Dome-Shaped Macula in Eyes with Myopic Posterior
Staphyloma
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 myopic
eyes 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 in
our OCT database to find similar cases. Fifteen eyes of 10
patients had a dome-shaped macula. These patients all
had undergone fluorescein angiography (FA), indocya-
nine green angiography (ICGA), and B-scan ultrasonog-
raphy examinations.
● RESULTS: The mean refractive error of the affected
eyes 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, and
metamorphopsia was noted in eight eyes. Four eyes were
asymptomatic. 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 visible
on both B-scan ultrasonography and OCT: a character-
istic bulge of the macular retina, RPE, and choroid
within the concavity of the moderate posterior staphy-
loma was present in all eyes. In 10 eyes, OCT also
showed a shallow foveal detachment at the top of the
dome-shaped macula.
● CONCLUSIONS: A dome-shaped macula within a myo-
pic staphyloma is an unreported type of myopic posterior
staphyloma. The dome-shaped macula often is associated
with RPE atrophic changes and foveal retinal detach-
ment, which may explain the visual impairment in these
eyes. (Am J Ophthalmol 2008;145:909 –914. © 2008
by Elsevier Inc. All rights reserved.)
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, Service
d’Ophtalmologie and Centre d’Imagerie et de Laser, Paris, France
(S.-Y.C.).
Inquiries to Alain Gaudric, Service d’Ophtalmologie de l’Hôpital
Lariboisière, 2, rue Ambroise Paré, 75475 Paris cedex 10, France; e-mail:
alain.gaudric@lrb.aphp.fr
0002-9394/08/$34.00 © 2008 BY ELSEVIER INC. ALL
doi:10.1016/j.ajo.2008.01.012
D
URING THE LAST DECADE, OPTICAL COHERENCE
tomography (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 has
proved useful in detecting asymptomatic macular hole
better than biomicroscopy.4
We describe in this study an entity characterized by a
convex 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 a
dome-shaped macula.
METHODS
BECAUSE WE SAW SEVERAL MYOPIC PATIENTS REFERRED
for unexplained visual loss whose OCT examination
revealed the unusual feature of a dome-shaped macula
(Figures 1 to 3), we reviewed the images of the 140
myopic eyes from our OCT database in a search for
similar profiles. We found 15 eyes in 10 patients having
such a dome-shaped macula. A retrospective study of
these patients’ files revealed that they all had reported
loss 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 pressure
measurement, slit-lamp examination of the anterior
segment, and fundus biomicroscopy. All patients also
underwent 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 as
follows: cross-sectional images of the posterior pole were
recorded with mapping software and multiple line soft-
ware with 4-, 5-, 6-, or 8-mm long scans. Short scans
were used to obtain better contrast because they made it
easier to pass through mild media opacities and to
reduce signal disturbances. Incident light intensity,
polarization, and dioptric controls of the optic system
RIGHTS RESERVED. 909
FIGURE 1. Images showing a dome-shaped macula within a myopic posterior staphyloma in the moderately myopic right eye of a
41-year-old man with visual acuity (VA) of 20/40 and a refractive error of !5; this unusual macular feature can be combined with
a foveal retinal detachment. (Top Left) Fundus photograph showing pigmentary changes present in the fovea. Arrowheads delimit
the 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 pigment
epithelium (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 vascularization
and no sign of choroidal new vessels. (Middle right) Late-phase ICGA image showing well-demarcated foveal oval-shaped
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 showing the macular profile exhibiting an unusual convexity within the general
concavity of the staphyloma. 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 (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.

always were adjusted to obtain the finest possible im- RESULTS

ages. A 2-dimensional ultrasonic examination was per-
formed in 11 of the 15 eyes studied using a 20-MHz THE STUDY COHORT CONSISTED OF 15 EYES OF 10 PATIENTS
probe (Cinescan; Quantel-Medical SA, Clermont-Fer- (eight women and two men). The mean age of the patients was
rand, France). 51 years (range, 26 to 83 years). No history of systemic disease

910 AMERICAN JOURNAL OF OPHTHALMOLOGY MAY 2008

FIGURE 2. Images showing a dome-shaped macula within a myopic posterior staphyloma in the highly myopic left eye of a
50-year-old woman with VA of 20/50 and refractive error of !12.5; another example of a dome-shaped macula combined with a
foveal retinal detachment. (Top left) Color fundus photograph showing a mild staphyloma with some pigmentary disturbances in
the fovea. White arrow indicates the direction of the OCT scan below. (Top right) FA image showing hyperfluorescence in the fovea
resulting from a RPE defect, but no leakage point. (Middle left) Fundus autofluorescence image showing mild atrophy of the RPE
clearly 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 showing
the anterior bulge of the macula involving the retina, RPE, and inner choroid. A shallow retinal detachment is present at the top
of the macular bulge. (Bottom right) B-scan ultrasonography image showing the alteration of the macular curvature within the
posterior staphyloma.

