1. health and fitness
2. disease

keratoconjunctivitis should be suspected in patients who have ocular
surface symptoms and report a metallic taste in their mouth and who
handle zoanthids, as occurred in case
1. After excluding the infectious
causes, toxic keratoconjunctivitis
might be treated with aggressive use
of topical corticosteroids as well as
topical cyclosporine and lubricants.
Majid Moshirfar, MD
Yousuf M. Khalifa, MD
Ladan Espandar, MD
Mark D. Mifflin, MD
Author Affiliations: Moran Eye Center, University of Utah, Salt Lake
City.
Correspondence: Dr Moshirfar,
Moran Eye Center, University of
Utah, S65 Mario Capecchi Dr, Salt
Lake City, UT 84132 (Majid
[email protected]).
Financial Disclosure: None reported.
Funding/Support: This study was
supported by a grant from Research
to Prevent Blindness, Inc, to the Department of Ophthalmology and Visual Sciences, University of Utah.
1. Wiles JS, Vick JA, Christensen MK. Toxicological evaluation of palytoxin in several animal
species. Toxicon. 1974;12(4):427-433.
2. Nordt SP, Wu J, Zahller S, Clark RF, Cantrell FL.
Palytoxin poisoning after dermal contact with zoanthid coral [published online ahead of print June
20, 2009]. J Emerg Med. doi:10.1016/j.emermed
.2009.05.004.
3. Hoffmann K, Hermanns-Clausen M, Buhl C, et al.
A case of palytoxin poisoning due to contact with
zoanthid corals through a skin injury. Toxicon.
2008;51(8):1535-1537.
4. Keamy J, Umlas J, Lee Y. Red coral keratitis.
Cornea. 2000;19(6):859-860.
5. SteelDHW.‘Deadman’sfinger’keratoconjunctivitis.
Br J Ophthalmol. 1993;77(1):63.
6. Reef Central Web site. http://www.reefcentral
.com/forums/showthread.php?s=&threadid
=1083843. Accessed April 19, 2010.
7. Wet Web Media.com Web site. FAQs about zoanthid reproduction/propagation. http://www
.wetwebmedia.com/zoanthidreprfaqs.htm. Accessed April 19, 2010.
8. Vale-Gonzalez C, Pazos MJ, Alfonso A, Vieytes MR,
Botana LM. Study of the neuronal effects of
ouabain and palytoxin and their binding to
Na,K-ATPases using an optical biosensor [published online ahead of print May 10, 2007].
Toxicon. 2007;50(4):541-552.
Familial Congenital
Grouped Albinotic Retinal
Pigment Epithelial Spots
Congenital grouped albinotic retinal pigment epithelial spots
(CGARPES), or polar bear tracks, is
A
B
C
D
Figure 1. Ocular fundus and fluorescein angiogram (FA) of case 1. A, The right eye had multiple yellow
and white deep retinal lesions with sharp edges of various sizes and configurations in each of the
4 quadrants from the posterior pole to the periphery. B, The left eye had similar fundus findings. The
smaller lesions are more homogeneous (black arrows) than the larger flecks (white arrows show the
difference between the center and the edge of a large fleck). C and D show the window defects in the
arterial phase of the FA.
a rare anomaly characterized by multiple grouped, white, variably sized,
albinotic spots. They generally involve the peripheral retina, similar
to that of bear track grouped pigmentation.1 Usually the macula is
spared, and the spots may occur in
one or both eyes. The lesions seem
to be stable, and visual acuity, visual fields, color examination, dark
adaptation, and electrophysiologic
findings are normal. There is only
1 report of a decrease in visual acuity in this entity.2(pp614-615) Fluorescein angiography revealed a variable pattern related to the choroidal
fluorescence seen through these lesions that was considered sporadic
and rare in frequency. In this article, we describe 4 cases that occurred in a Brazilian family of Italian descent. This is the first reported
instance of familial CGARPES.
Report of Cases. Case 1. A 19year-old woman was referred for
evaluation after her general ophthalmologist noted areas of retinal abnormality on ophthalmoscopy after a routine refraction. She had
occasional headaches but no specific ocular symptoms, and she was
in excellent general health. Visual
(REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 10), OCT 2010
1362
acuity was 20/20 in both eyes with
−0.50 cylinder correction in the left
eye. Slitlamp examination of the anterior segments and vitreous cavity
was unremarkable. Intraocular pressures were normal. Funduscopy of
both eyes revealed multiple white
and sometimes yellow flecks of variable size and configuration affecting all parts of the retina (periphery, equator, posterior pole, and
fovea) (Figure 1). The size of the
flecks was highly variable, from the
diameter of one vessel to 4 times
the diameter of the optic nerve but
the flecks had a consistent color pattern: in the smaller lesions, the color
was more homogeneous, and in the
larger lesions, it was concentrated at
the edge of the flecks (Figure 1).
Fluorescein angiography showed a
normal choroidal and retinal vascular perfusion. The arterial phase revealed a transmitted hyperfluorescent lesion (a window defect of the
retinal pigment epithelium) that corresponded to the lesions seen during the ophthalmic examination. No
intraretinal fluid was seen during
the late phase of the angiogram
(Figure 1).
