A new insight into pachychoroid diseases: Remodeling of choroidal vasculature.

PURPOSE: Pachychoroid spectrum diseases are regarded as being different manifestations of a common pathogenic process. We suggest that pachychoroid diseases are consequences of chronic vortex vein stasis. METHODS: We describe how we came to this conclusion based on our own recent reports as well as a search of the related literature. RESULTS: Central serous chorioretinopathy (CSC) is the first stage of pachychoroid spectrum diseases. CSC is caused by congestion of choroidal veins, which are branches of the vortex veins. The venous outflow tract of the choroid is divided into four quadrants, based on horizontal and vertical watershed zones, with one or two vortex veins in each quadrant being independently responsible for venous outflow. In acute CSC, vortex vein stasis frequently causes asymmetric dilatation of the vortex veins in the horizontal watershed. The area of geographic filling delay in the choriocapillaris coincides with the area of this asymmetrically dilated vortex veins. With chronic stasis of the vortex veins, venous anastomosis occurs in the watershed zone as a means of compensating for the stasis, and the choriocapillaris becomes occluded in the area of filling delay. The anastomotic vessels dilate, becoming often hyperpermeable, and are then recognizable as pachyvessels. With the development of choriocapillaris ischemia, choroidal neovascularization (CNV) occurs at the site of pachyvessels. This is termed pachychoroid neovasculopathy (PNV). Polypoidal choroidal vasculopathy is regarded as a variant of PNV. CONCLUSIONS: Intervortex venous anastomosis is among the key factors underlying the development of pachychoroid diseases. Remodeling of the venous drainage route though the anastomosis across the watershed zones is apparently a common response to chronic vortex vein stasis.

Variation of vortex veins at the horizontal watershed in normal eyes.

PURPOSE: To assess the vascular pattern of choroidal vortex veins at the horizontal watershed zone in normal eyes using optical coherence tomography (OCT). METHODS: We retrospectively studied 207 normal eyes of 207 patients whose fellow eyes were diagnosed with unilateral retinal diseases without choroidal involvement. Venous anastomosis between the superior and inferior vortex veins and deviation of the horizontal watershed zone were evaluated using 12 × 12-mm en face OCT images. Central choroidal thickness (CCT) was measured on B-mode OCT images. RESULTS: Vortex vein anastomosis was observed in 92 eyes (44.4%) at the horizontal watershed zone. Superior or inferior deviation of the horizontal watershed was ascertained in 69 eyes (33.3%). The frequency of the anastomosis and deviation did not differ significantly between age groups (P = 0.56 and 0.96, respectively). Mean CCT of all eyes was 221 ± 80 µm. CCT was significantly greater in eyes with anastomosis than in those without (233 ± 73 µm vs 210 ± 83 µm, P < 0.05). However, CCT did not differ significantly between eyes with and without deviation of the horizontal watershed zone (223 ± 74 µm vs 219 ± 82 µm). CONCLUSIONS: Venous anastomosis at the horizontal watershed zone as well as superior or inferior deviation of the zone were frequently observed in normal eyes. CCT was greater in eyes with than in those without anastomosis, suggesting subclinical vortex vein congestion.

Clinical characteristics of pachydrusen in central serous chorioretinopathy.

