Other Choroidal Disorders




Choroidal Rupture



Jorge Ruiz-Medrano
Jay Chhablani
Clínico San Carlos University Hospital, Ophthalmology Unit, Madrid, Spain
LV Prasad Eye Institute, Hyderabad, India

Abstract


Choroidal ruptures involve a tear of the retinal pigment epithelium, Bruch’s membrane, and choroidal tissue as a consequence of trauma to the eye. Choroidal rupture caused by blunt trauma to the eye may occur at the site of the impact in the case of direct ruptures, or as a consequence of a countercoup, just opposite to it, in indirect tears. Most of them take place temporal to the optic disc and usually affect the macular area and the fovea. Diseases affecting the consistency of Bruch’s membrane are more susceptible to choroidal ruptures after trauma, like angioid streaks.


Visual prognosis is marked by location, presence of intraocular hemorrhage, retinal involvement (retinal tears and detachment, retinal edema), and development of choroidal neovascularization. Current therapeutic approaches are focused on the management of complications.


Keywords: Choroidal rupture; Trauma; Choroidal neovascularization; Bruch’s membrane; Angioid streaks


Introduction


The first report of a choroidal rupture goes back to 1854, when von Graefe described it for the first time. It involves a tear of the retinal pigment epithelium (RPE), Bruch’s membrane, and choroidal tissue as a consequence of trauma to the eye. Choroidal rupture caused by blunt trauma to the eye may occur at the site of the impact in the case of direct ruptures, or as a consequence of a countercoup, just opposite to it, in indirect tears.


According to published series, up to 80% of choroidal ruptures are classified as indirect, and the vast majority of these are caused by blunt trauma. Most of them take place temporal to the optic disc and usually affect the macular area and the fovea, showing a crescent shape. On the other hand, open-globe injuries appear to be the cause of choroidal ruptures in up to 28% of the cases. In these cases lesions are more typically located anteriorly, parallel to the ora serrata .


There are several theories about the mechanism that may cause an indirect choroidal break. The most accepted suggests that antero-posterior compressions and deformations cause an equatorial expansion of the globe. This force is conducted to the optic nerve and radiated from the peripapillary uveo-scleral tether. Bruch’s membrane tissue has properties that make it the most susceptible target to be damaged in indirect traumas. While retinal tissue is more elastic than Bruch’s membrane, the sclera shows more resistance to tension thanks to its stiffness. This is why they are both usually spared.


Diseases affecting the consistency of Bruch’s membrane are more susceptible to choroidal ruptures after trauma, like angioid streaks, as will be discussed later. Older theories relating the tears to vascular necrosis as a consequence of the trauma are not as supported as the previous one.


Ocular Manifestations and Clinical Course


The most common clinical presentation is a subretinal hemorrhage that may hide the actual tear in the early phases ( Fig. 19.1.1 ) and, in rare cases, there may be multiple breaks. After blood is resorbed, the choroidal rupture becomes visible taking a crescent shape, concentric to the optic nerve head ( Fig. 19.1.2 ). Although it has not been found to be a predictive factor by some authors, initial visual acuity (VA) ranges from 20/20 to light perception, depending on the affected area.




Figure 19.1.1


Fundus photograph showing choroidal rupture with subretinal hemorrhage.



Figure 19.1.2


Fundus photograph shows peripapillary choroidal rupture (A) and corresponding SD-OCT (B).


Visual prognosis is marked by location, presence of intraocular hemorrhage, retinal involvement (retinal tears and detachment, retinal edema), and development of choroidal neovascularization (CNV).


Secretan et al. published a review of 79 cases reaching several conclusions: They stated that ruptures located less than 200 µm from the foveal avascular zone (FAZ) and between 200 and 1500 µm from the FAZ were significantly more likely to develop CNV than those located farther than 1500 µm from the FAZ. They also found breaks larger than 4000 µm to predispose for the development of CNV, wherever they were located; 82% of their patients developed CNV during the first year of follow-up.


Treatment


Although traumatic CNV usually regress spontaneously, several treatments have been proposed through the years, from photodynamic therapy (PDT), going through surgical extraction and laser photocoagulation until the age of anti-vascular endothelial growth factor (VEGF) drugs ( Fig. 19.1.3 ).




