Long-term follow-up demonstrates change in conformation shape of the focal choroidal excavation lesions.

PURPOSE: This study aims to present long-term observation of 5 eyes with focal choroidal excavation (FCE), focusing on morphological changes in conformity of the lesion. METHODS: A retrospective case series was conducted, including 5 eyes of 5 patients with FCE. The study utilized multimodal imaging including color fundus photography, optical coherence tomography (OCT), enhanced depth imaging OCT (EDI-OCT), fundus fluorescein angiography (FFA), fundus autofluorescence (FAF), red free imaging, and OCT angiography. RESULTS: The mean age at diagnosis was 51 ± 10.65 years, with a mean follow-up period 37 ± 13.59 months. All cases were unilateral, with 1 presenting FCE as an isolated lesion, and one patient exhibiting 2 FCEs in one eye. The mean choroidal thickness measured by EDI-OCT was 268.2 ± 63.39 µm in the affected eye. One patient displayed choroidal thickening and pachyvessels. Of the 5 eyes, one had conforming and 4 non-conforming FCE. We observed a conversion in conformity in all patients, with 4 cases transitioning from non-conforming FCE to conforming type (3 spontaneously, 1 treatment-induced). In conforming FCE, a hyporeflective space appeared twice between neuroretina and retinal pigment epithelium with spontaneous regression. CONCLUSION: We observed change in shape from the conforming to non-conforming FCE and vice versa in all patients. We consider this small change in the hyporeflective space as non-pathologic and clinically insignificant.

Choroidal manifestations of non-ocular sarcoidosis: an enhanced depth imaging OCT study.

BACKGROUND: Although choroidal thickening was reported as a sign of active inflammation in ocular sarcoidosis, there has been no research on the choroidal changes in non-ocular sarcoidosis (defined as systemic sarcoidosis without overt clinical signs of ocular involvement). Therefore, this study aimed to investigate choroidal structural changes in patients with non-ocular sarcoidosis. METHODS: This retrospective case-control study was conducted at Asan Medical Center, a tertiary referral center. We evaluated 30 eyes with non-ocular sarcoidosis and their age- and spherical equivalent-matched healthy control eyes. The subfoveal choroidal thickness, area ratio (Sattler layer-choriocapillaris complex [SLCC] area to Haller layer [HL] area), and choroidal vascularity index (CVI, luminal area to choroidal area) were analyzed using enhanced depth imaging in optical coherence tomography. Systemic and ocular factors associated with the choroidal thickness were investigated. RESULTS: Compared with the healthy control group, the non-ocular sarcoidosis group had significantly thicker subfoveal choroid (total and all sublayers [SLCC and HL]) and lower area ratio. There were no significant differences in the CVIs at all sublayers between groups. In the non-ocular sarcoidosis group, eyes under oral steroid treatment had thinner choroid than eyes under observation. In the control group, eyes with older age and more myopic spherical equivalent had thinner choroidal thickness. CONCLUSION: Total and all sublayers of the subfoveal choroid were significantly thicker without significant vascularity changes in non-ocular sarcoidosis eyes than in healthy control eyes. The degree of choroidal thickening was disproportionally greater at HL than at SLCC. These characteristic choroidal changes may be the subclinical manifestations in non-ocular sarcoidosis.

[Can ultra-widefield optical coherence tomography angiography replace traditional fundus angiography].

Fundus imaging plays a pivotal role in diagnosing retinal and choroidal diseases. Optical coherence tomography angiography (OCTA), by capturing signals to reconstruct vascular structures, offers a clear depiction of retinal vasculature with notable advantages such as rapid scanning and non-invasiveness. Although OCTA, due to its underlying principles, cannot dynamically assess vascular function, exploring its future applications and potential to eventually replace traditional fundus angiography remains a key focus in the medical community. OCTA provides multiple parameters that conventional fundus angiography cannot obtain. With the expanding coverage area of OCTA scans and improvements in artifact elimination, the detection rate of various retinal and choroidal diseases has significantly increased, making the widespread clinical application of OCTA an inevitable trend. Although ultra-widefield OCTA cannot yet fully replace angiography in clinical practice, with continued clinical practice, expanded clinical research, and ongoing technological innovation, OCTA is expected to gradually replace fundus angiography in the future.

