Dome-Shaped Macula in Eyes with Myopic PosteriorStaphyloma

DAVID GAUCHER, ALI ERGINAY, AMELIE LECLEIRE-COLLET, BELKACEM HAOUCHINE, MICHEL PUECH,SALOMON-YVES COHEN, PASCALE MASSIN, AND ALAIN GAUDRIC

! PURPOSE: To describe an unusual feature in myopiceyes responsible for visual loss, which we call a dome-shaped macula.! DESIGN: Retrospective, observational case series.! METHODS: After observing isolated cases of dome-shaped macula, we analyzed optical coherence tomogra-phy (OCT) scans of 140 highly myopic eyes present inour OCT database to find similar cases. Fifteen eyes of 10patients had a dome-shaped macula. These patients allhad undergone fluorescein angiography (FA), indocya-nine green angiography (ICGA), and B-scan ultrasonog-raphy examinations.! RESULTS: The mean refractive error of the affectedeyes was !8.25 diopters (D; range, !2 to !15 D).Median visual acuity was 20/50. Recent visual impair-ment was noted in 11 of the 15 eyes studied, andmetamorphopsia was noted in eight eyes. Four eyes wereasymptomatic. FA showed atrophic changes in the mac-ular retinal pigment epithelium (RPE) in all eyes, com-bined with focal points of leakage in seven of the 15 eyes.The dome-shaped appearance of the macula was visibleon both B-scan ultrasonography and OCT: a character-istic bulge of the macular retina, RPE, and choroidwithin the concavity of the moderate posterior staphy-loma was present in all eyes. In 10 eyes, OCT alsoshowed a shallow foveal detachment at the top of thedome-shaped macula.! CONCLUSIONS: A dome-shaped macula within a myo-pic staphyloma is an unreported type of myopic posteriorstaphyloma. The dome-shaped macula often is associatedwith RPE atrophic changes and foveal retinal detach-ment, which may explain the visual impairment in theseeyes. (Am J Ophthalmol 2008;145:909–914. © 2008by Elsevier Inc. All rights reserved.)

D URING THE LAST DECADE, OPTICAL COHERENCEtomography (OCT) helped to define new dis-turbances in the posterior pole of myopic eyes

such as foveoschisis,1 peripapillary intrachoroidal cavi-tation,2 and retinal vascular microfolds.3 OCT also hasproved useful in detecting asymptomatic macular holebetter than biomicroscopy.4

We describe in this study an entity characterized by aconvex elevation of the macula within a myopic staph-yloma. OCT images will show this particular morpho-logic feature of the posterior pole, which we call adome-shaped macula.

METHODS

BECAUSE WE SAW SEVERAL MYOPIC PATIENTS REFERREDfor unexplained visual loss whose OCT examinationrevealed the unusual feature of a dome-shaped macula(Figures 1 to 3), we reviewed the images of the 140myopic eyes from our OCT database in a search forsimilar profiles. We found 15 eyes in 10 patients havingsuch a dome-shaped macula. A retrospective study ofthese patients’ files revealed that they all had reportedloss of vision in at least one eye.

All these patients had undergone one or more com-plete ophthalmologic examinations during their follow-up, which included best-corrected visual acuity (VA)assessment with Snellen VA charts, intraocular pressuremeasurement, slit-lamp examination of the anteriorsegment, and fundus biomicroscopy. All patients alsounderwent one color photograph of the fundus session,at least one fluorescein angiography (FA) examination,and indocyanine green angiography (ICGA) examina-tion of both eyes during their follow-up.

Optical coherence tomography 3 examination (Stra-tus OCT; Carl Zeiss Meditec, Inc, Dublin, California,USA) was performed in both eyes of each patient asfollows: cross-sectional images of the posterior pole wererecorded with mapping software and multiple line soft-ware with 4-, 5-, 6-, or 8-mm long scans. Short scanswere used to obtain better contrast because they made iteasier to pass through mild media opacities and toreduce signal disturbances. Incident light intensity,polarization, and dioptric controls of the optic system

Accepted for publication Jan 5, 2008.From the Assistance Publique–Hôpitaux de Paris, Hôpital Lari-

boisière, Service d’Ophtalmologie; Université Paris 7, Paris, France(D.G., A.E., A.L.-C., B.H., P.M., A.G.); the Centre Explore Vision,Paris, France (M.P.); and the Hôpital Lariboisière, Serviced’Ophtalmologie and Centre d’Imagerie et de Laser, Paris, France(S.-Y.C.).

