Almeida R et al. Bilateral ring-shaped corneal opacity.

Bilateral ring-shaped corneal opacity:

case report and review of the literature
Raquel Almeida, MD1,2, Miguel Ruo, MD1,3, Ins Almeida, MD1,2, Filipa D. Corresponding author: Raquel Sofia Ribeiro Almeida, MD
Rodrigues, MD1,2, Cludia Costa-Ferreira, MD1,2, Joo Chibante-Pedro, MD1,2 Rua Dr. Cndido de Pinho, 4520-211 - Santa Maria da Feira, Portugal
Telephone: 00351918200664
Department of Ophthalmology, Centro Hospitalar de Entre o Douro e Email: raquelribeiroalmeida@gmail.com
Vouga, Santa Maria da Feira, Portugal
1
Ophthalmology Department of Centro Hospitalar de Entre o Douro e Vouga, Santa
Maria da Feira, Portugal
2
Ophthalmologist
3
Resident in Ophthalmology
Funding: None
Proprietary/financial interes: None Date of submission: 09/01/2016 Date of Approval: 05/02/2016

Abstract
Purpose: To describe a rare bilateral corneal ring-
shaped opacity, not categorized as corneal dystrophy
or degeneration occurring without any associated
ocular or systemic disease.
Methods: Case report and review of the literature.
Results: The authors describe the clinical history
and examination of an 80 years old man, with an A B
asymptomatic bilateral and symmetrical ring-shaped
corneal opacity in the deep stroma of the corneal
mid-periphery. The surrounding corneal stroma was
clear, and the tear film, epithelium and its basement
membrane, Descemets membrane and endothelium
were normal. This rare corneal anomaly was
discovered by chance and its etiology is unknown.
Conclusions: These rings may result from
deposits of unknown origin or possibly a rare corneal
dystrophy.
C
Keywords: Corneal ring opacity, Ascher ring,
peripheral ring opacity. Figure 1. Slit lamp photographs of the right (A) and left eye (B). Both corneas show
granular deposits with the configuration of a perfect ring that is sharply demarcated
Introduction from the clear surrounding cornea (C).

Stromal deposits are frequently found in corneal
dystrophies or degenerations, in many metabolic
disorders, in active infection or immune reaction and
after corneal surgery. Nevertheless, bilateral stromal
ring-shaped deposits in eyes without any history of
the previously mentioned causes are very rare. In the
literature, we have found a total of twelve similar cases
in size and appearance to the one we describe in this
paper, all without a known etiology (Table 1). Here
we present a case of a bilateral, mid peripheral ring
opacity, in a healthy patient, as a coincidental finding
during routine ocular examination.
Case presentation
80 years old male was sent to our department
given a progressive loss of visual acuity in his left eye.
He worked as a farmer, however denied any contact
with toxic products. Had a personal history of arterial
hypertension controlled with amlodipine (calcium
channel blocker) and was also medicated with triflusal
(platelet aggregation inhibitor). He denied ocular
disease or trauma, previous ocular, orbital or eyelid
surgery and, in particular, any type of
excimer lasers photoablation treatment.
Ophthalmological evaluation
revealed a best corrected visual acuity
(BCVA) of 8/10 in the right eye (OD)
and 3/10 in the left eye (OS). Slit lamp
examination showed a mild arcus
senilis and corticonuclear cataracts in
both eyes (OU). In the deep stroma
of the mid-peripheral cornea, he had
a ring-shaped grey-white corneal
opacity, bilateral and symmetrical,
with an average diameter of 7mm
horizontally and 6.3mm vertically.
No evidence of thinning, scarring
or vascularization. The surrounding
corneal stroma was clear and the tear
film, epithelium and its basement
membrane, Descemets membrane
and endothelium were normal (Figure
1). Corneal esthesiometry, fundoscopy,
gonioscopy and intraocular pressure were
normal. Pentacam (OCULUS) evaluation
showed a normal topographic map.
Scheimpflug analysis and optical coherence
tomography images (Cirrus HD-OCT)
confirmed the deep stromal location (Figure
2). Specular microscopy (Tomey EM-3000)
showed an endothelial cell count of 2168
cels/mm2 in OD and 2334 cels/mm2 in OS,
with typical hexagonal shape.
Systemic work-up revealed a normal
hematological, biochemical and lipid profile;
urinalysis and 24-hour urine sample showed
no abnormalities and kidney function was
normal; markers of autoimmune disorders
and serologic tests for syphilis, Lyme disease
and sarcoidosis (serum level of angiotensin
converting enzyme and serum lysozyme)
were negative. Following ophthalmological
and systemic work-up, patient underwent
phacoemulsification in both eyes. There has

