島田 佳明

OBJECTIVE: To report a case of bilateral reversible optic neuropathy as the first sign of Waldenström macroglobulinemia (WM). METHODS: Observational case report. RESULTS: A 52-year-old man had a sudden loss of vision in the left eye. Examinations revealed the presence of a serum monoclonal immunoglobulin (IgM kappa) in the serum. Even after a session of steroid pulse therapy, optic neuropathy became bilateral and then resolved almost completely after 4 months. The condition progressed to WM with multiorgan lesions years later. There was no evidence of optic neuropathy recurrence. The literature revealed two cases of monoclonal gammopathy (MG): a 64-year-old man with multiple myeloma (MM) with IgA lambda and a 51-year-old man with MM with IgG kappa. These cases have similar conditions: 1) visual reduction as an initial symptom of MG, 2) bilateral involvement, 3) no sign of central nervous system (CNS) infiltration shown by normal brain magnetic resonance images, and 4) recovery to a visual acuity of ≥1.0 bilaterally with no reoccurrence. The excessive Igs or B-cell hyperactivity may activate an autoimmune mechanism that reversibly interferes with the bilateral optic nerves. CONCLUSION: Bilateral optic neuropathy was the initial symptom of WM. There was no evidence of CNS infiltration; it recovered and then did not reoccur. The pathogenesis remained unknown, but two cases of MG were reported in the literature with remarkably similar conditions.

PURPOSE: This study reports a case of unilateral retinopathy with extinguished full-field ERGs (ffERGs), wherein the visual acuity was 16/16 and the visual field was spared. METHODS: Observational case report. RESULTS: A 39-year-old female had developed nyctalopia in her left eye. Two years later, she visited an ophthalmologist who noted a bilaterally reduced pigmentation of the fundus. Her best-corrected visual acuity was 16/16 in both the eyes. Goldmann perimetry demonstrated that her visual field was bilaterally fully spared. ffERGs measurement was performed in accordance with the ISCEV standard protocol and indicated that her right eye was normal. However, all ERG responses were severely attenuated in her left eye. Multifocal ERG responses were found to be normal in the right eye and extinguished in the left eye except for residual responses that were exclusively located at the center. During the 7 years of the follow-up period, the visual field in the left eye, which was once normal, became shaded, and the development of a ring scotoma was identified. The visual field in the right eye is still full. CONCLUSIONS: The pathogenesis of this patient's condition still remains unknown, while unilateral retinitis pigmentosa, unilateral pigmentary retinopathy, acute zonal occult outer retinopathy, and autoimmune retinopathy can all be considered as possible explanations. The uniqueness of this case study is that the extinguished ERG responses are predictive of the functional alteration in the affected eye, when the initial visual acuity and the visual field were normal.

Background: The acute physiological changes induced by focal retinal photocoagulation (PC) have been largely unexplored. Methods: This was a case-series study. We recorded multifocal electroretinograms (mfERGs) just before PC, and mfERGs were also recorded 5′, 15′, one hour, 24 h, and one week after the PCs. Transient changes of mfERGs were analyzed in eyes which underwent PCs to treat diabetic macular edema. The mfERGs recorded from the predominantly irradiated area and that from non-irradiated areas were analyzed separately. Results: Fifteen eyes of 15 patients were included in this study. The mfERGs elicited from non-irradiated areas did not change after PC, but the mfERGs elicited from the irradiated area changed with time; the amplitude was larger at 60′ than that before (p < 0.05) and at 5′ after PC (p < 0.01) and significantly smaller at 24 h and 1 week than that before and at 60′ after the PC (p < 0.01). The implicit time was significantly prolonged after PC. mfERG on irradiated area with the severe diabetic change was less altered after PCs. Conclusions: The transient increase in the amplitude at 60′ likely resulted from a biological amplification of partially damaged cells adjacent to the PC spots. The mfERGs manifested the dynamic alterations of the retinal function following PCs.

Objective: To report a case of unintentional retina artery amputation during macular peeling. Methods: Observational case report Results: A 73-year-old female underwent surgery to treat epiretinal membrane (ERM) associated with severe staphyloma in her left eye. While the ERM/posterior hyaloid membrane visually enhanced with triamcinolone (TA) was peeled, a movement of the forceps unintentionally involved the inferior temporal branch artery near the inner edge of the staphyloma. The artery was avulsed and amputated. Oozing from the retinal cleft that had once enfolded the artery and microscopic bleeding from the amputation stump were observed. The vitreous hemorrhage obscured a view of the fundus more than 4 weeks after the surgery. After 8 weeks, postoperative visual acuity was improved; however, the superior nasal visual field was lost, and the patient was aware of the broken vessel as a floater in her vision. Conclusions: Macular peeling is technically challenging, so meticulous attention must be paid to avoid any damage on vessels. The retina tissue was stretched in a staphyloma and vessels were bulged into the vitreous space especially at the inner edge of the staphyloma. High levels of TA dye here buried the texture of the retina. Excessive TA should be removed prior to macular peeling.

