Journal of Neuro-Ophthalmology, December 2011, Volume 31, Issue 4

Periodic Unilateral Eyelid Retraction in a Pediatric Patient

A healthy 11-year-old girl presented with right upper eyelid retraction since birth. An evaluation including thyroid function studies and neuroimaging was negative, and the patient was scheduled for a right levator recession to address the eyelid malposition. Intraoperatively, after the induction of inhalational general anesthesia, the patient displayed cyclic right upper eyelid retraction. Occurring in intervals of exactly 48 seconds, these cycles involved a rapid elevation of the right eyelid from a position of half-closure to a retracted position just above the superior limbus. There was no change in pupil size or eye position during these cyclic spasms, and the contralateral eyelid was unaffected. The patient underwent an uncomplicated levator recession, which improved the upper eyelid retraction. Postoperative testing, including external motility video and infrared pupillometry, demonstrated no cyclic variation in eyelid position, eye position, or pupil size in the waking state. This is a unique case of unilateral eyelid retraction with periodic spasms under conditions of anesthesia without a preexisting oculomotor paresis; it represents an unusual variation on congenital eyelid retraction and classically described cyclic oculomotor palsy.