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Carpal Tunnel Syndrome Due to Fibrolipomatous Hamartoma of the Median Nerve in Klippel-Trénaunay SyndromeA Case Report
Mitsuhiro Matsubara, MD1; Hirotaka Tanikawa, MD1; Yuuji Mogami, MD1; Shunichi Shibata, MD1; Shigeharu Uchiyama, MD2; Hiroyuki Kato, MD2
1 Department of Orthopaedic Surgery, Azumi General Hospital, Ikeda 3207-1, Ikeda-Town, Kitaazumi-County, 399-8601, Nagano, Japan
2 Department of Orthopaedic Surgery, Shinshu University School of Medicine, Asahi 3-1-1, Matsumoto-City, 390-8621, Nagano, Japan. E-mail address for S. Uchiyama: sigeuti@shinshu-u.ac.jp
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Disclosure: The authors did not receive any outside funding or grants in support of their research for or preparation of this work. Neither they nor a member of their immediate families received payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, division, center, clinical practice, or other charitable or nonprofit organization with which the authors, or a member of their immediate families, are affiliated or associated.
Investigation performed at Azumi General Hospital, Nagano, Japan

The Journal of Bone and Joint Surgery, Inc.
J Bone Joint Surg Am, 2009 May 01;91(5):1223-1227. doi: 10.2106/JBJS.H.00813
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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.
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