PURPOSE: To evaluate the clinical characteristics of pachydrusen in central serous chorioretinopathy (CSC) and investigate the relationship between choroidal circulation and pachydrusen. METHODS: In a retrospective case series of 302 eyes of 151 patients with treatment-naïve CSC, we assessed the incidence of pachydrusen and their features on indocyanine green angiography (ICGA) and optical coherence tomography (OCT). RESULTS: Pachydrusen were observed in 82 of the 302 eyes (27.2%). The patients with pachydrusen were significantly older than those without pachydrusen. In 36 of the 82 eyes with pachydrusen, the choriocapillaris perfusion phase of ICGA was recorded. Pachydrusen were localized within the geographic filling delay of the choriocapillaris in 26 of the 36 eyes (72.2%). In the late phase of ICGA, pachydrusen corresponded to punctate hyperfluorescent spots in 69 of the 82 eyes (84.1%) and localized within sites of choroidal vascular hyperpermeability in 45 eyes (54.9%). En face OCT revealed pachydrusen to be localized over the dilated outer choroidal vessels in 70 of the 82 eyes (85.4%). B-mode OCT showed pachydrusen under the retinal pigment epithelium (RPE) in 72 of the 82 eyes (87.8%). There was no significant difference in central choroidal thickness between eyes with and without pachydrusen. CONCLUSIONS: Pachydrusen in patients with CSC were frequently localized within the choriocapillaris filling delay and over the dilated outer choroidal vessels. Moreover, they were frequently observed under the RPE and corresponded to punctate hyperfluorescent spots on ICGA. These findings suggest that inner choroidal circulation impairment due to dilatation of outer choroidal vessels might induce pachydrusen.

Optical coherence tomography patterns and outcomes of contusion maculopathy caused by impact of sporting equipment.

BACKGROUND: To describe the patterns and outcomes of contusion maculopathy after ocular contusions resulting from accidental impact with sporting equipment. METHODS: We conducted a retrospective study of interventional case series. PATIENT POPULATION: Twenty-one eyes of 21 patients who sustained blunt ocular trauma while playing a sport. Intervention/Observation Procedure(s): Surgery or observation by optical coherence tomography (OCT). MAIN OUTCOME MEASURE(S): The morphologic changes within the macula in the early stages after injury and changes in visual function in the early and recovery stages after injury. RESULTS: In the early stage, OCT visualized four injury patterns: type Ι, commotio retinae (14.3%, 3 eyes) with increased reflectivity of the ellipsoid zone and retinal pigment epithelium; type II, incomplete macular hole(38.1%, 8 eyes) with three structural changes, i.e., a partial V-shaped macular hole, a jar-shaped macular hole with retinal tissue at the bottom, and a connective bridge attached to retinal tissues; type III, full-thickness macular hole (33.3%, 7 eyes); and type IV, foveal hemorrhage (14.3%, 3 eyes). During recovery, OCT images of types Ι and II showed almost normal macular morphology with better visual acuity (mean ± SD,0.02 ± 0.1 and 0.14 ± 0.21logMAR.). In types III and IV, the visual prognosis was poor (0.52 ± 0.34 and 0.22 ± 0.16), OCT images showed retinal atrophy at the fovea despite vitrectomy and sulfur hexafluoride (SF6) gas tamponade. CONCLUSION: Early OCT images identified four patterns of contusion maculopathy with different treatment outcomes. In types Ι and II, the visual function and retinal morphology remained intact. With types III and IV, respectively, the treatments of vitrectomy and SF6 gas tamponade for patients were effective.

Genetic analysis of varicella-zoster virus in the aqueous humor in uveitis with severe hyphema.

