[Development of thrombocytopenic purpura following BNT162b2 mRNA COVID-19 vaccination].

Because the coronavirus disease 2019 (COVID-19) pandemic is still rampant, vaccination is being promoted worldwide. However, the safety of various COVID-19 vaccines remains poorly understood. We herein report the case of a 37-year-old woman who experienced thrombocytopenia following BNT162b2 mRNA COVID-19 vaccination. The patient presented with purpura on the extremities 10 days after the first vaccination. She had marked thrombocytopenia and no thrombosis. Thrombocytopenia resolved spontaneously. Given the possibility of occurrence of post-vaccination thrombocytopenia, vaccinated persons should be instructed to consult a medical institution if they experience bleeding symptoms.

Prevalence and spatial distribution of cystoid spaces in retinitis pigmentosa: investigation with spectral domain optical coherence tomography.

PURPOSE: To investigate the prevalence and spatial distribution of cystoid spaces (CS) in retinitis pigmentosa patients with spectral domain optical coherence tomography. METHODS: A total of 529 eyes of 275 patients with retinitis pigmentosa were examined with spectral domain optical coherence tomography. The presence or absence of CS was judged for each eye. Retinal layer and outer retinal status where the CS existed were also investigated. Statistical analysis was performed using 1 eye per 1 patient. RESULTS: Cystoid spaces were present in 119 of 529 eyes (22.5%) of 74 of 275 patients (26.9%). There were no significant differences between the cases with and without CS except for central foveal thickness (P < 0.001). Cystoid spaces were noted in the inner nuclear layer in almost all eyes (98.6%), and outer nuclear layer/outer plexiform layer was also involved in many eyes (27.8%). Cystoid spaces were sometimes seen in ganglion cell layer (6.9%). Cystoid spaces were predominantly (78.9%) distributed in the relatively preserved retina where external limiting membrane was retained. The presence of epiretinal membrane or posterior vitreous adhesion was associated with the presence of CS (P < 0.001) but showed no relationship with the spatial location of CS (P = 1.000). CONCLUSION: The prevalence of CS in patients with retinitis pigmentosa was 26.9% and contrary to previous reports, most CS were present in inner nuclear layer. In addition, most CS were observed in relatively retained retina, which is compatible to the prevailing notion. Epiretinal membrane or posterior vitreous adhesion was also associated with the development of CS. The distribution of CS in inner and preserved retina may provide insight for the pathogenesis of CS in retinitis pigmentosa.

Low-dose-rate, low-dose irradiation delays neurodegeneration in a model of retinitis pigmentosa.

The existence of radiation hormesis is controversial. Several stimulatory effects of low-dose (LD) radiation have been reported to date; however, the effects on neural tissue or neurodegeneration remain unknown. Here, we show that LD radiation has a neuroprotective effect in mouse models of retinitis pigmentosa, a hereditary, progressive neurodegenerative disease that leads to blindness. Various LD radiation doses were administered to the eyes in a retinal degeneration mouse model, and their pathological and physiological effects were analyzed. LD gamma radiation in a low-dose-rate (LDR) condition rescues photoreceptor cell apoptosis both morphologically and functionally. The greatest effect was observed in a condition using 650 mGy irradiation and a 26 mGy/minute dose rate. Multiple rounds of irradiation strengthened this neuroprotective effect. A characteristic up-regulation (563%) of antioxidative gene peroxiredoxin-2 (Prdx2) in the LDR-LD-irradiated retina was observed compared to the sham-treated control retina. Silencing the Prdx2 using small-interfering RNA administration reduced the LDR-LD rescue effect on the photoreceptors. Our results demonstrate for the first time that LDR-LD irradiation has a biological effect in neural cells of living animals. The results support that radiation exhibits hormesis, and this effect may be applied as a novel therapeutic concept for retinitis pigmentosa and for other progressive neurodegenerative diseases regardless of the mechanism of degeneration involved.

Granulocyte colony-stimulating factor attenuates oxidative stress-induced apoptosis in vascular endothelial cells and exhibits functional and morphologic protective effect in oxygen-induced retinopathy.