Inquiries to Alain Gaudric, Service d’Ophtalmologie de l’HôpitalLariboisière, 2, rue Ambroise Paré, 75475 Paris cedex 10, France; e-mail:alain.gaudric@lrb.aphp.fr

© 2008 BY ELSEVIER INC. ALL RIGHTS RESERVED.0002-9394/08/$34.00 909doi:10.1016/j.ajo.2008.01.012

always were adjusted to obtain the finest possible im-ages. A 2-dimensional ultrasonic examination was per-formed in 11 of the 15 eyes studied using a 20-MHzprobe (Cinescan; Quantel-Medical SA, Clermont-Fer-rand, France).

RESULTS

THE STUDY COHORT CONSISTED OF 15 EYES OF 10 PATIENTS(eight women and two men). The mean age of the patients was51 years (range, 26 to 83 years). No history of systemic disease

FIGURE 1. Images showing a dome-shaped macula within a myopic posterior staphyloma in the moderately myopic right eye of a41-year-old man with visual acuity (VA) of 20/40 and a refractive error of !5; this unusual macular feature can be combined witha foveal retinal detachment. (Top Left) Fundus photograph showing pigmentary changes present in the fovea. Arrowheads delimitthe area of the staphyloma; white arrow indicates the direction of the optical coherence tomography (OCT) scan below. (Top right)Late-phase fluorescein angiography (FA) image showing a leakage point (arrow) at the margin of the area of retinal pigmentepithelium (RPE) disturbance. The contour of a serous retinal detachment extending above the macula also was visualized(arrowheads). (Middle left) Early-phase indocyanine green angiography (ICGA) image showing normal choroidal vascularizationand no sign of choroidal new vessels. (Middle right) Late-phase ICGA image showing well-demarcated foveal oval-shapedhypofluorescence in the area of RPE disturbance and focal hyperfluorescence (arrow) corresponding to the leakage point observedon FA image. (Bottom left) Vertical OCT scan showing the macular profile exhibiting an unusual convexity within the generalconcavity of the staphyloma. This bulge affects the retina, the RPE, and the inner choroid. The foveal retina is slightly detachedat the top of the macular bulge (asterisk) and above the fovea (arrow), corresponding to the serous detachment seen on FA image.(Bottom right) Ultrasonography (20-MHz probe) image confirming the abnormal anterior bulge at the posterior pole of the globe,but not showing whether this bulge also affects the sclera.

AMERICAN JOURNAL OF OPHTHALMOLOGY910 MAY 2008

was reported for any patient. The median refractive error of theeyes studied was !8.25 diopters [D] (range, !2 to !15 D). Thecharacteristics of the eyes are reported in the Table.

The dome-shaped appearance of the macula was bilat-eral in five patients and unilateral in five. All patientsreported visual loss in at least one eye. In all, visual loss waspresent in 11 eyes, and metamorphopsia was present ineight eyes. Four fellow eyes of the five bilateral cases ofdome-shaped macula were asymptomatic. The median VAof the eyes studied was 20/50 (median logarithm of theminimum angle of resolution [logMAR] VA, 0.4).

On fundus biomicroscopy, all eyes exhibited a type I orII myopic staphyloma, according to the Curtin classifica-tion, that is, tessellation and pallor extended over ahorizontal elliptical area nasal to the optic nerve, up to avariable distance from the temporal side of the macula.

Five eyes also exhibited some degree of papillary dysver-sion, but without an inferior staphyloma. Posterior vitreousdetachment (PVD) was present in four of the 15 eyes, inwhich a Weiss ring was seen. No PVD was detected ineight other eyes, and PVD was questionable in the remain-ing three. In all 15 eyes, FA showed various degrees ofchange in the retinal pigment epithelium (RPE), combin-ing atrophy and pigment clumping. In seven of the 15 eyes,points of focal leakage in the macular area were detected atleast once during the follow-up (Figure 1). However,choroidal neovascularization was ruled out in all cases byFA and ICGA and by OCT. Late-phase ICGA framesshowed hypofluorescence of the central macula corre-sponding to the areas of RPE disturbance (Figures 1 and 2).

