Article Text

Idiopathic parafoveal telangiectasis associated with a foveal pseudovitelliform lesion
  1. E Margalit1,
  2. R S Apte1,
  3. S R Sadda2
  1. 1Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD, USA
  2. 2Doheny Retina Institute, University of Southern California Keck School of Medicine, Los Angeles, CA, USA
  1. Correspondence to: Srinivas R Sadda, MD, The Doheny Retina Institute, University of Southern California Keck School of Medicine,1450 San Pablo Street, Suite 3610, Los Angeles, CA 90033, USA; ssadda{at}dei.hsc.usc.edu.edu

Statistics from Altmetric.com

Idiopathic parafoveal telangiectasis (IPT) and basal laminar drusen with pseudovitelliform lesions are both uncommon ocular findings.1,2 Gass classified parafoveal telangiectasis into several groups. Group 2 is characterised by bilateral acquired parafoveal telangiectasis that usually becomes clinically apparent later in life, in the fifth and sixth decade. The zones of telangiectasis tend to be symmetric and measure up to one disc diameter, with preferential involvement of the temporal parafoveal region. Typical findings are minimal retinal oedema, “right angle” veins, which drain the telangiectatic areas, underlying retinal pigment epithelial (RPE) alterations and superficial retinal refractile deposits. Gass has also described the development of a small, foveal yellow pseudovitelliform lesion in some patients, but no associated basal laminar drusen were noted.1 Abnormal blood glucose metabolism may play a part in the development of IPT.3

Basal laminar or cuticular drusen are focal thickenings of the basement membrane of the RPE. Although they are sometimes difficult to detect on clinical examination, these basal laminar drusen present a striking fluorescein angiographic picture of innumerable uniform, small, slightly raised, hyperfluorescent nodules.2 Patients with basal laminar drusen may develop a yellowish exudative macular detachment (“pseudovitelliform lesion”) that may resemble the foveal lesion characteristic of Best’s vitelliform dystrophy. In some cases, the yellow material may gravitate to the inferior part of the detachment, producing a “pseudohypopyon” appearance. Typically, the pseudovitelliform material shows progressive staining with fluorescein dye during the course of the angiogram.2

This case report describes the presence of these two relatively uncommon conditions in the same eye of one patient. To the best of our knowledge, there is no previously reported case of simultaneous presentation of basal laminar drusen with pseudovitelliform lesion and parafoveal telangiectasis in one eye.

Case report

A 48 year old white woman presented for refraction with no other complaints. Around the age of 10, she presented with ptosis and a dilated pupil on the right eye and was diagnosed with a third nerve palsy. A examination ultimately revealed a craniopharyngioma for which she underwent resection. She did not receive any postoperative radiation treatment. Since the surgery, she has continued to have persistent ocular motility problems in the right eye. She also felt that her vision has been poor, though stable, since the time of the surgery in the right eye. No other ocular findings were reported at that time.

After the surgery, she developed secondary panhypopituitarism for which she received hormone replacement therapy. A recent fasting blood sugar was normal, and diabetes was ruled out by her primary care physician. Her current medications include hormone replacement therapy (for the panhypopituitarism), consisting of prednisone 5 mg/day, levothyroxine sodium, desmopressin acetate, conjugated oestrogens, and medroxyprogesterone. Her family history was notable for cataract and diabetes mellitus. She has a 14 pack year smoking history, but quit 18 years ago.

On examination, best corrected visual acuity measured 20/40 in the right eye and 20/20 in the left eye. Visual fields were full to confrontation in both eyes. She had a non-reactive pupil on the right, but, by reverse testing, she did not have an afferent pupillary defect. She had marked limitation of movements of the right eye, particularly depression, elevation, and adduction. Abduction was also mildly reduced. Extraocular movements of the left eye were full. She had approximately 30 prism dioptres of exotropia of the right eye in primary position. Colour vision was 14 out of 14 in each eye, although somewhat slower on the right side.