Granulocyte colony-stimulating factor (G-CSF) is a known hematopoietic glycoprotein, and recent studies have revealed that G-CSF possesses other interesting properties. Oxidative stress is involved in many diseases, such as atherosclerosis, heart failure, myocardial infarction, Alzheimer disease, and diabetic retinopathy. This study was designed to examine whether G-CSF has a protective effect on endothelial cells against oxidative stress and to investigate whether G-CSF has a therapeutic role in ischemic vascular diseases. Expression of G-CSF (P < .01) and G-CSF receptor (P < .05) mRNA in human retinal endothelial cells (HRECs) was significantly up-regulated by oxidative stress. Treatment with 100 ng/mL G-CSF significantly reduced H(2)O(2)-induced apoptosis in HRECs from 61.7% to 41.4% (P < .05). Akt was phosphorylated in HRECs by G-CSF addition, and LY294002, a PI3K inhibitor, significantly attenuated the antiapoptotic effect of G-CSF (by 44.1%, P < .05). The rescue effect was also observed in human umbilical vein endothelial cells. In mouse oxygen-induced retinopathy model, G-CSF significantly reduced vascular obliteration (P < .01) and neovascular tuft formation (P < .01). G-CSF treatment also clearly rescued the functional and morphologic deterioration of the neural retina. A possibility of a novel therapeutic strategy for ischemic diseases through attenuating vascular regression using G-CSF was proposed.

Two-year outcome of photodynamic therapy combined with intravitreal injection of bevacizumab and triamcinolone acetonide for polypoidal choroidal vasculopathy.

PURPOSE: To compare the 2-year results after photodynamic therapy (PDT) alone and PDT combined with intravitreal injections of bevacizumab and triamcinolone acetonide (triple therapy) for polypoidal choroidal vasculopathy (PCV). METHODS: We retrospectively reviewed the medical records of 40 consecutive patients (40 eyes) with subfoveal PCV. Of these 40 eyes, 16 were treated with PDT alone and 24 were treated with triple therapy. RESULTS: The change in visual acuity in the triple therapy group was significantly better than that in the PDT group (P < 0.001). At 24 months, improvement in visual acuity was seen in only two eyes (12.5 %) of the PDT group, while it was seen in ten eyes (41.7 %) of the triple therapy group. Retreatment was given to 12 eyes (75.0 %) in the PDT group and to nine eyes (37.5 %) in the triple therapy group, although the retreatment-free period was significantly longer in the triple therapy group than in the PDT group (P < 0.001). Post-treatment vitreous hemorrhage was seen in only two eyes (12.5 %), all of which were in the PDT group. CONCLUSION: Compared with PDT alone, triple therapy appears to reduce the postoperative hemorrhagic complications and recurrences of PCV and to improve the 2-year visual outcomes of PCV.

The time course changes of choroidal neovascularization in angioid streaks.

PURPOSE: To assess the clinical course of choroidal neovascularization (CNV) in patients with angioid streaks using optical coherence tomography and fluorescein angiography/indocyanine green angiography. METHODS: We examined a consecutive series of 88 eyes of 44 patients with angioid streaks using color fundus photography, optical coherence tomography, and fluorescein angiography/indocyanine green angiography. RESULTS: At the initial visit, 33 eyes exhibited no CNV, 2 exhibited polypoidal choroidal vasculopathy, 8 exhibited Type 1 CNV, 32 exhibited active Type 2 CNV, and 13 exhibited a fibrotic scar. In addition to the 2 eyes that exhibited macular polypoidal choroidal vasculopathy at the initial visit, 3 exhibited peripapillary polypoidal lesions, and 2 exhibited polypoidal lesions at the edge of the preexisting Type 2 CNV/fibrosis. During the follow-up, Type 2 CNV developed in 4 eyes on the basis of Type 1 CNV. Visual acuity was worse in eyes with Type 2 CNV and fibrosis than in those with Type 1 CNV, while polypoidal choroidal vasculopathy did not affect the visual acuity. CONCLUSION: Eyes with angioid streaks can develop any form of CNV including polypoidal choroidal vasculopathy. Considering the worse visual acuity in eyes with Type 2 CNV and fibrosis, patients should be carefully observed so as to treat them promptly when Type 2 CNV occurred beneath the fovea.

