Carpal Tunnel Syndrome Associated with Klippel-Trénaunay Syndrome: A Case Report

Background and Importance: 

Klippel-Trénaunay syndrome is a rare congenital condition that rarely affects peripheral nerves. Median nerve involvement at the carpal tunnel level has only been reported on four occasions of this syndrome in the medical literature.

A 61-year-old Caucasian female patient with Klippel-Trénaunay syndrome presented with a 10-month history of paraesthesia and numbness affecting the median nerve distribution area of her left hand. The clinical and neurophysiological examination confirmed a moderately severe carpal tunnel syndrome that required surgical decompression.

Pre-operative findings demonstrated diffuse vascular infiltration and engorgement of the median nerve. Flexor tendons had a normal appearance. The patient had a satisfactory post-operative period with full resolution of her symptoms 4 weeks after the procedure.Although peripheral nerves are rarely affected in patients with Klippel-Trénaunay syndrome, neurological symptoms could indicate nerve involvement and magnetic resonance imaging (MRI) examination should be considered to further assess the extension of the lesion.