Optical coherence tomography examination showed anunusual feature of the macular profile, especially clear on

FIGURE 2. Images showing a dome-shaped macula within a myopic posterior staphyloma in the highly myopic left eye of a50-year-old woman with VA of 20/50 and refractive error of !12.5; another example of a dome-shaped macula combined with afoveal retinal detachment. (Top left) Color fundus photograph showing a mild staphyloma with some pigmentary disturbances inthe fovea. White arrow indicates the direction of the OCT scan below. (Top right) FA image showing hyperfluorescence in the fovearesulting from a RPE defect, but no leakage point. (Middle left) Fundus autofluorescence image showing mild atrophy of the RPEclearly visible as a dark oval area in the fovea. (Middle center) Early-phase ICGA image with normal results. (Middle right)Late-phase ICGA image showing only mild hypofluorescence in the area of RPE atrophy. (Bottom left) Vertical OCT scan showingthe anterior bulge of the macula involving the retina, RPE, and inner choroid. A shallow retinal detachment is present at the topof the macular bulge. (Bottom right) B-scan ultrasonography image showing the alteration of the macular curvature within theposterior staphyloma.

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vertical scans: the macula exhibited a convex, curved,elevated profile within the concavity of the staphyloma.Both the RPE and choroid also appeared to be elevated.Retinal thickness was normal, but the retina was slightlydetached in 10 of the 15 eyes studied (Figures 1 and 2).One eye was examined with spectral-domain OCT (3DOCT-1000; Topcon, Tokyo, Japan), which allowed 3-di-mensional (3D) reconstruction of the elevated macula inthe staphyloma (Figure 3). No epiretinal membrane, par-tially adherent posterior hyaloid, or vitreous traction onthe macula were detected in any of the 15 eyes studied.

B-scan ultrasonography examinations were performedusing a 20-MHz probe, which also confirmed the convexchange in the curvature of the posterior pole in themacular area (Figures 1 and 2). There was no sign of tumoror of an infiltration process in the choroidal layer. How-ever, it was not possible to see whether this convexcurvature of the macular profile was the result of thechoroid, which seemed to be thickened in some eyes(Figure 1), or to a change in the scleral wall shape orthickness. The impression was that the eye wall kept itsconcave shape. A shallow retinal detachment, sometimespresent on OCT, was not detected by ultrasonography.

The mean axial length of the eyes was 26.92 mm (range,24.4 to 29.2 mm). On ultrasonography, PVD was presentonly in two of the 11 eyes studied.

DISCUSSION

IN THIS CASE SERIES OF MYOPIC EYES, WE REPORT A PAR-ticular type of macular profile within a posterior myopicstaphyloma. This feature may be responsible for visual loss.This abnormal feature, which we called a dome-shapedmacula, may be responsible for visual loss. It is character-ized by an inward bulge inside the chorioretinal posteriorconcavity of the eye, in the macular area. This macularbulge was hardly detectable on fundus biomicroscopy, andOCT and ultrasonography greatly helped to diagnose it.

Of interest is the fact that we have never observed suchan anomaly either in nonmyopic eyes or in myopic eyeswithout posterior staphyloma. Here, all cases indeed hadtype I or II primary myopic staphylomas combined with thedome-shaped macula, as evidenced by the tessellation andpallor extended over a horizontal elliptical area involvingthe optic disk and macula. The authors are unaware of any

FIGURE 3. Images showing a dome-shaped macula within a myopic posterior staphyloma in the highly myopic right eye of a83-year-old woman with VA of 20/32 and a refractive error of !13; this unusual macular feature is not always combined with afoveal detachment. (Top left) Fundus photograph showing a mild staphyloma and discrete RPE changes in the fovea. White arrowcorresponds to the direction of the OCT scan. (Bottom left) Late-phase FA image showing mild hyperfluorescence without leakageat the temporal edge of the fovea. (Top right) On a horizontal 9-mm OCT scan, the dome-shaped profile of the macula ischaracteristic. No serous retinal detachment is present and central macular thickness is normal (219 "m). (Bottom right)Three-dimensional (3D) reconstruction based on spectral-domain OCT images showing the bulge formed by the macula within thestaphyloma.