was reported for any patient. The median refractive error of the
eyes studied was !8.25 diopters [D] (range, !2 to !15 D). The
characteristics 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 patients
reported visual loss in at least one eye. In all, visual loss was
present in 11 eyes, and metamorphopsia was present in
eight eyes. Four fellow eyes of the five bilateral cases of
dome-shaped macula were asymptomatic. The median VA
of the eyes studied was 20/50 (median logarithm of the
minimum angle of resolution [logMAR] VA, 0.4).
On fundus biomicroscopy, all eyes exhibited a type I or
II myopic staphyloma, according to the Curtin classifica-
tion, that is, tessellation and pallor extended over a
horizontal elliptical area nasal to the optic nerve, up to a
variable distance from the temporal side of the macula.
Five eyes also exhibited some degree of papillary dysver-
sion, but without an inferior staphyloma. Posterior vitreous
detachment (PVD) was present in four of the 15 eyes, in
which a Weiss ring was seen. No PVD was detected in
eight other eyes, and PVD was questionable in the remain-
ing three. In all 15 eyes, FA showed various degrees of
change 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 at
least once during the follow-up (Figure 1). However,
choroidal neovascularization was ruled out in all cases by
FA and ICGA and by OCT. Late-phase ICGA frames
showed hypofluorescence of the central macula corre-
sponding to the areas of RPE disturbance (Figures 1 and 2).
Optical coherence tomography examination showed an
unusual feature of the macular profile, especially clear on

VOL. 145, NO. 5 THE DOME-SHAPED MACULA 911

FIGURE 3. Images showing a dome-shaped macula within a myopic posterior staphyloma in the highly myopic right eye of a
83-year-old woman with VA of 20/32 and a refractive error of !13; this unusual macular feature is not always combined with a
foveal detachment. (Top left) Fundus photograph showing a mild staphyloma and discrete RPE changes in the fovea. White arrow
corresponds to the direction of the OCT scan. (Bottom left) Late-phase FA image showing mild hyperfluorescence without leakage
at the temporal edge of the fovea. (Top right) On a horizontal 9-mm OCT scan, the dome-shaped profile of the macula is
characteristic. 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 the
staphyloma.

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 slightly
detached in 10 of the 15 eyes studied (Figures 1 and 2).
One eye was examined with spectral-domain OCT (3D
OCT-1000; Topcon, Tokyo, Japan), which allowed 3-di-
mensional (3D) reconstruction of the elevated macula in
the staphyloma (Figure 3). No epiretinal membrane, par-
tially adherent posterior hyaloid, or vitreous traction on
the macula were detected in any of the 15 eyes studied.
B-scan ultrasonography examinations were performed
using a 20-MHz probe, which also confirmed the convex
change in the curvature of the posterior pole in the
macular area (Figures 1 and 2). There was no sign of tumor
or of an infiltration process in the choroidal layer. How-
ever, it was not possible to see whether this convex
curvature of the macular profile was the result of the
choroid, which seemed to be thickened in some eyes
(Figure 1), or to a change in the scleral wall shape or
thickness. The impression was that the eye wall kept its
concave shape. A shallow retinal detachment, sometimes
present 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 present
only 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 myopic
staphyloma. This feature may be responsible for visual loss.
This abnormal feature, which we called a dome-shaped
macula, may be responsible for visual loss. It is character-
ized by an inward bulge inside the chorioretinal posterior
concavity of the eye, in the macular area. This macular
bulge was hardly detectable on fundus biomicroscopy, and
OCT and ultrasonography greatly helped to diagnose it.
Of interest is the fact that we have never observed such
an anomaly either in nonmyopic eyes or in myopic eyes
without posterior staphyloma. Here, all cases indeed had
type I or II primary myopic staphylomas combined with the
dome-shaped macula, as evidenced by the tessellation and
pallor extended over a horizontal elliptical area involving
the optic disk and macula. The authors are unaware of any

912 AMERICAN JOURNAL OF OPHTHALMOLOGY MAY 2008

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

Visual
Axial Spherical Impairment Metamorphopsia, Focal
Length Equivalent Reported by Central Scotoma, Foveal Leakage
Patient No. Age (yrs) Gender Eye (mm) (D) VA (Snellen) the Patient or Both Detachment on FA

1 50 Female Right 28.6 !12 20/20 None No Present No

Left 29.2 !15 20/63 Yes No Present Yes
2 56 Female Right 25.2 !2 20/50 Yes No Present No
Left 25.9 !6 20/200 Yes No Present Yes
3 41 Male Right 27.5 !5 20/40 Yes Yes Present Yes
4 83 Female Right — !13 20/32 None No Absent No
Left — !12 20/63 Yes Yes Absent No
5 50 Female Right 27.2 !9.5 20/25 None No Absent No
Left 28.8 !12.5 20/50 Yes Yes Present No
6 59 Male Right — !9 20/80 Yes Yes Present Yes
7 26 Female Right 24.4 !5 20/100 Yes Yes Present Yes
Left 24.4 !6.5 20/400 Yes Yes Absent No
8 59 Female Right 29.9 !12 20/40 None No Absent No
9 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.

