Share this article on:

Periodic Unilateral Eyelid Retraction in a Pediatric Patient

Gandhi, Nandini G. MD; Rogers, Gina M. MD; Kardon, Randy H. MD, PhD; Allen, Richard C. MD, PhD

doi: 10.1097/WNO.0b013e31822db30b
Clinical Observation

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.

Supplemental Digital Content is Available in the Text.

Department of Ophthalmology and Visual Sciences (NGG, GMR, RHK, RCA), University of Iowa Hospitals and Clinics, Iowa City, Iowa

Department of Ophthalmology (NGG), Duke University Medical Center, Durham, North Carolina

Department of Veterans Affairs (RHK), Iowa City, Iowa

Department of Otolaryngology-Head and Neck Surgery (RCA), University of Iowa Hospitals and Clinics, Iowa City, Iowa.

Supported in part by an unrestricted grant from the Research to Prevent Blindness, New York, NY.

The authors report no conflicts of interest.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (

Address correspondence to Nandini G. Gandhi, MD, Duke University Eye Center, DUMC 3802, Durham, NC 27710; E-mail:

Congenital eyelid retraction in children has been reported in isolation and in association with disease processes, such as thyroid dysfunction, aberrant innervation of the third nerve, and levator fibrosis (1,2). Cyclic spasms associated with eyelid retraction have been reported almost exclusively in the setting of pre-existing oculomotor paresis and are thought to be the result of aberrant innervation (3–8). We present the case of a child with unilateral eyelid retraction and no pre-existing oculomotor paresis who demonstrated periodic spasms of eyelid retraction after the administration of inhalational anesthesia. We discuss the differential diagnosis and appropriate workup for congenital eyelid retraction and propose possible mechanisms for this patient's unusual presentation.

Back to Top | Article Outline


A healthy 11-year-old girl presented for evaluation and management of right upper eyelid retraction since birth. The patient was the full-term product of an uncomplicated pregnancy and nontraumatic vaginal delivery. The family reported that the right upper eyelid had been retracted since birth, with no noticeable change in the position over time. They also noted that the patient's eyelids remained closed during sleep without abnormal movement of the eyelid while asleep. There was no history of ptosis, strabismus, or periocular/facial trauma, and she took no medications and had no known medication allergies.

On initial examination, the patient had right upper eyelid retraction without proptosis (Fig. 1A). The palpebral fissure measured 11.5 mm on the right eye and 9 mm on the left eye. The margin reflex distance-1 measured 6 mm on the right eye and 3.5 mm on the left eye. Levator function was normal on each side at 15 mm, and the patient demonstrated right lid lag on downgaze (Fig. 1B). The patient had full ductions and versions, and there was no variation in upper eyelid position, with changes in gaze position. The right lower eyelid elevated slightly on upgaze (Fig. 1C), and there was no change in eyelid position with jaw movement. Pupils were isocoric without variation in pupil size in different gaze positions. The anterior and posterior segment examination of both the eyes was normal, with the exception of mild superficial punctate epitheliopathy of the right cornea.

FIG. 1

FIG. 1

Thyroid function studies and MRI of the brain and orbits were normal. It was assumed that the eyelid retraction was idiopathic and isolated, and the decision was made to proceed with a right levator recession.

After the induction of general anesthesia with inhaled sevoflurane and before the administration of local anesthetic, the patient displayed periodic spasms of right upper eyelid retraction (see Video, Supplemental Digital Content 1, 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. The eyelid slowly drifted down to a position of half-closure after 8 seconds of full retraction. There were no fasciculations, changes in pupil size, or alterations in eye position during these spasms. The left eyelid was unaffected. The patient was observed for a total of 10 minutes, and these cycles occurred in exactly the same time interval. Of note, the patient had not received any other medications in the preceding 24 hours. Given that these spasms were not seen during wakefulness, the decision was made to proceed with surgery.

