Childhood fibrous dysplasia presenting as blindness: a skull base approach for resection and immediate reconstruction.
Fibrous dysplasia is an abnormal fibroosseous process of bone of unknown cause. The incidence of skull involvement varies, painless enlargement being the most common presenting symptom. Change in vision is a rare but recognized finding. We report a 3-year-old boy with extreme fibrous dysplasia involving the skull base, who presented with blindness. He underwent exposure osteotomies of the frontal bones and orbits to provide access for skull base tumor removal. The orbital roofs were reconstructed with microplate-fixed cranial grafts. One and one half years after tumor excision followed by immediate reconstruction, the boy retains facial symmetry, and his ocular function has not deteriorated.
Department of Surgery, Hospital for Sick Children, Toronto, Canada.
J C
JC
Posnick
M D
MD
Wells
J M
JM
Drake
J R
JR
Buncic
D
D
Armstrong
D001766:Blindness / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D016025:Bone Transplantation; D002675:Child, Preschool; D005357:Fibrous Dysplasia of Bone / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D008297:Male; D009896:Optic Atrophy / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D009916:Orbital Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D010027:Osteotomy; D011183:Postoperative Complications / Q000000981:diagnostic imaging; D013100:Sphenoid Bone / Q000000981:diagnostic imaging / Q000601:surgery; D014057:Tomography, X-Ray Computed