topography (HRT) in normal, normal tension glaucoma (NTG), and high tension glaucoma (HTG).

Methods

Normal, NTG and HTG subjects who met inclusion and exclusion criteria were evaluated retrospectively. One hundred seventy eyes of 170 patients (30 normal, 40 NTG, and 100 HTG) were enrolled. Complete ophthalmologic examination, HRT, OCT, and automated perimetry were evaluated.

Results

Disc area, cup area and cup/disc area ratio measured with HRT were significantly different between NTG and HTG (all p<0.05). Mean RNFL thickness measured by OCT with ascanning diameter of 3.4 mm was larger in NTG than HTG (84.97±24.20 µm vs. 73.53±27.17 µm, p=0.037). Four quadrant RNFL thickness measurements were not significantly different between NTG and HTG (all p>0.05). Mean deviation and corrected pattern standard deviation measured by automated perimetry was significantly correlated with mean and inferior RNFL thickness in both NTG and HTG (Pearson''s r, p<0.05). Mean RNFL thickness/disc area ratio was significantly larger in HTG than NTG (35.21±18.92 vs. 31.30±10.91, p=0.004).

Conclusions

These findings suggest that optic disc and RNFL damage pattern in NTG may be different from those of HTG.     

Using ImageJ to Evaluate Optic Disc Pallor in Traumatic Optic Neuropathy

Purpose

To evaluate optic disc pallor using ImageJ in traumatic optic neuropathy (TON).

Methods

This study examined unilateral TON patients. The optic disc was divided into 4 quadrants (temporal, superior, nasal, and inferior), consistent with the quadrants on optical coherence tomography (OCT) retinal nerve fiber layer (RNFL) thickness maps. Optic disc photography was performed and disc pallor was quantified using gray scale photographic images imported into ImageJ software. The correlation between optic disc pallor and RNFL thickness was examined in each quadrant.

Results

A total of 35 patients (31 male, 4 female) were enrolled in the study. The mean participant age was 34.8 ± 15.0 years (range, 5 to 63 years). Overall RNFL thickness decreased in 6 patients, with thinning most often occurring in the inferior quadrant (28 of 35 eyes). There was a significant correlation between optic disc pallor and RNFL thickness (superior, rho = -0.358, p = 0.04; inferior, rho = -0.345, p = 0.04; nasal, rho = -0.417, p = 0.01; temporal, rho = -0.390, p = 0.02). The highest level of correspondence between disc pallor and RNFL thickness values outside of the normative 95th percentiles was 39.3% and occurred in the inferior quadrant.

Conclusions

Optic disc pallor in TON was quantified with ImageJ and was significantly correlated with RNFL thickness abnormalities. Thus, ImageJ evaluations of disc pallor may be useful for evaluating RNFL thinning, as verified by OCT RNFL analyses.     

Analyses of shape of eyes and structure of optic nerves in eyes with tilted disc syndrome by swept-source optical coherence tomography and three-dimensional magnetic resonance imaging

Purpose

To evaluate the deeper structures of the optic nerve and to analyze the shape of eyes with tilted disc syndrome (TDS) by swept-source optical coherence tomography (OCT) and three-dimensional magnetic resonance imaging (3D MRI).

Methods

The medical records of 54 eyes of 36 patients with TDS were reviewed. The patients with TDS and high myopia were analyzed separately from those without high myopia. All the eyes were examined with a swept-source OCT, and 22 of the eyes were examined by 3D MRI.

Results

A total of 38 eyes of 29 patients were highly myopic and 16 eyes of 15 patients were not highly myopic. The representative OCT findings of the optic disc were: a sloping of the lamina cribrosa posteriorly from the upper part to the lower part, a protrusion of the upper edge of Bruch''s membrane, and choroid. The distance and the depth of the most protruded point from the fovea were significantly greater in the eyes with non-highly myopic TDS than those with highly myopic TDS. In the 3D MRI, the lower part of the posterior segment was protruded outward, and the optic nerves attached at the upper nasal edge of the protrusion.

Conclusions

The abnormalities detected by swept-source OCT and 3D MRI analyses indicate the possibility that the essential pathology of TDS is a deformity of the inferior globe below the optic nerve, and the positional relation between the fovea and the inferior protrusion determines the degree of myopia.     

Factors associated with the signal strengths obtained by spectral domain optical coherence tomography

Purpose

The aim of this study was to investigate factors associated with the signal strengths (SS, image quality scores) of optic disc and macular images obtained using Cirrus spectral domain optical coherence tomography (OCT).

Methods

Ninety-two glaucomatous eyes were imaged using the Cirrus OCT macular and optic disc cube modes after pupil dilation. The influences of patient age, spherical equivalent, cataract presence, and cataract and glaucoma severity (visual field mean deviation), on the SS of images obtained using the two cube modes were compared between patients whose images showed high SS (SS ≥7) and low SS (SS <7).

