Encephalofacial angiomatosis (Sturge-Weber syndrome): report of three cases.

The purpose is to show and discuss the findings in three cases of Sturge-Weber syndrome. One adult and two children were examined in the last three years. The girl was scanned after diode laser treatment for her right eye glaucoma. Ultrasonographic examinations were performed with 20- and 25-MHz probes (nominal frequencies) for posterior pole and 35-MHz probe (closed system, HiScan Optikon 2000, Rome, Italy) for anterior segment. All cases were characterized by choroid thickening due to capillary hemangioma. The thickening was not regular and the use of 25-MHz probe proved superior in visualizing the two layers (choroid vs. sclera), showing a striking reflectivity difference between them. All cases also showed enlarged vortex veins and some anomalous vessels adjacent to the external surface of the sclera, i.e. posterior episcleral dilated vessels. In one case only, very anomalous tortuous vessels were found in the orbit. Disk cupping and/ or pseudo cupping was always present in all cases; in the oldest patient only, who had a thinner choroid, it was possible to display a concave bottom of the cup instead of the straight, small, highly reflective segment representing the cribrosa. Serous retinal and choroid detachments were present as temporary complications after glaucoma treatment. In conclusion, choroid, facial and encephalon involvement in Sturge-Weber syndrome is widely described in ocular oncology books, whereas by far less attention is paid to orbital involvement. Anterior dilated episcleral vessels are visible and till now the posterior vessel involvement has only been demonstrated in some pathology specimens.