Abstract

P 180

Combined hamartomas of the retina and retinal pigment epithelium

Sebastian Kupferschmid, Gerhand K. Lang, Gabriele E. Lang
Universitäts-Augenklinik Ulm

Objective
Combined hamartomas of the retina and retinal pigment epithelium (CHR) are rare benign tumors that may cause significant visual loss. The clinical appearance depends on the location of the tumor and its composition with varying amounts of retinal pigment epithelial, vascular, and glial tissue. Fluorescein angiography and optical coherence tomography (OCT) can be helpful to find the diagnosis.
Methods
This interventional case report describes three patients aged between 40 and 56 years with CHR. All patients were first diagnosed CHR. We characterize CHR lesions clinically, angiographically and with OCT and correlate this to previously published histological findings. We describe treatment of a secondary CNV with photodynamic therapy (PDT) with Verteporfin®.
Results
All three Patients presented clinically a unilateral, slightly elevated, pigmented lesion juxtapapillary.  Angiography demonstrates vascular tortuosity and teleangiectasis in the area of the pigmented tumor. One of the three patients had CHR associated with classical subfoveal choroidal neovascularisation (CNV). This patient was successfully treated with PDT. OCT presented an elevated lesion with high reflectivity of the inner retina. The innermost layer of the retina showed denticulate wrinkling. This correlates to histological findings with proliferation of glial tissue with focal areas of intense gliosis in the nerve fiber layer and wrinkling of the internal limiting membrane. Deeper retinal or choroidal layers showed hypo reflective shadowing of the underlying tissue. The amount of shadowing is dependent on the composition with varying amounts of retinal pigment epithelium, vascular and glial components of the CHR. The elevation of CHR in OCT scan was less than 1 millimeter. Ultrasonography presented a hyper reflective structure.
Conclusions
Clinical examination, angiography and OCT present typical characteristics that can be very helpful to discriminate between CHR and choroidal melanoma.  CHR lesions do not show characteristic sonographic patterns. CNV at the margin of the lesion is a complication in CHR that can be successfully treated with PDT.

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