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Clinical Observation

Monocular Elevation Deficiency (Double Elevator Palsy): A Cautionary Note


Michael C. Brodsky, MD, Virginia Karlsson, CO

Abstract: Monocular elevation deciency (or double elevator palsy) is a descriptive term denoting a congenital deciency of monocular elevation that is equal in abduction and adduction. We describe a child with monocular elevation deciency who displayed tethering and buckling of the central lower eyelid in downgaze. We caution that this manifestation of inferior rectus contracture can simulate impaired infraduction in the involved eye.
Journal of Neuro-Ophthalmology 2011;31:5657 doi: 10.1097/WNO.0b013e3181fb4a18 2011 by North American Neuro-Ophthalmology Society

CASE REPORT
A 4-year-old boy was referred to us for evaluation of an inability to elevate the right eye since birth. He was born prematurely at 28.5 weeks gestation, weighing 2 lb 13 oz, and required hospitalization for 3 months. He was currently meeting his developmental milestones. On examination, visual acuity was 20/25, right eye, and 20/20, left eye, using Allen cards. He maintained xation with the left eye but did not do so with the right eye. Extraocular movements showed absent elevation of the right eye in primary position and right and left gaze. Mild right upper eyelid pseudoptosis was noted, which resolved with xation of the right eye. An enhanced right lower eyelid fold was present. Titmus stereoacuity was 60 seconds of arc. Fixing at near, he had 5 prism diopter right hypotropia, which was horizontally comitant but increased to 25 prism diopter in upgaze and resolved in downgaze. Upward saccades from infraduction to primary position were brisk in the right eye. Bielschowsky head tilt testing produced no detectable change, and Bell phenomenon was diminished in the right eye. The patient maintained a slight chin-up head position when viewing objects of interest. There was no associated jaw winking, and retinal examination showed mild extorsion in both eyes. Cycloplegic refraction was +1.75 +2.00 3 15 in the right eye, and +1.25 +0.50 3 175 in the left eye. He was prescribed glasses and treated with part-time occlusion therapy of the left eye. On his follow-up visit, his ndings were unchanged except that version testing showed what appeared to be decient downward rotation of the right eye compared to the left eye (Fig. 1). However, prism and alternate cover testing showed no hyperdeviation in downgaze. On closer examination, it became apparent that the mid portion of the right lower eyelid had tethered and buckled in downgaze, producing retraction of the right lower eyelid. The greater visibility of the right lower iris created the appearance of an infraduction decit of the right eye (Fig. 1). This focal
Brodsky and Karlsson: J Neuro-Ophthalmol 2011; 31: 56-57

onocular elevation deciency (or double elevator palsy) is a descriptive term denoting a congenital deciency of monocular elevation that is equal in abduction and adduction (1,2). In general, it can be paretic or restrictive, with reduced Bell phenomenon, normal upward saccadic velocities, and a prominent lower eyelid fold in the affected eye considered to be positive signs of inferior rectus contracture (3,4). We describe a child with monocular elevation deciency who displayed tethering and buckling of the central lower eyelid in downgaze. We caution that this manifestation of inferior rectus contracture can simulate impaired infraduction in the involved eye.

Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota. Supported in part by an unrestricted grant to the Department of Ophthalmology at Mayo from Research to Prevent Blindness. The authors report no conicts of interest. Address correspondence to Michael C. Brodsky, MD, Mayo Clinic, 200 First Street SW, Rochester, MN 55905; E-mail: brodsky. michael@mayo.edu

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Clinical Observation
eyelid retraction was attributed to contracture of the right inferior rectus muscle.

DISCUSSION
In 1977, Scott and Jackson (4) described an accentuated lower eyelid fold that became more prominent in attempted upgaze as a sign of inferior rectus contracture in children with monocular elevation deciency or double elevator palsy. In our patient, an accentuated lower eyelid fold in upgaze was accompanied by focal tethering and buckling of the lower eyelid in downgaze, creating the appearance of an infraduction decit in the involved eye. If not recognized as a correlative sign of inferior rectus contracture, this unusual clinical manifestation could lead to unnecessary neurodiagnostic evaluation.

REFERENCES
1. Metz HS. Double elevator palsy. Arch Ophthalmol. 1979;97: 901903. 2. Brodsky MC. Pediatric Neuro-Ophthalmology. New York, NY: Springer, 2010:348350. 3. Ziffer AJ, Rosenbaum AL, Demer JL, Yee RD. Congenital double elevator palsy: vertical saccadic velocity utilizing scleral search coil technique. J Pediatr Ophthalmol Strabismus. 1992;29:142149. 4. Scott WE, Jackson OB. Double elevator palsy: the signicance of inferior rectus restriction. Am Orthopt J. 1977;27:510.

FIG. 1. Vertical gaze positions showing absent supraduction with enhanced right lower eyelid fold in the right eye (A), vertical alignment near primary position (B), and focal tethering and retraction of the midportion of the right lower eyelid in downgaze (C). Note the greater visibility of the lower right iris, which creates the appearance of an infraduction decit in the right eye (C).

Brodsky and Karlsson: J Neuro-Ophthalmol 2011; 31: 56-57

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Copyright North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.