Macrodystrophia lipomatosa involving multiple nerves

Journal of Orthopaedics and Traumatology

Seema Rohilla¹  Rambaksh Sharma¹  Dhara B. Dhaulakhandi¹  Nitin Jain¹ 

[1] Pt. B.D. Sharma University of Health Sciences$$

关键词: Proteus syndrome;   

DOI  :  10.1007/s10195-011-0159-6

学科分类：骨科学

来源: Springer

【 摘 要 】

Macrodystrophia lipomatosa (MDL), a rare congenital disorder, is considered by some to be a localized form of Proteus syndrome. The implication of the PTEN (phosphatase and tensin homolog deleted on chromosome 10) gene in both strengthens this belief. We present a case who had MDL in multiple nerve territories—all on the same side of the body—with hypertrophy of mainly fibroadipose tissue throughout their distribution, thus pointing to a form of localized hemihypertrophy; both hemihypertrophy and lipomatous tumors are components of Proteus syndrome.

【 授权许可】

Unknown