LONGITUDINAL ANALYSIS OF DIABETIC CHOROIDOPATHY IN PROLIFERATIVE DIABETIC RETINOPATHY TREATED WITH PANRETINAL PHOTOCOAGULATION USING WIDEFIELD SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY.

PURPOSE: Widefield swept-source optical coherence tomography (OCT) imaging was used to characterize choroidal thickness and vascularity at baseline in proliferative diabetic retinopathy (PDR) and longitudinally after panretinal photocoagulation (PRP). METHODS: Patients with treatment-naive PDR were imaged at baseline and at 1 week, 1 month, and 3 months after PRP. Previously validated algorithms were used to calculate the mean choroidal thickness (MCT) and choroidal vascularity index (CVI) in 5 regions of 12 mm × 12 mm scans. RESULTS: Fourteen PDR eyes were included. Baseline MCT in PDR eyes did not differ significantly from normal eyes, but CVI measurements in PDR eyes were lower in all regions (P < 0.001-0.008). After PRP, MCT measurements in PDR eyes were significantly lower at 1 month and 3 months in all regions (P < 0.001-0.005) except the fovea (P = 0.074). However, CVI measurements did not change over time in any region after PRP. CONCLUSION: The choroid in PDR eyes has a smaller CVI than that in normal eyes. After PRP, the choroidal thickness decreases outside the fovea, but the CVI remains constant, which suggests that a relative decrease in choroidal vascularity persists. These widefield swept-source OCT results are consistent with choroidal alterations found in histopathological reports of diabetic choroidopathy.

CHORIOCAPILLARIS VASCULAR PARAMETERS IN NORMAL EYES AND THOSE WITH PACHYCHOROID WITH AND WITHOUT DISEASE.

PURPOSE: To evaluate the vascular characteristics of the choriocapillaris in eyes with pachychoroid as compared with normal controls. METHODS: Eyes with pachychoroid disease were defined as those with a history of central serous chorioretinopathy or peripapillary pachychoroid syndrome. Pachychoroid without disease was defined as eyes with no history of disease with a subfoveal choroidal thickness ≥ the age-adjusted 95th percentile thickness. Frame-averaged optical coherence tomography angiography images of the choriocapillaris obtained with a Zeiss Plex Elite were binarized, skeletonized, and evaluated for vascular branching parameters. RESULTS: There were 7 normal control subjects, 10 subjects with pachychoroid without disease, and 17 pachychoroid disease subjects. Mean choriocapillaris vessel segment length was 12.19 µm in eyes with pachychoroid disease as compared with 11.48 µm in normal controls and 11.62 µm in pachychoroid without disease (P = 0.003 and P = 0.006, respectively). The branches per square millimeter were fewer in pachychoroid disease (1,215), as compared with normal controls (1,471) or pachychoroid without disease (1,384; P < 0.001, and P = 0.002, respectively). The choriocapillaris vessel diameter was larger, but the fractal dimension was smaller in pachychoroid disease eyes as compared with normal eyes or pachychoroid without disease eyes. There was no statistically significant difference between normal controls and pachychoroid without disease for any measured vascular parameter of the choriocapillaris. CONCLUSION: Choriocapillaris vascular parameters suggest that pachychoroid is not necessarily pathologic. It is possible that choroidal thickening is an epiphenomenon, and there are more significant vascular parameters that are related to disease. These concepts may help guide future prospective studies.

Case Report: Focal Choroidal Excavation: Conversion from Conforming to Nonconforming in a Pregnant Female.

