Combined central serous chorioretinopathy, hypermetropia, short axial length, chorioretinal folds, enlarged/thickened ocular coats, with varying association of scleral changes (CHAFES).

PURPOSE: To describe a condition with the following features: chronic central serous chorioretinopathy (CCSC), chorioretinal folds, scleral changes (including any of the following flattened or 'squared off' posterior pole, 'T sign', or thickened ocular coats), accompanied by a short axial length and hypermetropia in a series of 7 patients. METHODS: The case notes of 7 patients presenting with a combination of CSC, choroidal folds scleral changes and hypermetropia were reviewed as part of a retrospective case series. Corrected visual acuities, serial refraction, colour imaging, fluorescein and indocyanine green angiography findings, together with B-ultrasound scan features were recorded, with axial length measurements as available (< 23.3 mm was defined as short). RESULTS: The study included 14 eyes of 7 subjects (2 females and 5 males) with a primary presentation of central vision disturbance. All patients showed signs of previous or current episodes of the following features in at least one eye: CSC (5/7 bilateral); choroidal folds (6/7 bilateral), thickening of ocular coats in the 5 in whom this was measured, at least one scleral abnormality on ultrasound in at least one eye. A short axial length at final appointment was recorded in 13/14 eyes. CONCLUSIONS AND RELEVANCE: The combination of CCSC with choroidal folds, hypermetropia with apparent shortening of the eyeball associated with one or more scleral abnormalities such as a flattened or 'squared off 'appearance of the B ultrasound may be a specific ocular condition. The aetiology of this particular combination of posterior segment manifestations is unknown; the choroid could be the primary focus of disease with secondary involvement of the sclera. Alternatively, the features observed may result from a chronic inflammatory process affecting the sclera with secondary effects on the choroid, retinal pigment epithelium and retina. In our case series, the final vision was not significantly different from vision at presentation.

Thyroid eye disease with choroidal folds.

Purpose: To determine the clinical course of patients with chorioretinal folds (CRF) in thyroid eye disease (TED).Methods: A multi-center retrospective case series of patients with TED who developed CRF.Results: Ten patients (17 eyes) with CRF related to TED were identified. The mean age of presentation was 59.3 ± 8.3 years old. The majority of patients were male (70%), hyperthyroid (70%), hyperopic (53%), had a history of radioactive iodine (60%), and currently on methimazole treatment (30%). Three patients (3 eyes) had unilateral involvement of CRF with bilateral TED. The average clinical activity score was 3.6 ± 2.1 at the time of presentation. The most commonly enlarged extraocular muscles were medial (76%), inferior (64%), superior (64%) and lateral rectus (35%). Compressive optic neuropathy was seen in 47% of eyes. Treatment included oral prednisone (70%), orbital decompression (59%), thyroidectomy (20%) and tocilizumab (10%). The CRF did not resolve over a follow up period of 24.7 ± 23.7 months in 70% of eyes. There was no significant difference in average axial length (25.7 ± 4.9 mm) and optic nerve to optic strut distance (37.8 ± 3.9 mm) between patients with CRF and the eight age-and sex-matched TED control patients without CRF (p = 0.81 and 0.65 respectively). A univariable and multivariable analysis found an enlarged inferior rectus as a factor in TED patients with persistent CRF.Conclusions: CRF are often an indicator of visually threatening situations and often do not resolve despite treatment of TED.

Chorioretinal Folds in the Trabeculectomized Eye with Normal Intraocular Pressure after Phacoemulsification.

