Neurofibromatosis PTOSIS- Complete Reconstruction
As per our literature search management of neurofibromatosis having ptosis with absence of wing of sphenoid has been described in some journals of neurosurgery and plastic surgery. Plexiform neurofibromas are infiltrative non-capsulated lesions of lid causing hypertrophy of the local tissues ultimately leading to mechanical ptosis. Complete excision of tumor is not possible because of its infiltrative nature and recurrence is a major issue in long term follow-up. Management of ptosis and mass excision in cases of neurofibromatosis is not a difficult procedure but it requires systematic approach. Careful and meticulous dissection is the key of surgery for functional and aesthetic benefit.
Summary of Content
Patient had normal vision in both eyes. On examination he had moderate ptosis prominent laterally. Lid was S shaped and showed a mass laterally. On examination of left eye (LE) his margin reflex distance (MRD) I was plus Two, MRD II was plus Five and margin limbal distance (MLD) was 8mm. His Palpebral aperture height was 7mm and LPS action was 11 mm (good). There was no lid lag, the bells phenomenon was normal and there was no jaw winking phenomena. He had a cafe au lait spot on his forearm and back along with lisch nodules on iris of LE. On supine position he had visible pulsation of the left orbit. A diagnosis of neurofibromatosis type I was made with absence of sphenoid wing.
Plan of surgery in this case was correction of ptosis with debulking of mass. For this lid incision was taken on skin side at expected lid crease but it was shifted slightly latterly because mass was present on lateral side. Skin and orbicularis oculi muscle was separated from mass which was infiltrated to the levator aponeurosis. Mass and extra levator was resected followed by advancement with fixation of levator to the tarsal plate. Upper lid blepheroplasty was done. Horizontal lid laxity was managed by pentagonal excision of lid followed by 3 layer repair. The final result was good. There was no exposure. There was mild lid lag in down gaze. Lid closure was good and the lid height was same in both lids.
[ Keyword ]
Neurofibromatosis, Ptosis, Lid reconstruction
[ Conflict of Interest ]