BACKGROUND: Genetic variations have been identified in the genome of varicella-zoster virus (VZV) strains using vesicle fluid, varicella scabs and throat swab samples. We report a rare case of VZV-associated uveitis with severe hyphema, which was immediately diagnosed by polymerase chain reaction (PCR) using the aqueous humor, in which we were able to analyze the VZV genotype for the first time. CASE PRESENTATION: A 16-year-old Japanese boy was referred to our hospital with a 20-day history of unilateral anterior uveitis and 11-day history of hyphema. At presentation, details of the iris, the iridocorneal angle, and the fundus were not visible due to the severe hyphema. Serum anti-VZV IgG and anti-VZV IgM were elevated, and 1.61 × 109 copies/mL of VZV-DNA were detected by real-time PCR using the aqueous humor. As there were no eruptions on his face or body, we diagnosed zoster sine herpete and started intravenous administration of prednisolone and acyclovir. The hyphema completely disappeared 2 weeks after presentation, while sectorial iris atrophy and mild periphlebitis of the fundus became gradually apparent. Anterior inflammation and periphlebitis gradually improved and VZV-DNA in the aqueous humor was reduced to 1.02 × 106 copies/mL at 4 weeks after presentation. Examination by slit lamp microscope revealed no inflammation after 5 months, and VZV-DNA could no longer be detected in the aqueous humor. Serum anti-VZV IgG and anti-VZV IgM also showed a gradual decrease along with improvement in ocular inflammation. The genetic analysis of multiple open reading frames and the R5 variable repeat region in the VZV genes, using DNA extracted from the aqueous humor at presentation, showed that the isolate was a wild-type clade 2 VZV strain (prevalent in Japan and surrounding countries) with R5A allele and one SNP unique to clade 1 (both are major types in Europe and North America). CONCLUSIONS: VZV-associated uveitis may develop hyphema that obscures ocular inflammation, thus PCR analysis using the aqueous humor is the key investigation necessary for the diagnosis. The measurement of VZV-DNA copies by real-time PCR would be useful for evaluation of therapeutic effects. We could amplify and analyze VZV genotype using the aqueous humor including a very large number of VZV-DNA copies (1.61 × 109 copies/mL).

Cytomegalovirus retinitis followed by immune recovery uveitis in an elderly patient with rheumatoid arthritis undergoing administration of methotrexate and tofacitinib combination therapy.

Cytomegalovirus (CMV) retinitis is an opportunistic ocular infection most commonly observed in patients infected with human immunodeficiency virus (HIV). We present a rare case of CMV retinitis that developed in a non-HIV patient with rheumatoid arthritis (RA). Over the preceding 5 months, a family doctor had been treating the 78-year-old male patient with a combination therapy of methotrexate (MTX) and tofacitinib (TOF). CMV retinitis occurred when the patient's CD4+ T cells were low (196 cells/µl), and preceded the onset of Pneumocystis pneumonia. MTX and TOF were stopped after the diagnosis of CMV retinitis. While intravenous and intravitreal ganciclovir administration significantly improved the CMV retinitis, uveitis developed 3 months later during the maintenance therapy with oral valganciclovir, concomitantly with the recovery of the CD4+ T cell counts. As we believed this uveitis was caused by the immune reconstitution mechanism, we treated the patient with a retrobulbar injection of corticosteroids. During the 6 months following the cessation of MTX and TOF, there was no flare-up of the RA. Cases of CMV retinitis and immune recovery uveitis in RA patients have been rarely reported in the literature. In the current case, the intensive immunosuppressive therapy in this elderly patient might have been the cause of this unusual opportunistic complication of RA.

Acute ganglion cell loss during rapid visual recovery in optic neuritis.

PURPOSE: To report dynamic changes in the retinal ganglion cell layer (GCL) and visual function in acute and chronic optic neuritis (ON). METHODS: Sixteen eyes (15 patients) with acute ON were followed for 3.5 to 31 months (average, 10.2). The best-corrected visual acuity (BCVA) and thickness of the GCL plus the inner plexiform layer (GCL+IPL) were measured 4 to 13 times between baseline and the final visit using the ganglion cell analysis software in the Cirrus HD-OCT [high-definition optical coherence tomography] instrument. Goldmann perimetry was performed at baseline and at the final visit. RESULTS: The thickness of the GCL+IPL at baseline was within normal limits in the affected (80.4 ± 4.9 microns) and unaffected fellow eyes (80.5 ± 5.0 microns). Rapid thinning to 69 ± 7.3 microns occurred during month 1 in the affected eyes, slowing during month 2, and then reaching a minimum level (63.6 ± 8.7 microns). In contrast, BCVA was lowest (mean ± standard deviation logarithm of the minimum angle of resolution, -1.29 ± 0.96) in 11 eyes at baseline, increased markedly to -0.15 ± 0.37 during month 1, and reached a maximum level (-0.18 ± 0.19) during month 2 and (-0.02 ± 0.23) at the final visit. The BCVA in the other five eyes fluctuated during month 1, increased markedly during month 2, and then reached a maximum plateau (-0.07 ± 0.20). The patterns of visual field defects at baseline were varied, and were determinants of BCVA. The visual field largely recovered in 11 eyes, but small central scotomas in four eyes and an enlarged blind spot in one eye remained at the final visit. Eyes with the least GCL+IPL thinning at month 1 or 2 had the least depression in the final deviation map. CONCLUSIONS: In acute ON, the progression toward irreversible ganglion cell loss occurs rapidly during months 1 and 2. In contrast, visual function recovers rapidly during the same period. Remodeling of the neural network may occur between the photoreceptors and the reduced numbers of ganglion cells during the first months of ON. The small number of residual ganglion cells appears to compensate for the initial visual dysfunction.

