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Neovascular Membranes Associated With Idiopathic Juxtafoveolar Telangiectasis
Nicholas E. Engelbrecht, MD;
Thomas M. Aaberg, Jr, MD;
Jennie Sung, MD;
Mary Lou Lewis, MD
Arch Ophthalmol. 2002;120:320-324.
ABSTRACT
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Objective To report the visual outcome in patients with a neovascular membrane
(NVM) associated with idiopathic juxtafoveolar telangiectasis (IJFT).
Methods We performed a retrospective, noncomparative analysis of 26 eyes of
16 patients with an NVM associated with bilateral IJFT (Gass classification
group 2A). Eyes were divided into 2 groups: group WO (n = 11) included eyes
with IJFT without evidence of an NVM on initial examination; eyes in group
W (n = 15) had an NVM at the initial diagnosis of IJFT. In group WO, the initial
visual acuity and the time between the initial examination to the diagnosis
of an NVM were evaluated. Characteristic fundus findings, including the presence
or absence of a chorioretinal anastomosis, intraretinal pigmentary plaques,
and crystalline deposits, as well as the final visual acuity were reviewed
for both groups.
Results The initial visual acuity for eyes in group WO ranged from 20/20 to
20/70 (median, 20/30); in group W, from 20/20 to 4/200 (median, 20/70). The
average time from initial diagnosis of IJFT to the development of an NVM was
73 months (range, 5-142 months). In group WO, chorioretinal anastomosis and
concurrent perivascular retinal pigment epithelial hyperplasia were observed
before the development of an NVM. The final visual acuity for all eyes ranged
from 20/40 to 2/200 (median, 20/200). Eighty-one percent of eyes (21/26) had
a final visual acuity of 20/200 or worse.
Conclusions The stable final visual acuity in patients with an NVM associated with
IJFT is generally poor, with 80% of eyes in this series having a final visual
acuity of l20/200 or worse. In patients with IJFT, the presence of a chorioretinal
anastomosis and retinal pigment epithelial hyperplastic plaques always preceded
the development of an NVM.
INTRODUCTION
IDIOPATHIC juxtafoveolar telangiectasis (IJFT) is characterized by the
presence of an area of ectatic and incompetent retinal capillaries in the
foveolar region, in the absence of other known causes for retinal telangiectasis.
Idiopathic juxtafoveolar telangiectasis may be congenital or acquired, and
has been classified by Gass and Blodi1 into
3 groups. Under Gass's classification, group 2A describes patients with acquired,
typically bilateral areas of occult capillary telangiectasis who generally
seek care because of complaints of mild blurred vision in the fifth or sixth
decade of life.1 Vision loss generally occurs
slowly over many years, but may be rapid with the development of a neovascular
membrane (NVM).
The natural course of eyes with NVMs associated with IJFT is not well
known. Therefore, the potential benefits and indications for treatment of
the NVM are poorly defined. One previous report by Park et al2
found a mean final visual acuity of 20/70, in which they questioned the role
of laser photocoagulation of the fibrovascular tissues given the relatively
mild level of vision loss. The purpose of our study was to review the natural
course of NVMs in eyes with IJFT and the associated visual outcomes in these
patients. Serial fundus photographs were evaluated for characteristics that
may predict the development of an NVM. The potential role of treatment of
these lesions, including its indications, timing, and methods, is also discussed.
PATIENTS AND METHODS
We reviewed the charts of all patients with the diagnosis of IJFT with
an associated NVM who were seen at the Bascom Palmer Eye Institute, Miami,
Fla, between May 1, 1966, and January 31, 1996. The eyes included in this
series have been previously reported by Gass and Blodi1;
however, in that report, the outcomes were not evaluated. The patient medical
records, including the clinical chart, as well as serial fundus photographs
and fluorescein angiograms were reviewed. One patient was excluded because
the NVM was treated with focal laser photocoagulation. We reviewed 26 eyes
of 16 patients with an NVM in association with IJFT who received no treatment.
The eyes were divided into 2 groups on the basis of the presence or absence
of an NVM at the initial diagnosis of IJFT. Group WO included eyes that developed
an NVM after the initial diagnosis of IJFT; eyes in group W had an NVM at
initial examination.
The patients' age, sex, and medical history were reviewed for both groups.
The initial visual acuity and time between initial diagnosis of IJFT and the
development of an NVM were examined for the eyes in group WO. Serial fundus
photographs and fluorescein angiograms were reviewed for eyes in group W to
attempt to establish a pattern of progression leading to the development of
an NVM. The final stable visual acuity and length of follow-up were reviewed
for both groups.
RESULTS
Twenty-six eyes of 16 patients were diagnosed as having an NVM in association
with IJFT (Table 1). The age of
the patients in this series ranged from 33 to 81 years (mean age, 58 years).
