Focal Functional and Microstructural Changes of Photoreceptors in Eyes with Acute Zonal Occult Outer Retinopathy

Purpose: Acute zonal occult outer retinopathy (AZOOR) is characterized by an acute zonal loss of outer retinal function with minimal ophthalmoscopic changes in one or both eyes. We present a patient with AZOOR whose ultrastructural and functional findings were followed for 8 months. Case: A 22-year-old woman developed an acute central scotoma in her right eye. Her best-corrected visual acuity (BCVA) was 0.5 OD and 1.2 OS. The ophthalmoscopic examinations, fluorescein angiography, and full-field electroretinograms (ERGs) were normal in both eyes. The amplitudes of the multifocal ERGs (mfERGs) were attenuated in the area corresponding to the scotoma. Spectral domain optical coherence tomography showed an absence of both the inner and outer segment (IS/OS) line of the photoreceptors and the cone outer segment tip (COST) line between the IS/OS line and the retinal pigment epithelium. These changes were seen in the area corresponding to the scotoma. One month later, the scotoma disappeared and the BCVA improved to 1.2 OD. The mfERGs increased to almost the same amplitude as the fellow eye. The IS/OS line became discernible but the COST line was still absent. The ophthalmological findings of the right macula remained normal during the 11-month follow-up period. Conclusions: Our findings indicate that the selective loss of the IS/OS and the COST lines is probably the morphological alterations corresponding with the reduced BCVA and the mfERGs in the areas of the visual field defects in the acute phase of AZOOR. But in the recovery phase, only the abnormality of the COST line is a subclinical sign for the disease. These findings should be important in understanding and evaluating the pathological mechanism in other outer retinal diseases

Case of Unilateral Peripheral Cone Dysfunction

Purpose: Peripheral cone dystrophy is a subgroup of cone dystrophy, and only 4 cases have been reported. We present a patient with unilateral peripheral cone dysfunction and report the functional changes determined by electrophysiological tests and ultrastructural changes determined by spectral domain optical coherence tomography (SD-OCT). Case: A 34-year-old woman complained of blurred vision in both eyes. Our examination showed that her visual acuity was 0.05 OD and 0.2 OS. A relative afferent pupillary defect was present in her right eye. The results of slit-lamp examination, ophthalmoscopy, and fluorescein angiography were normal except for pallor of the right optic disc. SD-OCT showed a diffuse thinning of the retina in the posterior pole of the right eye. A severe constriction of the visual fields was found in both eyes but more in the right eye. The photopic full-field electroretinograms (ERGs) were reduced in the right eye but normal in the left eye. The multifocal ERGs were severely reduced throughout the visual field except in the central area of the right eye. The multifocal ERGs from the left eye were normal. The pattern visual evoked responses were within the normal range in both eyes. She had a 5-year history of sniffing paint thinner. Results: Although the visual dysfunction was initially suspected to be due to psychological problems from the results of subjective tests, objective tests indicated a peripheral cone dysfunction in the right eye. The pathophysiological mechanism and the relationship with thinner sniffing were not determined. Conclusions: Our findings indicate that peripheral cone dysfunction can occur unilaterally. Electrophysiology and SD-OCT are valuable tests to perform to determine the pathogenesis of unusual ocular findings objectively

Pars Plana Vitrectomy Combined with Focal Endolaser Photocoagulation for Idiopathic Macular Telangiectasia

Background. To report the outcome of pars plana vitrectomy (PPV) combined with intraoperative endolaser focal photocoagulation (PC) on eyes with idiopathic macular telangiectasis (MacTel) type 1. Methods. This was a retrospective study of two female patients with MacTel type 1 who were resistant to focal photocoagulation, sub-Tenon triamcinolone injection, and/or antiangiogenic drugs. The best-corrected visual acuity (BCVA) was determined, and fluorescein angiography (FA) and spectral domain optical coherence tomography (SD-OCT) were performed before and after surgery for up to 19 months. Results. After surgery, the BCVA gradually improved from 20/100 to 20/20 at 19 months in Case 1 and from 20/50 to 20/13 at 13 months in Case 2. Fluorescein angiography (FA) showed leakage at the late phase, and OCT showed that the cystoid macular edema was resolved and the fovea was considerably thinner postoperatively. Conclusion. Patients with MacTel type 1 who are refractory to the other types of treatments can benefit from PPV combined with intraoperative endolaser focal PC with functional and morphological improvements

Effect of Intraocular Lens Diameter Implanted in Enucleated Porcine Eye on Intraocular Pressure Induced by Scleral Depression