Results

The signal strength was significantly higher in images obtained using the macular cube compared to the optic disc cube mode (7.8 ± 1.3 vs. 6.9 ± 1.1, respectively; p = 0.001). Age and visual acuity of patients differed significantly between the high- and low-SS groups when data acquired using the optic disc (p = 0.027 and 0.012, respectively) and macular cube modes (p = 0.046 and 0.014, respectively) were analyzed. When the optic disc cube mode was employed, the extent of cataracts was significantly related to SS, whereas when the macular cube mode was used, none of the factors analyzed was significantly associated with SS.

Conclusions

Age, visual acuity, and the extent of cataracts were significantly associated with images of higher SS when the Cirrus OCT optic disc cube mode was employed.     

Refractory Coats’ Disease of Adult Onset

Purpose

We present the case of an 18-year-old Caucasian male with a unilateral macular star and retinal vascular anomalies compatible with adult onset Coats’ disease.

Methods

Diagnosis was based on fundoscopic, fluorescein angiography and optical coherence tomography findings.

Results

The patient presented to our emergency department with complaints of low vision in his left eye (LE) detected 10 days before. The best-corrected visual acuity in the LE was 20/50. Fundoscopy of the LE evidenced a complete macular star. Optical coherence tomography showed increased retinal thickness, infiltration of the retinal wall, and detachment of the neuroepithelium. Angiography revealed no appreciable diffusion in the macula. Above the superior temporal (ST) arcade, anomalies in the retinal vasculature were found, with interruption of the peripheral vessels and vessels which were ‘sausage’-like. After 1 month, the LE vision evolved to hand movements. Laser photocoagulation was performed in the ST quadrant. Intravitreal injection of bevacizumab 1.25 mg/0.05 ml and photodynamic therapy were performed without any significant changes, progression of ST serous detachment of the neuroepithelium, and finally progression to macular fibrosis.

Discussion

Coats’ disease is usually diagnosed in childhood, but rare cases may occur in adults. Those cases usually have a more indolent course which was not observed in our patient. When there is macular involvement, prognosis is more guarded, despite treatment.Key words: Coats’ disease, Adult onset, Macular star, Laser photocoagulation, Photodynamic therapy, Intravitreal bevacizumab     

Long-term outcomes of pars plana vitrectomy without internal limiting membrane peeling for optic disc pit maculopathy

Purpose

To evaluate the results of surgical treatment of maculopathy secondary to congenital optic pit anomaly with pars plana vitrectomy (PPV), endolaser to the temporal edge of the optic disc and C3F8 tamponade without internal limiting membrane (ILM) peeling.

Patients and methods

Thirteen eyes of 12 patients with serous macular detachment and/or macular retinoschisis secondary to congenital optic disc pit (ODP) were included in the study. All eyes underwent PPV, posterior hyaloid removal, endolaser photocoagulation on the temporal margin of the optic disc and 12% C3F8 gas tamponade. Anatomic success and functional outcome determined retrospectively by optical coherence tomography and measurement of best corrected visual acuity (BCVA), respectively were the main outcome parameters.

Results

Two lines or more improvement in BCVA was obtained in 11 eyes and 6 of these eyes had 20/40 or better BCVA at the final visit. Subretinal or intraretinal fluid was completely resorbed postoperatively in 12 eyes but a little intraretinal fluid persisted in one eye at the 16-month follow-up. Better visual improvement was observed in patients treated by earlier surgical intervention.

Conclusion

PPV, C3F8 gas tamponade and endolaser to the optic disc margin without ILM peeling may yield favourable results in the treatment of ODP maculopathy.     

Diabetic Retinopathy and Peripapillary Retinal Thickness

Purpose

To assess the diagnostic efficacy of macular and peripapillary retinal thickness measurements for the staging of diabetic retinopathy (DR) and the prediction of disease progression.

Methods

In this prospective study, 149 diabetic patients (149 eyes) and 50 non-diabetic control subjects were included. Baseline optical coherence tomography was employed to measure retinal thickness in the macula (horizontal, vertical, and central) and the peripapillary zone (superior, inferior, nasal, and concentric to the optic disc). Seven baseline parameters were correlated with the DR stages identified by fluorescein angiography. Baseline retinal thickness was compared between groups of patients requiring panretinal photocoagulation (PRP) within 6 months (PRP group) and patients not requiring PRP (No-PRP group).

Results

Macular and peripapillary retinal thicknesses in diabetic subjects were significantly greater than that in normal controls (p<0.05). All retinal thickness parameters, and particularly peripapillary circular scans, tended to increase with increasing DR severity (p<0.05). The baseline thicknesses of the peripapillary circular scans were greater in the PRP group than in the no-PRP group (p<0.05).

Conclusions

Peripapillary retinal thickness may prove to be a useful criterion for DR severity and may also serve as an indicator of disease progression.     

