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

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 abnor mally formed and was not ossified ipsilateral to the dysmorphic eye and und erdeveloped cerebral hemisphere. Maldevelopment of the sphenoid wing may in terfere with the normal closure of the optic vesicle and normal growth of e ncephalic structures, possibly by disturbing developmental tissue interacti ons. These patients may exhibit a type of restricted primary sphenoid dyspl asia, while the sphenoid dysplasia of neurofibromatosis type 1 may be secon dary to orbital or ocular neurofibromas and other factors associated with t hat disease.