Multifocal intradural spinal AVF and renal artery aneurysms in a case of Klippel Trenaunay Syndrome (KTS).

We report an interesting case of a young patient who had hypertrophy of right leg and nevoid geographic skin lesion on the dorsal aspect of the right foot and leg suggestive of Klippel Trenaunay syndrome (KTS) and who presented for spinal digital subtraction angiography (DSA) to investigate the cause of progressive weakness of bilateral lower limbs. DSA revealed spinal arteriovenous fistulae (AVFs) at 3 levels and bilateral renal artery aneurysms. Although multiple intradural spinal cord AVFs and renal artery aneurysms are considered a feature of KTS, their clear demonstration in a single case either alone or together is not available in literature to the best of this author's knowledge. Existence of multifocal intradural fistulae in KTS calls for a detailed and complete spinal DSA even after a fistula is detected.