SIGNIFICANCE: Although rarely seen, clinicians should closely monitor patients, especially pregnant patients with focal choroidal excavation (FCE), as it can convert between subtypes and has been linked to pachychoroidal disease and potential for vision loss. PURPOSE: This study aimed to report a case of the conversion of conforming FCE to nonconforming FCE with spontaneous resolution in a pregnant female. CASE REPORT: A 35-year-old Indian woman presented with a slightly decreased vision in the right eye. The patient was 3 months pregnant at this visit. Her ocular history included stable conforming FCE in both eyes that was diagnosed 1 year earlier. Retinal pigmentary changes were noted in both eyes and consistent with previous examinations as being conforming FCEs in both eyes. Optical coherence tomography through the pigmented changes revealed FCE in the right eye with overlying serous fluid, with the left eye showing stable conforming FCE. She was diagnosed with a nonconforming FCE in the right eye secondary to her pregnancy. She was monitored with subsequent visits showing spontaneous resolution of the fluid and conversion back to a conforming FCE. CONCLUSIONS: This case highlights the conversion of a conforming FCE to a nonconforming FCE with spontaneous resolution in a pregnant female. Similarities are seen in terms of pathophysiology with central serous chorioretinopathy, a fellow pachychoroidal disease, which also has pregnancy as a risk factor. Risk factors in pregnant patients such as increased cortisol and increased ocular blood flow may play an important part in the pathophysiology of the conditions, as they both result in choroidal hyperpermeability. Frequent monitoring and follow-up times are suggested for the patients. Lifelong monitoring is also indicated, as reoccurrences have been reported. Further research is needed at this time to elucidate the exact etiology of FCE and conversions between conforming and nonconforming FCE.

Multimodal imaging in sclerochoroidal calcification: a case report and literature review.

BACKGROUND: Sclerochoroidal calcification (SCC), a rare condition found in elderly people, is idiopathic or occasionally secondary to disorders affecting calcium metabolism. Findings of multimodal imaging including choroidal circulation are, however, largely unknown. We present a patient of SCC with systemic background, who underwent multimodal imaging evaluations. CASE PRESENTATION: A 70-year-old Japanese man was referred to our clinic because of bilateral fundus lesions. He had a history of chronic kidney disease (CKD) and secondary hyperparathyroidism. Fundus photography showed a cluster of choroidal folds in the superotemporal extra-macular region OS. Swept-source optical coherence tomography demonstrated ellipsoid zone disruption OD, retinal pigment epithelium undulation OS, dilated Haller layer veins OU, and central choroidal thickening OU and thinning of the overlying choroid due to scleral elevation OS. Fluorescein angiography detected macular hyperfluorescence OD. Indocyanine green angiography demonstrated choroidal vascular hyperpermeability together with numerous scattered hypofluorescent lesions OU. Fundus autofluorescence showed multiple hypoautofluorescent spots surrounded by hyperautofluorescent areas OD. Laser speckle flowgraphy exhibited choroidal blood flow reduction represented by a cold color pattern OU. B-mode echography displayed hyperechoic solid lesions with acoustic shadowing and orbital computed tomography revealed high density areas in the sclera, both of which were consistent with calcification. The patient was diagnosed with SCC, and these imaging findings remained unchanged 7 months after the diagnosis. CONCLUSIONS: We reported a case of SCC with the background of CKD. Our detailed multimodal observations indicated choroidal hypoperfusion possibly caused by mechanical compression due to calcium deposition in the sclera.

[The application and progress of optical coherence topography angiography in polypoidal choroidal vasculopathy].

Optical coherence tomography angiography (OCTA) is a novel, non-invasive imaging technology, which could acquire volumetric angiographic information. Numerous studies have reported the potential clinical use of OCTA in a variety of common retinal disorders. Polypoidal choroidal vasculopathy (PCV) is characterized by the formation of branching choroidal vascular networks (BVN) with terminal dilatations (polyps). Indocyanine green angiography (ICGA) remains the golden diagnostic standard for PCV. The clinical application of OCTA in PCV is also widely investigated recent years. But the results are controversially interpreted. In addition to various diagnostic accuracy of PCV from different studies, the clinical application of OCTA in PCV is limited. With the constant innovation of fundus imaging techniques, OCTA is reaching greater investigation depth and become more accurate at picking up blood flow signals, which also improves the diagnostic accuracy of PCV. In this paper, we reviewed the clinical application and research progress of OCTA in PCV, in order to provide some assistant for clinical practice and correct interpretation of the reports. (Chin J Ophthalmol, 2020, 56:950-955).

MULTIMODAL IMAGING OF CHOROIDAL LESIONS IN DISSEMINATED MYCOBACTERIUM CHIMAERA INFECTION AFTER CARDIOTHORACIC SURGERY.

