Delayed presentation of morning glory disc anomaly and transsphenoidal encephalocele: A management dilemma

ABSTRACT

Background Morning glory disc anomaly (MGDA) is a rare developmental abnormality of the optic disc that is associated with many other neurological and vascular conditions. Most cases are diagnosed in childhood.

Case Report We report a 57-year-old woman who presented to the ophthalmology department for assessment of long-standing poor vision in the left eye and exotropia. Examination showed a left MGDA and bitemporal hemianopsia. These findings prompted magnetic resonance imaging, revealing a transsphenoidal basal meningoencephalocele with herniation of the optic chiasm and inferior hypothalamus into the dural sac. Due to the eloquence of the neurovascular structures it contained, a decision was made not to reduce the meningoencephalocele. Instead, a ventriculoperitoneal shunt was placed. The patient’s ophthalmologic examination remained stable over the following year.

Conclusion While rare, MGDA can be first diagnosed in late adulthood and a thorough evaluation should be completed to assess for midline cranial defects, vascular abnormalities, and other associated abnormalities. Patients presenting late in life with basal encephalocele, herniation of the optic chiasm, and bitemporal hemianopsia present a management dilemma. In this case, a ventriculoperitoneal shunt was placed with the intention of lowering intracranial pressure to prevent further herniation and reduce the risk of cerebrospinal fluid leak.

KEYWORDS:

• Morning glory disc anomaly
• basal encephalocele
• transsphenoidal encephalocele
• optic neuropathy
• skull base
• bitemporal hemianopsia
• ventriculoperitoneal shunt