External eye examination was within normal limits. Slit lamp examination disclosed trace nuclear opacities bilaterally. Contact lens biomicroscopy showed a cup:disc ratio of 0.3 in each eye. Both eyes showed evidence of numerous basal laminar hard drusen (best seen with indirect illumination), which formed typical clusters in numerous areas. There was no evidence of a pseudovitelliform lesion on the left. On the right side, there was a central yellowish lesion deep to the retina, which was consistent with a pseudovitelliform lesion. In addition, she appeared to have fine telangiectatic capillaries in the parafoveal retina most prominent temporally. The parafoveal retina was mildly thickened, and had a slight greyish sheen. Crystalline changes were also observed in the superficial retina (Fig 1).

Figure 1

(A) Fundus photograph of the right eye. The pseudovitelliform lesion is seen in the centre of the fovea (long arrow). Crystalline changes (short arrows) and temporal parafoveal telangiectasis (arrowhead) are also present. (B) Fundus photograph of the left eye. Multiple basal laminar drusen are seen. No pseudovitelliform lesion, telangiectatic vessels, or crystalline deposits can be observed.

A fluorescein angiogram was obtained transiting the right eye. The transit revealed telangiectasia of the parafoveal capillaries (most prominent temporally) with intraretinal leakage from these capillaries in later frames (Fig 2). The dye did not accumulate in cystoid spaces. There was no disc hyperfluorescence. Blocked fluorescence was observed under the fovea, corresponding to the pseudovitelliform lesion noted clinically. There was also punctate hyperfluorescence associated with the numerous basal laminar drusen present in both eyes (Figs 2A and 3). No evidence of telangiectasia or at least no evidence of leakage from telangiectatic capillaries was evident in the left eye (Fig 3).

Figure 2

(A) Early frame of a fluorescein angiogram. Subtle telangiectatic vessels are seen temporal to the foveal avascular zone in the right eye. Early fluorescence from multiple basal laminar drusen is observed deep to the retina. An area of blocked fluorescence is noted in the foveal centre corresponding to the pseudovitelliform lesion noted clinically. (B) Late frame of the same angiogram shows diffuse intraretinal leakage around the foveal avascular zone and staining of clusters of drusen.

Figure 3

Late frame of a fluorescein angiogram of the left eye shows punctate hyperfluorescence associated with numerous basal laminar drusen. There is no evidence of telangiectasia or retinal vascular leakage.

Comment

This 48 year old woman with evidence of basal laminar drusen in both eyes and a vitelliform lesion in the right eye also has parafoveal telangiectasis in the same right eye.

She does not have any history of radiation treatment and she does not have diabetes. In addition, the distribution of telangiectatic capillaries and retinal leakage does not conform to the distribution of a branch retinal vein occlusion. Thus, the most likely explanation for the central vascular changes in the right eye is idiopathic parafoveal telangiectasis.3 Most of the patients with acquired IPT (group 2A according to Gass classification) present with bilateral disease.1 However, some patients present with unilateral disease and some may present with asymmetric lesions and develop IPT in the other eye later in life.1,3 Our patient had typical findings including RPE changes, mild thickening of the retina, greyish sheen, characteristic crystalline deposits, and a typical fluorescein angiogram.3

Gass also noted that IPT could sometimes be associated with a yellow central spot.1 He described five patients in group 2A that had such a spot, 100–300 μm in diameter in the centre of the foveolar region.1 The pseudovitelliform lesion in our patient, however, did not resemble the lesions described by Gass. Gass identified a yellow precipitate within the retinal layers. Our patient’s lesion was deep to the retina and had the typical appearance of lesions associated with basal laminar drusen.2 In addition, our patient, unlike those described by Gass, had clusters of basal laminar drusen, seen both clinically and angiographically.

References

View Abstract

Footnotes

  • Financial interest: none.

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.