Carpal tunnel syndrome due to fibrolipomatous hamartoma of the median nerve in Klippel-Trénaunay syndrome. A case report.

Klippel-Trenaunay syndrome is a rare congenital disorder that is characterized by the triad of cutaneous capillary malformation, varicose veins, and hypertrophy of bone and soft tissue1. It is associated with numerous digital anomalies, including macrodactyly, which is observed in 24% of patients2. Carpal tunnel syndrome is rarely associated with Klippel-Trenaunay syndrome, and only a few cases have been reported3-7. The proposed pathogenesis of carpal tunnel syndrome in a patient with Klippel-Trenaunay syndrome is either lymphatic obstruction or abnormal lymphatic proliferation, which causes an increase in pressure inside the carpal tunnel, or cavernous hemangioma surrounding the median nerve. We report a rare case of carpal tunnel syndrome due to fibrolipomatous hamartoma of the median nerve in a patient with Klippel-Trenaunay syndrome. Fibrolipomatous hamartoma is characterized by the expansion of the epineurium by fibrofatty tissue that surrounds and separates the nerve fascicles8; it usually involves the median nerve3,8-10. The pathophysiology of the structures inside the carpal tunnel was investigated in our patient, and attempts were made to compare the findings in the present case with those of idiopathic carpal tunnel syndrome. The patient was informed that data concerning the case would be submitted for publication, and he consented. A forty-six-year-old man with bilateral macrodactyly presented with pain and numbness in the thumb and the index and long fingers of the right (dominant) hand. He had been diagnosed with Klippel-Trenaunay syndrome at birth. The family history was unremarkable for congenital abnormalities. At twelve years of age, the patient began to experience numbness in the thumb and the index and long fingers of the right hand. At fifteen years of age, he noticed a soft mass in the right palm, although the symptoms remained unchanged. At the age …