TREAT-AND-EXTEND REGIMEN WITH AFLIBERCEPT FOR RETINAL ANGIOMATOUS PROLIFERATION.

PURPOSE: To evaluate the effects of aflibercept therapy using a treat-and-extend regimen on treatment-naïve retinal angiomatous proliferation (RAP) and development of retinal pigment epithelium (RPE) atrophy. METHODS: We retrospectively studied 17 treated eyes with RAP and 13 untreated fellow eyes. We assessed best-corrected visual acuity (BCVA) in logarithm of the minimal angle of resolution (logMAR) units and recorded the total number of injections for 12 months. Central macular thickness (CMT) and central choroidal thickness (CCT) were assessed by optical coherence tomography (OCT), and RPE atrophy extent in the macular area was assessed by fundus autofluorescence. RESULTS: Average BCVA in eyes with RAP was 0.57 logMAR units (Snellen 20/74 or approximately 56.5 ETDRS letters) before treatment and significantly improved to 0.38 (Snellen 20/48 or approximately 66 ETDRS letters, P < 0.01) after 3 months and 0.32 (Snellen 20/42 or approximately 69 ETDRS letters, P < 0.01) after 12 months. Average CMT was 340 µm before treatment and significantly reduced to 133 µm (P < 0.001) after 3 months and 130 µm (P < 0.001) after 12 months. Average CCT was 147 µm before treatment, 123 µm (P < 0.01) after 3 months, and 131 µm (P < 0.01) after 12 months. Average total number of injections was 7.2. Average area of RPE atrophy enlarged by 1.00 mm in treated eyes compared with 0.34 mm in fellow eyes (P < 0.01). The enlarged area of RPE atrophy was inversely correlated with central choroidal thickness after 12 months (rs = -0.49, P < 0.01) and positively correlated with the number of injections (rs = 0.58, P < 0.01). CONCLUSION: Treat-and-extend intravitreal therapy with aflibercept may be effective for improvement and stabilization of visual acuity and exudative change in eyes with RAP. However, choroidal thinning during the treatment regimen may accelerate enlargement of RPE atrophy.

A Case of Blunt Trauma of the Eyeball Associated With an Inferior Oblique Muscle and an Inferior Rectus Muscle Rupture.

Rupture of the extraocular muscle in the absence of significant injury to the eyeball and adnexa is uncommon. The authors report a case of blunt trauma of the eyeball associated with an inferior oblique muscle and an inferior rectus muscle rupture. A 55-year-old man slipped and fell down hitting his eye on an extended windshield wiper blade. Although he had treatment in the emergency room, he complained of diplopia in the primary position 1 day postoperatively. After noticing ruptures of the inferior oblique muscle and an inferior rectus muscle during exploratory surgery, the authors carefully repaired it. Diplopia in the primary position had disappeared within 1 month after the operation and by 6 months postoperatively. The movement of the eye had almost completely recovered.

Contact Lens-Related Infectious Keratitis with White Plaque Formation Caused by Corynebacterium propinquum.

