Severe enophthalmos following intracranial decompression in a von Recklinghausen patient

A patient with neurofibromatosis type I and absence of the sphenoid wing developed proptosis of the right eye secondary to expansion of the intracranial contents from an intracranial astrocytoma associated with cerebral edema and hydrocephalus. Marked enophthalmos developed following excision of the intracranial tumor and placement of a ventriculoperitoneal shunt. Absence of the sphenoid bone limited the orbit's ability to halt posterior refraction of the orbital contents when the intracranial volume decreased.