Mizuo Matsui

Bietti's crystalline retinopathy

The authors previously reported four cases with crystalline retinopathy and suggested that it be classified into three stages: 1) retinal pigment epithelium (RPE) atrophy in the posterior pole, 2) choriocapillaris atrophy appearing in the enlarged areas of RPE atrophy, and 3) prominent diffuse atrophy of both the RPE and choriocapillaris. We followed up 4 cases, including 2 cases which were previously been reported, and have now evaluated the clinical features of this disease. The results indicated that the above classification was clinically useful. Enlargement of the RPE atrophy from stage 2 to 3 was due to enlargement and confluence of the RPE atrophy between the posterior pole and the periphery. In addition, disturbances in ERG and color vision were thought to correlate with secondary dysfunction at the level of the photoreceptor cell layer within the diseased lesions.

Indocyanine Green Videoangiographic Findings in Detachment of the Retinal Pigment Epithelium

BACKGROUND Several forms of retinal pigment epithelial detachment have been reported. The authors used indocyanine green (ICG) videoangiography, which is useful to study the choroidal vasculature and Bruch membrane, to study pigment epithelial detachments. METHODS Ninety-eight pigment epithelial detachments in 75 eyes were classified based on the appearance of choroidal neovascular membranes or late phase findings of ICG videoangiography done at the initial examination. The authors also followed the evolution of 51 such detachments not associated with choroidal neovascularization (CNV). RESULTS Sixty-four pigment epithelial detachments without CNV were divided into five groups. Among eyes with pigment epithelial detachments that showed intense hyperfluorescence, all except one of the patients had both eyes involved and had several pigment epithelial detachments, sometimes with exudative retinal detachments. Weak hyperfluorescence was observed more often in younger patients. During follow-up of eyes with pigment epithelial detachments that showed irregular hypofluorescence, a neovascular membrane developed in one eye, microrips developed in four eyes and retinochoroidal folds in one eye. Most eyes that showed irregular hyperfluorescence developed atrophy of the retinal pigment epithelium. In 34 pigment epithelial detachments with CNV, either irregular hypofluorescence or absence of fluorescence was observed in areas that corresponded to the pigment epithelial detachment. CONCLUSION The intense hyperfluorescence is thought to be due to the accumulation of protein-rich fluid within the pigment epithelial detachment. Most pigment epithelial detachments that showed weak fluorescence probably represent variants of central serous choroidopathy. Pigment epithelial detachments that showed irregular hypofluorescence or hyperfluorescence were associated with age-related macular degeneration, and the former was correlated closely with CNV. Close follow-up therefore is recommended for eyes with pigment epithelial detachments that show irregular hypofluorescence.

Indocyanine green video angiographic findings in acute posterior multifocal placoid pigment epitheliopathy

Abstract. The placoid lesions of acute posterior multifocal placoid pigment epitheliopathy consist of two distinct areas: a light yellow area surrounding a dark yellow center. On two occasions, the authors compared indo‐cyanine green video angiographic with fluorescein angiographic findings in a typical case of acute posterior multifocal placoid pigment epitheliopathy. In the acute stage, the multiple placoid lesions observed in the early phase of indocyanine green video‐angiography showed hypofluorescent lesions corresponding to those seen with fluorescein angiography which blocked choroidal vessels. Some fluorescent intermediate sized choroidal vessels, which traversed the hypofluorescent areas, were dark due to a slight degree of blocked fluorescence. On both indocyanine green video and fluorescein angiography, hypofluorescent lesions were almost the same size as the light yellow area and the dark yellow center of a placoid lesion. A hypofluorescent lesion, seen on fluorescein angiography, obscured a retinal vessel. When the placoid lesions had disappeared, fewer hypofluorescent lesions, corresponding to some of the hypofluorescent lesions originally observed, were seen in the early phase of indocyanine green video angiography. They were also smaller in size. These hypofluorescent lesions decreased in size and some had completely disappeared in the late phase. No hypofluorescent lesions were seen in the early phase of fluorescein angiography. Findings in the acute stage suggest that the hypofluorescence observed in the early phase of indocyanine green video and fluorescein angiography is mainly blockage due to a light yellow area of placoid. When the placoid lesion disappeared, filling delays, which were detectable on only indocyanine green video angiography, persisted in the choriocapillaris.

Multicenter genetic study of retinitis pigmentosa in Japan: I. Genetic heterogeneity in typical retinitis pigmentosa☆

A nationwide, multicenter study of typical retinitis pigmentosa (RP) was carried out in collaboration with 18 hospitals throughout Japan to obtain current information for genetic counseling. We analyzed the genetic heterogeneity of RP based on the parental consanguinity of 434 probands registered during a 6-month period in 1990. A gradual decline in the frequency of consanguineous marriage was recognized among the normal parents of RP patients. The relative frequencies of inheritance patterns were estimated as: autosomal recessive, 25.2%; autosomal dominant, 16.9%; X-linked, 1.6%; and simplex, 56.3%. A comparison of these results with previous reports in Japan revealed a decline in the relative frequency of autosomal recessive cases and an increase in simplex cases. This suggests a decrease in the incidence of autosomal recessive retinitis pigmentosa in Japan, as well as the necessity for exhaustive investigations aimed at identifying inheritance patterns for RP patients seeking genetic counseling.