Twelve patients (75%) were female, and the right and left eyes were affected
with equal frequency. Ten patients (62%) developed bilateral NVMs. The medical
history was significant for hypertension in 1 patient, diabetes mellitus in
7, thyroid disease in 1, chronic obstructive pulmonary disease in 1, and coronary
artery disease in 1. One additional patient had a history of a positive glucose
tolerance test but was not treated for diabetes mellitus. Of the 7 patients
with diabetes mellitus, none showed retinal changes consistent with diabetic
retinopathy; specifically, microaneurysms, blot hemorrhages, and neovascularization
of the disc or elsewhere were absent.
Group WO included 11 eyes that developed an NVM after the initial diagnosis
of IJFT. The initial visual acuity for eyes in group WO ranged from 20/20
to 20/70 (median, 20/30). The time between the initial diagnosis of IJFT and
the development of the NVM ranged from 5 to 142 months (mean, 73 months).
A review of serial fundus photographs for eyes in group WO showed the development
or presence of a chorioretinal anastomosis in all eyes before the development
of the NVM. Hyperpigmented plaques were also noted in all eyes before the
diagnosis of an NVM. Two eyes in group WO with stage 3 IJFT were treated with
focal laser photocoagulation for persistent macular edema associated with
the IJFT. Both eyes developed an NVM, one at 1 month and the other at 3 months
after the laser treatment.
Group W included 15 eyes with an NVM present at initial examination,
with a median visual acuity of 20/70 (range, 20/20 to 4/200). A chorioretinal
anastomosis was seen in all eyes in group W, and all but 1 eye showed hyperpigmented
plaques at the site of the NVM.
The NVMs found in these patients were most commonly located temporal
to the fovea in association with hyperplastic plaques and appeared to originate
from the retinal vasculature as opposed to the choroidal vasculature (Figure 1). Initially, the NVMs enlarged with
concurrent retinal edema and subretinal hemorrhage before becoming a cicatrix.
Once in the cicatricial stage, the membranes tended to contract.
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Idiopathic juxtafoveolar telangiectasis with associated neovascular
membrane (NVM). A, Color fundus photograph of the NVM. B through D, Corresponding
fluorescein angiograms showing progressive hyperfluorescence of the NVM. B,
Note the retinal vessel (arrow) filling before the appearance of the NVM.
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Groups WO and W each had a median final visual acuity of 20/200 (range,
20/200 to 2/200 and 20/40 to 5/200 in groups WO and W, respectively). The
stable final visual acuity for all eyes ranged from 20/40 to 2/200 (median,
20/200). There were no identifiable characteristics of the NVM that predicted
a better visual outcome. Twenty-one (81%) of the eyes in this series had a
final visual acuity of 20/200 or worse. The average length of follow-up was
107 months (range, 14-212 months).
COMMENT
Vision loss in patients with IJFT is generally mild and occurs over
many years. The development of an NVM may occur late in the disease process
and lead to more dramatic vision loss. The natural course of an NVM associated
with IJFT is not well understood, and therefore the utility of treatment is
not well defined.
Idiopathic juxtafoveolar telangiectasis involves the presence of ectatic
retinal capillaries in the absence of other known causes of retinal telangiectasis,
such as diabetic retinopathy, radiation retinopathy, carotid occlusive disease,
or following a branch retinal vein occlusion.3-4
In 1982, Gass and Oyakawa5 originally proposed
a classification system of idiopathic juxtafoveolar retinal telangiectasis.
This classification system was revised in 1992 by Gass and Blodi1
and includes 3 classification groups. Under the current classification, group
2A includes patients with bilateral IJFT. Group 2A represents the most common
type of IJFT, and patients are usually first examined in the fifth and sixth
decades of life because of the complaint of mild vision loss in one or both
eyes. Idiopathic juxtafoveolar telangiectasis is an acquired disease that
usually occurs bilaterally and has no sex predilection. The presence of exudation
from the telangiectatic vessels is also characteristically absent.1, 4
Gass and Blodi1 described 5 stages in
the progression of group 2A IJFT. In stage 1, there is evidence of juxtafoveolar
telangiectasis on fluorescein angiography, but with no abnormality seen on
clinical examination. Biomicroscopic evidence of the disease begins in stage
2 with graying of the parafoveolar retina; also, minimal telangiectatic changes
may be visible. Patients most commonly are first seen with stage 3 disease
and a complaint of mild vision loss that appears to be secondary to progressive
foveolar atrophy. Stage 3 is clinically characterized by the presence of right-angle–draining
retinal venules or chorioretinal anastomosis. Stage 4 is characterized by
the development of plaques of hyperplastic retinal pigment epithelium that
are commonly associated with the chorioretinal anastomosis. The final stage,
stage 5, involves the development of an NVM, which is believed to originally
develop from the retinal vasculature but may later also include connections
to the choroidal vasculature or choroidal neovascularization.1-2
Superficial retinal crystalline deposits of unknown origin have also been
described in eyes with IJFT and have been reported to occur in 40% to 45%
of eyes.1, 5-6 The
significance of these refractile deposits is unknown and they may be seen
in stages 2 through 5 in the progression of the disease.