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previous reports of such a myopic staphyloma, combiningtype I or II primary staphyloma with a macular anteriorbulge. They could find no reference to such a feature,which may correspond to another type of compoundstaphyloma described by Curtin.5

Posterior staphyloma is associated more frequently witha high level of myopia or elevated eye axial length.6 In fiveof the 15 eyes studied, spherical equivalent refraction wasless than 6 D, and axial length was less than 26.5 mm infive of the 11 eyes measured. Although posterior staphy-lomas already have been reported in eyes with mild ormoderate myopia, one may wonder whether the lowspherical equivalent refractions and short axial lengths weobserved in these eyes are not the result of the unusualform of the dome-shaped macula. For instance, in one ofour patients, the axial lengths measured, respectively, fromthe cornea to the foveola and from the cornea to the opticdisk differed by more than 1 mm. It looked as if thismacular bulge enabled the eye to be less myopic than itwould have been because of the staphyloma.

Ultrasonography failed to show whether the scleraexhibited a local convex inversion of its curvature at themacular level. On the contrary, in some eyes, the shape ofthe scleral staphyloma did not seem affected, and theconvexity of the macula seemed to be the result of thechoroid, which looked as if it was thickened in the maculararea in some eyes (Figure 1), although no choroidalhyperperfusion was detected on ICGA.

The dome-shaped macula seems to be a new cause ofvisual impairment in myopic eyes: in this series, all thepatients reported visual loss and metamorphopsia. How-

ever, in four patients who had bilateral lesions and whoreported visual loss in one eye, the fellow eye wasasymptomatic.

Atrophic changes in the RPE were present in all theeyes studied, and foveal detachment was present in 10 ofthem. These changes may explain the visual impairmentand metamorphopsia reported by the patients. On FA,foveal detachment was associated with focal leakage inseven of these 10 eyes and extended over the sloping areasaround the macula in two cases (Figure 1). Foveal detach-ment resolved spontaneously in two cases, and in anothercase, focal laser treatment was performed, but withoutsuccess. We postulated that in eyes with a dome-shapedmacula, foveal detachment was a complication of theabnormal dome-shaped curvature of the macula. Themechanism of such a complication is unknown. Thefluorescein dynamics of the leaking points may mimicthose of chronic idiopathic central serous chorioretinopa-thy. Here, when present, they indeed appeared relativelylate in the sequence, were moderately active, and occurredin an area of atrophic changes in the RPE. However, theydisplayed the particular feature of being localized at the topof the dome-shaped macula. This area was hypofluorescenton the late frames of ICGA, indicating the presence ofsome degree of choriocapillaris and RPE atrophy. Thesame areas also appeared dark on autofluorescence photo-graphs in the cases in which it was performed. Thesefeatures also resemble those sometimes seen in tilted disksyndrome,7 in which defective RPE and leakage pointsappear at the edge of the inferior staphyloma associatedwith tilted disks. Last, no evidence of vitreous traction,

TABLE. Characteristics of the 15 Eyes (10 Patients) that Exhibited a Dome-Shaped Macula within a Myopic PosteriorStaphyloma

Patient No. Age (yrs) Gender Eye

AxialLength(mm)

SphericalEquivalent

(D) VA (Snellen)

VisualImpairmentReported bythe Patient

Metamorphopsia,Central Scotoma,

or BothFoveal

Detachment

FocalLeakageon FA

1 50 Female Right 28.6 !12 20/20 None No Present NoLeft 29.2 !15 20/63 Yes No Present Yes

2 56 Female Right 25.2 !2 20/50 Yes No Present NoLeft 25.9 !6 20/200 Yes No Present Yes

3 41 Male Right 27.5 !5 20/40 Yes Yes Present Yes4 83 Female Right — !13 20/32 None No Absent No