Multicenter genetic study of retinitis pigmentosa in Japan: II. Prevalence of autosomal recessive retinitis pigmentosa

Retinitis pigmentosa (RP) is a group of genetically heterogeneous diseases with autosomal recessive (AR), autosomal dominant, and X-linked modes of inheritance. Autosomal recessive retinitis pigmentosa (ARRP) is the most common form in Japan. A genetic analysis was done to determine the prevalence of ARRP indirectly, to provide an estimation of changing trends in the overall prevalence of RP. Data on the frequency of consanguinity and marriage year of normal parents of 59 ARRP patients were obtained from a nationwide multicenter survey of typical retinitis pigmentosa conducted in 1990. The gene frequency of ARRP was 0.01145 (Dahlbergs formula). In 1990, the number of young symptomatic ARRP patients decreased, while the number of patients aged 40 years and older increased. The total number of symptomatic ARRP patients in 1990 was nearly 21% higher than in 1970. Despite a dramatic decline in consanguinity in recent decades in Japan, the number of ARRP patients has increased. This increase is attributed to greater longevity and overall population growth. Our results suggest that the total number of RP patients has not decreased, and may even have increased.

Indocyanine green video-angiographic findings in choroidal osteoma

Indocyanine green video-angiography (ICG) was done twice on three patients with choroidal osteoma. Choroidal osteoma consisted of two portions, a yellow-white area and a surrounding orange area. The early phase of ICG angiography showed hypofluorescence due to blockage, corresponding to the osteoma. However, the hypofluorescence was greater in areas corresponding to the orange portions of the lesions than in the yellow-white parts. In the late phase, tumor staining was present, corresponding to the orange area. Although long-standing yellow-white lesions blocked fluorescence in the late phase, some of these showed hyperfluorescence. ICG video-angiography showed areas of blocked fluorescence in the early phase as well as hyperfluorescent areas accompanied by several small hypofluorescent foci in the late phase, corresponding to very slight ophthalmoscopically observed changes in the vicinity of the osteoma. During follow-up observation, fluorescein angiography confirmed the extension of the osteoma into this area. These results confirm that the characteristic findings of choroidal osteoma can be observed with ICG video-angiography. This imaging technique showed the lesions were larger than they appeared with ophthalmoscopy and fluorescein angiography.

Clinical evaluation of indocyanine green video-angiography in the diagnosis of choroidal neovascular membrane associated with age-related macular degeneration

The Authors compared ICG video-angiographic findings in 85 eyes of 78 cases of presumed choroidal neovascular membrane (CNM) associated with age-related macular degeneration with those diagnosed by fluorescein angiography. The detection rate of CNM was 68% with indocyanine green (ICG) video-angiography and 59% with fluorescein angiography. ICG video-angiography can be especially useful for identifying hemorrhagic or exudative detachments of the retinal pigment epithelium. In these cases, detection of CNM by ICG video-angiography was generally far superior to that by fluorescein angiography. However, some CNM in which leakage was identified with fluorescein angiography could not be visualized by ICG video-angiography.

Vitrectomy for Diabetic Macular Heterotopia

PURPOSE The authors performed 15 vitrectomies for diabetic macular heterotopia, and then compared visual prognoses and postoperative complications with those of 88 macular detachments to determine the role of, and indications for, vitrectomy for diabetic macular heterotopia. METHODS Fifteen patients with diabetic macular heterotopia and 88 with traction macular detachment, in which the vitreous cavities were sufficiently clear for posterior poles to be observed, underwent vitrectomies. Preoperative and postoperative visual acuity and postoperative complications were assessed and documented retrospectively. RESULTS There was no statistically significant difference between the two groups in terms of eyes showing improvement in visual acuity postoperatively. However, a final postoperative visual acuity better than 20/20 was documented in 93% of patients with macular heterotopia and 48% of patients with macular detachment (P < 0.002), whereas 47% of the former and 10% of the latter had visual acuities better than 20/40 (P < 0.001). Postoperative neovascular glaucoma and retinal detachment developed in 10% and 13%, respectively, of patients with macular detachments. None of the patients with macular heterotopia experienced these complications. CONCLUSION Based on the above results, the authors conclude that diabetic macular heterotopia is a very good indication for early vitrectomy.

Symptomatology of neuroretinal dystrophy

Fluorescein angiographic findings of two cases are presented: one was probably a kind of expanded phakomatoses, and the other was a Battens disease. In the first case, fluorescein angiography and CT scan findings were presented; the latter suggested the existence of a hemangioma. In the second case fluorescein angiography and ophthalmoscopic findings were found to correspond well with the histo-pathological findings. It was also proved that in this disease, not only retinal neural elements, but also choriocapillaris and choroidal vessels were damaged and contributed to the development of the fundus abnormalities

Clinical Dose Finding Study of Indocyanine Green to Detect Choroidal Neovascularization in Patients with Exudative Age-related Macular Degeneration

PURPOSE To evaluate the proper clinical dosage of indocyanine green(ICG) in angiography for detecting choroidal neovascularization(CNV) of exudative age-related macular degeneration(AMD). SUBJECTS AND METHODS Indocyanine green angiography (IA) was performed using a randomized crossover method with two different doses on two occasions. Ease of detection, side effects and clinical serum data were also evaluated. RESULTS Among the 39 eyes, detection of CNV using 12.5 mg and 25 mg was most effective in 21 and 31 eyes respectively, showing a statistically significant difference. Slight vomiting was observed temporarily in one patient who had taken 25 mg. CONCLUSION A dose of 25 mg is appropriate for detection of CNV of exudative AMD and this dosage raises no safety concerns.