Dana C Holl1, Jose A U Hardillo2, Ruben Dammers3, Marc P van der Schroeff2, Aad van der Lugt4. 1. Department of Neurosurgery, Erasmus Medical Center and Sophia Children's Hospital, Rotterdam, The Netherlands. Electronic address: d.holl@erasmusmc.nl. 2. Department of Otolaryngology-Head and Neck Surgery, Erasmus Medical Center and Sophia Children's Hospital, Rotterdam, The Netherlands. 3. Department of Pediatric Neurosurgery, Erasmus Medical Center and Sophia Children's Hospital, Rotterdam, The Netherlands. 4. Department of Radiology and Nuclear Medicine, Erasmus Medical Center, Erasmus Medical Center and Sophia Children's Hospital, Rotterdam, The Netherlands.
Abstract
BACKGROUND: Fibrous dysplasia (FD) is most often a slowly progressive benign disease in which the normal bone structure is replaced by fibrous and osteoid tissue. CASE DESCRIPTION: A 16-year-old adolescent, known with FD in the sphenoid bone, suffered an acute decreased visual acuity with papilledema on the left eye. The radiologic images were best compatible with cystic degeneration of the known FD with optic nerve compression in the optic canal. Decompression of the optic nerve was performed through an endoscopic exploration of the left sphenoid sinus. The visual acuity recovered completely. CONCLUSIONS: In FD with cystic changes, leading to acute signs of optic nerve compression, early aggressive surgical decompression is strongly recommended. Cystic degeneration of the FD, although rare, should be considered.
BACKGROUND:Fibrous dysplasia (FD) is most often a slowly progressive benign disease in which the normal bone structure is replaced by fibrous and osteoid tissue. CASE DESCRIPTION: A 16-year-old adolescent, known with FD in the sphenoid bone, suffered an acute decreased visual acuity with papilledema on the left eye. The radiologic images were best compatible with cystic degeneration of the known FD with optic nerve compression in the optic canal. Decompression of the optic nerve was performed through an endoscopic exploration of the left sphenoid sinus. The visual acuity recovered completely. CONCLUSIONS: In FD with cystic changes, leading to acute signs of optic nerve compression, early aggressive surgical decompression is strongly recommended. Cystic degeneration of the FD, although rare, should be considered.