Figure 19.1.3


Fundus photograph (A) shows choroidal rupture with suspicious CNV secondary to choroidal rupture. Fundus fluorescein angiography shows early hyperfluorescence (B) with late leakage (D). SD-OCT (E) suggests presence of CNV with subretinal fluid. After anti-VEGF therapy OCT shows scarred CNV with resolution of subretinal fluid.


Both bevacizumab and ranibizumab have shown good results for the treatment of CNV related to choroidal ruptures. Mean number of injections goes from 2.5 injections in 52 months up to monthly injections depending on the treatment regimen established. Rosina et al. state that patients free from CNV reactivation for about a year show a smaller recurrence rate. Furthermore, each recurrence increases the risk for further reactivation; therefore, frequent controls during follow-up are mandatory. Some authors report promising results, stabilizing or improving VA in 62.9% of the eyes treated with ranibizumab.




Angioid Streaks



Jorge Ruiz-Medrano
Jay Chhablani
Clínico San Carlos University Hospital, Ophthalmology Unit, Madrid, Spain
LV Prasad Eye Institute, Hyderabad, India

Abstract


Angioid streaks are degenerations and irregular breaks in Bruch’s membrane together with a consequent atrophy of the RPE above them, ophthalmoscopically visible as irregular lines that spread radially form the optic nerve. Calcium deposits make Bruch’s membrane more brittle and less resistant to trauma. Although they are not necessarily present in every affected patient, systemic associations of angioid streaks include pseudoxanthoma elasticum, Ehlers–Danlos and Marfan syndromes, Paget’s disease, acromegaly and several blood dyscrasias like thalassemia, spherocytosis, and sickle-cell anemia. Patients showing this disease are usually asymptomatic until complications arise. The most frequent of them is the development of traumatic CNV. Current therapeutic approaches are focused on the management of complications.


Keywords: Angioid streaks; Choroidal neovascularization; Choroidal rupture; Marfan; Pseudoxantoma elasticum


Introduction


Angioid streaks is the term used to describe visible irregular lines that spread radially form the optic nerve, with a variable width which funduscopically seem red-brown. It is their similarity to retinal vessels that is responsible for their name, and were first described in 1889 by Doyne during the exploration of traumatized eyes.


Angioid streaks are the ophthalmoscopic manifestations of calcific degeneration and irregular breaks in Bruch’s membrane together with a consequent atrophy of the RPE above them. Calcium deposits make Bruch’s membrane more brittle and less resistant to trauma (external, muscle traction, pressure on the eye), being responsible for the choroidal ruptures involved in these patients. Latest theories demonstrate an absence of a systemic antimineralization factor that leads to the calcification Bruch’s membrane and other connective tissues that are rich in elastic fibers. Although they are not necessarily present in every affected patient, systemic associations of angioid streaks include pseudoxanthoma elasticum, Ehlers–Danlos and Marfan syndromes, Paget’s disease, acromegaly and several blood dyscrasias like thalassemia, spherocytosis, and sickle-cell anemia. Other less common conditions associated to this pathology are listed in Table 19.2.1 .



Table 19.2.1

Systemic Associations of Angioid Streaks











































Pseudoxanthoma elasticum
Marfan syndrome
Ehlers–Danlos syndrome
Paget’s disease
Acromegaly
Sturge–Weber syndrome
Spherocytosis
Sickle-cell anemia
Thalassemia
Hemolytic anemia
Neurofibromatosis
Tuberous sclerosis
Epilepsy
Trauma hypertension
Diabetes
Diffuse lipomatosis
Hypercalcinosis
Hyperphosphatemia
Hemochromatosis
Alpha–beta-lipoproteinemia


Patients showing this disease are usually asymptomatic until complications arise. The most frequent of them is the development of traumatic CNV, and prognosis depends on the macular involvement.


Ocular Manifestations and Clinical Course


As described earlier, angioid streaks are funduscopically diagnosed and identified as irregular subretinal breaks or dehiscences that radiate from the optic nerve whose width is reported to vary from 50 to 500 μm. They may remain stable or increase their size, but they do not regress.


