RT Journal
A1 Matsubara, Mitsuhiro
A1 Tanikawa, Hirotaka
A1 Mogami, Yuuji
A1 Shibata, Shunichi
A1 Uchiyama, Shigeharu
A1 Kato, Hiroyuki
T1 Carpal Tunnel Syndrome Due to Fibrolipomatous Hamartoma of the Median Nerve in Klippel-Trénaunay SyndromeA Case Report
JF The Journal of Bone & Joint Surgery
JO The Journal of Bone & Joint Surgery
YR 2009
FD May 1
VO 91
IS 5
SP 1223
OP 1227
DO 10.2106/JBJS.H.00813
UL http://dx.doi.org/10.2106/JBJS.H.00813
AB 
Klippel-Trénaunay 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-Trénaunay syndrome, and only a few cases have been reported3-7. The proposed pathogenesis of carpal tunnel syndrome in a patient with Klippel-Trénaunay 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-Trénaunay 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.