[A case of Klippel-Trenaunay-Weber syndrome associated with intracranial multiple angiomas]

A case of Klippel-Trenaunay-Weber syndrome associated with multiple intracranial angiomas was reported. Intracranial angiomas with Klippel-Trenaunay-Weber syndrome is very rare. Only 4 cases are known to this day. A 24-year-old man fell at his workplace and suffered a head injury. He was admitted to our hospital. Neurological examination revealed no abnormality. However, physiological examination demonstrated hyperplasia and cutaneous hemangiomas of left lower leg, malformation of the left eyelid and clouding of the left cornea. CT showed left hemispheric atrophy and subdural effusion. MRI demonstrated multiple flow voids at the left cerebellar hemisphere and at pons. Cerebral angiogram demonstrated left cerebellar AVM and pontine angioma. Feeding arteries of the AVM were left superior cerebellar artery and left posterior inferior cerebellar artery. Those of the pontine angioma could not be identified. No spinal angiomas were revealed. While spinal angiomas associated with this syndrome are known, association of intracranial lesions are rare. Reported anomalies are angiomas, hemi-hypertrophy of skull and brain, carotid occlusion, megadolicoanomaly of the basilar artery, meningioma and glioma. Our case report with cerebral angiomas is the first one in Japan. We postulate that anomaly of our case originated at the Streeter's 2nd-3rd stage or that multiple anomalies occurred. We emphasize that intracranial, spinal or visceral angiomas, which can be fatal if it bleeds, should be routinely examined in Klippel-Trenaunay-Weber syndrome.