JBJS | Carpal Tunnel Syndrome Due to Fibrolipomatous Hamartoma of the Median Nerve in Klippel-Trénaunay Syndrome: A Case Report

The Journal of Bone & Joint Surgery, Volume 91, Issue 5

Case Reports | May 01, 2009

Carpal Tunnel Syndrome Due to Fibrolipomatous Hamartoma of the Median Nerve in Klippel-Trénaunay Syndrome: A 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

The Journal of Bone & Joint Surgery. 2009; 91:1223-1227 doi:10.2106/JBJS.H.00813

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Extract

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 reported^3-7. 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 nerve^3,8-10. 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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Topics

carpal tunnel syndrome ; hamartoma ; median nerve

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