[Genotype screening of retinal dystrophies in the Japanese population using a microarray].

PURPOSE: To investigate the pathogenic variants of retinal dystrophies in the Japanese population using microarray analysis. SUBJECTS AND METHODS: DNA extracted from the blood samples of 84 families (87 patients) with retinal dystrophies (retinitis pigmentosa, Leber congenital amaurosis, cone-rod dystrophy and Bietti's crystalline retinopathy) was screened by Asper Biotech services. All the variants detected by microarray analysis were verified by direct sequencing. RESULTS: Mutations were detected in 2 of 36 families with autosomal dominant retinitis pigmentosa, 2 of 4 with Leber congenital amaurosis, 11 of 24 with cone-rod dystrophy, 3 of 7 with macular dystrophy and 6 of 7 with Bietti's crystalline retinopathy. CONCLUSION: Genotype screening using microarray analysis can be effectively used to determine the variants of retinal dystrophies, except retinitis pigmentosa, in the Japanese population.

Patient with Adult T-cell Leukemia and Lung Infection caused by Mycobacterium abscessus: Successful Treatment with Intensive Chemotherapy Followed by Haploidentical Hematopoietic Stem Cell Transplantation: A Case Report.

A 63-year-old woman with adult T-cell leukemia (ATL) lymphomatous type developed a mild dry cough. Computed tomography revealed lung lesions with a tree-in-bud appearance during intensive chemotherapy. Antibodies against Mycobacterium avium complex were positive. Bronchoalveolar lavage culture showed growth of M. abscessus complex. Finally, M. abscessus subsp. massiliense was also identified. Sequential use of antimicrobials, including macrolides, was introduced during intensive chemotherapy, and the patient successfully underwent allogeneic hematopoietic stem cell transplantation (AHSCT). This is the first case report of a patient with ATL complicated by M. massiliense lung infection, who was successfully treated with haploidentical AHSCT using various combinations of antimicrobials.

Knockout of ccr2 alleviates photoreceptor cell death in a model of retinitis pigmentosa.

Neuroinflammation involving CC chemokines such as monocyte chemoattractant protein-1 (MCP-1) has been demonstrated in the pathological process of retinitis pigmentosa (RP), an inherited degenerative retinal disease. However, the mechanism of MCP-1 and its receptor CCR2 involvement in the disease remains unclear. To investigate the role of MCP1/CCR2 in RP pathogenesis, ccr2 mutant RP mice (ccr2(-/-) rd10) were created and analyzed. The expression of MCP-1, RANTES, stromal cell-derived factor (SDF-1), and tumor necrosis factor-α (TNF-α) in the retinas of wild-type, rd10, and ccr2(-/-) rd10 mice was analyzed using quantitative RT-PCR. Photoreceptor apoptosis (TUNEL staining) and the number of microglia (positive for the F4/80 antibody) in the retina were examined. Retinal function was assessed using electroretinograms, and the structure of the whole retina was analyzed from images obtained using optical coherence tomography (OCT) and by histological examination. The expression levels of MCP-1, RANTES, and SDF-1 increased with time in the rd10 mice but not in the wild-type mice. Rearing the mice in the dark prevented degeneration and resulted in thicker photoreceptor layers at each time point. In those mice, the peaks of chemokine expression shifted to a later time with degeneration, suggesting that the expression of these chemokines was induced during the progression of degeneration. Although the difference was not so obvious, the retina in the ccr2(-/-) rd10 mice was consistently and significantly thicker than that in the rd10 (ccr2(+/+) rd10) mice at all time points. Rhodopsin gene expression was also higher in the ccr2(-/-) rd10 mice than in rd10 (ccr2(+/+) rd10) mice, suggesting photoreceptor survival in the former. Retinal function was also better preserved in the ccr2(-/-) rd10 mice than in the rd10 mice. The number of microglia in the retinas of the ccr2(-/-) rd10 mice was significantly lower than that in the retinas of the rd10 mice. Interestingly, the MCP-1 induction that was observed in the retinas of the rd10 mice was diminished in the retinas of the ccr2(-/-) rd10 mice. Our results suggest that the MCP-1/CCR2 system plays a role in retinal degeneration in rd mouse retinas. Retinal MCP-1 expression in the rd mouse retina may be partially controlled by ccr2-positive circulating cells.

