A NUMBER of bone diseases may affect the orbital bones decreasing the orbital volume and narrowing the optic canal, giving rise to unilateral exophthalmos and usually optic atrophy, simulating clinically an orbital tumour. Among such diseases are osteitis deformans (Paget, 1876); leontiasis ossea (Virchow, 1896); osteopetrosis (Albers-Schonberg, 1904), and the generalized and localized osteitis...

We illustrate a case of a giant primary intraosseous meningiomas (PIMs) with optic nerve compression treated by partial resection. A 48-year-old female presented with visual disturbances exophthalmus, diplopia and eye pain due to optic nerve compression. The patient had a past history of fibrous dysplasia treated surgically with partial resection by ENT in 2010. Histology confirmed the diagnosi...

Neurofibromatosis type 1 (NF-1) is a significant autosomal dominant disorder with wide spectrum of clinical findings. These signs (Café au lait spots, bone dysplasia, Lisch nodules) usually start to emerge after the first months life and most are benign in nature. On other hand, neoplasms (optic glioma, neurofibroma, malignant peripheral nerve sheath tumor, soft tissue sarcoma, leukemia, breast...

Fibrous dysplasia was first described by Lichtenstein in 1938 as a disorder characterized by progressive replacement of normal bone elements by fibrous tissue. It is a bone tumor that, although benign, has the potential to cause significant cosmetic and functional disturbance, particularly in the craniofacial skeleton. Its management poses significant challenges to the surgeon. Its compression ...

Neurofibromas are the hallmark of neurofibromatosis type 1 (NF1). Interestingly, generalised and localised interference or dysfunction of bone is also a key element of the NF1 phenotype. In the skull, NF1-associated orbital dysplasia often results in a severe disfigurement of affected individuals. However, the underlying pathology of orbital dysplasia is a complex phenomenon and up to now poorl...

OBJECTIVE Fibrous dysplasia (FD) of bone may occur solely as a skeletal condition or it may occur in association with extraskeletal manifestations, including growth hormone (GH) excess. Uncertainty exists as to the management of FD involving the optic nerves. In an effort to clarify management, the authors studied a large population of patients. METHODS One hundred four patients underwent an ...