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DOME SHAPED MACULOPATHY Ιωάννης Ν. Βαγγελόπουλος Χειρ. Οφθαλμίατρος - Βόλος

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Page 1: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

DOME SHAPED MACULOPATHY

Ιωάννης Ν. Βαγγελόπουλος

Χειρ. Οφθαλμίατρος - Βόλος

Page 2: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

DOME SHAPED MACULOPATHY-DEFINITIONS

• The entity Dome Shaped Macula ( DSM ) was first described by Gaucher

and associates in 2008 by the use of time-domain optical coherence

tomography (OCT) as an inward convexity or bulge of the macula within the

concavity of the posterior staphyloma

“We describe in this study an entity characterized by a convex elevation of

the macula within a myopic staphyloma.”

• Only in myopic eyes and always with Posterior Staphyloma, Type I or II

• It is seen in approximately 10.7% of eyes with high myopia

Gaucher D, Erginay A, Lecleire-Collet A, et al. Dome-shaped macula in eyes

with myopic posterior staphyloma. Am J Ophthalmol. 2008;145(5):909-914.

Page 3: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

SYMPTOMS

• The dome-shaped macula seems to be a new cause of visual impairment in myopic

eyes

Symptoms

• Visual loss and

• Metamorphopsia

Page 4: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

EXAMPLES

(Top Left) Fundus photograph showing pigmentary

changes present in the fovea. Arrowheads delimit

the area of the staphyloma;

(Top right) Late-phase fluorescein angiography (FA)

image showing a leakage point (arrow)

Serous retinal detachment (arrowheads).

(Middle left) ICGA showing no sign of choroidal new

vessels.

(Middle right) Late-phase ICGA image showing

hypofluorescence in the area of RPE disturbance

and focal hyperfluorescence (arrow) corresponding

to the leakage point observed on FA image.

(Bottom left) Vertical OCT scan. This bulge affects

the retina, the RPE, and the inner choroid. The

foveal retina is slightly detached at the top of the

macular bulge (asterisk) and above the fovea

(arrow), corresponding to the serous detachment

seen on FA image.

(Bottom right) Ultrasonography image confirming

the abnormal anterior bulge at the posterior pole of

the globe,.

Page 5: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

(B, C), OCT images across the central fovea. The sclera is thickened in the macula and shows aninward bulge in the horizontal section (B) and vertical section (C). The fovea (arrowhead in B) islocated temporal to the top of the dome in the horizontal section.

(E, F), OCT image across the central fovea. The sclera is thickened in the macula and shows aninward bulge in both the horizontal (E) and vertical (F) sections. The fovea (arrowhead in E) is locatedslightly temporal to the top of the dome

Page 6: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

EXAMPLES – DSM seen only in Horizontal OCT section

An inward bulge due to a DSM is observed only in the horizontal OCT section (B) and not inthe vertical section (C). The central fovea (arrowhead in B) is located temporal to the top ofthe dome.

Page 7: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

A staphyloma is defined as an abnormal local protrusion of thin sclera and uveal tissue

and was categorized into 10 types by Curtin

Drawings showing the classification of the different types of

posterior staphyloma according to Curtin

Curtin BJ, Karlin DB. Axial length measurements and fundus changes of

the myopic eye. Am J Ophthalmol 1971;1:42–53.

Page 8: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Curtin BJ, Karlin DB. Axial length measurements and fundus

changes of the myopic eye. Am J Ophthalmol 1971;1:42–53.

Because the DSM feature could not be categorized to any subtypes of staphyloma, it

should be considered a novel, posterior anatomic characteristic of some highly myopic

eyes and not as a staphyloma subtype.

It is believed that the dome-shaped macula is not related to the type of staphyloma, but

rather is related to an anatomic or structural change within the sclera and can occur in eyeswith any type of staphyloma.

Three-Dimensional Tomographic Features of Dome-Shaped Macula by Swept-Source

Optical Coherence Tomography

Am J Ophthalmol 2013;155:320–328.

Page 9: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Clues that a dome-shaped macula (DSM) might be present

A, B, Macular pigmentation (arrow) is seenfunduscopically as increased pigmentation (A)and as hypoautofluorescence (B).C-E, Horizontal ridge-like lesion connecting theoptic disc and the fovea (between arrows).

1 - Macular pigmentation2 - Ridge-like connection3 - Oval optic disc

The horizontal ridge is observed funduscopicallyas a yellowish-white linear lesion (C), ashyperautofluorescent (D), and ashyperfluorescent (E).In stereoscopic observations, the horizontalridge seems to slightly protrude toward thevitreous.F, G, Horizontally oval optic disc (arrowheads).