At the 3.5-month postoperative visit, the right upper eyelid position had improved (Fig. 2). There were no cycles of eyelid retraction detected after prolonged observation in the clinic. Upon questioning, the family denied any changes in eyelid position during wakefulness or during sleep. The patient was videotaped with the eyelid position observed in primary position and in downgaze for 5 minutes each: no cyclic variation of eyelid position was detected. Infrared pupillometry performed for 5 minutes likewise demonstrated no cycles of miosis or mydriasis of either eye. Ocular motility was once again confirmed as being normal.

FIG. 2

FIG. 2

Back to Top | Article Outline


We were unable to find other reports of unilateral eyelid retraction with periodic spasms without a pre-existing oculomotor paresis under conditions of inhalational general anesthesia. This case represents an unusual variation on isolated pediatric upper eyelid retraction and classically described oculomotor palsy with cyclic spasms.

Upper eyelid retraction in children has been reported in isolation and in association with systemic and local disease processes (1,2,9–13). The diagnosis of primary, isolated congenital eyelid retraction can be made only when systemic conditions and neurological injury have been excluded. In a review of 16 pediatric patients with unilateral and bilateral eyelid retraction, Stout and Borchert (1) found that the most common causes were hyperthyroidism (either acquired or congenital), aberrant innervation, and local tissue changes (orbital lesions or fibrosis of the levator complex). Abnormally dense extensions of the lateral and medial horn of the levator at the level of the Whitnall ligament have been documented in cases of isolated congenital eyelid retraction (2). Posttraumatic upper eyelid retraction secondary to adhesions between the levator complex and other orbital structures has also been reported (10).

Oculomotor palsy with cyclic spasms is a rare condition first described in 1884 by Rampoldi (8) as “oculo-palpebral imbalance” in a healthy 4-year-old girl. In their review of the literature through 1975, Loewenfeld and Thompson (6) identified only 54 cases; Miller and Lee (7) identified an additional 31 cases between 1975 and 2003. The majority are characterized by the presence of an oculomotor palsy with regular cyclic spasms of varying degrees of eyelid retraction, adduction of the globe, and/or pupillary mydriasis (3–8,14). Loewenfeld and Thompson (6) calculated the average time of each cycle is 73.6 seconds (with a range of 20 seconds to 3.5 minutes), with the paretic phase being consistently longer than the spastic phase in nearly all cases. The exact mechanism of cyclic oculomotor palsy is unknown. Most theories invoke the principle of aberrant innervation following an initial insult to the oculomotor nerve, but the question of whether the process is primarily supranuclear or infranuclear remains controversial (3,6,14,15).

Our case combines features of isolated eyelid retraction and cyclic oculomotor palsy but is distinct from all previously reported cases. First, in spite of the idiosyncratic cyclic spasms of eyelid retraction, our patient had no evidence or history of pre-existing oculomotor paresis. Second, our patient's eyelid was affected in isolation without involvement of the extraocular muscles or pupillary muscles. Finally, the cycles were only present during inhalational general anesthesia and were not observed during wakefulness or sleep.

The mechanism for our patient's clinical findings is unclear. If we assume a neurological basis, both peripheral and central factors may be involved. The unilaterality of the eyelid spasms speaks to peripheral nerve involvement, whereas the rhythmic cycles that were unmasked with general anesthesia suggest disinhibition of central control. It is possible that the patient had an injury in utero or in the perinatal period that affected some fibers of the superior division of the third nerve. The patient's eyelid retraction suggests sustained overaction of injured axons that have re-innervated the levator muscle, and the presence of normal levator function is consistent with a population of uninjured, neurons capable of transmitting normal physiologic impulses (6).

The periodic nature of our patient's eyelid retraction may be due to development of abnormal supranuclear connections. These connections might transmit impulses that overcome levator inhibitors (6,16). Levator inhibition is unnecessary while awake but is required during general anesthesia.

