Ear deformity due to neurofibromatosis type 1.

Aesthetic Plastic Surgery 31(4):403 (2007) PMID 17576502

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.