PURPOSE: To explore morphologic characteristics of choroidal lesions in patients with disseminated Mycobacterium chimaera infection subsequent to open-heart surgery. METHODS: Nine patients (18 eyes) with systemic M. chimaera infection were reviewed. Activity of choroidal lesions were evaluated using biomicroscopy, fundus autofluorescence, enhanced depth imaging optical coherence tomography, fluorescein angiography/indocyanine green angiography, and optical coherence tomography angiography. Relationships of choroidal findings to systemic disease activity were sought. RESULTS: All 9 male patients, aged between 49 and 66 years, were diagnosed with endocarditis and/or aortic graft infection. Mean follow-up was 17.6 months. Four patients had only inactive lesions (mild disease). In all five patients (10 eyes) with progressive ocular disease, indocyanine green angiography was superior to other tests for revealing new lesions and active lesions correlated with hyporeflective choroidal areas on enhanced depth imaging optical coherence tomography. One eye with a large choroidal granuloma developed choroidal neovascularization. Optical coherence tomography angiography showed areas with reduced perfusion at the inner choroid. All 5 patients with progressive ocular disease had evidence of systemic disease activity within ±6 weeks' duration. CONCLUSION: Choroidal manifestation of disseminated M. chimaera infection indicates systemic disease activity. Multimodal imaging is suitable to recognize progressive ocular disease. We propose ophthalmologic screening examinations for patients with M. chimaera infection.

FOCAL DISRUPTIONS IN ELLIPSOID ZONE AND INTERDIGITATION ZONE ON SPECTRAL-DOMAIN OPTICAL COHERENCE TOMOGRAPHY IN PACHYCHOROID PIGMENT EPITHELIOPATHY.

PURPOSE: To describe a distinctive focal disruption of the ellipsoid zone (EZ)/interdigitation zone (IZ) determined by spectral-domain optical coherence tomography in eyes with pachychoroid pigment epitheliopathy. METHODS: Twenty patients with focal EZ/IZ disruptions associated with pachychoroid without history or findings indicative of subretinal fluid were evaluated. Detailed clinical/imaging characteristics and their longitudinal changes were evaluated. RESULTS: A total of 27 lesions from 21 eyes were included. The mean subfoveal choroidal thickness was 450 µm. In six of seven eyes with previous imaging data to compare, characteristic drusenoid lesions associated with thick choroid preceded focal EZ/IZ disruptions at the corresponding sites. Dilated outer choroidal vessels were noted beneath the focal EZ/IZ disruption in 24 lesions (88.9%), with attenuation of choriocapillaris in 18 lesions (66.7%). The external limiting membrane was intact in all lesions. All except three eyes did not show morphologic changes, and mean visual acuity was maintained (Snellen equivalent, 20/25) during a mean follow-up period of 20 months. CONCLUSION: Focal disruptions of the EZ/IZ band can develop in pachychoroid eyes in the absence of subretinal fluid, with a stable clinical course, and possibly as a result of regression of drusenoid lesions. This may represent an atrophic form of pachychoroid manifestation.

Morphological characteristics of ocular toxoplasmosis and its regression pattern on swept-source optical coherence tomography angiography: a case report.

BACKGROUND: To report the successful treatment of ocular toxoplasmosis and present the use of multimodal imaging to describe the changes in ocular toxoplasmic lesions subsequent to treatment. CASE PRESENTATION: A 73-year-old female visited the clinic with decreased visual acuity in the left eye. Fundus examination showed severe vitreous haze with yellow-white infiltrates near the foveal center. Spectral-domain optical coherence tomography (SD-OCT) revealed disorganization of the retinal structure with markedly thickened choroid beneath the active lesion. Highly elevated serum titers of IgG antibodies against Toxoplasma gondii were observed. Topical and systemic steroids with oral Bactrim were administered after a diagnosis of ocular toxoplasmosis was made. After improvement in the severity of vitritis, structural en face swept-source optical coherence tomography (SS-OCT) imaging demonstrated diffuse choroidal dilation with many collateral vascular branches surrounding the active lesion. Eight intravitreal injections of clindamycin (1 mg/0.1 ml) were administered at 1- to 2-week intervals along with systemic antibiotics and steroids. After the treatment, the toxoplasmic lesion resolved to an atrophic chorioretinal scar. Dilated choroidal vessel size was normalized and collateral vascular branches were markedly constricted on structural en face SS-OCT images. CONCLUSIONS: This is the first detailed report on the morphological changes in the choroidal vasculature surrounding ocular toxoplasmic lesions that were characterized using SS-OCT-A imaging. Multimodal imaging with SS-OCT-A can be valuable in clinical diagnosis as well as in clarifying the mechanism of choroidal structural changes in ocular toxoplasmosis.

