Morning glory syndrome

Case contributed by Dr Nerses Nersesyan

Presentation

Admitted for the progressive loss of vision in the right eye.

Patient Data

Age: 16 years
Gender: Male
Photo

Fundus photos are illustrating normal left optic nerve (B) while right optic nerve is demonstrating Morning Glory anomaly (A). 

Of note are the more significant overall size, halo of pigmentary changes surrounding the nerve (yellow dashed circle), and the radial orientation of the blood vessels (black arrows) emanating from the anomalous optic nerve.

MRI

Axial fat-saturated T2WI of a patient with morning glory syndrome demonstrates a funnel-shaped morphologic pattern of the right optic disc with the elevation of the adjacent retinal surface.

T1 C+ sequence shows the presence of abnormal tissue associated with the right distal intraorbital segment of the ipsilateral optic nerve and discontinuity of the uveoscleral coat at the optic nerve insertion.

Coronal T2 FatSat and T1 C+ images show the persistence of craniopharyngeal canal and demonstrate hypoplasia of the contralateral (left) optic chiasm, which could result from retrograde axonal degeneration.

Annotated image

Annotated MRI

The red heart-shaped area demonstrates the presence of Persistent hypophyseal canal  in the coronal T2 FatSat image.

Red (pathologic) and yellow (normal) areas on both optic nerves in the axial T1 C+ image are reflecting the characteristic funnel-shaped morphologic pattern of the right optic disc.

MRI

Vascular reconstructions from MRA sequences demonstrate hypoplastic right ICA (yellow arrows), suggestive of Moyamoya disease. 

Case Discussion

Morning glory syndrome (MGS) is a congenital optic nerve anomaly termed by its resemblance of the malformed optic nerve to the morning glory flower. 

The funduscopic appearance includes three characteristic features:

  1. funnel-shaped excavation in the optic disc
  2. chorioretinal pigmentary annulus
  3. radial orientation of unusually straight and narrow retinal blood vessels

MGS is associated with other ocular anomalies and many intracranial abnormalities, such as skull base defects, vascular abnormalities, and cerebral malformations. Thus, the clinical diagnosis should prompt further imaging evaluation. 

On imaging studies there are four typical orbital findings 3:

  1. a funnel-shaped morphologic pattern of the optic disc associated with elevation of the adjacent retinal margins
  2. abnormal tissue associated with the distal intraorbital segment of the ipsilateral optic nerve
  3. effacement of the regional subarachnoid spaces on the basis of this soft tissue
  4. discontinuity of the normal uveoscleral coat at the optic nerve insertion

In our case, several of these anomalies were present. Persistent craniopharyngeal canal and hypoplasia of the left optic chiasm are noted. Additionally, hypoplastic right ICA artery can be observed, which should raise the awareness for the presence of Moyamoya disease (which is associated with MGS in up to 45% of patients). 

Moyamoya disease is an idiopathic, non-inflammatory, non-atherosclerotic progressive vascular-occlusive illness involving the arteries of the Circle of Willis. It should not be confused with Moyamoya syndrome, where another primary condition causes the arterial occlusion. The nature of the relationship between the MGS in the setting of a progressive disease such as Moyamoya disease is not yet clearly explained.

The case is submitted in collaboration with Dr Jose Luis Leon Guijarro and Dr Fernando Mas Estelles. 

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Case information

rID: 58571
Published: 23rd Feb 2018
Last edited: 16th Jul 2018
Inclusion in quiz mode: Included

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