Abnormal development of the lesser wing of the sphenoid with microphthalmos and microcephaly

• Published in: Neuroradiology Vol. 43; no. 2; pp. 178 - 182
• Main Authors: JACQUEMIN, C, MULLANEY, P, BOSLEY, T. M
• Format: Journal Article
• Language: English
• Published: Berlin Springer 01-02-2001
• Subjects: Biological and medical sciences; Child; Diseases of the osteoarticular system; Humans; Male; Malformations and congenital and or hereditary diseases involving bones. Joint deformations; Medical sciences; Microcephaly - complications; Microcephaly - diagnostic imaging; Microphthalmos - complications; Microphthalmos - diagnostic imaging; Neurofibromatosis 1 - diagnosis; Sphenoid Bone - abnormalities; Tomography, X-Ray Computed; Skin disease; Wing; Diseases of the osteoarticular system; Microcephaly; Neurofibromatosis Recklinghausen; Benign neoplasm; Child; Human; Sphenoid; Nervous system diseases; Dysplasia; Phacomatosis; Radiodiagnosis; Exploration; Congenital disease; Cerebral disorder; Genetic disease; Case study; Eye disease; Malformation; Central nervous system disease; Skull disease; Medical imagery; Computerized axial tomography; Microphthalmia
• ISSN: 0028-3940; 1432-1920
• DOI: 10.1007/s002340000455

We report two patients with abnormal development of the lesser wing of the sphenoid bone, globe, optic nerve and cerebral hemisphere without stigmata of neurofibromatosis type 1. The lesser wing of the sphenoid bone was abnormally formed and was not ossified ipsilateral to the dysmorphic eye and underdeveloped cerebral hemisphere. Maldevelopment of the sphenoid wing may interfere with the normal closure of the optic vesicle and normal growth of encephalic structures, possibly by disturbing developmental tissue interactions. These patients may exhibit a type of restricted primary sphenoid dysplasia, while the sphenoid dysplasia of neurofibromatosis type 1 may be secondary to orbital or ocular neurofibromas and other factors associated with that disease.