POLYPOIDAL CHOROIDAL VASCULOPATHY UPON OPTICAL COHERENCE TOMOGRAPHIC ANGIOGRAPHY.

PURPOSE: To study polypoidal lesions and branching choroidal vascular networks in eyes with polypoidal choroidal vasculopathy by optical coherence tomography (OCT)-based angiography (OCTA). METHODS: In the observational cross-sectional study, patients with polypoidal choroidal vasculopathy, as diagnosed by indocyanine green angiography, underwent OCTA. RESULTS: Thirty-two eyes of 31 patients with an age of 61.1 ± 7.6 years were included. Branching choroidal vascular networks were detected by indocyanine green angiography and OCTA in 25 of 32 (78 ± 73%) and in 30 of 32 (94 ± 4%) eyes, respectively, with a marginally significant difference (P = 0.06) in the detection rate between both techniques. A total of 72 polyps (area, 0.06 ± 0.06 mm; range, 0.01-0.27 mm) were detected by indocyanine green angiography, and they were consistently present on the OCTA images. By moving the reference level in the OCT angiograms to the corresponding layer, the polypoidal lesions showed cluster-like structures in 53 of 72 polypoidal lesions (74%). In 60 of the 72 polypoidal lesions (83%), cluster-like structures were detected in the en face structural OCT images at the reference plane of the OCTA images. On the cross-sectional OCT images, some internal channels of flow were seen in 50 of the 72 polypoidal lesions (69%). Larger size of the polypoidal lesions was associated with a higher prevalence of cluster-like structures on the OCTA images, some internal channels of flow on the en face structural images, and clustered vascular structures on the cross-sectional OCT images. CONCLUSION: In conclusion, OCTA is a useful technique for the noninvasive detection of branching choroidal vascular networks including visualization of details such as cluster-like structures and flow. In some eyes, OCTA was superior to indocyanine green angiography to detect polypoidal choroidal vasculopathy and to show branching choroidal vascular networks.

POLYPOIDAL CHOROIDAL VASCULOPATHY ASSOCIATED WITH CENTRAL SEROUS CHORIORETINOPATHY: Pachychoroid Spectrum of Diseases.

PURPOSE: To study multimodal imaging features of polypoidal choroidal vasculopathy (PCV) associated with central serous chorioretinopathy (CSC) in the same eye. METHODS: A retrospective observational study of cases of suspected PCV which underwent indocyanine green angiography, fundus fluorescein angiography and optical coherence tomography was done, to look for simultaneous typical CSC-like active leak in the same eye. The relevant history, best-corrected visual acuity, optical coherence tomography, fundus fluorescein angiography, and indocyanine green angiography findings were analyzed. RESULTS: From 226 patients reviewed, 195 patients had PCV from which 6 eyes (3.07%) with features of PCV associated with typical CSC-like active leak in the same eye were identified; 3 men and 3 women with a mean age of 62.6 years. Optical coherence tomography showed notched pigment epithelial detachment in two and irregular peaked pigment epithelial detachment in four cases with subretinal fluid and/or subretinal haemorrhage over a thick choroid with dilated outer choroidal vessels. Fundus fluorescein angiography showed stippled hyperfluorescence at polyp area and a separate typical CSC-like active leak-Inkblot in three and Smokestack in three cases. On indocyanine green angiography, multiple polyps were noted with choroidal hyperpermeability in late phase at the site of CSC leak. CONCLUSION: The coexistence of PCV with typical CSC-like active leaks and a thick choroid in the same eye simultaneously provides strong evidence that these diseases have an association and support the hypothesis that these conditions may originate from predisposed thick choroid.

Multi-modality imaging findings of huge intrachoroidal cavitation and myopic peripapillary sinkhole.