We report the first case of Corynebacterium propinquum keratitis in the compromised cornea of a diabetic patient wearing therapeutic contact lenses. The strain was identified to the species level based on sequencing of the 16S rRNA gene and RNA polymerase ß-subunit-encoding gene (rpoB). Ophthalmologists should be aware of nondiphtherial corynebacterial infection of compromised corneas.

Repair mechanism of retinal pigment epithelial tears in age-related macular degeneration.

PURPOSE: To investigate repair mechanisms of retinal pigment epithelial (RPE) tears in age-related macular degeneration. METHODS: The authors retrospectively studied 10 eyes with age-related macular degeneration that developed RPE tears during follow-up or after treatment with an anti-vascular endothelial growth factor drug or photodynamic therapy combined with ranibizumab. After development of the RPE tears, all follow-ups exceeded 13 months. Spectral domain or swept-source optical coherence tomography have been used to examine consecutive retinal changes where the RPE tears developed and attempted to determine the repair mechanisms. RESULTS: Retinal pigment epithelial tears developed during the natural course (n = 4) after ranibizumab treatment (n = 2) and after photodynamic therapy and ranibizumab (n = 4). Subretinal fluid persisted for more than 6 months after the RPE tears developed (n = 4), with the area where the RPE was lost found to be covered with thickened proliferative tissue. In 6 eyes where the subretinal fluid was absorbed within 2 months, optical coherence tomography showed the outer retina appeared to be directly attached to Bruch membrane, and there was attenuation of the normal hyperreflective band attributable to normal RPE during follow-up. CONCLUSION: Results suggest that two repair processes may be present in the area where RPE tears developed. Persistent subretinal fluid may lead to repair with thick proliferative tissue, while the outer retina appears to attach to Bruch membrane when there is early subretinal fluid resolution after RPE tear development.

[The vitreous and the macula].

The macula is a site where various vitreoretinal disorders occur. In 1983 we started to observe the retinal surface of postmortem eyes with a scanning electron microscope (SEM). We investigated the anatomy of the vitreous in postmortem eyes by slit lamp biomicroscopy. The novel anatomy of the premacular vitreous led us to conduct a clinical study of vitreomacular interface diseases. In 1997, time domain optical coherence tomography(OCT) became available which facilitated visualization of the vitreoretinal interface. Swept source OCT which was introduced in 2012 can depict liquefied lacunae in the vitreous. It enabled us to elucidate the mechanism of vitreoretinal diseases. I. SEM revealed the remnants of vitreous cortex at fovea with high incidence (44%), which suggests strong vitreoretinal attachment at the fovea and vitreous cortex origin of the epiretinal membrane. II. We studied the anatomy of the vitreous in postmortem eyes. The vitreous of bisected eye balls was stained by fluorescein and immersed in water and observed by slit-lamp biomicroscopy. We discovered a "posterior precortical vitreous pocket (PPVP)" in adult eyes without posterior vitreous detachment (PVD). III. We performed clinical study in various vitreoretinal diseases based on the novel vitreous anatomy and explained their mechanism. 1. In diabetic retinopathy, ring shaped fibrovascular tissue surrounding the macula is formed along the outer margin of the PPVP. Although PVD progresses outside the PPVP, its posterior wall remains attached to the retina, which causes macular traction or cystoid macular edema. 2. In eyes with idiopathic epimacular membrane (IEM), detached vitreous cortex had an oval defect corresponding to the IEM. Posterior wall of the PPVP that is premacular vitreous cortex appeared to be the framework of IEM. 3. During vitrectomy for macular hole, premacular round defect appears when PVD is created. The residual cortex on the macula is fibrous membrane with elasticity. The tangential contraction of premacular cortex may generate anterior traction to the fovea, which leads to macular hole. IV. Using time domain OCT, we demonstrated the evolution of macular hole, myopic foveoschisis and lamellar macular hole. After 2007, we investigated age related changes of vitreoretinal interface by spectral domain OCT V. We demonstrated whole structure of the PPVP using swept source OCT. PPVP is a boat shaped premacular liquefied space which has a connecting channel to Cloquet's canal. PPVP develops during childhood. Visualization of vitreous structure proved that our previous assumptions are reasonable. Although the physiological function of the PPVP is unclear, we speculate that the aqueous flows into the PPVP though Cloquet's canal and the connecting channel.