In our series, all patients had the characteristic findings on initial
examination that were described by Gass and Blodi1
for patients with group 2A IJFT. All eyes included in our series also had
an NVM present (stage 5). Patients in group WO of this series did not have
an NVM present at the time the patient was initially diagnosed as having IJFT.
All eyes in group WO that had adequate serial fundus photographs available
showed the progression of disease as described by Gass and Blodi. The development
of a chorioretinal anastomosis in the area of the juxtafoveolar telangiectasis
was followed by the development of hyperpigmented retinal pigment epithelial
plaques before the presence of an associated NVM in all eyes in group WO.
Also, all eyes with an NVM at the initial diagnosis of IJFT (group W) had
a chorioretinal anastomosis with hyperplastic plaques present, except 1 eye
that did not show a hyperplastic plaque. No eyes in our series with stage
5 IJFT had crystalline deposits present, although 1 patient had crystalline
deposits present in the opposite eye without an NVM.
An association between systemic diseases, such as diabetes mellitus,
and IJFT has not been well established. The presence of parafoveal telangiectasis
and diabetic retinopathy in patients has been previously reported,7 but the eyes in this series had other manifestations
of nonproliferative diabetic retinopathy. Although 7 of the 16 patients in
this series had a diagnosis of diabetes mellitus and 1 additional patient
had a history of a positive glucose tolerance test, none of these patients
had any typical manifestations of diabetic retinopathy. Also, the development
of subretinal NVM is uncharacteristic of the manifestations of diabetic retinopathy.
Although familial cases of bilateral IJFT have been reported,8-9
none of the patients in our series were related.
Park et al2 reported the visual outcome
in a series of eyes with IJFT and associated fibrovascular tissue. They found
little change in the size of the fibrovascular tissue and an average stable
final visual acuity of approximately 20/70 for the 11 eyes in their series.
Because of the relatively mild vision loss associated with the presence of
neovascular tissue and the apparent stability of the membranes, they questioned
the utility of treatment in these eyes. In contrast to the previously reported
series, we found a median stable final visual acuity of eyes in this series
of 20/200, with 80% of eyes having a final visual acuity of 20/200 or worse.
Only 3 of 26 eyes maintained a visual acuity of 20/70 or better. Because 80%
of the eyes in our series had poor visual acuity with long-term follow-up,
we believe that treatment, if available, should be considered before significant
vision loss. In our series, there were no identifiable characteristics of
the NVM that predicted a better visual outcome.
Previous studies have reported the use of focal laser photocoagulation
to areas of macular edema associated with juxtafoveolar telangiectasis.1-2,5, 9-11
Park et al10 reported the results of 10 eyes
treated with grid laser photocoagulation for macular edema in bilateral juxtafoveolar
telangiectasis and showed that treatment did not improve or stabilize long-term
visual acuity. Gass and Blodi1 reported the
results of 8 eyes with stages 3 and 4 disease treated with laser photocoagulation;
they reported no improvement in vision in 4 eyes and worse vision in 4 eyes.
Laser treatment may be complicated by subretinal hemorrhage or the development
of an NVM.10-11 The development
of an NVM after laser treatment has been reported.2, 10
In our series, 2 eyes that were treated with laser for macular edema developed
an NVM, one at 1 month and the other at 3 months after treatment. Because
previous reports have shown no significant improvement in visual outcomes
with focal laser photocoagulation, and because there may be an increased risk
of the development of an NVM following treatment, the use of laser photocoagulation
for the treatment of macular edema associated with IJFT is not recommended.
The role of treatment of NVMs associated with IJFT is not well defined.
The NVMs in IJFT in our series initially grew with associated retinal edema
and subretinal hemorrhage before entering a cicatricial stage in which the
membrane contracted or remained relatively unchanged. Therefore, intervention
would appear to be potentially most beneficial in the early stages of neovascularization.
