제 1 형 다발성신경섬유종과 동반된 접형골 형성부전 1예

Sphenoid dysplasia is rare and has reported to occur in 13.5% of patients with neurofibromatosis type 1 (NF-1). It is characterized by a defect in all or part of the greater wing, an elevation of the lesser wing of sphenoid, the distortion of the sella and the enlargement of the ipsilateral orbit. A 33-year-old man was admitted to our hospital with right zygomatic, maxillar fracture after trauma to right periorbital area. While treating the patient, pulsatile exophthalmos was found, and through computer tomographic scan, the absense of the wing of the sphenoid bone was found, along with the enlargement of the orbital cavity. He had skin problems since he was very young and a long history of decreased visual acuity in his right eye, but went untreated for them. After treating for the right zygomatic, maxillar fracture, and further discussion with the neurosurgery, he underwent sphenoid wing reconstruction with a number of surgicels and gelfoams. Postoperatively, his pulsating exophthalmos was not seen. We report successful treatment of sphenoid dysplasia associated with neurofibromatosis type 1. (J Clinical Otolaryngol 2004;15:303-306)