Retinal varicosity in Klippel Trenaunay syndrome

In 1869, Trelat and Monod recognized the association between limb hypertrophy and vascular dilation. However, in 1900, Klippel and Trenaunay described the syndrome of mesodermal phakomatosis characterized by cutaneous hemangiomata, varicose veins, and hypertrophy of soft tissue and bone of the affected limb. This same syndrome was also described in 1907 by Parkes Weber. Subsequently, various ophthalmological manifestations of this rare syndrome have been reported in a sporadic fashion. At this time, the authors report a case illustrating an unusual example of retinal varicosity in the ipsilateral macular area that has not, to our knowledge, been previously recorded.