We assume autosomal dominant heredity with varying expression for this "pterygoid corneal dystrophy". It does not seem to differ histologically from normal pterygia, but recurrences appear to be more frequent and more aggressive.
If swelling of the lid is found orbito-palpebral emphysema should be thought of to initiate appropriate therapy to avoid possible severe complications, for example, occlusion of the retinal central artery.
Even children with mild ptosis, especially in combination with motility disorders, require a complete ophthalmological and orthoptic examination to avoid amblyopia also of the fellow eye.
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