Left — !12 20/63 Yes Yes Absent No5 50 Female Right 27.2 !9.5 20/25 None No Absent No

Left 28.8 !12.5 20/50 Yes Yes Present No6 59 Male Right — !9 20/80 Yes Yes Present Yes7 26 Female Right 24.4 !5 20/100 Yes Yes Present Yes

Left 24.4 !6.5 20/400 Yes Yes Absent No8 59 Female Right 29.9 !12 20/40 None No Absent No9 29 Female Right 25 !2.5 20/20 Yes Yes Present Yes

10 40 Female Right — !2 20/32 Yes Yes Present Yes

D " diopters; FA " fluorescein angiography; VA " visual acuity; yrs " years.

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which may have been responsible in part for the fovealdetachment, was found in any of the studied eyes on OCTexamination.

The evolution of the dome-shaped macula is not known,because the present study included only a few caseswithout a long follow-up. VA and macular profiles re-mained stable in the eyes of the eight patients followed upfor more than one year.

In this series, we reported cases of myopic eyes exhibit-ing a dome-shaped macula within a myopic posteriorstaphyloma. A dome-shaped macula is characterized by aninflexion of the concavity of the staphyloma in themacular area, which bulges into the ocular cavity. Thistype of staphyloma may be associated with macular serousretinal detachment and may cause visual impairment inmyopic patients.

THE AUTHORS INDICATE NO FINANCIAL SUPPORT OR FINANCIAL CONFLICT OF INTEREST. INVOLVED IN DESIGN ANDconduct of study (D.G., A.G.); collection and management of the data (D.G., A.E., A.L.-C., B.H., S.-Y.C., M.P., P.M.); analysis and interpretation ofthe data (D.G., A.G.); and preparation and review of the manuscript (D.G., A.G.). The Institutional Review Board (IRB) “Comité de protection despersonnes, Ile de France V” has declared that IRB approval was not required for this retrospective study, which was in conformity with all European andFrench law. Informed consent was obtained from the patients included in the study, who were aware that they were involved in a research study andagreed about the collect of data from their chart. This study adhered to the tenets of the Declaration of Helsinki.

REFERENCES

1. Takano M, Kishi S. Foveal retinoschisis and retinal detach-ment in severely myopic eyes with posterior staphyloma. Am JOphthalmol 1999;128:472–476.

2. Toranzo J, Cohen SY, Erginay A, Gaudric A. Peripapillaryintrachoroidal cavitation in myopia. Am J Ophthalmol 2005;140:731–732.

3. Sayanagi K, Ikuno Y, Gomi F, Tano Y. Retinal vascularmicrofolds in highly myopic eyes. Am J Ophthalmol 2005;139:658–663.

4. Coppe AM, Ripandelli G, Parisi V, Varano M, Stirpe M.Prevalence of asymptomatic macular holes in highly myopiceyes. Ophthalmology 2005;112:2103–2109.

5. Curtin BJ. The posterior staphyloma of pathologic myopia.Trans Am Ophthalmol Soc 1977;75:67–86.

6. Curtin BJ, Karlin DB. Axial length measurements and funduschanges of the myopic eye. Am J Ophthalmol 1971;1:42–53.

7. Cohen SY, Quentel G, Guiberteau B, Delahaye-Mazza C,Gaudric A. Macular serous retinal detachment caused bysubretinal leakage in tilted disc syndrome. Ophthalmology1998;105:1831–1834.

AMERICAN JOURNAL OF OPHTHALMOLOGY914 MAY 2008

Biosketch

David Gaucher obtained his MD degree at the Nancy Medical School (France) and graduated in 1999 with a Master ofBiology and Human Sciences. In 2003, he was approved by the French Board of Ophtalmology, and was awarded aone-year medical research fellowship on diabetic retinopathy in Paris. In 2004, he graduated with a Master ofNeurosciences and was held a fellowship in Prof Gaudric’s Department of Ophthalmology at the Lariboisiere Hospital inParis. Since November 2007, Dr Gaucher working as a vitreoretinal surgeon in Prof Speeg-Schatz’s Department ofOphthalmology at the Teaching Civilian Hospital in Strasbourg, France.

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