Color vision tests with the Farnsworth dichotomous (D-15) test,
WWW.ARCHOPHTHALMOL.COM
©2010 American Medical Association. All rights reserved.
Downloaded From: https://archopht.jamanetwork.com/ on 10/28/2014
A
Figure 2. The pedigree of the family. Affected
individuals are identified by solid circles (women),
deceased individuals by a slash (/). The mother
and 3 daughters were affected. We were unable to
examine the patient’s maternal grandmother and
uncle, who are indicated by a star.
electrooculogram, electroretinogram, and visual field were normal.
Follow-up 1 and 5 years later
showed the same ocular fundus findings without progression of the
flecks, and the headaches had disappeared. There was no history of
consanguineous marriage. When
other members of the family were
examined, this unusual fundus was
observed in her 2 sisters and her
mother (Figure 2).
Case 2. A 39-year-old woman,
the mother of the patient described
in case 1, described no ocular or systemic symptoms. Her visual acuity
was 20/20 in both eyes, with normal visual fields and no clinical night
blindness. Results of anterior biomicroscopy examination, color testing (Farnsworth dichotomous
D-15), electroretinogram, and electrooculogram were normal. Color
fundus photographs showed deep lesions of the fundus, similar to those
seen in case 1. The same color pattern was seen: more homogenous
color in the smaller lesions, and color
concentrated at the edges of the
larger lesions (Figure 3). The ocular coherence tomographic findings of a small fleck showed no defect in the inner or outer retina.
Case 3. This 16-year-old girl is the
sister of the patient described in case
1. Her corrected visual acuities were
20/20 in both eyes. Her visual fields
were normal, and she had no night
blindness. Retinal flecks were present in the posterior pole and all fundus quadrants up to the periphery.
However, the lesions were smaller
than those seen in either case 1 or
case 2 (Figure 4).
Case 4. A 12-year-old girl, the
youngest sister of cases 1 and 3, was
C
B
D
Figure 3. The mother of case 1. A, Right ocular fundus photograph shows deep retinal flecks in the
periphery. The arrows indicate the different coloration between the center and the edge of the large
lesion; black line, the site of optical coherence tomographic scan. B, Note the deeper penetration of the
optical coherence tomographic scan in the center of the lesion (arrows). C, Retinal flecks spread in the
nasal quadrant of the left eye; the black line indicates the site of optical coherence tomography. D, Optical
coherence tomography could not differentiate the small flecks from the rest of the retina.
also healthy, with 20/20 visual acuity and normal visual field without
clinical night blindness. Both eyes
were affected with fleck lesions,
comparable in shape and distribution with the flecks seen in the eyes
of her 2 sisters and mother. As in her
16-year-old sister (case 3), the fleck
lesions were smaller but were distributed from the macula up to the
periphery in all quadrants.
Comment. The retinal condition
in the patients described in our article is compatible and consistent
with a diagnosis of congenital
grouped retinal pigment epithelial
spots. The 4 cases were characterized by bilateral, multiple, deeply
situated, white-yellow fleck lesions
with a panretinal distribution (fovea, posterior pole, peripapillary,
equator, and periphery). However,
the lesions in original reports of
CGARPES tended to be white, spare
the macula, and uniocular.1,2 On the
other hand, our and more recent articles3,4 found those spots to be bilateral, affecting the macula and present with some yellow lesions,
particularly in the posterior pole
area. We hypothesize that yellow
macular lesions could be explained
by the increased amount of xanthophyll in these areas compared
with the periphery, where the spots
are white. Like in all reports of
CGARPES, the lesions vary in size
(REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 10), OCT 2010
1363
and configuration; however, we believe there is a color pattern: larger
lesions tend to have heterogeneous
coloration between the edge and the
central part; smaller lesions tend to
be more homogeneous.
According to Gass,2 the spots may
represent focal thickening of the retinal pigment epithelium that is filled
with a white material that may be diffusely distributed or more concentrated in the periphery of the lesion. Histologic findings have not
been reported so far to clarify the
true composition of this material.
Fluorescein angiography showed
hyperfluorescent spots with the initial phase of the angiogram (window defect) correlating exactly with
the lesions observed in the ocular
fundus. This finding is well documented in previous articles.1,2,4 The
differential diagnosis of CGAREPS
includes fundus flavimaculatus, fundus albipunctatus, and familial drusen. Gass2 recognized that CGAREPS
is identical to the entity reported by
Kandori and colleagues, 5 sometimes referred to as the flecked retina
of Kandori.
In contrast to previous studies that
found this entity to be sporadic, we
found an obvious familial component in our cases. The hereditary pattern in the presently described family is most likely an autosomal
dominant form of the disease since
only the mother and 3 daughters pre-
WWW.ARCHOPHTHALMOL.COM
©2010 American Medical Association. All rights reserved.
Downloaded From: https://archopht.jamanetwork.com/ on 10/28/2014
A
following ARN has never been
described.