However, to our knowledge, there are no reports in the peer-reviewed literature
that discuss the role of focal laser photocoagulation for the treatment of
NVM associated with IJFT. Berger et al12 reported
the results of 2 eyes that were treated with surgical removal of a subfoveal
NVM. The visual outcome in both eyes was poor secondary to marked adherence
between the NVM and the sensory retina. Therefore, as they reported, the surgical
removal of these membranes may be contraindicated. Photodynamic therapy may
play a role in the treatment of subfoveal NVMs in IJFT. Recently, photodynamic
therapy has been reported to result in resolution of leakage from an NVM,
with no effect on the edema associated with the telangiectasis.13
The natural course of the visual acuity in eyes with NVMs associated
with IJFT has not been well defined. Therefore, the indications for treatment
of these membranes are also not well established. The use of laser photocoagulation
for macular edema associated with IJFT has not been shown to stabilize or
improve visual outcomes in these patients and may hasten the development of
NVMs. Therefore, this treatment is not recommended. The use of laser photocoagulation
or photodynamic therapy for the treatment of NVMs associated with IJFT may
improve the visual outcomes in these patients; however, there are insufficient
data at this time to support their use in these patients. Eighty-one percent
of the eyes in our series with an NVM associated with IJFT had a stable final
visual acuity of 20/200 or worse. Therefore, we believe that it is important
to investigate the potential role of treatment of these eyes to attempt to
prevent significant vision loss.
AUTHOR INFORMATION
Submitted for publication May 31, 2001; final revision received October
25, 2001; accepted November 16, 2001.
This study was supported in part by Core Grant P30 EY06360 from the
National Institutes of Health, Bethesda, Md, and by Research to PRevent Blindness
Inc, New York, NY. Dr Engelbrecht is supported by the Heed Ophthalmic Fellowship
Fund, Cleveland, Ohio.
This study was presented in part at the annual meeting of the Association
for Research in Vision and Ophthalmology, Ft Lauderdale, Fla, March 15, 2001.
Corresponding author: Thomas M. Aaberg, Jr, MD, Associated Retinal
Consultants, 1000 E Paris Rd, Suite 150, Grand Rapids, MI 49546 (e-mail: aaberg3{at}attbi.com).
From the Department of Ophthalmology, Emory University Eye Center,
Atlanta, Ga (Drs Engelbrecht and Aaberg); Associated Retinal Consultants,
Grand Rapids, Mich (Dr Aaberg); Moorfields Eye Hospital, London, England (Dr
Sung); and the Bascom Palmer Eye Institute, Miami, Fla (Dr Lewis).
REFERENCES
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1. Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis: update of classification
and follow-up study. Ophthalmology. 1993;100:1536-1546.
ISI
| PUBMED
2. Park D, Schatz H, McDonald HR, Johnson RN. Fibrovascular tissue in bilateral juxtafoveal telangiectasis. Arch Ophthalmol. 1996;114:1092-1096.
ABSTRACT
3. Gass JDM. Retinal capillary diseases. In: Stereoscopic Atlas of Macular Diseases.
4th ed. St Louis, Mo: CV Mosby Co; 1997:506-510.
4. Casswell AG, Chaine G, Rush P, Bird AC. Paramacular telangiectasis. Trans Ophthalmol Soc U K. 1986;105:683-692.
5. Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol. 1982;100:769-780.
ABSTRACT
6. Moisseiev J, Lewis H, Bartov E, Fine SL, Murphy RP. Superficial retinal refractile deposits in juxtafoveal telangiectasis. Am J Ophthalmol. 1990;109:604-605.
ISI
| PUBMED
7. Chew EY, Murphy RP, Newsome DA, Fine SL. Parafoveal telangiectasis and diabetic retinopathy. Arch Ophthalmol. 1986;104:71-75.
ABSTRACT
8. Oh KT, Park DW. Bilateral juxtafoveal telangiectasis in a family. Retina. 1999;19:246-247.
PUBMED
9. Hutton WL, Snyder WB, Fuller D, Vaiser A. Focal parafoveal retinal telangiectasis. Arch Ophthalmol. 1978;96:1362-1367.
ABSTRACT
10. Park DW, Schatz H, McDonald HR, Johnson RN. Grid laser photocoagulation for macular edema in bilateral juxtafoveal
telangiectasis. Ophthalmology. 1997;104:1838-1846.
ISI
| PUBMED
11. Friedman SM, Mames RN, Stewart MW. Subretinal hemorrhage after grid laser photocoagulation for idiopathic
juxtafoveolar retinal telangiectasis. Ophthalmic Surg. 1993;24:551-553.
PUBMED
12. Berger AS, McCuen II BW, Brown GC, Brownlow RL Jr. Surgical removal of subfoveal neovascularization in idiopathic juxtafoveolar
retinal telangiectasis. Retina. 1997;17:94-98.
ISI
| PUBMED
13. Potter MJ, Szabo SM, Chan EYY, Morris AHC. Photodynamic therapy for subretinal neovascular membranes in type 2A
idiopathic juxtafoveolar retinal telangiectasis [ARVO abstract]. Invest Ophthalmol Vis Sci. 2001;42:S438. Abstract 2363.
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