Background and Objectives: This case report discusses possible causes of chorioretinal fold (CRF) formation. Materials and Methods: A case report. Results: A 48-year-old man presented with a history of high myopia and primary open-angle glaucoma in both eyes. He underwent a trabeculectomy followed by phacoemulsification in both eyes. Two months later, he complained of blurred vision in the right eye. The intraocular pressure (IOP) was 17 mmHg in the left eye and 9 mmHg in the right eye. Refraction showed a hyperopic shift in both eyes. Slit-lamp examination showed a deep anterior chamber without cells and a well-functional bleb without leakage. Fundus examination revealed CRFs in the macula of the right eye. No papilledema, choroidal lesions, or other retinal lesions were found. Wrinkling of CRFs at the macula, an increase in central foveal thickness, and a fluid cleft were demonstrated by spectral-domain optical coherence tomography. After using steroid eye drops, the IOP in the right eye and refraction in both eyes recovered to the baseline level. Visual acuity improved in both eyes. Conclusions: CRFs in trabeculectomized eyes with normal IOP after phacoemulsification have not been reported. This case demonstrated that the trabeculectomized eye remains at risk of CRF formation, even if the IOP is normal without hypotony. The importance of a detailed fundus examination in patients with unexplained blurred vision may be necessary after having undergone these procedures. The early recognition of the cause of visual loss may facilitate immediate treatment and may avoid irreversible changes with permanent visual loss.

Multimodal imaging in a case of a congenital retinal macrovessel associated with a retinal cavernous hemangioma: a case report.

BACKGROUND: To report the results of multimodal imaging in a case of a congenital retinal macrovessel associated with a retinal cavernous hemangioma. CASE PRESENTATION: A 52-year-old female patient presented with progressive vision loss in the right eye. BCVA was 8/20 in the right eye and 18/20 in the left eye. Fundus examination of the right eye showed an aberrant retinal macrovessel arising from the inferior temporal major vein. It crossed the foveal area and overstepped to the superior retina. A "brunch of grapes" shaped retinal lesions arised from the macrovessel. Fluorescein angiography showed saccular lesions that filled slowly during the venous phase and became brightly hyperfluorescent saccular caps. SS-OCT of the right eye revealed a highly back-scattering hyper-reflective vessel across the fovea with shadow effect and adhesions between the vitreous and the aberrant macrovessel. It also revealed hypo reflective saccules with hyperreflective borders located in the inner retina corresponding to the cavernous retinal hemangioma. Retinal pigment epithelium undulations and vascular dilations at the level of Haller's layer were observed in both eyes. Indocyanine green angiography revealed chroidal vascular dilatations in both eyes in the late phase. OCT-A showed the aberrant vessel emerging from the inferior temporal vein and splitting the foveal avascular zone horizontally. RCH appeared as small heterogeneous saccular flow areas associated with focal capillary hypo perfusion areas. Asymmetry and distorsion of the foveal avascular zone were also noticed. A diagnosis of retinal macrovessel associated with a retinal cavernous hemangioma was made. CONCLUSIONS: Congenital retinal macrovessels and retinal cavernous hemangioma are benign lesions. Their association is rare. Abnormal vascular development is likely to be responsible for their association. Swept source OCT and OCT angiography may be of a great contribution to better evaluate these retinal vascular disorders.

Post-operative optical coherence tomography angiography features of chorioretinal folds resulting from pleomorphic adenoma of the lacrimal gland (PALG) of orbit- a case report.

BACKGROUND: Chorioretinal fold (CFs) is a rare condition resulting from undulations in the choriocapillaris, Bruch's membrane, retinal pigment epithelium and occasionally neurosensory retina. It can be idiopathic or due to different etiologies. The use of spectral-domain optical coherence tomography (SD-OCT) has increased the diagnosis of CFs and helped in differentiation from other etiologies. Recently, optical coherence tomography angiography (OCT-A) emerged as a non-invasive imaging technique allowing visualization of the individual layers of microvasculature of the retina and the choroid by comparing consecutive B-scans. We described a rare case of pleomorphic adenoma of the lacrimal gland (PALG) causing hyperopic shift and CFs with the new OCT-A technology, getting deeper insight into vascular changes of this disease. CASE PRESENTATION: A 40-year-old Asian man experienced progressive blurred vision in his right eye over 6 months. The patient's initial axial lengths were 25.55 mm in the right eye and 28.13 mm in the left eye. Fundus examination in the right eye revealed oblique CFs as well as the SD-OCT displayed. Magnetic resonance imaging showed intraconal mass extended from superior temporal side of the right orbit. The patient then received tumor removal surgery through lateral orbitotomy and histopathology confirmed a pleomorphic adenoma of the orbit. The patient had regular follow-up for 1 year. His best corrected visual acuity markedly improved from 20/50 to 20/20 with nearly stationary AXL. We performed OCT-A at one year after the surgery, which showed early visualization of deep choroidal vessels. The scleral remodeling due to mass effect of retrobulbar tumor also caused displacement of the deep large choroidal vessels over the superior macular area even after tumor removal. CONCLUSIONS: We reported a rare case of PALG with hyperopic shift and CFs as initial presentation. Surgical removal of the tumor partially resolved the CFs and contributes to impressive visual acuity recovery. The use of OCT-A provided a deeper insight to vascular architecture changes resulting from scleral remodeling after long-term tumor compression.

