Facial plexiform neurofibromatosis: A surgical challenge

Abstract

Plexiform neurofibromatosis is a relatively common but potentially devastating manifestation of neurofibromatosis type 1 (NF1). It produces very hideous deformity if the face is involved. Surgical management remains the mainstay of therapy, but in the head and neck region it is limited by the infiltrating nature of these tumors, inherent operative morbidity and high rate of regrowth. We present two cases of facial neurofibromatosis managed in our hospital. The first patient presented with overhanging mass of skin folds on the right side of her face, completely obliterating her right eye. The other patient was a young male having a huge, unsightly swelling over the right cheek, resulting in pulled down right eyelids and right pinna. Physical examination revealed the presence of café au lait macules, freckling in the axillary region and multiple neurofibromas over the trunk. Reconstructive surgical procedure in the form of subtotal excision of tumor mass followed by re draping of the facial skin was performed in both cases. There was evidence of regrowth of the tumor on review after 6 months.

Keywords

• Facial plexiform neurofibromatosis
• regrowth
• subtotal excision