Retinal structural alterations and macular sensitivity in idiopathic macular telangiectasia type 1.

PURPOSE: To evaluate the relationship between the abnormalities of retinal structures showed on spectral-domain optical coherence tomography and the changes in the macular sensitivity measured by microperimetry in eyes with idiopathic macular telangiectasia type 1. METHODS: Eleven eyes of 11 patients with macular telangiectasia type 1 were reviewed. Morphologic changes in the retina and retinal sensitivity of eyes with macular telangiectasia type 1 were studied using spectral-domain optical coherence tomography and microperimetry. RESULTS: Spectral-domain optical coherence tomographic images revealed disruptions in the photoreceptor inner segment-outer segment (IS/OS) junction in all the eyes and intraretinal cystoid spaces in 10 eyes. In the points that had intraretinal cystoid spaces, the mean retinal sensitivity was 6.8 ± 5.5 dB where the IS/OS was disrupted and 13.4 ± 4.0 dB where the IS/OS was intact (P < 0.001). In the points that had intact IS/OS and no cystoid spaces, the mean retinal sensitivity was 15.3 ± 4.3 dB, which was better than that of points that had intact IS/OS with cystoid spaces (P < 0.001). CONCLUSION: Retinal sensitivity is influenced not only by intraretinal cystoid spaces but also by IS/OS disruptions, and the IS/OS alterations reduce the visual function more severely.

Genetic variants in pigment epithelium-derived factor influence response of polypoidal choroidal vasculopathy to photodynamic therapy.

PURPOSE: To investigate whether photodynamic therapy (PDT) outcomes of polypoidal choroidal vasculopathy (PCV) are related to baseline clinical characteristics, smoking history, or genetic factors by analyzing the retreatment-free period after the first PDT. DESIGN: Retrospective cohort study. PARTICIPANTS: The study consisted of 167 patients with PCV who underwent PDT as their first treatment. METHODS: We targeted 638 single nucleotide polymorphisms (SNPs) in 42 possible susceptible genes for age-related macular degeneration to evaluate their relation to the effectiveness of PDT for PCV. For this evaluation, we used 2 methods: (1) survival analysis, with the retreatment-free period as the target; and (2) logistic regression test between the need for additional therapy within 3 months after the first PDT and the genotypes, with age, gender, smoking status, and greatest linear dimension (GLD) at baseline as covariates. The contributions of smoking status and GLD at baseline for the retreatment-free period also were evaluated. Contributions of these factors to visual prognosis were evaluated for 1 year after PDT. MAIN OUTCOME MEASURES: Retreatment-free period after the first PDT for PCV. Secondary outcome measures included correlation of the susceptible factor to the retreatment requirement within the 3-month follow-up and the mean visual acuity change. RESULTS: In survival analyses, SERPINF1 rs12603825 showed a significant association with the retreatment-free period after the first PDT; those patients homozygous for the minor allele A of rs12603825 received additional treatment after PDT within significantly shorter times than those with other genotypes (P = 0.0038). There was no significant difference in the retreatment-free period between baseline GLD and smoking status. Retreatment within 3 months was required significantly more in patients with the AA genotype, even after taking into consideration the effect of clinical characteristics (age, gender), baseline PCV lesion size, and smoking status (P = 0.0027). Furthermore, patients with the AA genotype showed significantly worse visual prognosis after PDT (P = 0.013). CONCLUSIONS: Pigment epithelium-derived factor (SERPINF1 or PEDF) polymorphisms may influence the initial response to and visual prognosis after PDT for PCV. Our findings may lead to understanding the pathogenesis of PCV and modification of the effects of PDT.

Bone marrow-derived stem cells target retinal astrocytes and can promote or inhibit retinal angiogenesis.