Similar to choroidal ruptures, patients stay asymptomatic as long as the macular area is not involved. Patients suffering from angioid streaks are very susceptible to choroidal breaks after the mildest of traumas, and 72–86% of cases present with CNV. These patients usually evolve to legal blindness as more than 70% of the cases are bilateral.


The early phase is characterized by metamorphopsia and/or blurred vision (ranging from 20/20 to light perception, depending on the location of the break) after mild trauma. The most common finding at this stage is a subretinal hemorrhage involving the macular area, which will later reveal CNV after a break in Bruch’s membrane at the affected spot. Fundus autofluorescence, fluorescein angiography (FFA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT) may help identify areas of angioid streaks or their complications ( Fig. 19.2.1 ).




Figure 19.2.1


Fundus photograph (A) and autofluorescence (B) images of an eye with angioid streaks and scarred choroidal neovascular membrane which is confirmed on SS-OCT scans (horizontal (C) and vertical (D)).


Treatment


There is no effective treatment to prevent the development of angioid streaks. Spaide et al. suggested the possibility that pyrophosphates could be used to treat pseudoxanthoma elasticum looking to halt abnormal mineralization of tissue including that seen in Bruch’s membrane, and so prevent the development of breaks and angioid streaks. Current therapeutic approaches are focused on the management of complications.


PDT, surgical extraction, laser photocoagulation, macular translocation, and transpupillary thermotherapy (TTT) were used with variable results and no significant long-term benefits. On the other hand, anti-VEGF intravitreal injections have surged as a new option during the last years as several studies have shown their potential benefits for the treatment of angioid streak-related CNV and seem to be able to stop the progression of CNV with significant improvement in visual outcomes. Tilleul et al. state that this kind of CNV seems to be more similar to myopia-related CNV than age-related macular degeneration (AMD), needing fewer injections than wet AMD. This group also highlights the importance of monitoring patients suffering from complications in one eye so as to be able to identify and treat the fellow eye in case of involvement as soon as possible and so prevent foveal scarring. Larger series with longer follow-up periods will be necessary to corroborate these reports of sustained visual improvement.




Choroidal Disorders: Staphyloma



Nan-Kai Wang
Chang Gung Memorial Hospital, Linkuo Medical Center, Kuei Shan, Taoyuan, Taiwan

Abstract


Staphyloma was first described by Antonio Scarpa in 1801 when he noticed pronounced outward bulges in the posterior portion of two eyes from a cadaver. In 1977, Curtin created a classification of staphyloma into 10 different types according to its location and size. Ohno-Matsui renamed the staphyloma into six types according to their location and distribution, which include wide macular, narrow macular, peripapillary, nasal, inferior, and other staphylomas.


Keywords: Staphyloma; Myopic refraction; Pathologic myopic; Peripapillary staphyloma; Tilted disc syndrome


Introduction


Staphyloma was first described by Antonio Scarpa in 1801 when he noticed pronounced outward bulges in the posterior portion of two eyes from a cadaver. In 1977, Curtin created a classification of staphyloma into 10 different types according to its location and size. Ohno-Matsui renamed the staphyloma into six types according to their location and distribution, which include wide macular, narrow macular, peripapillary, nasal, inferior, and other staphylomas.


Since Scarpa’s first description of staphyloma in 1801, he didn’t make the link to myopia. It is until 1856, when Arlt established a connection between a posterior staphyloma and myopic refraction. Therefore, the presence of a posterior staphyloma and high myopia were thought to be synonymous. However, staphyloma can exit in eyes without long axial length or high refractive error, such as tilted disc syndrome or age-related staphyloma.


