PMC4323507_01
Skin reformatted three-dimensional CT scan of the patient showing exophthalmos and dystopia in the right eye and increased distance between the eyeballs due to dystopia.
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F1
Our case was a 15-year-old female patient, who had progressive pulsatile exophthalmus and dystopia in the right eye, beginning at early age (Fig. 1).
Bone-reformatted three-dimensional CT scan of the patient showing enlargement of the right orbital cavity and large bone defect in the superior orbital fissure secondary to hypoplasia. The bone defect allows the petrous part of the temporal bone to be viewed in the posterior plain (arrows). The normal superior orbital fissure in the left orbita is shown with arrows.
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F2
On the three-dimensional (3D) image of CT, a large hypoplasic bone defect of 20.6x30.7 mm (35.8 cm2) was observed in the superior orbital fissure of the greater wing of the right sphenoid bone (Fig. 2).
T2-weighted coronal MRI showing the inferio-lateral displacement of the right bulbus oculi. The right bulbus oculi is markedly larger than the left bulbus oculi.
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F3
On MRI, the anterior-posterior diameter of the right bulbus oculi, which measured 27.9 mm, was relatively larger than the anterior-posterior diameter of the left bulbus oculi, which measured 22 mm; the distance between the two medial canthi, which measured 33.1 mm, was increased, and the right bulbus oculi was seen to have been displaced to the inferior by 5.9 mm (Fig. 3).
A : The T1-weighted sagittal MRI without contrast demonstrating a large bone defect and cerebral tissue within the defect protruding into the orbita. B : The T2-weighted sagittal MRI demonstrating no apparent dysplasia in cerebral tissue, but an increase in the levels of peripheral cerebrospinal fluid and marked exophthalmos and displacement toward the anterior of the right bulbus oculi.
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F4
The T1- and T2-weighted images obtained on MRI showed the left temporal lobe, with the subarachnoid membrane extending into the orbita through the defect (Fig. 4).