Staged Ear Transfer and Reconstruction in a Severe Case of Neurofibromatosis Type 1

Abstract

Summary: Cases of neurofibromatosis type 1 may present with varying degrees of craniofacial deformities. Involvement of the ear and surrounding soft tissues may include hypertrophy, distortion, and sagging. We present the case of a 40-year-old woman with a severe plexiform neurofibroma affecting her left face and ear. Ear position was extremely altered by the chronic disease process, with sagging almost to the level of the clavicle. The patient was subjected to a 3-stage surgical intervention aimed at transfer of the external ear to its native region and debulking of the involved facial soft tissue tumors. A large Z-plasty was performed to transpose a flap containing the external ear, with its underlying extremely stretched auditory canal, to a proper location. In the second stage, further tumor debulking was performed. In the final stage, the floppy ear was set back significantly by obliteration of the auriculocephalic sulcus and was suspended by suturing to the mastoid fascia. The patient showed a remarkable improvement. A significant part of her neurofibroma was debulked, and the ear was salvaged, reduced in size, and transferred to a near-normal location. This was quite a unique and challenging case in terms of extreme external ear ptosis and malposition; however, success was achieved through adherence to basic principles and techniques, staging, collaborative support, and full commitment.