Combined Hamartoma of the Retina and Retinal Pigment Epithelium imaged with Fluorescein, Indocyanine Green Angiography and Spectral Domain Optical Coherence Tomography (SD-OCT) in a Four Week Old Infant

Anand Vinekar
Kavitha Avadhani
Rachna Kumar
Padmamalini Mahendradas
Sivakumar Munusamy
Sribharghav Natesh

Narayana Nethralaya Postgraduate Institute of Ophthalmology, Bangalore, India

AIM: To report the youngest case of unilateral Combined Hamartoma of the Retina and Retinal Pigment Epithelium (CHR&RPE) imaged in a four week old premature infant using simultaneous fluorescein (FA) and indocyanine green angiography (ICGA) and spectral domain OCT.

CASE: A four week old premature baby was noted to have a solitary lesion along the inferior arcade during routine ROP screening. Multi-modality imaging was performed using the Spectralis (Hiedelberg, Germany) using a modified hand-held technique. The SD OCT horizontal section scan showed a hyper reflective lesion with homogenous reflectivity and no internal cystic alteration with intact deep retinal layers and a normal RPE layer. The macula showed cystoid changes resembling macular edema. FA revealed an early triangular hyperfluorescent lesion with intrinsic vascularity evident of an early lacy pattern with increasing hyperfluorescence in late phases. ICGA was essentially normal.

CONCLUSIONS: To the best of our knowledge, this is the earliest report of CHR&RPE demonstrating early, hitherto unknown characteristic features of this lesion before the development of the epiretinal membranes or RPE changes