Pathologic Myopia


Posterior staphyloma in pathologic myopia may present different types of myopic maculopathy, such as chorioretinal atrophy, Fuchs spots, patchy chorioretinal atrophy, and myopic traction maculopathy ( Fig. 19.3.1 ). Spectral-domain OCT (SD-OCT) is a very powerful tool to identify pathologic features in the retina and choroid, which may not be easy to observe using indirect ophthalmoscopy. In general, SD-OCT show decreased choroidal thickness with or without intraretinal lesion, such as macula retinoschisis ( Fig. 19.3.1A ), decreased refractivity of ellipsoid zone ( Fig. 19.3.1B ), patchy chorioretinal atrophy ( Fig. 19.3.1C ), CNV ( Fig. 19.3.1D ), macula hole, and retinal detachment. Wang et al. has reported that choroidal thickness is a better indicator of the severity of myopic maculopathy than axial length or refractive error. Further study from the same group showed that choroidal thickness has the strongest association with lacquer crack formation vs axial length and refractive error.




Figure 19.3.1


Fundus photography and SD-OCT of posterior staphyloma in pathologic myopia. SD-OCT shows decreased choroidal thickness and macula retinoschisis ( Fig. 19.3.1A , right); decreased refractivity of ellipsoid zone ( Fig. 19.3.1B ), patchy chorioretinal atrophy ( Fig. 19.3.1C ), and CNV ( Fig. 19.3.1D ).


Peripapillary Staphyloma


Peripapillary staphyloma has been classified as type III staphyloma by Curtin. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone ( Fig. 19.3.2A and B ).




Figure 19.3.2


Fundus photography and SD-OCT of peripapillary staphyloma. SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone (A and B).


Tilted Disc Syndrome


Fuchs first stressed the difference between the inferior location of the congenital crescent (Fuchs’ coloboma) and the temporal location of the acquired myopic type. In tilted disc syndrome, the retinal vessels emerge from the optic nerve in a nasal direction before turning toward to the temporal side of the fundus. Spaide has classified tilted disc syndrome as type V of inferior staphyloma. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone ( Fig. 19.3.3A and B ).




Figure 19.3.3


Fundus photography and SD-OCT of tilted disc syndrome. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone (A and B, left); A and B, right are fellows, eyes without titled disc syndrome.


Age-Related Staphyloma


Recently, Wang et al. reported 16 eyes from 10 elderly patients (mean age 70 years old) with posterior staphyloma resembling pathologic myopic maculopathy, but without an axial length longer than 26.5 mm ( Fig. 19.3.4 ). They found this disease is characterized by decreasing choroidal thickness, decreased ellipsoid zone reflectivity seen in SD-OCT scans, and lateral protrusions seen in three-dimensional (3D) magnetic resonance imaging (MRI) scans. However, it did not fit the criteria for pathologic myopia of excessive axial length or refractive error values. They assumed that this type of staphyloma might cause visual impairment in elderly patients and might be associated with the aging process, which is different from the axial elongation staphyloma in highly myopic eyes.




Figure 19.3.4


Fundus photography and SD-OCT of age-related staphyloma. SD-OCT shows decreased choroidal thickness and decreased refractivity of ellipsoid zone (A and B, left), macula retinoschisis (C, left), macular retinoschisis and foveal detachment (D), and CNV (E).




Choroidal Disorders: Staphyloma



Nan-Kai Wang
Chang Gung Memorial Hospital, Linkuo Medical Center, Kuei Shan, Taoyuan, Taiwan

Abstract


Staphyloma was first described by Antonio Scarpa in 1801 when he noticed pronounced outward bulges in the posterior portion of two eyes from a cadaver. In 1977, Curtin created a classification of staphyloma into 10 different types according to its location and size. Ohno-Matsui renamed the staphyloma into six types according to their location and distribution, which include wide macular, narrow macular, peripapillary, nasal, inferior, and other staphylomas.


Keywords: Staphyloma; Myopic refraction; Pathologic myopic; Peripapillary staphyloma; Tilted disc syndrome


Introduction


Staphyloma was first described by Antonio Scarpa in 1801 when he noticed pronounced outward bulges in the posterior portion of two eyes from a cadaver. In 1977, Curtin created a classification of staphyloma into 10 different types according to its location and size. Ohno-Matsui renamed the staphyloma into six types according to their location and distribution, which include wide macular, narrow macular, peripapillary, nasal, inferior, and other staphylomas.


Since Scarpa’s first description of staphyloma in 1801, he didn’t make the link to myopia. It is until 1856, when Arlt established a connection between a posterior staphyloma and myopic refraction. Therefore, the presence of a posterior staphyloma and high myopia were thought to be synonymous. However, staphyloma can exit in eyes without long axial length or high refractive error, such as tilted disc syndrome or age-related staphyloma.