This article describes 2 cases of
presumed reactive immune choroiditis following contralateral ARN.
B
C
D
Figure 4. The sister of case 1. A, In the right eye, note the smaller retinal flecks in the posterior pole. B,
Lesions in the posterior pole of the left eye are shown. C, Lesions are present up to the superior quadrant
periphery of the right eye. D, The superior temporal portion of the left eye contained the retinal flecks.
sent with the unusual fundus. However, because only 2 generations were
analyzed, the pattern is also consistent with autosomal recessive, Xlinked dominant, digenic, or mitochondrial inheritance. Because this is
the first report of CGARPES in more
than a single family member, we believe the appropriate name for this
unique presentation is familial congenital grouped albinotic retinal pigment epithelial spots.
Luı´s Augusto Arana, MD
Mario Sato, MD
Jayme Arana, MD
Submitted for Publication: January 11, 2010; final revision received April 25, 2010; accepted April
30, 2010.
Author Affiliations: Doheny Eye Institute, Doheny Retina Institute, Los
Angeles, California (Dr L. A. Arana);
Department of Ophthalmology, Federal University of Parana, Curitiba,
Brazil (Drs Sato and J. Arana).
Correspondence: Dr L. A. Arana,
Doheny Eye Institute, 1450 San
Pablo St, Ste 100, Los Angeles, CA
90033 (luisarana79@hotmail
.com).
Financial Disclosure: None reported.
1. Gass JDM. Focal congenital anomalies of the retinal pigment epithelium. Eye (Lond). 1989;3
(pt 1):1-18.
2. Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment. 3rd ed. St Louis,
MO: CV Mosby & Co; 1987.
3. Fuhrmann C, Bopp S, Laqua H. Congenital
grouped albinotic spots: a rare anomaly of the
retinal pigment epithelium. Ger J Ophthalmol.
1992;1(2):103-104.
4. Offret H, Offret M, Offret O, Labetoulle M.
Congenital grouped albinotic spots: a case report [in French]. J Fr Ophtalmol. 2007;30(9):
e26.
5. Kandori F, Tamai A, Kurimoto S, Fukunaga K.
Fleck retina. Am J Ophthalmol. 1972;73(5):673685.
Immune Choroiditis
Following Contralateral
Acute Retinal Necrosis
Acute retinal necrosis (ARN) syndrome is characterized by acute
panuveitis and retinal arteritis that
progresses to a diffuse necrotizing
retinitis with late-onset rhegmatogenous retinal detachment.
The contralateral eye is involved in 10% of patients despite systemic antiviral treatment. 1 It is
thought to occur because of retrograde axonal transport between
the suprachiasmatic nucleus of
the hypothalamus and the contralateral retina in an animal model.2
Bilateral ARN is characterized by
bilateral foci of retinal necrosis
associated with arteritis and panuveitis. However, a noninfective
choroiditis in the contralateral eye
(REPRINTED) ARCH OPHTHALMOL / VOL 128 (NO. 10), OCT 2010
1364
Report of Cases. Case 1. A 54-yearold white woman was referred for
treatment with a diagnosis of presumed ARN in her right eye. There
was no other relevant medical history. Her best-corrected visual acuity (BCVA) was 20/200 OD, 20/20
OS. On examination, the right eye
had panuveitis with retinal necrosis and associated arteritis. There was
also a relative afferent pupillary defect in the right eye. The left eye was
unaffected. Vitreous biopsy confirmed herpes simplex virus–
associated ARN by polymerase chain
reaction. She began taking 10 mg/kg
of intravenous acyclovir 3 times per
day for 7 days followed by 500 mg
of oral valacyclovir 3 times per day
for 3 months. Four months later, the
right eye became phthisical owing to
persistent inflammation despite topical corticosteroids. Ten months later,
the right eye was no longer inflamed but she had increasingly
blurred vision and mild discomfort
in her left eye. Her BCVA was 20/40
OS. Anterior segment examination
revealed a white eye with mild inflammation and a cataract. There was
minimal vitritis, and fundoscopy revealed disc swelling with multiple
deep pale lesions throughout the
fundus (Figure 1A). Fluorescein
angiography showed early hyperfluorescensce of the choroidal lesions with late staining accompanied by perivascular and optic disc
leakage of dye (Figure 1B). A vitreous biopsy was performed and was
negative for herpes simplex virus,
Varicella zoster virus, cytomegalovirus, and Epstein-Barr virus. The
sensitivity for detection of virus by
polymerase chain reaction in ARN
at our institution was 87.5%.3 With
suspicion of atypical presentation of
bilateral ARN, she started taking 500
mg of oral valacyclovir 3 times per
day and 40 mg of prednisolone once
per day. Her BCVA improved to
20/20 OS. During the next 12
months, her visual acuity diminished to 20/40, partly owing to a
cataract that was removed with a
perioperative intravitreal triamcino-
WWW.ARCHOPHTHALMOL.COM
©2010 American Medical Association. All rights reserved.
Downloaded From: https://archopht.jamanetwork.com/ on 10/28/2014