Orbital reconstruction for pulsatile exophthalmos secondary to sphenoid wing dysplasia.
Sphenoid wing dysplasia or absence of the greater sphenoid wing is a rare condition that is considered pathopneumonic for neurofibromatosis type 1 (NF1). It occurs in 4% to 11% of NF1 patients, and its precise cause is unclear. Some cases appear to be congenital, while others have demonstrated it to be a progressive degeneration of the orbital wall. In about half of cases, associated adjacent neurofibromas are described. Consistently, however, the clinical sequelae is herniation of the temporal lobe into the orbit, causing progressive proptosis and pulsatile exophthalmos. Reconstruction of the orbit has traditionally been with bone grafts, but due to problems with bone resorption and recurrence, titanium plates in conjunction with bone grafts have been reported. We present a case of a 6-year-old male patient who was first diagnosed with NF1 and associated absence of the greater sphenoid wing at the age of 2. Four years later, he was referred for reconstruction after the development of pulsatile exophthalmos. Surgical management included dissection of the dura of the temporal lobe off of the periorbita and skull base reconstruction with a combination of radial-shaped titanium mesh and split calvarial bone grafts. Postoperatively, there was near immediate resolution of the pulsatile exophthalmos, and follow-up at 1 year showed no recurrence.
From the Department of Plastic Surgery, University of Tennessee College of Medicine Chattanooga, Chattanooga, TN.
Elizabeth L
EL
Dale
Timothy A
TA
Strait
Larry A
LA
Sargent
D016025:Bone Transplantation; D002648:Child; D005094:Exophthalmos / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications; D009915:Orbit / Q000601:surgery*; D011673:Pulsatile Flow; D019651:Plastic Surgery Procedures / Q000379:methods*; D012886:Skull / Q000637:transplantation; D019291:Skull Base / Q000601:surgery; D013100:Sphenoid Bone / Q000002:abnormalities*; D013526:Surgical Mesh; D014057:Tomography, X-Ray Computed