Pathologic Myopia


Posterior staphyloma in pathologic myopia may present different types of myopic maculopathy, such as chorioretinal atrophy, Fuchs spots, patchy chorioretinal atrophy, and myopic traction maculopathy ( Fig. 19.3.1 ). Spectral-domain OCT (SD-OCT) is a very powerful tool to identify pathologic features in the retina and choroid, which may not be easy to observe using indirect ophthalmoscopy. In general, SD-OCT show decreased choroidal thickness with or without intraretinal lesion, such as macula retinoschisis ( Fig. 19.3.1A ), decreased refractivity of ellipsoid zone ( Fig. 19.3.1B ), patchy chorioretinal atrophy ( Fig. 19.3.1C ), CNV ( Fig. 19.3.1D ), macula hole, and retinal detachment. Wang et al. has reported that choroidal thickness is a better indicator of the severity of myopic maculopathy than axial length or refractive error. Further study from the same group showed that choroidal thickness has the strongest association with lacquer crack formation vs axial length and refractive error.




Figure 19.3.1


Fundus photography and SD-OCT of posterior staphyloma in pathologic myopia. SD-OCT shows decreased choroidal thickness and macula retinoschisis ( Fig. 19.3.1A , right); decreased refractivity of ellipsoid zone ( Fig. 19.3.1B ), patchy chorioretinal atrophy ( Fig. 19.3.1C ), and CNV ( Fig. 19.3.1D ).


Peripapillary Staphyloma


Peripapillary staphyloma has been classified as type III staphyloma by Curtin. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone ( Fig. 19.3.2A and B ).




Figure 19.3.2


Fundus photography and SD-OCT of peripapillary staphyloma. SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone (A and B).


Tilted Disc Syndrome


Fuchs first stressed the difference between the inferior location of the congenital crescent (Fuchs’ coloboma) and the temporal location of the acquired myopic type. In tilted disc syndrome, the retinal vessels emerge from the optic nerve in a nasal direction before turning toward to the temporal side of the fundus. Spaide has classified tilted disc syndrome as type V of inferior staphyloma. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone ( Fig. 19.3.3A and B ).




Figure 19.3.3


Fundus photography and SD-OCT of tilted disc syndrome. The SD-OCT scans show decreased choroidal thickness at the slope of staphyloma and decreased reflectivity of ellipsoid zone (A and B, left); A and B, right are fellows, eyes without titled disc syndrome.


Age-Related Staphyloma


Recently, Wang et al. reported 16 eyes from 10 elderly patients (mean age 70 years old) with posterior staphyloma resembling pathologic myopic maculopathy, but without an axial length longer than 26.5 mm ( Fig. 19.3.4 ). They found this disease is characterized by decreasing choroidal thickness, decreased ellipsoid zone reflectivity seen in SD-OCT scans, and lateral protrusions seen in three-dimensional (3D) magnetic resonance imaging (MRI) scans. However, it did not fit the criteria for pathologic myopia of excessive axial length or refractive error values. They assumed that this type of staphyloma might cause visual impairment in elderly patients and might be associated with the aging process, which is different from the axial elongation staphyloma in highly myopic eyes.




Figure 19.3.4


Fundus photography and SD-OCT of age-related staphyloma. SD-OCT shows decreased choroidal thickness and decreased refractivity of ellipsoid zone (A and B, left), macula retinoschisis (C, left), macular retinoschisis and foveal detachment (D), and CNV (E).




Focal Choroidal Excavation



Bindu Rajesh
Jay Chhablani
Giridhar Eye Institute, Kochi, Kerala, India
LV Prasad Eye Institute, Hyderabad, India

Abstract


A focal area of choroidal excavation without associated posterior staphyloma or ectasia has been termed a focal choroidal excavation, which has been suggested to be idiopathic or congenital in origin. Usually unilateral, it is best detected on SD-OCT as a focal excavation in the RPE choriocapillary band. Conforming and nonconforming types are the most common morphological patterns noted and associations with central serous chorioretinopathy, choroidal neovascular membrane and polypoidal choroidal vasculopathy have resulted in expansion of the clinical spectrum.