Page 10: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Horizontal RidgeA, Right fundus showing 2 yellowishlinear lesions connecting the opticdisc and the macula horizontally(arrows).

B, Fundus autofluorescence areobserved as hyperautofluorescence(arrows).

C, Infrared image shows a horizontalridge as hyperreflectance (arrows).

D, E, OCT images . An inward bulgedue to a DSM is observed only inthe vertical OCT section (E) and notin the horizontal section (D).

F, G, Vertical OCT images acrossthe horizontal ridge nasal to thecentral fovea.Two cross-sections of hyporeflectivestructures (arrowheads) areobserved in the sclera (F). There is anotch-like dent (arrow) between the2 horizontal protrusions (G).

Page 11: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Causes

Several authors have suggested possible causes for this abnormal morphology,

including

Scleral infolding similar to hypotony maculopathy,

Vitreous traction, and

Choroidal thickening.

However, more recent evidence provided by enhanced depth imaging OCT (EDI-

OCT), has shown that the usual cause is localized thickening of the sclera

underneath the macula in highly myopic eyes

Gaucher D, Erginay A, Lecleire-Collet A, et al. Dome-shaped

macula in eyes with myopic posterior staphyloma. Am J

Ophthalmol. 2008;145(5):909-914.

Mehdizadeh M, Nowroozzadeh MH. Dome-shaped macula in

eyes with myopic posterior staphyloma. [Correspondence].

Am J Ophthalmol. 2008;146(3):478.

Imamura Y, Tomohiro I, Maruko I, Zweifel SA, Spaide RF. Enhanced

depth imaging optical coherence tomography of the sclera in dome-shaped macula. Am J Ophthalmol. 2011;151(2):297-302

Page 12: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

CAUSE

The external surface of the sclera is seen clearly (white arrowheads), and the sclera shows uneventhinning in the posterior pole, being relatively thick beneath the fovea

Page 13: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Complications in the Eyes With a Dome-Shaped

Macula

• Choroidal neovascularization

• Serous retinal detachment

• Diffuse chorioretinal atrophy

• Patchy chorioretinal atrophy

• Lamellar macular hole

• Full-thickness macular hole

• Foveal schisis

• Extrafoveal retinal schisis

Page 14: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Macular abnormalities in DSM

• Foveal serous RD was the most common finding,

• Observed in almost one third of the eyes with dome-shaped macula and

• In 44% of the eyes with dome-shaped macula without myopic CNV

The origin of subretinal fluid in dome-shaped macula eyes without choroidalneovascularization is not known, as is the therapeutic mechanism of action of verteporfinPDT in this condition

Hypothesis - Subretinal fluid can occur in eyes because of the obstruction of outflow ofchoroidal fluid by a thick sclera

The effect is similar to central serous chorioretinopathy, in which PDT is presumed to closehyperpermeable vascular channels in the choroid

Serous RD is generally described in highly myopic eyes with

CNV

Tractional phenomena

Dome-Shaped Macula or

Tilted disc syndrome

Choroidal Findings in Dome-Shaped Macula in Highly Myopic Eyes: A Longitudinal Study

FRANCESCO VIOLA, LAURA DELL’ARTI, ELEONORA BENATTI, ALESSANDRO INVERNIZZI,

CHIARA MAPELLI,FABIO FERRARI, ROBERTO RATIGLIA, GIOVANNI STAURENGHI, AND

GIULIO BARTESELLI Am J Ophthalmol 2015;159:44–52.

Three-Dimensional Tomographic Features of Dome-Shaped Macula by Swept-Source Optical

Coherence Tomography

Am J Ophthalmol 2013;155:320–328.

Page 15: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Treatment

The only two studies reporting treatment of subretinal fluid in dome-shaped maculadescribe the unsuccessful use of argon laser photocoagulation

Anti-VEGF injections are without benefit.

• Although very limited, it is reasonable to consider half-fluence verteporfin PDT astreatment for patients with nonresolving subretinal fluid associated with dome-shapedmacula.

• Application of low-intensity laser photocoagulation, similar to that employed in centralserous retinopathy, seems a reasonable alternative for eyes with focal areas of leakagethat are located safely outside the fovea, particularly if PDT has failed to resolve the fluid.

• Although additional experience is needed to confirm the efficacy of these treatments indome-shaped macula,

• The risk-benefit ratio appears acceptable for patients with significant visual symptoms ordisability.