Intravitreal gas injection without vitrectomy for macular detachment associated with an optic disk pit.

PURPOSE: To evaluate the clinical outcomes after gas tamponade without vitrectomy for retinal detachment associated with an optic disk pit using optical coherence tomography. METHODS: Intravitreal gas injection was performed on 8 consecutive patients (mean age, 35.0 years; range, 15-74 years) with unilateral macular detachment associated with an optic disk pit. A 0.3-mL injection of 100% sulfur hexafluoride 6 gas was carried out without an anterior chamber tap. Patients treated with gas injection were instructed to remain facedown for 5 days. RESULTS: Complete retinal reattachment after only gas tamponade was achieved in four out of eight eyes. The mean number of gas injections was 1.8. The mean best-corrected visual acuity before and after the treatment with gas tamponade was approximately 30/100 and 20/20, respectively. The period required for reattachment after final gas treatment was 12 months. There were no incidences of recurrence after complete reattachment by gas tamponade in any of the cases during the 94-month average follow-up period (range, 64-132 months). CONCLUSION: Gas tamponade appears to be an effective alternative method for macular detachment associated with an optic disk pit, even though the mechanisms of optic disk pit maculopathy are still unknown.

A hyporeflective space between hyperreflective materials in pigment epithelial detachment and Bruch's membrane in neovascular age-related macular degeneration.

BACKGROUND: The purpose of this study was to investigate the clinical characteristics of a hyporeflective space between hyperreflective materials in pigment epithelial detachment (PED) and Bruch's membrane in neovascular age-related macular degeneration (AMD) using spectral-domain optical coherence tomography (SD-OCT) or swept source optical coherence tomography (SS-OCT). METHODS: Among 223 patients with neovascular AMD, 227 eyes were studied retrospectively. Using SD-OCT or SS-OCT, we reviewed clinical characteristics of the space. RESULTS: Twenty-two (10%) of the 227 eyes showed a space between hyperreflective materials in PED and Bruch's membrane. In all spaces, fibrovascular changes of the choroidal neovascularization (CNV) membrane were seen on funduscopy, with OCT images showing the retinal pigment epithelium (RPE) above the space adhering tightly and continuously to the CNV membranes. Nineteen (86%) of the 22 eyes with this cleft also had serous retinal detachment or cystoid macular edema. Five eyes (23%) had an RPE tear during follow-up. CONCLUSIONS: A hyporeflective space between hyperreflective materials in PED and Bruch's membrane sometimes appears in neovascular AMD. The appearance of such a space may indicate residual activities of the hyperreflective materials.

Retinal ganglion cell analysis in multiple evanescent white dot syndrome.

BACKGROUND: Multiple evanescent white dot syndrome (MEWDS) is an acute and usually unilateral retinopathy that occurs predominantly in young adults. This report presents the outcomes of ganglion cell analysis (GCA) in MEWDS. CASE PRESENTATION: A 41-year-old woman was diagnosed as MEWDS in right eye. At her initial visit, the deviation map of the ganglion cell analysis showed there was a decrease of the ganglion cell layer (GCL) + inner plexiform layer (IPL) thickness in both eyes, even though her left eye was not affected. A 29-year-old woman was also diagnosed as MEWDS in right eye. Although the deviation map of ganglion cell analysis showed there was a decrease of the GCL + IPL thickness in both eyes at her initial visit, her right eye was not affected. CONCLUSION: GCA indicated there was a decrease (<1% of the distribution of normals) of the ganglion cell layer + inner plexiform layer thickness in both the affected and fellow eyes in 7 of 9 patients diagnosed as MEWDS in our hospital. Although the lesions responsible for MEWDS are thought to disrupt the photoreceptor outer segments, we observed changes in the inner retina in both the affected and fellow eye of MEWDS patients.

