Journal of Japanese Society of Reconstructive Microsurgery
Online ISSN : 2185-9949
Print ISSN : 0916-4936
ISSN-L : 2185-9949
Case Report
A Case of Maffucci Syndrome Reconstructed with Free Fibular Osteocutaneous Flap for Chondrosarcoma in the Upper Arm
Shinichi HIGUCHI, Kunihiro IKUTA, Kazunobu HASHIKAWA, Katsumi EBISAWA, Miki KAMBE, Yuzuru KAMEI
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2024 Volume 37 Issue 3 Pages 133-136

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Abstract

 A 33-year-old man with Maffucci syndrome developed chondrosarcoma in the left upper arm, which required reconstructive surgery. The left fibula, which had been shortened and underwent bone lengthening in childhood, was not suitable for use as an osteocutaneous flap. Instead, the right fibula, which was free of enchondromas, was considered suitable for use as the donor site for the free-flap reconstruction. One year and three months post-surgery, the transferred bone achieved fusion, and upper limb function was preserved. Maffucci syndrome is characterized by multiple enchondromas and venous malformations due to somatic mosaic mutations in IDH1 and IDH2. Approximately 30% of cases involve malignant transformation of enchondromas. The bone affected by enchondromas is structurally fragile and prone to fractures. In the present study, we used the right fibula, which did not develop enchondromas, as a free flap. The absence of genetic mutations in this bone allowed for normal bone fusion and retention of its strength.

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© 2024 Japanese Society of Reconstructive Microsurgery
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