Chorioretinal folds: a proposed diagnostic algorithm.

PURPOSE: To create a diagnostic algorithm for the management of chorioretinal folds. METHODS: We reviewed the existing literature about chorioretinal folds focusing our attention on three specific conditions and created a diagnostic algorithm in order to otpimize the choice and the number of investigations. RESULTS: Chorioretinal folds are visible striations of the fundus usually arranged in parallel lines and disposed horizontally. They may be either unilateral or bilateral, symptomatic or asymptomatic and are often associated with different possible ocular and extra ocular pathologies, including systemic diseases like autoimmune disorders and intracranial hypertension. They are named idiopathic when no apparent cause for their development is detectable. However, with improved diagnostic testing, the patients with idiopathic choroidal folds are likely to represent only a smaller portion of the total. CONCLUSIONS: Since choroidal folds be the sole sign of an underlying disease possibly requiring a multidisciplinary approach, an appropriate work-up varying according to the specific clinical features of each case is needed to define the etiology and the treatment. A diagnosting algorithm may be useful in order to optimize the diagnostic approach and management.

Decreased macular choriocapillaris in thyroid-associated ophthalmopathy: focusing on chorioretinal folds with and without optic disc edema.

Purpose: The aim of this study was to evaluate the vessel density (VD) of the macular choriocapillaris (CC) and retina in thyroid-associated ophthalmopathy (TAO) patients with chorioretinal folds (CRFs) with and without optic disc edema (ODE) and the correlations of these characteristics with visual function. Method: This was a cross-sectional study. Twenty TAO patients with CRFs (35 eyes) and 20 normal subjects (normal group, 40 eyes) were recruited at the Ophthalmology Department of the Sun Yat-sen Memorial Hospital from March 2018 to October 2022. Then, CRF patients were divided into two groups, the ODE and non-ODE groups (NODE), based on the presence or absence of ODE. All the patients underwent optical coherence tomography angiography (OCTA) and the VD of the macular CC and retina was computed. The correlation of VD and visual function was analyzed. Results: Compared with the normal group, the macular whole-image VD in the retinal superficial layer (SLR-mwiVD: 49.82 ± 3.38 in the normal group, 42.44 ± 5.40 in the NODE group, and 42.51 ± 5.37 in the ODE group), deep layer (DLR-mwiVD: 51.05 ± 6.23 in the normal group, 45.71 ± 6.66 in the NODE group, and 46.31 ± 5.48 in the ODE group), and CC (CC-mwiVD: 70.23 ± 2.47 in the normal group, 68.04 ± 3.73 in the NODE group, and 63.09 ± 6.51 in the ODE group) was decreased in the NODE (all p < 0.05) and ODE group (all p < 0.01). There was no difference in these parameters except CC-mwiVD between the ODE and NODE groups. The CC-mwiVD in the ODE group (63.09 ± 6.51) was significantly reduced compared with that in the NODE group (68.04 ± 3.73, p = 0.004). All these VD parameters were negatively correlated with BCVA, VF-PSD, and P100 latency and positively associated with VF-MD, P100 amplitude, and HRR scores (all p < 0.05). Conclusions: There was a significant decrease in the VD of the macular CC and retina of patients with CRFs with or without ODE, which was correlated with visual dysfunction. The VD of the macular CC in CRF patients with ODE was significantly reduced compared with that in the NODE group, but similar results were not observed in the retina.

