Congenital Unilateral Upper Eyelid Retraction

Authors: Gavin Roberts, MD, David K. Coats, MDSeries Editor: David K. Coats, MD


April 07, 2003


The list of problems that can produce upper eyelid retraction is extensive. In neonates, the condition is rare, and a relatively small differential diagnosis explains most cases. The most common etiologies include thyroid ophthalmopathy, orbital hemangiomas, fibrosis of the levator muscle, and aberrant regeneration of the third cranial nerve.[1] In this child, the slightly elevated thyroxine level was not considered sufficient to diagnose a thyroid abnormality. MRI of the orbits demonstrated soft tissue abnormalities in the area of the superior rectus and levator muscles (Figure 2). There was no radiographic evidence of an orbital mass, such as orbital hemangioma, and there was no clinical evidence of aberrant regeneration of the third cranial nerve or synkinetic abnormalities characteristic of the Marcus-Gunn jaw winking phenomenon.

Thyroid ophthalmopathy has been shown to be among the most common etiologies of eyelid retraction in adults and children.[2] It may be unilateral or bilateral and may occur in the setting of elevated thyroid function tests or euthyroidism. Neonatal hyperthyroidism may be congenital or acquired, with congenital cases often associated with placental transfer of maternal TSH from a mother with hyperthyroidism.[1,2,3] Imaging studies can be helpful in distinguishing thyroid eye disease, as the affected muscles in thyroid ophthalmopathy typically show diffuse enlargement of the rectus muscle(s) with sparing of the tendons.[4]

Other causes for congenital eyelid retraction include orbital hemangiomas, which may be accompanied by proptosis with crying or straining, and are evident on orbital imaging.[5] Levator/superior rectus muscle fibrosis can be associated with difficult delivery, presumably due to pressure on the orbit during delivery. Patients with congenital fibrosis of the levator/superior rectus muscle complex have localized findings on imaging studies, which may be confirmed at the time of surgical correction.[1] Eyelid retraction associated with synkinetic movements in cases of third cranial nerve palsy occurs upon attempted depression or adduction, and relaxes on abduction.[6] Such patients would be expected to have normal imaging studies. It is also important to note that in some cases of congenital eyelid retraction, no identifiable cause may be found.[7]


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