Retinal astrocytic hamartomas (RAH)

Retinal astrocytic hamartomas (RAH)

👉Benign tumors of the RNFL

Occur in isolation (unilateral, unifocal) or in association with tuberous sclerosis complex (TSC) or neurofibromatosis (bilateral, multiple)

👉Three types have been described [Rowley SA, et al BJO 2001]:

Type 1 – semitransparent, flat, greyish lesions confined to the RNFL, no signs of calcification

Type 2 – multinodular calcified lesions with the classic mulberry-like appearance

Type 3 – combined lesions

👉OCT aids in diagnosis of RAH, picking up secondary changes such as SRF, ERM and in the follow up of these lesions. SD-OCT classification of RAH was proposed by Pichi F, et al Retina 2016 which aslo described relationship to systemic findings:

Type 1 –flat, gradual transition, tumor in NFL

Type 2 – elevated, sudden transition, tumor superficial to NFL, VR traction. Cutaneous fibrous plaques+

Type 3 – calcified, moth eaten appearance. SEGA+

Type 4 – dome shaped, optically empty. Pulmonary lymphangiomyomatosis+

Important to recognise RAH:

As these are benign and generally show minimal growth

Early diagnosis is imporatant for systemic evaluation and genetic counselling

We have published a large symptomatic RAH in a child with TSC –full text available at

Also published SD-OCT features of RAH in 2012 -

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