Adult bone marrow (BM) contains cells capable of differentiating along hematopoietic (Lin(+)) or non-hematopoietic (Lin(-)) lineages. Lin(-) hematopoietic stem cells (HSCs) have recently been shown to contain a population of endothelial precursor cells (EPCs) capable of forming blood vessels. Here we show that intravitreally injected Lin(-) BM cells selectively target retinal astrocytes, cells that serve as a template for both developmental and injury-associated retinal angiogenesis. When Lin(-) BM cells were injected into neonatal mouse eyes, they extensively and stably incorporated into forming retinal vasculature. When EPC-enriched HSCs were injected into the eyes of neonatal rd/rd mice, whose vasculature ordinarily degenerates with age, they rescued and maintained a normal vasculature. In contrast, normal retinal angiogenesis was inhibited when EPCs expressing a potent angiostatic protein were injected. We have demonstrated that Lin(-) BM cells and astrocytes specifically interact with one another during normal angiogenesis and pathological vascular degeneration in the retina. Selective targeting with Lin(-) HSC may be a useful therapeutic approach for the treatment of many ocular diseases.

Retinal microstructural abnormalities in central serous chorioretinopathy and polypoidal choroidal vasculopathy.

PURPOSE: To compare retinal morphologic alterations in eyes with polypoidal choroidal vasculopathy (PCV) and central serous chorioretinopathy (CSC) using speckle noise-reduced spectral-domain optical coherence tomography. METHODS: We retrospectively reviewed 63 eyes of 62 patients with active PCV and 38 eyes of 38 patients with active CSC. Patients underwent fundus photography, angiography, and speckle noise-reduced spectral-domain optical coherence tomography examinations, and retinal morphologic alterations were evaluated. RESULTS: Cystoid macular edema, lipid deposits, subretinal hemorrhage, and hemorrhagic pigment epithelial detachment were not seen in any eye with CSC but were seen in eyes with PCV. In PCV, mean visual acuity was significantly poorer in eyes with fibrin infiltration (P = 0.027) or hemorrhagic infiltration (P = 0.002) in the fovea than in eyes without fibrin or hemorrhagic infiltration. CONCLUSION: Differentiating factors between PCV and CSC noted on spectral-domain optical coherence tomography include a lack of cystoid macular edema, lipid deposition, subretinal hemorrhage, and hemorrhagic pigment epithelial detachment in eyes with CSC, which makes spectral-domain optical coherence tomography helpful in differentiating CSC from PCV. More severe retinal alterations were seen in PCV than in CSC because of infiltration of fibrin and hemorrhage in the outer retina, which also correlated with poorer vision.

Bone marrow-derived cells are differentially involved in pathological and physiological retinal angiogenesis in mice.

PURPOSE: Bone marrow-derived cells have been shown to play roles in angiogenesis. Although these cells have been shown to promote angiogenesis, it is not yet clear whether these cells affect all types of angiogenesis. This study investigated the involvement of bone marrow-derived cells in pathological and physiological angiogenesis in the murine retina. MATERIALS AND METHODS: The oxygen-induced retinopathy (OIR) model was used as a retinal angiogenesis model in newborn mice. To block the influence of bone marrow-derived cells, the mice were irradiated with a 4-Gy dose of radiation from a (137)Cs source. Irradiation was performed in four different conditions with radio dense 2-cm thick lead disks; (1) H group, the head were covered with these discs to protect the eyes from radiation; (2) A group, all of the body was covered with these discs; (3) N group, mice were completely unshielded; (4) C group, mice were put in the irradiator but were not irradiated. On P17, the retinal areas showing pathological and physiological retinal angiogenesis were measured and compared to the retinas of nonirradiated mice. RESULTS: Although irradiation induced leukocyte depletion, it did not affect the number of other cell types or body weight. Retinal nonperfusion areas were significantly larger in irradiated mice than in control mice (P<0.05), indicating that physiological angiogenesis was impaired. However, the formation of tuft-like angiogenesis processes was more prominent in the irradiated mice (P<0.05), indicating that pathological angiogenesis was intact. CONCLUSIONS: Bone marrow-derived cells seem to be differentially involved in the formation of physiological and pathological retinal vessels. Pathological angiogenesis in the murine retina does not require functional bone marrow-derived cells, but these cells are important for the formation of physiological vessels. Our results add a new insight into the pathology of retinal angiogenesis and bolster the hypothesis that bone marrow cells are involved in the pathology or severity of retinal angiogenic diseases.