Topical voriconazole therapy of Purpureocillium lilacinum keratitis that occurred in disposable soft contact lens wearers.

The objective of this study was to describe 2 cases of keratitis caused by Purpureocillium lilacinum (formerly Paecilomyces lilacinus) that occurred in disposable contact lens users, which were successfully treated with topical voriconazole. Case 1 was a healthy 44-year-old woman, who wore weekly disposable contact lenses and had developed a superficial corneal infection in her right eye. For diagnosis, corneal scraping and molecular identification of the cultured pathogen were performed. A corneal smear revealed the presence of fungi. The pathogen was identified as P. lilacinum by traditional morphological identification of fungal culture, and this identification was confirmed by DNA sequencing of the ribosomal internal transcribed spacer (ITS) sequence. Therapy with topical fluconazole, topical pimaricin (natamycin), and oral itraconazole were ineffective. Topical voriconazole showed a significant effect, and the keratitis was successfully treated. Case 2 was a 43-year-old woman with bilateral recurrent peripheral corneal ulcers by meibomian gland dysfunction, who used therapeutic bandage contact lenses on her left eye. However, a corneal abscess with hypopyon occurred in the eye after 3 months. The microbial smear examination showed the presence of fungi and the fungal culture, and the DNA sequence of ITS region revealed that the causative agent was P. lilacinum. The susceptibility testing against antifungal agents showed that voriconazole was effective. The lesion improved gradually by topical voriconazole. As a conclusion, P. lilacinum keratitis can occur in disposable soft contact lens wearer. Early and accurate detection of the pathogenic organism is essential. Topical voriconazole was effective against P. lilacinum keratitis.

Inversion of Asymmetric Vortex Vein Dilatation in Pachychoroid Spectrum Diseases.

Purpose: Intervortex venous anastomosis is widely recognized as compensating for vortex vein congestion in pachychoroid spectrum diseases. However, determining the blood flow direction within the compensated drainage route is often challenging. Herein, we investigated the morphological patterns of vortex veins in eyes showing retrograde pulsatile vortex venous flow. Design: Retrospective observational case series. Subjects: Six hundred eighty-nine consecutive eyes with treatment-naive central serous chorioretinopathy, pachychoroid neovasculopathy, or polypoidal choroidal vasculopathy. Methods: We reviewed the clinical records of patients with these pachychoroid spectrum diseases. Multimodal images including indocyanine green angiography (ICGA) and en face OCT were analyzed. Main Outcome Measures: Intervortex venous anastomosis between superotemporal and inferotemporal vortex veins and the dominant site of dilated temporal vortex veins were determined in the eyes with retrograde pulsatile vortex venous flow in the temporal vortex veins. Results: Twenty-two eyes with retrograde pulsatile vortex venous flow in the temporal vortex veins were identified utilizing early phase ICGA videos. In 9 eyes, retrograde pulsatile flow was detected in the superotemporal vortex veins, which were connected to the inferotemporal vortex veins via intervortex venous anastomoses. Among these cases, contralateral inferotemporal vortex vein dilatation was dominant in 7 eyes (77.8%), while superotemporal and inferotemporal vortex veins were symmetrically dilated in the other 2 eyes (22.2%). On the other hand, in 13 eyes, the retrograde pulsatile flow was detected in the inferotemporal vortex veins, which were linked to the superotemporal vortex veins via intervortex venous anastomoses. In these eyes, contralateral superotemporal vortex vein dilatation was dominant in 10 eyes (76.9%). Superotemporal and inferotemporal vortex veins were symmetrically dilated in 2 eyes (15.4%), while mainly inferotemporal vortex veins were dilated in 1 eye (7.7%). Conclusions: In pachychoroid spectrum diseases, there are cases wherein congested venous blood might drain into the contralateral vortex veins via intervortex anastomoses. Overloaded contralateral vortex veins may, as a consequence, become more dilated than the primary congested vortex veins. Inversion of asymmetric vortex vein dilatation might thereby develop in pachychoroid spectrum diseases. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Alterations of posterior precortical vitreous pockets with positional changes.

