Frosted Branch Angiitis

What is primary frosted branch angiitis?

Frosted Branch Angiitis (FBA) is a rare eye condition characterized by distinct and striking features in the retina and surrounding blood vessels. It is typically characterized by the following key characteristics:

Perivascular Sheathing: FBA causes florid, translucent perivascular sheathing, which means that the blood vessels in the retina, both arterioles (small arteries) and venules (small veins), become surrounded by a frosted or hazy appearance.
Variable Uveitis: Uveitis refers to inflammation of the uvea, which is the middle layer of the eye. FBA is often associated with variable uveitis, indicating that there is inflammation within the eye itself.

Retinal Edema: The condition can lead to retinal edema, which is the accumulation of fluid in the layers of the retina. This can further affect vision and contribute to visual loss.
Visual Loss and Recovery: Individuals with primary frosted branch angiitis may experience visual loss as a result of the condition. However, one notable feature is that, with appropriate treatment, there is often a good chance of recovery of vision.
Age Group: FBA typically affects children or young adults.

Rare Incidence: Primary FBA is exceedingly rare, with only a limited number of cases reported worldwide. As mentioned, your study represents the first report of a case from the UK.
Possible Infectious Cause: The exact cause of primary frosted branch angiitis is not well understood, but it is believed to be associated with an immune response triggered by various infectious agents. This suggests that it may be a response to multiple infective agents.
Secondary FBA: It’s important to note that FBA can also occur as a secondary condition in response to other causes of intraocular inflammation, particularly in cases of cytomegalovirus retinitis.

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Frosted Branch Angiitis: An Overview

Description: Frosted branch angiitis is a descriptive term used to characterize a specific type of retinal vasculitis.

Characteristics: It is characterized by a severe infiltration of the perivascular space with lymphoplasmacytic infiltrates.
Visual Appearance: This infiltration gives the appearance of frosted branches, resembling the branches of a tree.

pic credit : Eyewiki.aao

Associations with Frosted Branch Angiitis

Lymphoproliferative Diseases: Frosted branch angiitis can be associated with lymphoproliferative diseases such as lymphoma or leukemia.
- Accumulation of Malignant Cells: In cases associated with lymphoproliferative diseases, the condition is often due to the accumulation of malignant cells within the perivascular space.

Other Reported Associations: Frosted branch angiitis has also been reported in various other medical conditions, including:
- Systemic Lupus Erythematosus
- Crohn’s Disease
- Toxoplasmosis
- AIDS (Acquired Immunodeficiency Syndrome)

Classification of Patients with Frosted Branch Angiitis

Malignant Infiltration-Related Frosted Branch Angiitis
- Subgroup 1: Patients with Lymphoma or Leukemia
Infection-Related and Autoimmune Frosted Branch Angiitis
- Subgroup 2: Patients with Associated Viral Infections or Autoimmune Disease
  - Examples include:
    - Systemic Lupus Erythematosus
    - Crohn’s Disease
    - Toxoplasmic Retinochoroiditis
    - Human T Cell Lymphoma Virus Type 1 Infection
    - AIDS with Small Patches of Retinitis
    - HIV without CMV Retinitis
    - Herpes Simplex Virus Infection
    - Epstein-Barr Virus Infection
  - Frosted Branch Angiitis as a Clinical Sign, Possibly Due to Immune Complex Deposition (Secondary Frosted Branch Angiitis)
Acute Idiopathic Angiitis
- Subgroup 3: Otherwise Healthy Young Patients

This classification helps to categorize patients with Frosted Branch Angiitis based on the underlying causes or associations of the condition.

Frosted Branch Angiitis case report

Four case series and an extensive literature review of 236 cases were conducted by ncbi to clarify the characteristics of this rare condition.

An analysis of the reported cases revealed that a majority (54.6%) developed FBA in the presence of an underlying disease, with recurrence and complications requiring surgical intervention being rare. The frequency of bilateral occurrence (55.0%) and prevalence in female patients (45.0%) were noted to be lower than previously reported.

Frosted Branch Angiitis findings

The retinal findings include swelling of the retina and severe sheathing of the retinal venules, creating the appearance of frosted tree branches.
Additional findings include intraretinal hemorrhages, hard exudates, and serous exudative detachments of the macula and periphery.
Fluorescein angiography demonstrates leakage of dye from the vessels, but no evidence of decreased blood flow or occlusion.
Credit: Uveitis Atlas.
Photo credit: comprehensive_uveitis.