Orbital Dysplasia in Neurofibromatosis

Autor: Stephen H. Martin, Stephen A. Kieffer, Eugene F. Binet, Harold O. Peterson
Rok vydání: 1969
Předmět:
Zdroj: Radiology. 93:829-833
ISSN: 1527-1315
0033-8419
DOI: 10.1148/93.4.829
Popis: A dysplasia of the bony walls of the orbit is one of the congenital anomalies associated with generalized neurofibromatosis (1–6). Orbital dysplasia may be mistaken clinically and radiographically for neoplasm or chronic subdural hematoma within the middle cranial fossa unless its characteristic plain film and angiographic findings are appreciated. Plain Skull Film Findings The plain skull film findings of 7 patients with neurofibromatosis and orbital dysplasia, ranging in age from eighteen months to fifty-seven years, are summarized in Table I. All 7 showed obvious stigmata of neurofibromatosis, e.g., cafe au lait spots, subcutaneous tumors, or skeletal anomalies. The major presenting complaint in all cases was pulsating exophthalmos. Radiographs of the skull in all 7 patients demonstrated a dysplastic sphenoid bone complex. This consists of hypoplasia of the greater and lesser sphenoid wings, resulting in (a) elevation of the lesser sphenoid wing, (b) widening of the superior orbital fissure, and (c) la...
Databáze: OpenAIRE