Presentation
Hypocortisolism. History of "pituitary abnormality" in childhood.
Patient Data
A defect is present through the anteroinferior wall of the sphenoid bone, communicating directly with an enlarged pituitary fossa, through which soft tissue protrudes into the nasopharynx.
Through a defect anteroinferior wall of the sphenoid bone, communicating directly with the enlarged pituitary fossa, a meningoencephalocele protrudes which contains the floor of the third ventricle. This can be traced anteriorly from the depressed mammillary body.
Note the flow void seen extending into the meningoencephalocele.
Additional findings include:
Conclusion
The constelation of optic disc abnormality, ICA stenosis and basal encephalocele suggest the diagnosis of morning glory syndrome.
Case Discussion
Transsphenoidal encephaloceles are rare. They can be acquired (e.g. following trauma or secondary to transsphenoidal surgery) or congenital through a defect in the base of the skull (e.g. a large craniopharyngeal canal).
Such a mass can lend itself to medical misadventure. For example, it may be biopsied if believed to be a mucosal mass or entered inadvertently by nasogastric instrumentation. This patient had been previously intubated and the ETT could be seen to indent the meningoencephalocele (scan not shown).
In this instance, the presence of left-sided optic nerve head abnormality and internal carotid artery stenosis suggest the unifying diagnosis of morning glory syndrome. This is known to be associated with persistence of the craniopharyngeal canal and basal encephaloceles.
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