Rare and Unusual Choroidal Abnormalities in a Patient with Systemic Lupus Erythematosus

Purpose

To report a case of rare and unusual choroidal abnormalities in a 42-year-old woman with systemic lupus erythematosus (SLE).

Methods

Images were obtained using fundus photography, fluorescein angiography, near-infrared reflectance (NIR) imaging, and optical coherence tomography (OCT).

Results

The patient had a history of SLE and central retinal artery occlusion in her right eye. Fundus examination showed no specific retinochoroidal abnormalities, with the exception of optic disc atrophy in her right eye and a peripapillary small hemorrhage in her left eye. However, NIR revealed multiple bright patchy lesions in the choroid of the posterior pole and the mid-periphery of the fundus in both eyes. OCT demonstrated irregular hyperreflectivity at the lesion sites.

Conclusions

The observed choroidal abnormalities are highly specific findings and therefore indicative of neurofibromatosis type 1 (NF1). Since the coexistence of SLE and NF1 is extremely rare, this case provided the chance to examine the relationship between SLE and NF1.Key words: Systemic lupus erythematosus, Choroidal abnormality, Near-infrared reflectance, Neurofibromatosis type 1     

En face OCT angiography demonstrates flow in early type 3 neovascularization (retinal angiomatous proliferation)

Introduction

The characteristics of type 3 neovascularization (NV), also known as retinal angiomatous proliferation, have been well described clinically, as well as with fluorescein angiography (FA), indocyanine green angiography, and optical coherence tomography (OCT). OCT angiography (OCT-A) is a novel and non-invasive technique for imaging retinal microvasculature by detecting changes, with respect to time, in reflectivity related to blood flow.

Method

In this case series, we describe two patients who presented with type 3 NV and underwent clinical examination and multimodal imaging, including OCT-A.

Results

In the first patient, OCT-A demonstrated flow within two separate lesions in the same eye, one of which was only weakly detected by FA. In the second patient, sequential OCT-A demonstrated a reduction in intralesional flow following intravitreal therapy.

Conclusions

OCT-A may have a role in the early diagnosis of type 3 NV and in assessing the response to treatment. Further studies are needed to determine sensitivity and specificity.     

Long-term efficacy of infliximab on background vascular leakage in patients with Beh?et's disease

Purpose

To evaluate the effect of infliximab over the initial 4 years of treatment on inflammatory ocular attacks and background retinal/disc vascular leakage in patients with refractory uveoretinitis associated with Behçet''s disease.

Methods

Clinical records of nine patients were retrospectively reviewed. The main outcomes analyzed were frequency of ocular inflammatory attacks, background retinal and disc vascular leakage as assessed by fluorescein angiography during periods of clinical quiescence, best-corrected visual acuity, and adverse effects.

Results

The median follow-up on infliximab was 50 months (range 48–58 months). Mean frequency of attacks decreased significantly in years 1, 2, 3, and 4 compared with the baseline 1-year period before infliximab use. Mean background retinal and disc vascular leakage scores also decreased significantly at the end of each 1-year period compared with baseline. Visual acuity improved or was unchanged at the end of 4 years in 17 of 18 eyes. No serious adverse effects were observed.

Conclusion

Infliximab reduced the mean frequency of ocular attacks and mean background retinal/disc vascular leakage in a long-term sustained manner over 4 years of treatment in Behçet''s disease patients.     

Changes in retinal nerve fibre layer, optic nerve head morphology, and visual field after acute primary angle closure

Aims/Purpose

To determine and correlate the long-term changes in retinal nerve fibre layer (RNFL) thickness, optic nerve head (ONH) morphology, and visual fields after a single episode of acute primary angle closure (APAC).

Methods

This was a cross-sectional comparative study of patients at National University Hospital (Singapore) from 2000 to 2006 after an episode of unilateral APAC. The peripapillary and macular RNFL were measured using Stratus optical coherence tomography (OCT) and ONH configuration was assessed using Heidelberg Retina Tomography (HRT)-III. Humphrey perimetry was also performed, and the presence of disc pallor was noted. APAC eyes were compared with fellow eyes as matched controls.

Results

Twenty-five patients were assessed at a median of 33 months (range, 11–85 months) after APAC. OCT showed that there was a reduction in the peripapillary and outer macular RNFL thickness in APAC eyes compared with controls. Humphrey perimetry revealed significantly reduced mean deviation (P=0.006) and increased pattern standard deviation (P=0.045) in APAC eyes compared with controls. HRT-III showed no difference in mean rim area, rim volume, or cup–disc ratio between APAC eyes and controls. Disc pallor was present in nine APAC eyes (36%) but was absent in fellow eyes (P=0.002), and was associated with peripapillary RNFL thinning, visual field loss, and an increased interval between the onset of symptoms and normalization of intraocular pressure (P=0.023).

Conclusion

APAC results in peripapillary and outer macular RNFL loss, visual field defects, and optic disc pallor, even in cases in which the ONH configuration remains unchanged.