Gaucher D, Erginay A, Lecleire-Collet A, et al. Dome-shaped macula in eyes with myopic

posterior staphyloma. Am J Ophthalmol. 2008;145(5):909-914.

Pardo-Lopez D, Gallego-Pinazo R, Meteo C, et al. Serous macular detachment associated

with dome-shaped macula and tilted disc. Case Report Ophthalmol. 2011;2(1):111-115.

Treatment of Subretinal Fluid Associated With Dome-Shaped Macula

Nicholas D. Chinskey, MD; Mark W. Johnson, MD

Ophthalmic Surg Lasers Imaging Retina. 2013;44:593-595

Page 16: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

RADIANCE Study

• Patients with visual impairment due to myopic CNV

• A total of 277 baseline scans have been analyzed for the presence of DSM.

• Identified a total of 50 patients (18%) with the DSM feature.

• Of the 50 patients with DSM, 38 were treated with intravitreal ranibizumab and 12

with verteporfin photodynamic therapy (vPDT) at baseline.

• Comparing visual outcome in patients treated with ranibizumab showed no

differences in visual gain at month 3, in patients with DSM compared with patients

without DSM.

• The number of injections at month 12 was similar in both groups.

• At month 12 follow-up, no relevant differences in visual gain between the groups were

detected.

• Comparison of patients treated with vPDT having DSM or no DSM showed

numerically better outcomes at month 3 for patients with DSM at baseline

• This is an important finding, because the DSM feature is a frequent finding in patients

with myopic CNV.

RADIANCE: A Randomized Controlled Study of Ranibizumab in Patients with Choroidal

Neovascularization Secondary to Pathologic Myopia

Ophthalmology 2014;121:682-692

Page 17: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Case report-Treatment with PDT

Early (A) and late (B) fluorescein angiographyimages of the right eye show mild late leakage nasalto the fovea without definable choroidalneovascularization.

(C) OCT demonstrates DSM with subretinal fluid inthe right eye and

(D) without fluid in the left eye.

(E) OCT of right eye 6 months after PDT showsresolution of subretinal fluid.(F) Subretinal fluid has reaccumulated in the righteye 2 years after PDT.

(G) Six months after a second PDT treatment,subretinal fluid has decreased but not completelyresolved .

(H) OCT imaging 3 months after grid laser showscomplete resolution of subretinal fluid .

Page 18: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Case report-Treatment with half-fluence verteporfin PDT

Case 2. Fundus photographs of the right (A) and left(B) eye demonstrate absence of drusen and pigmentmottling.

Early (C) and late (D) fluorescein angiographyimages of the left eye show mild diffuse late leakagein the central macula without definable choroidalneovascularization.

OCT of the right (E) and left (F) eyes shows bilateraldome-shaped macula with subretinal fluid in the lefteye.

Four months after PDT (G), the subretinal fluid hasresolved completely in the left eye.

Page 19: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Differential Diagnosis

• Idiopathic central serous chorioretinopathy,

• Neovascular Age-related Macular Degeneration, or

• Myopic Choroidal Neovascularization

Page 20: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

FIBROVASCULAR PED

Gass JD. Biomicroscopic and histopathologic considerations

regarding the feasibility of surgical excision of subfoveal neovascular membranes.

Am J Ophthalmol 1994;118:285–298.

Type 1 Type 2

Page 21: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Central Serous Retinopathy - EDI OCT

The conventional OCT scans before (E )and 1 month after (F ) PDT show completeresolution of central serous retinaldetachment.

The EDI-OCT scans before (G) and after(H) PDT show that the choroid wasthickened initially and decreased 1 monthafter PDT (H, double arrowhead ).

Choroidal vascular changes seen only inside the inward bulge within the posterior

staphyloma and not widely spread in the entire choroid like CSR

Enhanced depth imaging optical coherence tomography of the

sclera in dome-shaped macula. Am J Ophthalmol.

2011;151(2):297-302

Page 22: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Central Serous Retinopathy

Serous detachments of the neurosensory retina in the macular region.

Page 23: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Differential diagnosis for myopicchoroidal neovascularisation(CNV):

(A and B) haemorrhage due tolacquer cracks;

(C) dome-shaped macula withserous retinal detachment; and

(D and E) macular fluid due tostaphyloma.

Page 24: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

Conclusion

• New Entity

• Symptomatic patients

• Exclude CNV

• Anti – VEGF ineffective

• PDT and Argon laser

Page 25: DOME SHAPED MACULOPATHY - Livemedia.gr · the macula within a myopic staphyloma.” • Only in myopic eyes and always with Posterior Staphyloma, Type I or II • It is seen in approximately

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