previous reports of such a myopic staphyloma, combining
type I or II primary staphyloma with a macular anterior
bulge. They could find no reference to such a feature,
which may correspond to another type of compound
staphyloma described by Curtin.5
Posterior staphyloma is associated more frequently with
a high level of myopia or elevated eye axial length.6 In five
of the 15 eyes studied, spherical equivalent refraction was
less than 6 D, and axial length was less than 26.5 mm in
five of the 11 eyes measured. Although posterior staphy-
lomas already have been reported in eyes with mild or
moderate myopia, one may wonder whether the low
spherical equivalent refractions and short axial lengths we
observed in these eyes are not the result of the unusual
form of the dome-shaped macula. For instance, in one of
our patients, the axial lengths measured, respectively, from
the cornea to the foveola and from the cornea to the optic
disk differed by more than 1 mm. It looked as if this
macular bulge enabled the eye to be less myopic than it
would have been because of the staphyloma.
Ultrasonography failed to show whether the sclera
exhibited a local convex inversion of its curvature at the
macular level. On the contrary, in some eyes, the shape of
the scleral staphyloma did not seem affected, and the
convexity of the macula seemed to be the result of the
choroid, which looked as if it was thickened in the macular
area in some eyes (Figure 1), although no choroidal
hyperperfusion was detected on ICGA.
The dome-shaped macula seems to be a new cause of
visual impairment in myopic eyes: in this series, all the
patients reported visual loss and metamorphopsia. How-
ever, in four patients who had bilateral lesions and who
reported visual loss in one eye, the fellow eye was
asymptomatic.
Atrophic changes in the RPE were present in all the
eyes studied, and foveal detachment was present in 10 of
them. These changes may explain the visual impairment
and metamorphopsia reported by the patients. On FA,
foveal detachment was associated with focal leakage in
seven of these 10 eyes and extended over the sloping areas
around the macula in two cases (Figure 1). Foveal detach-
ment resolved spontaneously in two cases, and in another
case, focal laser treatment was performed, but without
success. We postulated that in eyes with a dome-shaped
macula, foveal detachment was a complication of the
abnormal dome-shaped curvature of the macula. The
mechanism of such a complication is unknown. The
fluorescein dynamics of the leaking points may mimic
those of chronic idiopathic central serous chorioretinopa-
thy. Here, when present, they indeed appeared relatively
late in the sequence, were moderately active, and occurred
in an area of atrophic changes in the RPE. However, they
displayed the particular feature of being localized at the top
of the dome-shaped macula. This area was hypofluorescent
on the late frames of ICGA, indicating the presence of
some degree of choriocapillaris and RPE atrophy. The
same areas also appeared dark on autofluorescence photo-
graphs in the cases in which it was performed. These
features also resemble those sometimes seen in tilted disk
syndrome,7 in which defective RPE and leakage points
appear at the edge of the inferior staphyloma associated
with tilted disks. Last, no evidence of vitreous traction,

VOL. 145, NO. 5 THE DOME-SHAPED MACULA 913

which may have been responsible in part for the foveal
detachment, was found in any of the studied eyes on OCT
examination.
The evolution of the dome-shaped macula is not known,
because the present study included only a few cases
without a long follow-up. VA and macular profiles re-
mained stable in the eyes of the eight patients followed up
for more than one year.
In this series, we reported cases of myopic eyes exhibit-
ing a dome-shaped macula within a myopic posterior
staphyloma. A dome-shaped macula is characterized by an
inflexion of the concavity of the staphyloma in the
macular area, which bulges into the ocular cavity. This
type of staphyloma may be associated with macular serous
retinal detachment and may cause visual impairment in
myopic patients.

THE AUTHORS INDICATE NO FINANCIAL SUPPORT OR FINANCIAL CONFLICT OF INTEREST. INVOLVED IN DESIGN AND
conduct 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 of
the data (D.G., A.G.); and preparation and review of the manuscript (D.G., A.G.). The Institutional Review Board (IRB) “Comité de protection des
personnes, Ile de France V” has declared that IRB approval was not required for this retrospective study, which was in conformity with all European and
French law. Informed consent was obtained from the patients included in the study, who were aware that they were involved in a research study and
agreed about the collect of data from their chart. This study adhered to the tenets of the Declaration of Helsinki.

REFERENCES 4. Coppe AM, Ripandelli G, Parisi V, Varano M, Stirpe M.

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5. Curtin BJ. The posterior staphyloma of pathologic myopia.
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6. Curtin BJ, Karlin DB. Axial length measurements and fundus
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914 AMERICAN JOURNAL OF OPHTHALMOLOGY MAY 2008

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

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