Macular and peripapillary optical coherence tomography angiography metrics in thyroid-associated ophthalmopathy with chorioretinal folds.

PURPOSE: To evaluate macular and radial peripapillary capillary (RPC) microvascular densities and the thickness of the retinal nerve fiber layer (RNFL) in thyroid-associated ophthalmopathy (TAO) with chorioretinal folds (CRFs) and the associations of these characteristics with visual function. METHOD: A cross-sectional study was performed at the Ophthalmology Department of the Sun Yat-sen Memorial Hospital from March 2018 to August 2021. All patients underwent ocular examination, ophthalmic function tests and optical coherence tomography angiography (OCTA). The microvascular densities in the macula and optic papilla in the TAO with CRFs or without CRFs. Correlation analyses were used to examine the association of microvascular density and visual function. RESULTS: Ten TAO patients with CRFs (CRF group, 20 eyes) and 10 TAO patients without CRFs (NCRF group, 20 eyes) were recruited for the study. Visual function measurements, including best-corrected visual acuity (BCVA), were found to be worse in the CRF group (all p < 0.05). The macular whole-image vessel density in the superficial layer (SLR-mwiVD) was significantly decreased in the CRF group (p < 0.05). The RPC whole-image vessel density (RPC-wiVD) was significantly decreased in the CRF group (p < 0.05), particularly in the temporal subfields. The P100 amplitude of visual evoked potentials (VEPs) was positively associated with SLR-mwiVD and RPC-wiVD. The thickness of RNFL in the CRF group was obviously thicker than that in the NCRF group (p < 0.05). CONCLUSIONS: Our study showed decreased microvascular density of the macula and RPC and thicker RNFL in TAO patients with CRFs. CRFs with decreased microvascular density should be regard as an indicator of visually threatening conditions.

Optical coherence tomography enhanced depth imaging of chorioretinal folds in patients with orbital tumors.

AIM: To characterize spectral-domain optical coherence tomography (SD-OCT) features of chorioretinal folds in orbital mass imaged using enhanced depth imaging (EDI). METHODS: Prospective observational case-control study was conducted in 20 eyes of 20 patients, the uninvolved eye served as a control. All the patients underwent clinical fundus photography, computed tomography, EDI SD-OCT imaging before and after surgery. Two patients with cavernous hemangiomas underwent intratumoral injection of bleomycin A5; the remaining patients underwent tumor excision. Patients were followed 1 to 14mo following surgery (average follow up, 5.8mo). RESULTS: Visual acuity prior to surgery ranged from 20/20 to 20/200. Following surgery, 5 patients' visual acuity remained unchanged while the remaining 15 patients had a mean letter improvement of 10 (range 4 to 26 letters). Photoreceptor inner/outer segment defects were found in 10 of 15 patients prior to surgery. Following surgical excision, photoreceptor inner/outer segment defects fully resolved in 8 of these 10 patients. CONCLUSION: Persistence of photoreceptor inner/outer segment defects caused by compression of the globe by an orbital mass can be associated with reduced visual prognosis. Our findings suggest that photoreceptor inner/outer segment defects on EDI SD-OCT could be an indicator for immediate surgical excision of an orbital mass causing choroidal compression.

Intermittent and Unilateral Chorioretinal Folds due to Combined Chiari 1 Malformation and Basilar Invagination.

We report the case of a 35-year-old female with combined Chiari 1 malformation and basilar invagination, who presented with intermittent conjunctival chemosis and unilateral chorioretinal folds that were temporally correlated. She denied any flashes, floaters, eye redness, or pain. She also denied nausea or vomiting. Clinical exam and optical coherence tomography imaging revealed conjunctival chemosis and chorioretinal folds in the left eye. Subsequent magnetic resonance imaging of the brain and the orbits were consistent with combined Chiari 1 malformation and basilar invagination. The unilateral and intermittent chorioretinal folds and conjunctival chemosis presentation of combined Chiari 1 malformation and basilar invagination is unusual. To the best of our knowledge, this is the first case to be reported with this unique clinical presentation. It is most important to be aware that unilateral and intermittent chorioretinal folds associated with conjunctival chemosis may be signs of intracranial disease.

