title: Reconstruction of skull base defects in sphenoid wing dysplasia associated with neurofibromatosis I with titanium mesh.
abstract: Sphenoid wing dysplasia occurs in 3-7% of patients with neurofibromatosis type 1 (NF1). The typical radiological features are partial or complete absence of the greater wing of the sphenoid. This condition is slowly progressive and may result in temporal lobe herniation into the orbital cavity, producing pulsating exophthalmos and gross facial deformity. Thus, reconstruction of the orbit is important for both cosmetic and functional reasons. Traditional surgical treatment of sphenoid dysplasia involves split bone grafting and repair of the anterior skull base defect. However, several reports have demonstrated complications of graft resorption and recurrence of proptosis and pulsating exopthalmos. In this case series, we present two patients suffering from pulsating exophthalmos due to sphenoid dysplasia. Radiological and MRI studies demonstrated orbital enlargement and complete absence of the greater wing of the sphenoid. Surgical management of these patients involved dural defect repair, and the use of titanium mesh in conjunction with bone graft to act as a barrier between the orbit and the middle cranial fossa. The mesh was fixed by fine screws. Proptosis improved markedly post-operatively and resolved within a few weeks. Ocular pulsation subsided and remained quiescent with at least 1-year follow-up.
authors.affiliation: Department of Neurosurgery, Cairo University, Egypt.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Lotfy
authors.forename: Risheng
authors.initials: R
authors.lastname: Xu
authors.forename: Matthew
authors.initials: M
authors.lastname: McGirt
authors.forename: Sameh
authors.initials: S
authors.lastname: Sakr
authors.forename: Basim
authors.initials: B
authors.lastname: Ayoub
authors.forename: Ali
authors.initials: A
authors.lastname: Bydon
mesh_terms: D001672:Biocompatible Materials; D001848:Bone Diseases, Developmental / Q000473:pathology / Q000601:surgery*; D001863:Bone Screws; D003399:Craniotomy; D005094:Exophthalmos / Q000209:etiology / Q000601:surgery; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000473:pathology; D019651:Plastic Surgery Procedures; D019291:Skull Base / Q000473:pathology / Q000601:surgery*; D013100:Sphenoid Bone / Q000473:pathology / Q000601:surgery*; D013526:Surgical Mesh; D014025:Titanium; D014057:Tomography, X-Ray Computed; D055815:Young Adult
chemical_list: D001672:Biocompatible Materials; D014025:Titanium