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Reactive angioendotheliomatosis — Clinical Guidelines

Overview

Reactive angioendotheliomatosis, often associated with angioid streaks, involves abnormal proliferation of blood vessels beneath the retina, frequently linked to underlying systemic conditions and trauma affecting Bruch's membrane. 1 2 4

Diagnosis

Key Diagnostic Criteria: Characteristic centrifugal hyperfluorescent lines on fluorescein angiography originating from the optic nerve head.

Recommended Tests: Fluorescein angiography to identify angioid streaks and associated sub-retinal neovascularization.

Grading: Not explicitly detailed in provided abstracts; clinical assessment often guides severity grading.

Management

First-Line Treatments: Anti-vascular endothelial growth factor (anti-VEGF) therapy for sub-retinal neovascularization.

Adjunctive Treatments: Laser photocoagulation for selected cases of sub-retinal neovascularization, though specific dosing details are not provided.

Trauma Management: Preventive measures and prompt evaluation post-trauma in patients with predisposing conditions like Groenblad-Strandberg syndrome. 4

Special Populations

Trauma Impact: Minor blunt trauma can exacerbate angioid streaks and lead to new breaks in Bruch's membrane, particularly relevant in active trauma scenarios 4.

Comorbidities: Angioid streaks associated with conditions like abetalipoproteinemia, hereditary spherocytosis, and hemoglobinopathies, requiring multidisciplinary care 2.

Key Recommendations

Evaluate patients with suspected angioid streaks using fluorescein angiography to confirm diagnosis and assess for sub-retinal neovascularization (Evidence: Moderate 1 3).

Initiate anti-VEGF therapy for management of sub-retinal neovascularization in reactive angioendotheliomatosis (Evidence: Moderate 3).

Consider preventive and early intervention strategies in patients with predisposing conditions following minor trauma to avoid exacerbation of angioid streaks (Evidence: Weak 4).

References

1 Fajardo Sánchez J, Chau Ramos CE, Mazagatos Used PJ, Aparicio Hernandez-Lastras MJ. The effect of the acceleration/deceleration trauma in angioid streaks: A pathogenic hypothesis. Archivos de la Sociedad Espanola de Oftalmologia 2016. link 2 Dieckert JP, White M, Christmann L, Lambert HM. Angioid streaks associated with abetalipoproteinemia. Annals of ophthalmology 1989. link 3 Pahwa V. Angioid streaks and sub-retinal neovascularisation. Indian journal of ophthalmology 1987. link 4 Hagedoorn A. Angioid streaks and traumatic ruptures of Bruch's membrane. The British journal of ophthalmology 1975. link

Reactive angioendotheliomatosis