Alternatively, it is possible that this phenomenon is a pharmacologic effect of inhaled sevoflurane. Sevoflurane has been implicated in causing localized or generalized tonic–clonic movements at the end of induction (17–19). These signs are often accompanied by burst suppression on electroencephalography, indicating altered cortical activity as a possible cause for the seizure-like movements (18). Possibly, our patient's eyelid retraction was due to myopathic changes in the levator, making it susceptible to the effects of sevoflurane. However, we were unable to find any reports of periodic muscle spasms with the inhalational anesthetics in conditions of generalized myopathy.

Infants and children presenting with unilateral eyelid retraction without an obvious ocular syndrome warrant a careful history and workup to identify an underlying cause. Thyroid function studies should be performed to rule out congenital or acquired thyroid disease. Imaging of the brain and orbits should be performed with special attention to the entire course of the third nerve to rule out a mass lesion, evidence of trauma, or other structural abnormalities. The possibility of cyclic eyelid retraction should be considered, especially in those patients with evidence of a pre-existing oculomotor palsy. Careful observation of these patients in the outpatient setting is advised prior to workup and surgical planning.

Back to Top | Article Outline


1. Stout AU, Borchert M. Etiology of eyelid retraction in children: a retrospective study. J Pediatr Ophthalmol Strabismus. 1993;30:96–99
2. Ballen PH, Rochkopf L. Congenital retraction of the upper lid. Ophthalmic Surg. 1987;18:689–690
3. Bateman DE, Saunders M. Cyclic oculomotor palsy: description of a case and hypothesis of the mechanism. J Neurol Neurosurg Psychiatry. 1983;46:451–453
4. Fells P, Collin JR. Cyclic oculomotor palsy. Trans Ophthalmol Soc U K. 1979;99:192–196
5. Friedman DI, Wright KW, Sadun AA. Oculomotor palsy with cyclic spasms. Neurology. 1989;39:1263–1264
6. Loewenfeld IE, Thompson HS. Oculomotor paresis with cyclic spasms. A critical review of the literature and a new case. Surv Ophthalmol. 1975;20:81–124
7. Miller NR, Lee AG. Adult-onset acquired oculomotor nerve paresis with cyclic spasms: relationship to neuromyotonia. Am J Ophthalmol. 2004;137:70–76
8. Rampoldi R. Casuistica clinica. II. Singolarissimo caso di equilibrio motorio oculopalpebrale. Ann Ottalmol. 1884;13:463–469
9. Bartley GB. The differential diagnosis and classification of eyelid retraction. Trans Am Ophthalmol Soc. 1995;93:371–387
10. Collin JR, Allen L, Castronuovo S. Congenital eyelid retraction. Br J Ophthalmol. 1990;74:542–544
11. Kwon SI, Kim Y. Upper eyelid retraction after periorbital trauma. Korean J Ophthalmol. 2008;22:255–258
12. Rovit AJ, Deupree DM, Zang Y, Biglan AW. Treatment of congenital unilateral upper eyelid retraction with a marginal myotomy procedure. Ophthalmic Surg. 1988;19:872–875
13. Spierer A, Bourla N. Primary congenital upper eyelid retraction in infants and children. Ophthal Plast Reconstr Surg. 2004;20:246–248
14. Yazici B, Unal M, Koksal M, Ozdek S. Oculomotor palsy with cyclic spasms: a case report. Orbit. 2000;19:129–133
15. Freedman HL, Kushner BJ. Congenital ocular aberrant innervation—new concepts. J Pediatr Ophthalmol Strabismus. 1997;34:10–16
16. Keane JR. Spastic eyelids: failure of levator inhibition in unconscious states. Arch Neurol. 1975;32:695–698
17. Akenson J, Didkirsson I. Convulsions on anaesthetic induction with sevoflurane in young children. Acta Anaesthesiol Scand. 2004;48:405–407
18. Constant I, Seeman R, Murat I. Sevoflurane and epileptiform EEG changes. Paediatr Anesth. 2005;15:266–274
19. Eger E. New inhaled anesthetics. Anesthesiology. 1994;80:906–922

Supplemental Digital Content

Back to Top | Article Outline
© 2011 Lippincott Williams & Wilkins, Inc.