[Combined hamartoma of the retina and retinal pigment epithelium. CLinical Aspects and differential diagnosis to other melanocytic processes of the fundus].

BACKGROUND

The combined hamartoma of the retina and the retinal pigment epithelium is a rare but important differential diagnosis of the melanocytic lesions of the fundus.

PATIENTS AND METHODS

We present a patient with the typical clinical picture and characteristic fluorescein angiography findings. The case report is followed by the discussion of the differential diagnoses and their specific signs.

RESULTS

The combined hamartoma of the retina and the retinal pigment epithelium is a congenital malformation without growth potential, which may lead to a slow reduction of visual acuity due to secondary changes in the vitreoretinal interface followed by retinal traction. The hamartoma shows a typical clinical picture: unnoticed reduction of visual acuity, grayish tumor on the level of the retina with feathery margins, secondary epiretinal proliferation and traction of the neurosensory retina. The fluorescein angiography findings are pathognomonical for the hamartoma: early blockage of the choroidal hyperfluorescence due to the pigmentation of the tumor, a fine network of abnormal retinal capillaries with leakage, epiretinal traction, tortuositas of the larger retinal vessels without leakage.

CONCLUSION

The retinal and pigmentepithelial hamartoma can be diagnosed in most of the cases by mere visual inspection. The diagnosis can be confirmed by fluorescein angiography and always has to be included to evaluate the differential diagnoses of all pigmented fundus lesions.