The use of a conjunctival retractor that expands a small incision in the conjunctiva/Tenon's capsule facilitated the performance of strabismus surgery through a fornix incision. A wide field of exposure revealed the entire area of the muscle insertion. While there is a risk of conjunctival tearing in elderly patients, the use of the retractor is valuable.

PURPOSE: To determine adherence to face-down positioning (FDP) among patients who underwent vitrectomy and gas tamponade for macular hole (MH) repair. METHOD: A total of 69 patients (37 females and 32 males) who underwent primary vitrectomy for MH repair were studied. Nurses recorded whether the patient complied with FDP each time they examined the patient. FDP score was obtained from the nursing records; patients were given a score of 1 if they complied with the FDP and 0 if they did not. The score was recorded four times per day for the first 3 postoperative days. A perfect FDP score was 12. RESULTS: The mean ± standard deviation of the FDP scores was 10.6±1.8 (range: 4-12). Overall, 32 (46.4%) patients scored a perfect 12, and 7 (10.1%) patients scored <8. Failure of the MH closure was observed in only one patient (1.4%), who showed the poorest adherence to FDP (score =4). Consequently, the closure rate in patients with FDP score <7 (2/3, 66.7%) was significantly lower than in patients with an FDP score ≥7 (66/66, 100%) (P<0.05, Fisher's exact probability test). CONCLUSION: While adherence to FDP after MH surgery was better than that observed after vitrectomy for rhegmatogenous retinal detachments in our previous study, the percentage of patients with FDP scores <8 did not differ. Statistically, the poor adherence to FDP can negatively impact the effectiveness of the surgery for MH repair.

Leber hereditary optic neuropathy (LHON) causes visual loss, predominantly in healthy young men. We recently examined a patient who previously had bilateral macular holes and subsequently developed LHON at 74 years of age. Although his central scotomas were initially attributed to the macular holes, his visual acuity declined following an initial improvement after operative closure of the macular holes; thus, other diagnoses, including LHON, were considered. Furthermore, macular optical coherence tomography (OCT) images remained unchanged in this time. A mitochondrial genetic analysis identified a 11778G→A mutation. From this case, we propose that LHON remains in the differential diagnosis even in older patients, as has previously been reported.

We report a case of a 38-year-old man who presented with a recently self-detected lump under his left eyebrow. Previous ophthalmological history was unremarkable except for unilateral high myopia (left eye) since childhood. The appearance of the left eye was seemingly normal; however, with the top lid pulled up on downward gaze, a dark brown bulge emerged. The bulge was 10 × 7 mm and approximately 4 mm in height, and was covered by the extended superior rectus muscle. The diagnosis of equatorial staphyloma was made after coronal T1-weighted magnetic resonance imaging of the orbit revealed the dilatation of the vitreous cavity. Ocular movements were fully maintained and visual acuity was largely spared: 20/15 in the right eye without correction and 20/25 in the left eye with -10.00 spheres and -4.00 × 80 degrees cylinders. His past and family histories were unremarkable; however, small neurofibromas and café au lait spots all over his body led to the diagnosis of neurofibromatosis type 1 (NF1). From this case, similar to previous reports, we suggest that manifestations of NF1 are extremely variable and unpredictable.

A healthy 5-year-old girl presented with bilateral hyphema as an initial symptom of uveitis. The ocular findings and the patient's age suggested that the uveitis was due to juvenile rheumatic arthritis or chronic iridocyclitis.

<jats:p>To investigate the macular contribution to the amplitude and latency of pattern visual evoked cortical potentials (PVECPs), we recorded PVECPs in patients with unilateral macular disease (retinal diseases involving the macular region) and optic neuritis. We selected patients with visual acuities better than 0.3, which is thought to be the minimum acuity to provide clearly discernible responses. The visual stimulus was a checkerboard. We varied the check size in 4 steps as 7, 14, 28, and 56 min of arc, and the contrast was 20% or 80%. The amplitude of the sinusoidal wave of steady‐state VECPs (12 Hz) and the P100 component of a transient VECP (3 Hz) were measured. Significant attenuation and delay of PVECPs in the affected eye were found in the macular disease group. Macular disease may cause an increase of VECP latency. However, VECP seemed to be a more sensitive test of optic nerve disease than of macular disease, when patients had similar visual acuities.</jats:p>