The effect of the diameter of an intraocular lens (IOL) implanted in enucleated porcine eyes on the intraocular pressure induced by scleral depression was investigated. Two IOLs of 6 mm and 7 mm optic diameter were implanted. The intraocular pressure (IOP) was monitored during scleral depression by a transducer placed in the midvitreous through a sclerotomy at 6 o’clock. The area under the curve (AUC) of the IOP changes from the beginning of the indentation to the point when the peripheral retinal surface was observed through the IOL optics was measured. The AUC was significantly larger in eyes with a 6 mm IOL than in eyes with a 7 mm IOL (p<0.05). The IOP elevation at the endpoint was higher in eyes with the 6 mm IOL than in eyes with the 7 mm IOL. We conclude that the AUC may represent the degree of stress induced by scleral depression. The higher AUC value with the X-60 may be because of the longer distance from the peripheral retina to the edge of the IOL optics

Tissue Plasminogen Activator-Assisted Vitrectomy for Ruptured Eye with Suprachoroidal Hemorrhage

Purpose: To report a case of a ruptured eye with a suprachoroidal hemorrhage (SCH) in which tissue plasminogen activator (tPA)-assisted vitrectomy was successful in reconstructing the globe and restoring good vision. Case: A 32-year-old man was struck on the right eye by a surfboard. His eye was ruptured and his visual acuity decreased to hand movements. Surgery was immediately performed to successfully close the ruptured globe. Nine days later, a second surgery was performed, and tPA (25 µg/0.1 ml monteplase) was used to liquefy and drain the SCH. This freed enough vitreous space for a more comprehensive vitrectomy. Eighteen months after the injury, the retina remained attached, and the decimal best-corrected visual acuity improved to 0.8. Conclusion: tPA was helpful in lysing a massive SCH, thereby contributing to the excellent visual outcome. tPA-assisted drainage should be considered in cases of massive SCH when drainage is difficult due to an incomplete lysis of the clot

Assessment of Macular Function during Vitrectomy: New Approach Using Intraoperative Focal Macular Electroretinograms.

To describe a new technique to record focal macular electroretinograms (FMERGs) during vitrectomy to assess macular function.Intraoperative FMERGs (iFMERGs) were recorded in ten patients (10 eyes) who undergo vitrectomy. iFMERGs were elicited by focal macular stimulation. The stimulus light was directed to the macular area through a 25 gauge (25G) glass fiber optic bundle. Background light was delivered through a dual chandelier-type light fiber probe. Focal macular responses elicited with combinations of stimulus and background luminances were analyzed.A stimulus luminance that was approximately 1.75 log units brighter than the background light was able to elicit focal macular responses that were not contaminated by stray light responses. Thus, a stimulus luminance of 160 cd/m2 delivered on a background of 3 cd/m2 elicited iFMEGs from only the stimulated area. This combination of stimulus and background luminances did not elicit a response when the stimulus was projected onto the optic nerve head. The iFMERGs elicited by a 10° stimulus with a duration of 100 ms and an interstimulus interval of 150 ms consisted of an a-, b-, and d-waves, the oscillatory potentials, and the photopic negative response (PhNR).Focal ERGs with all components can be recorded from the macula and other retinal areas during vitreous surgery. This new technique will allow surgeons to assess the function of focal areas of the retina intraoperatively

Heads-Up 3D Surgery under Low Light Intensity Conditions: New High-Sensitivity HD Camera for Ophthalmological Microscopes

Purpose. To determine the feasibility of performing intraocular surgeries in a heads-up position with low illuminance conditions by observing a display of the surgical field created by a three-dimensional imaging (3D) system. Methods. Seventy-four eyes of 56 patients underwent cataract surgery (72 eyes) with the heads-up 3D surgery system; 60 eyes with cataract surgery alone, 7 eyes with combined cataract and glaucoma microdevice implant surgery, 5 eyes with combined cataract and vitrectomy surgery, and two eyes with vitrectomy surgery alone were studied. The illuminance from the surgical microscope was set to be dimmer (Leica M822F40 main light 2%; otto-flex 6%) than the usual setting to minimize the discomfort and glare for the patient. The surgeries were performed under topical anesthesia. The luminance of the images observed through the eyepieces of the operating microscope and the image of a 3D system created by a high-sensitivity sensor Exmor R 3CMOS HD camera (Sony MCC-1000MD) were measured. Results. All surgeries were completed without any complications under the low illumination conditions. The surgical field on the display monitor was created by a 3D system using a high-sensitivity sensor camera and was observed in a heads-up position. The patients did not report any intolerable discomfort or glare during the surgery. Cataract surgeries were performed with a good view of the surgical field under the extremely low illumination from the surgical microscope. The high-sensitivity sensors and electronic amplifications of the image signals made the surgical field brighter and allowed the surgeon to perform the surgery confidently and safely. Conclusions. Heads-up, 3D-assisted intraocular surgeries can be performed safely and efficiently with low illuminance of the surgical field. This trial is registered with UMIN000037838