Sterile endophthalmitis after intravitreal injection of bevacizumab obtained from a single batch.

PURPOSE: The purpose of this study was to report 14 consecutive cases of endophthalmitis after intravitreal injection of bevacizumab (Avastin; Roche, Basel, Switzerland) obtained from a single batch. METHODS: One vial of bevacizumab (100 mg/4 mL) was divided into 20 sterile injections and kept at 4 degrees C before use. Bevacizumab (1.25 mg/0.05 mL) was injected intravitreally into 19 eyes of 15 patients to treat macular edema or choroidal neovascularization. All treatments were performed within 1 week of the bevacizumab, which was from a single batch, being aliquotted into the 20 doses. RESULTS: Of the 19 eyes, 14 showed moderate to severe ocular inflammation immediately after injection. Cultures of aqueous humor and vitreous from 5 eyes were negative for bacteria and fungi. Eyes with moderate inflammation received topical or systemic antibiotics and steroid treatment. Five eyes with severe inflammation underwent pars plana vitrectomy because of dense vitreous opacity. Visual acuity returned to preendophthalmitis levels in 12 eyes but had decreased in 2 eyes at 1 month after the injection. CONCLUSION: Intravitreal injection of bevacizumab can cause sterile endophthalmitis. Most inflammation occurred within a few days after the intravitreous injection of the bevacizumab, but treatment with antibiotics, steroids, and/or vitrectomy was effective, and the prognosis was good in most cases.

Activation of bone marrow-derived microglia promotes photoreceptor survival in inherited retinal degeneration.

The role of microglia in neurodegeneration is controversial, although microglial activation in the retina has been shown to provide an early response against infection, injury, ischemia, and degeneration. Here we show that endogenous bone marrow (BM)-derived microglia play a protective role in vascular and neural degeneration in the retinitis pigmentosa model of inherited retinal degeneration. BM-derived cells were recruited to the degenerating retina where they differentiated into microglia and subsequently localized to the degenerating vessels and neurons. Inhibition of stromal-derived factor-1 in the retina reduced the number of BM-derived microglia and accelerated the rate of neurovascular degeneration. Systemic depletion of myeloid progenitors also accelerated the degenerative process. Conversely, activation of BM-derived myeloid progenitors by systemic administration of both granulocyte colony-stimulating factor and erythropoietin resulted in the deceleration of retinal degeneration and the promotion of cone cell survival. These data indicate that BM-derived microglia may play a protective role in retinitis pigmentosa. Functional activation of BM-derived myeloid progenitors by cytokine therapy may provide a novel strategy for the treatment of inherited retinal degeneration and other neurodegenerative diseases, regardless of the underlying genetic defect.

Improved visualization of polypoidal choroidal vasculopathy lesions using spectral-domain optical coherence tomography.

PURPOSE: To report the tomographic features of vascular lesions beneath the retinal pigment epithelium in eyes with polypoidal choroidal vasculopathy by using spectral-domain optical coherence tomography (SD-OCT). DESIGN: Retrospective observational case series. METHODS: Angiograms and images obtained using the prototype SD-OCT system were compared for 21 eyes of 21 patients with polypoidal choroidal vasculopathy to identify sub-retinal pigment epithelium abnormalities visible on three-dimensional and enhanced SD-OCT images. RESULTS: On angiography, a branching vascular network and at least 1 polypoidal lesion were visible in all 21 eyes; 10 eyes also had pigment epithelial detachment (PED). SD-OCT revealed a thin straight line of high reflectivity-Bruch's membrane-associated with the branching vascular network in all 21 eyes, polypoidal lesions, 19 (90%) of the 21 eyes; and PED, 9 (90%) of the 10 eyes with PED. The vascular abnormalities of polypoidal choroidal vasculopathy (polypoidal lesion and branching vascular network) identified with angiograms were visualized on SD-OCT images in 20 of the 21 eyes (95%) as areas of moderate reflectivity between the clearly delineated abnormal section of retinal pigment epithelium and Bruch's membrane. CONCLUSIONS: Enhanced SD-OCT imaging clearly depicted Bruch's membrane beneath areas of abnormal retinal pigment epithelium in the same locations where the vascular abnormalities of polypoidal choroidal vasculopathy were evident on angiography.