PURPOSE: To investigate the morphologic features of posterior precortical vitreous pockets (PPVPs) with positional changes using spectral domain optical coherence tomography. METHODS: The authors measured the distance between the fovea and anterior PPVP border on spectral domain optical coherence tomography scans in both eyes of 20 consecutive individuals and compared the differences with changes in position from sitting to supine. RESULTS: A PPVP was identified in both eyes of 14 individuals (70%). In the vertical scan, the superior portion of the pocket was larger than the inferior portion in all 28 eyes when the participants were sitting. The mean distances between the fovea and the anterior PPVPs that border in the right and left eyes, respectively, were 477.6 ± 40.7 µm and 497.1 ± 31.8 µm when the participants were sitting and 665.6 ± 51.6 µm and 750.5 ± 48.2 µm when the participants were supine. The differences between the 2 positions were significant (P < 0.005). CONCLUSION: The superior portion of the PPVPs enlarged when the participants were sitting. The anterior border of the pocket moved anteriorly when the participants were supine.

Submacular choroidal neovascularization at the margin of staphyloma in tilted disk syndrome.

PURPOSE: To clarify the nature of submacular choroidal neovascularization (CNV) and underlying choroidal circulatory disturbances in tilted disk syndrome. METHODS: We retrospectively examined patients with submacular CNV associated with tilted disk syndrome using fluorescein angiography and indocyanine green angiography in this observational case series. The axial lengths were measured by ultrasonography. Eyes with myopia exceeding -6.0 diopters were excluded. RESULTS: Eleven consecutive eyes (mean patient age ± standard deviation, 60.5 ± 12.1 years) were included. The axial lengths ranged from 21.7 mm to 24.6 mm (mean, 23.61 ± 0.95 mm). Classic CNV in nine eyes and polypoidal choroidal vasculopathy in two eyes developed at the macula along the superior margin of the inferior posterior staphyloma that crossed the macula in all eyes. The superior margin of the inferior posterior staphyloma was hyperfluorescent on fluorescein angiography and hypofluorescent on indocyanine green angiography, with the latter larger than the former. The inferior staphyloma contained fewer and smaller choroidal arteries and veins. During follow-up, the submacular CNV and polypoidal choroidal vasculopathy spontaneously regressed and the subretinal hemorrhage resorbed, leaving a fibrotic scar in eight eyes. Newly developed CNV at the initial no-leakage site on fluorescein angiography developed in one eye and the CNV located in the area of band-shaped hypofluorescence on indocyanine green angiography. CONCLUSION: Stretching of the ocular wall might result in occluded choriocapillaris and degeneration of the retinal pigment epithelium. These changes along the border of the inferior posterior staphyloma predispose to submacular CNV and polypoidal choroidal vasculopathy in tilted disk syndrome.

Alteration of choroidal thickness in a case of carotid cavernous fistula: a case report and a review of the literature.

BACKGROUND: To measure the alterations of the choroidal thickness in Carotid cavernous fistula (CCF) using enhanced depth imaging optical coherence tomography (EDI-OCT). CASE PRESENTATION: A 64-year-old woman was referred to us for redness, exophthalmos and visual disturbance in her right eye. She was diagnosed with CCF by magnetic resonance imaging (MRI) and magnetic resonance angiography.Observations; Embolization resulted in improvement of ocular symptoms, and there was a reduction of the subfoveal choroidal thickness in the right eye from 351 µm preoperatively to 142 µm postoperatively in EDI-OCT. CONCLUSION: EDI-OCT demonstrated that the choroidal thickness increases occurred due to congestion in a CCF case.