i-File: Idiopathic Juxtafoveal Retinal Telangiectasia

Dr. Bikramjit Pal
Published Online: April 12th, 2021 | Read Time: 5 minutes, 2 seconds


A 61-year-old presented with OU diminution of vision. He complains of distortion of letters which he has noticed in the last few months. He is a well-controlled diabetic for 13 years. There are no other systemic issues. Examination revealed a BCVA of OD 6/18(P) N8, OS 6/24 N8. The anterior segment is unremarkable except for pseudophakia OU (no e/o PCO). Fundus and FFA as shown

  1. What is the most probable diagnosis (give your differential)?
  2. Which other investigation will help in the diagnosis
  3. How will you treat the patient?


Idiopathic Juxtafoveal Retinal Telangiectasia(IJRT)



  1. Subretinal exudation without vascular abnormalities
  2. Subretinal exudation with vascular abnormalities
  3. Exudation with AV malformation

This classification is mostly for interest and not followed anymore

GASS and BLODI ( 1993) This classification was a modification of the original Gass and Oyakawa classification of 1982

1A) Congenital in nature, having a UNILATERAL presentation in MALES

1B) Similar to above having a better prognosis and limited retinal abnormality

2A) Occult Non-exudative IJRT: This is the commonly known parafoveal telangiectasia and will be dealt with here

2B) Juvenile Occult familial IJRT

3A) Occlusive IJRT

3B) Occlusive IJRT with CNS vasculopathy

YANUZZI (Currently followed)

Type 1 Macular Telangiectasia: This was the original Gass type 1A and 1 B combined together

Type 2 Macular Telangiectasia: This the Gass type 2A

This is again divided into:

  1. Non-proliferative: without e/o CNVM
  2. Proliferative: having e/o CNVM

The original 2B and 3 of the Gass classification were omitted in view of their rarity

Type 2 is further divided into 5 stages

Stage1: Difficult to appreciate but the temporal parafoveal area has a greyish appearance with loss of transparency

Stage 2: The transparency increases with the appearance of telangiectatic vessels

Stage 3: Presence of right-angled venules

Stage 4: Presence of RPE hyperplasia

Stage 5: the presence of CNVM

The presence of intraretinal crystals can occur in any stage.

Salient Points about Macular Telangiectasia Type 2

The functional parameter most important for assessing the condition is not Visual acuity, but microperimetry. The earliest sign is the occurrence of greyish discoloration of the temporal fovea

Why: This is thought to occur secondary to nutritional and ischemic damage to the middle layers.

  • The telangiectatic vessels are generally not associated with exudation until CNVM develops.
  • The RPE clumps occur along the right-angled venules and are often thought to increase the chances of CNVM

Presence of Crystals: why???

  • Formed by degenerating MULLER cells
  • Retinal capillary telangiectasia surrounded by lipofuschin cells
  • The presence of crystals does not correlate with the severity of the condition
  • Located mostly in the inner retinal layers


  1. The classical FFA pattern is thought to be dilated ectatic capillaries that leak dye in the parafoveal area
  2. In the early stages of the disease, there are no dilated capillaries, only minimal staining.
  3. There is characteristic oval-shaped hyperfluorescence.
  4. The leakage is not related to any cystic spaces


  1. Outer retinal atrophy with outer retinal thinning and loss of architecture
  2. The occurrence of hyporeflective spaces, they can occur in the outer or inner retinal layers
  3. Presence of hyperreflective foci
  4. Presence of other features s/o CNVM

Fundus autofluorescence: It’s a newer modality of investigation which is supposed to be the most sensitive modality to verify IJRT in the initial stages

Other imaging modalities are:

  1. Adaptive optics
  2. Confocal reflectance imaging
  3. Microperimetry

Clinical signs suggestive of development of CNVM

  1. Sudden onset loss of vision
  2. Presence of subretinal hemorrhage
  3. Presence of exudation
  4. Cystoid edema
  5. Retinochoroidal anastomosis

In this case, since there are no signs of CNVM only a close watch is needed.There is no role of the laser at this stage.If and when any signs of CNVM appear anti-VEGF can be used with or without PDT.

Dr. Bikramjit Pal
Pal's Retina Centre
Dr Bikramjit P Pal has done his post graduation DO from RIO Chennai and then did his DNB from Aravind Eye care Tirunelveli. With keen interest in retina , he pursued his retinal training from SN Chennai. Dr Pal was the first Indian ophthalmologist to be chosen by Eye cancer Foundation NY to undergo Oncology fellowship under their fellowship program. He completed the same from Helsinki and then set up the oncology unit in SN Kolkata branch. Dr Pal has started his own exclusive Retina clinic in Ranchi and aims to make the same the best in the region
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