[Circulating bone marrow-derived stem cells in patients with polypoidal choroidal vasculopathy].

PURPOSE: The current study was designed to investigate the role of circulating bone marrow (BM)-derived stem cells in the pathogenesis of polypoidal choroidal vasculopathy (PCV), a distinct type of neovascular age-related macular degeneration (AMD). METHODS: Thirty one clinically documented PCV patients were enrolled. Circulating BM-derived stem cells were collected from the patients' peripheral blood and cultured. Colony forming capacity (Hill assay) and migration activity (Boyden chamber system) were examined and analyzed. RESULTS: Colony forming units (CFU-Hill) were significantly fewer in bilateral PCV patients than in unilateral PCV patients. CFU-Hill was impaired in patients with larger (> 5000 microm) PCV lesions compared with patients with smaller PCV lesions. Migration activity of BM-derived stem cells was also reduced significantly in the bilateral PCV patients than in the unilateral PCV patients. CONCLUSIONS: Similar to CNV associated with AMD, impaired functional activity of circulating BM-derived stem cells was observed in PCV patients with bilateral or larger lesions. Circulating BM-derived stem cells may have a role in the pathogenesis of PCV.

Macular complications on the border of an inferior staphyloma associated with tilted disc syndrome.

PURPOSE: To describe the clinical characteristics of macular complications on the border of an inferior staphyloma associated with tilted disk syndrome. METHODS: We reviewed retrospectively the medical records of 32 consecutive eyes of 20 patients with tilted disk syndrome and an inferior staphyloma lying across the macula. RESULTS: In 21 (66%) eyes, fluorescein angiography showed window defects on the border of the staphyloma, where the early phase of indocyanine green angiography showed hypofluorescence due to atrophy of the choriocapillaris. On the late phase of indocyanine green angiography, 19 eyes (59%) showed hyperfluorescence along the border of the staphyloma, which often extended beyond the area of the window defect. Of the 32 eyes, 25 (78%) had macular complications: polypoidal choroidal vasculopathy in 7 (22%), classic choroidal neovascularization in 1 (3%), focal serous retinal detachment without polypoidal choroidal vasculopathy or choroidal neovascularization in 13 (41%), and atrophy of the retinal pigment epithelium alone in 4 (13%). Visual acuity in eyes with polypoidal choroidal vasculopathy or choroidal neovascularization was significantly worse than that in eyes with other complications (P < 0.001). CONCLUSIONS: Eyes with tilted disk syndrome often have macular complications on the border of the inferior staphyloma, which can cause severe visual loss.

Macular polypoidal choroidal vasculopathy with a remote lesion.

PURPOSE: To report cases of the macular type of polypoidal choroidal vasculopathy with a remote lesion. METHODS: We report six patients (seven eyes) with polypoidal choroidal vasculopathy who had macular and remote lesions. These eyes were examined with angiography and tomography. RESULTS: All seven eyes showed an exudative macular lesion beneath the fovea. In addition, all eyes showed remote polypoidal lesions that were not connected to the macular lesions; the remote lesion was detected outside of the vascular arcade in five eyes, superotemporally beside the optic disc in one eye and on the nasal side of the optic disc in one eye. Indocyanine green angiography, fluorescein angiography and optical coherence tomography failed to reveal any sign of a branching vascular network or choroidal neovascularization that connected the macular lesion with the more remote lesion. At the initial visit, visual acuity in the seven eyes ranged from 6/150 to 6/9 (median, 6/15). Four eyes underwent photodynamic therapy to the exudative macular lesion. During 27.6 +/- 14.3 months of follow up, no worsening was detected in any of the remote lesions. Median visual acuity was 6/60 at the final visit. CONCLUSIONS: Some patients with macular polypoidal choroidal vasculopathy also have a remote lesion, although the remote lesion seems to have only a minor effect on visual outcome.