Optic disc edema and chorioretinal folds develop during strict 6° head-down tilt bed rest with or without artificial gravity.

Spaceflight associated neuro-ocular syndrome (SANS) is hypothesized to develop as a consequence of the chronic headward fluid shift that occurs in sustained weightlessness. We exposed healthy subjects (n = 24) to strict 6° head-down tilt bed rest (HDTBR), an analog of weightlessness that generates a sustained headward fluid shift, and we monitored for ocular changes similar to findings that develop in SANS. Two-thirds of the subjects received a daily 30-min exposure to artificial gravity (AG, 1 g at center of mass, ~0.3 g at eye level) during HDTBR by either continuous (cAG, n = 8) or intermittent (iAG, n = 8) short-arm centrifugation to investigate whether this intervention would attenuate headward fluid shift-induced ocular changes. Optical coherence tomography images were acquired to quantify changes in peripapillary total retinal thickness (TRT), retinal nerve fiber layer thickness, and choroidal thickness, and to detect chorioretinal folds. Intraocular pressure (IOP), optical biometry, and standard automated perimetry data were collected. TRT increased by 35.9 µm (95% CI, 19.9-51.9 µm, p < 0.0001), 36.5 µm (95% CI, 4.7-68.2 µm, p = 0.01), and 27.6 µm (95% CI, 8.8-46.3 µm, p = 0.0005) at HDTBR day 58 in the control, cAG, and iAG groups, respectively. Chorioretinal folds developed in six subjects across the groups, despite small increases in IOP. Visual function outcomes did not change. These findings validate strict HDTBR without elevated ambient CO2 as a model for investigating SANS and suggest that a fluid shift reversal of longer duration and/or greater magnitude at the eye may be required to prevent or mitigate SANS.

Recognition, Diagnosis and Treatment of Chorioretinal Folds: Current Perspectives.

Chorioretinal folds (CRFs) are undulations of the choroid and overlying Bruch's membrane, retinal pigment epithelium and neurosensory retina. CRFs represent a clinical sign that is mandatory to investigate assuming their association with several ocular and extra-ocular disorders. Recent advances in retinal imaging have improved the characterization of CRFs. More importantly, retinal imaging may be useful to detect ocular complications secondary to chronic CRFs, including the development of choroidal neovascularization.

Staphyloma-related chorioretinal folds.

PURPOSE: To report a case of bilateral idiopathic chorioretinal folds that seemed to be related to an atypical staphyloma. OBSERVATIONS: A 49-year old man without medical history consulted for slight vision loss and metamorphopsia in the left eye. The ophthalmologic examination revealed moderate myopia and bilateral chorioretinal folds in the posterior pole, confirmed by multimodal imaging. Orbital and systemic examinations ruled out all the known etiologies of chorioretinal folds. 3-D optical coherence tomography and B-scan suggested that the folds were related to an atypical staphyloma that developed in the temporal part of the fundus, while sparing the peripapillary area. The peripapillary area, spared by the staphyloma process, appeared as a "dome-shaped disc" compared to the staphylomatous area. CONCLUSION AND IMPORTANCE: This case suggests that myopic patients with unusual staphyloma located outside the peripapillary area could develop chorioretinal folds.

Optical coherence tomography imaging of chorioretinal folds associated with hypotony maculopathy following pars plana vitrectomy.

Chorioretinal folds may occur as a consequence of hypotony and can be a cause of vision loss when associated with macular involvement. In this report, the spectral domain ocular coherence tomography imaging of three patients with chorioretinal folds before and after management are presented. The cases had unique presentations and each underwent different management approaches, but the results included improved visual acuities and lessened chorioretinal folds.