TY  - JOUR
T1  - Carpal Tunnel Syndrome Due to Fibrolipomatous Hamartoma of the Median Nerve in Klippel-Trénaunay SyndromeA Case Report
AU  - Matsubara, Mitsuhiro
AU  - Tanikawa, Hirotaka
AU  - Mogami, Yuuji
AU  - Shibata, Shunichi
AU  - Uchiyama, Shigeharu
AU  - Kato, Hiroyuki
Y1  - 2009/05/01
N1  - 10.2106/JBJS.H.00813
JO  - The Journal of Bone & Joint Surgery
SP  - 1223
EP  - 1227
VL  - 91
IS  - 5
N2  - 
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.

SN  - 0021-9355
M3  - doi: 10.2106/JBJS.H.00813
UR  - http://dx.doi.org/10.2106/JBJS.H.00813
ER  -