Ear deformity due to neurofibromatosis type 1

被引:4
作者
Erguen, Selma Soenmez
Atilganoglu, Ulviye
Yasar, Husamettin
机构
[1] Vakif Gureba Hosp, Dept Plast & Reconstruct Surg, Istanbul, Turkey
[2] Vakif Gureba Hosp, Dept Dermatol, Istanbul, Turkey
[3] Vakif Gureba Hosp, Dept Ear Nose & Throat, Istanbul, Turkey
关键词
ear deformity; elephantiasis neurofibromatosa; neurofibromatosis; plexiform neurofibromas;
D O I
10.1007/s00266-007-0020-8
中图分类号
R61 [外科手术学];
学科分类号
摘要
Neurofibromatosis is an autosomal dominant neurogenetic disorder. It is associated with significant morbidity and results in substantial loss of function as well as significant cosmetic problems. Elephantiasis neurofibromatosa is the most impressive manifestation of the disease because of its size. In this setting, the lesion leads to massive overgrowth of both the skin and the associated soft tissue, causing disturbing deformities. The reported patient had huge plexiform neurofibromas involving both the external right ear and neck regions. Deformed external ear and neck regions were restored surgically with satisfactory cosmetic results.
引用
收藏
页码:403 / 405
页数:3
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