BACKGROUND: Peripapillary intrachoroidal cavitation was described as the presence of an asymptomatic, well-circumscribed, yellow-orange, peripapillary lesion at the inferior border of the myopic conus in eyes with high myopia. CASE PRESENTATION: A 66-year-old myopic Chinese man was enrolled and his multi-color imaging examination showed a well-circumscribed, caesious, peripapillary lesion coalesced with the optic nerve head vertically rotated and obliquely tilted, together with an inferotemporal sinkhole in the myopic conus. The optical coherence tomography images showed an intrachoroidal hyporeflective space, schisis, an intracavitary septum located below the retinal pigment epithelium and inserted beneath the optic nerve head, as well as a sinkhole between the peripapillary intrachoroidal cavitation and the vitreous space. CONCLUSIONS: Both myopic colobomas and sinkhole in myopic conus may contribute the coalescence of intrachoroidal cavitation with optic nerve head. These qualitative and quantitative new findings will be beneficial for understanding its pathomorphological mechanism, and the impact on optic nerve tissue of myopic patients.

The blood flow characteristics of polypoidal choroidal vasculopathy and the choroidal remodelling process after photodynamic therapy.

OBJECTIVES: To investigate the blood flow characteristics of polypoidal choroidal vasculopathy (PCV) using optical coherence tomography angiography (OCTA) and to analyse photodynamic therapy (PDT) effects on choroidal remodelling in PCV. MATERIALS AND METHODS: Diagnostic indocyanine green angiography and OCTA were performed. All patients underwent PDT with full-dose verteporfin and were followed up with enhanced-depth imaging optical coherence tomography. RESULTS: At baseline, a branching vascular network (BVN) was clearly demonstrated in all patients with PCV who underwent OCTA examinations as opposed to polyps. Additionally, the choroidal thickness (266 [range: 74-456] µm) showed a positive relationship with polyp size (0.59 [range: 0.33-0.94] mm2 , r = 0.679, P = 0.0022). The subfoveal choroidal thickness and choroidal thickness at polyp sites increased within 1 day after PDT and atrophied 1-3 months after PDT. CONCLUSIONS: The differences in imaging characteristics between BVNs and polyps on OCTA were presumably due to both blood turbulence (different flow orientations) within polyps and the velocities detectable on OCTA. Moreover, the choroidal remodelling effects of PDT in PCV suggested the occurrence of transitional reactive inflammatory choroidal vascular hyperpermeability and choroidal exudation. PCV involved the entire choroids rather than only focal lesions. Lasers Surg. Med. 50:427-432, 2018. © 2018 Wiley Periodicals, Inc.

[Peripheral Exudative Haemorrhagic Chorioretinopathy: Course of Disease and Diagnosis - Including Wide-field Imaging of Associated Diseases Age-related Macular Degeneration and Polypoidal Choroidal Vasculopathy, Therapy]. / Peripher exsudative hämorrhagische Chorioretinopathie: Krankheitsverlauf, Diagnosestellung inklusive Weitwinkelbildgebung der assoziierten Erkrankungen altersabhängige Makuladegeneration und polypoidale Choriovaskulopathie, Therapie.

PEHCR (peripheral exudative haemorrhagic chorioretinopathy) is a disease manifested clinically, particularly by subretinal bleeding, retinal exudates, retinal pigment epithelium detachments (RPE detachments), exudative retinal detachment and sub-RPE bleeding. The PEHCR lesion is often characterized by its polypoidal pattern, which is very similar to PCV (polypoidal choroidal vasculopathy) polyps. Diagnosis is best made with a wide-field ICGA (indocyanine green angiography). In approximately half of patients, macular changes in the form of drusen, up to exudative AMD (age-related macular degeneration), are detected in the affected eye or partner eye. Since there is very little literature directly available on PEHCR, this work also discusses the peripheral changes described in the context of AMD that were investigated with wide-field imaging.

Choroidal thickening in a case of carotid cavernous fistula.

A 47-year-old woman was diagnosed with dural carotid cavernous fistula (CCF) in her right eye (RE). Scans of the choroid using Spectralis optic coherence tomography (OCT) demonstrated significant asymmetry in subfoveal choroidal thickness (RE 451 µm, left eye (LE) 367 µm). This asymmetry disappeared after the fistula was embolizated through the ophthalmic artery (RE 341 µm; LE 340 µm). This case suggests that OCT should be considered as an ancillary test in the diagnosis of CCF.

