History

61 year old male presents for eye examination. He states that his vision has been hazy centrally in both eyes. He stated that this problem has being going on for some time and it happened gradually.

Medical History

Examination

• Best –corrected vision: OD: 20/50, OS: 20/60
• Pupils: Round and reactive with no APD
• Motility: Full OU
• Intraocular pressures: Normal OU
• Slit lamp examination: Significant for PCIOL, OU.
• Fundus Examination:
• There anomalous vessels at the optic nerve in both eyes. The macula is significant for few pinpoint red dots in the perifoveal area, OU. No exudates seen in both macula.

Ancillary Tests

Fluorescein Angiogram:

Figure 1A		Figure 1B
Figure 1C		Figure 1D

Figure 1 (A,B,C & D): Fluorescein angiogram of the right eye (early and late A&B) and the left eye (early and late C&D) show the presence of dilated telangiectatic capillaries with small microaneurysms. There is leakage in the late phases of the angiogram.

Differential Diagnosis

• Diabetic retinopathy
• Hypertensive retinopathy
• Old branch vein occlusion
• Radiation retinopathy
• Idiopathic juxtafoveal telangeiectasis

Diagnosis

Idiopathic juxtafoveal elangeiectasis:

• An idiopathic retinal vascular disorder that involves the capillary network in the perifoveal area. It is mainly characterized by dilated and tortuous capillaries and is clinically divided into 3 main groups.



• Symptoms: The main symptom of JFT is blurred central vision. The age of onset and laterality is dependent on the subtype. Patients with type 1 complain of blurred vision in one eye only while type 2 patients have bilateral complaints. Type 3 is a rare condition and usually presents with bilateral blurred vision and can be associated with central nervous system symptoms.



• Signs: Type 1 patients are typically males and have unilateral retinal telangiectasis in the parafoveal area in the form of a single or multiple small aneurysms. Macular edema is usually present and hard exudates could also be seen. Type 2 usually has bilateral blunting of the retina in the foveal area with crystals seen early or late in the disease. RPE changes may develop later in the disease process and can cause vision loss. Type 3 typically demonstrate progressive capillary nonperfusion along with few aneurysms in the center of the macula.



• Ancillary testing: Fluorescein angiogram is useful in making the diagnosis. It typically shows prompt filling of the dye by the telangiectasis vessels with late phase leakage of the dye. Type 3 patients also demonstrate enlargement and irregularity in the FAZ. CNV is likely to develop in this condition and could be detected by fluorescein angiogram.



• Treatment: Laser photocoagulation is the treatment of choice when leaky microaneurysms are found. Vision may still be limited in case of RPE changes were present. CNV associated with JFT may benefit from treatment with confluent laser. The role of PDT has not been identified. Intravitreal injection of steroid as a treatment option for the macular edema in this condition has been reported to produce good results. The role of this new modality is being evaluated in multicenter trial currently underway.