Keywords: Choroidal excavation; Central serous chorioretinopathy; Choroidal neovascular membrane; Conforming; Focal; Nonconforming


A focal choroidal excavation (FCE) is defined as a focal area of choroidal excavation at the macula, which can be detected on an OCT scan without associated posterior staphyloma or scleral ectasia. This condition was first reported by Jampol et al. in 2006 using time domain OCT. The presence of good visual acuity and normal overlying retina were associated salient features initially observed. The absence of history of trauma, retinal, or choroidal vascular or inflammatory etiologies probably supported an idiopathic or a congenital etiology. As FCE is said to involve the RPE, the choriocapillaries, and the photoreceptor layers, Lui et al. have suggested that it could also result from possible aberrant regeneration of the chorioretina.


FCE in general is unilateral in nature but bilateral cases have been reported in 16% with an Asian preponderance noted in various studies. These excavations are best detected on SD-OCT as an outward curve in the RPE choriocapillary band in the 2D image or as a choroidal excavation in the 3D image. It is usually located in the perifovea around the horizontal raphe. The location and extent of the lesion usually determines affection of visual acuity.


Clinically the fundus appearance is either normal or reveals a focal area with localized pigmentary changes. Two basic morphological types of FCE have been described by Wakabayashi et al. : the “ conforming ” type wherein the outer retinal layers conform to the FCE without any intervening hyporeflective space or outer retinal layer disruption ( Fig. 19.4.1 ) and the “ non conforming ” type ( Fig. 19.4.2 ) which reveals disruption of the photoreceptor tips with a hyporeflective clear space between the RPE and the photoreceptor tips.




Figure 19.4.1


Conforming type of focal choroidal excavation (FCE) showing no disruption of outer retinal structures with the outer retinal layers in continuity with the excavation.



Figure 19.4.2


Nonconforming type of focal choroidal excavation (FCE) revealing a hyporeflective clear space between the FCE and the outer retinal layers with disruption of the photoreceptors.


Based on the contour, Guo et al. have also classified this excavation into two types: Type 1 : small excavation associated with a sharp cut down contour and Type 2 : slightly larger excavation with a gradual sloping edge. Shinojima classified FCE into three types based on their shapes: cone-shaped , bowl-shaped , and mixed . In their series, the cone-shaped type was the most common while the bowl-shaped and mixed patterns revealed retinal pigment epithelial irregularities within the FCE lesion. Among these various classifications, the conforming and nonconforming subtypes are the most commonly used and these subtypes have usually been reported to undergo mutual conversion from conforming into nonconforming types and vice versa. Weakening of the choroid with age with resultant enlargement of the excavation or resolution of the subretinal fluid or coexistent pathology may probably explain the mutual conversion that occurs over the years.


Initially FCE was considered as an isolated etiology, however with the advent of SD-OCT, conditions like central serous chorioretinopathy (CSCR), age related macular degeneration with choroidal neovascular membrane (AMD CNVM), and polypoidal choroidal vasculopathy (PCV) have been reported with FCE resulting in an expansion of the clinical spectrum. Other rare reported associations include cone dystrophy, Best macular dystrophy, vitreomacular traction with macular hole, and inflammatory disorders like multifocal choroiditis, punctate inner choroidopathy, and Vogt Koyanagi Harada syndrome. Among these, the highest prevalence of FCE has been observed in eyes with CSCR.


Though choroidal vascular changes and hyperpermeability are common to both CSCR and FCE, why CSCR occurs in FCE is still unknown. Suzuki et al. suggested that probably the disruption of the choroidal circulation and atrophic RPE at the FCE would be etiological in the development of CSCR in these patients. Occurrence of CNV membrane ( Fig. 19.4.3 ) is one of the causes of vision loss in FCE. An aberrant choroidal vasculature with a probable defect in the Bruch’s membrane was a possible risk factor for occurrence of a CNV membrane in these eyes.


Sep 8, 2018 | Posted by in OPHTHALMOLOGY | Comments Off on Other Choroidal Disorders

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