Primary acute angle-closure glaucoma complicating ciliochoroidal detachment: report of four cases and review of the literature.

PURPOSE: To describe the ultrasound biomicroscopy (UBM) and B-scan ultrasonography findings and therapeutic approach for ciliochoroidal detachment secondary to acute primary angle-closure glaucoma (APACG) in four patients. We also reviewed the literature. METHODS: Case report and systematic literature review. RESULTS: The four patients were referred to our department for further management of APACG. The UBM and B-scan ultrasonography examinations were conducted 2 days after the beginning of medical treatment and demonstrated ciliochoroidal detachment in four eyes of the four patients. The patients all received intravenous infusion of corticosteroid therapy (10 mg dexamethasone once daily for 5-7 days). We reviewed the UBM findings, which confirmed that the ciliochoroidal detachment disappeared. The patients then underwent trabeculectomy combined with peripheral iridotomy surgery. The patients all ultimately recovered very well. CONCLUSION: APACG with ciliochoroidal detachment is rare and has hidden clinical manifestations, and the pathophysiological mechanism is not yet fully understood. Anti-glaucoma surgery may increase the detachment. UBM and B-scan ultrasounds are useful tools for ciliochoroidal detachment diagnosis in APACG patients before operative treatment.

The absence that makes the difference: choroidal abnormalities in Legius syndrome.

Neurofibromatosis type 1 (NF1) is an hereditary disorder characterized by abnormal proliferation of multiple tissues of neural crest origin, and presents mainly with multiple café-au-lait macules, axillary freckling and neurofibromas. Choroidal involvement in NF1 patients has been studied, thanks to the development of non-invasive tools such as infrared monochromatic light during fundus examination, which showed bright patchy lesions consistent with choroidal nodules. Choroidal abnormalities identified with near-infrared reflectance have reported with a frequency of up to 100% in NF1, and have been recently been proposed as a novel diagnostic criterion for NF1. Legius syndrome can be clinically indistinguishable from NF1 and results in a small percentage of individuals being misdiagnosed. We investigated the presence of choroidal abnormalities in Legius syndrome to determine their specificity to NF1 and their potential usefulness as a novel diagnostic criterion for NF1. We examined the fundus of 16 eyes by confocal scanning laser ophthalmoscopy with infrared monochromatic light in eight patients with molecularly confirmed Legius syndrome. No abnormalities were observed, confirming the diagnostic value of choroidal abnormalities for the diagnosis of NF1.

Intraoperative B-scan ultrasonography and pars plana vitrectomy for severe open globe injury with hemorrhagic retinal and choroidal detachment.

PURPOSE: Our purpose was to report the initial clinical experience of intraoperative B-scan ultrasonography in combination with 25-gauge pars plana vitrectomy for severe open globe injury with hemorrhagic retinal and choroidal detachment. METHODS: Six eyes of six consecutive patients with severe open globe injury underwent intraoperative B-scan ultrasonography and 25-gauge pars plana vitrectomy at Osaka University Hospital in Japan. The feasibility of intraoperative B-scan ultrasonography, best-corrected visual acuity (BCVA), retinal reattachment, and intraoperative and postoperative complications were evaluated. RESULTS: Five patients presented with a ruptured globe and one patient with double penetration. Preoperative best-corrected visual acuity was no light perception in four eyes and light perception in two eyes. All patients underwent intraoperative B-scan ultrasonography and 25-gauge pars plana vitrectomy within 12 h after open globe injury. Intraoperative B-scan ultrasonography was feasible in all cases and was useful for diagnosing choroidal hemorrhage (four eyes), massive subretinal hemorrhage (two eyes), and retinal detachment (five eyes). In addition, serial real-time B-scan imaging facilitated successful evacuation of the choroidal hemorrhage and massive subretinal hemorrhage by external drainage, resulting in opening of the vitreous space to allow subsequent pars plana vitrectomy without entry site-related complications. After surgery, all patients had successful retinal attachment, and there was no loss of light perception. CONCLUSION: Intraoperative B-scan ultrasonography is technically feasible and may potentially improve the safety and efficacy of severe open globe injury repair.