V I S I O N P A N - A M E R I C A N T H E P A N - A M E R I C A N J O U R N A L O F O P H T H A L M O L O G Y
 Vis. Pan-Am. 2016; 15(2): 61-62.

melanin-like pigments in alkaptonuria, copper at the level

Year of Patient age at Uni- or of Descemets membrane in Wilsons disease and epithelial
Reported cases Sex
publication time of diagnosis bilateral crystals in Waldenstrms macroglobulinaemia, all of them very
Ascher1 1963 39 M Bilateral different from our patient.3,4,6
Corneal dystrophies have been historically defined as a group
Bron 8 1969
of inherited corneal diseases that are typically bilateral, symmetric
Case 1 59 M Bilateral and slowly progressive, without relationship to environmental or
Case 2 66 F Bilateral systemic factors.4,7 As knowledge has improved, exceptions to
each of these definitions have been noted and in our patient, the
Rieger9 1987 65 M Unilateral overall appearance of the ring opacity did not fit in with any of
Boop/Laqua10,11 1971/1991 the known corneal stromal dystrophies.
Case 1 80 F Bilateral Corneal degenerations are often a manifestation of aging,
inflammation or environmental insult and usually occur later in life
Case 2 65 M Bilateral than a dystrophy. Degenerations most often begin in the peripheral
Melles3 1998 25 M Bilateral cornea, although central vision may eventually be affected.
Khan JC12 2000 56 F Bilateral Inflammation is sometimes involved early in the degenerative
Rohrbach JM13 2001 63 M Bilateral process and may be accompanied by corneal vascularization.
Anterior embryotoxon, or arcus senilis, is the most common lipid
Caroline PJ14 2001 68 F Bilateral deposition in the cornea and is considered a normal change of
Silvestri A15 2003 82 F Bilateral aging unless it appears before the mid-30s, when it is suggestive
Nguyen DQ2 2007 80 M Bilateral of hyperlipoproteinemia. Other ring-shaped degenerations of the
Present study 2016 80 M Bilateral cornea with advancing age include mosaic crocodile shagreen and
Vogts white limbal girdle. In our patient, the corneal ring opacity
Table 1. Reported cases of mid peripheral corneal ring opacity
can be easily distinguished from the arcus senilis and other stromal
degenerations because of its location in the mid-peripheral cornea,
well demarcated from the limbus by a clear cornea.
In reference to the origin of this peripheral ring opacity of
the cornea, the bilateral symmetry of the rings and absence
A B of vessels suggest a dystrophic or degenerative basis for their
formation. The appearance of this type of rings in other studies,
particularly on confocal microscopy, suggested that they
represent anomalous exogenous or endogenous extracellular
microdeposits in the corneal stroma. The apparent stability,
discrete positioning in the midperipheral cornea and lack
of any associated infiltrative or inflammatory response were
suggestive of altered collagen fibril formation.2
C
To our knowledge, this asymptomatic, mid peripheral corneal
Figure 2. Scheimpflug images of right (A) and left (B) eye showing deep stromal opacification. Optical coherence ring opacity has only been observed in twelve adult healthy
tomography (C) shows a V-shaped opacification with the widest part close to Descemets membrane. The apex, patients. Mainly manifested bilaterally, as a sharply outlined,
anteriorly, is slightly displaced towards the center of the cornea. The central and peripheral cornea is clear.
grey-white opacity in the corneal stroma. This opacity probably
resulted from a deposition of an unknown material. Like others,
been no progression on the cornea over Corneal opacities induced by
we found that the ring did not show progression, which may
more than 12 months follow-up with topical or systemic drugs usually
suggest an exposition in a limited period of time.
the patient remaining asymptomatic involve both the central and peripheral
and with a BCVA of 10/10 OU. His only cornea and accumulation of a drug in a References
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