Duane’s Syndrome with CompressiveDenervation of the Lateral Rectus MuscleMark Silverberg, MD, andJoseph Demer, MD, PhD

PURPOSE: To describe an unusual case of Duane’s syn-drome.METHODS: Individual case report.RESULTS: A skull base meningioma was discovered in awoman with Duane’s syndrome who presented withrecurrent, large-angle esotropia and uncharacteristic at-rophy of the lateral rectus muscle on magnetic resonanceimage (MRI) scan.CONCLUSION: Neuroimaging may be useful in unusualcases of Duane’s syndrome. (Am J Ophthalmol 2001;131:146–148. © 2001 by Elsevier Science Inc. Allrights reserved.)

DUANE’S RETRACTION SYNDROME CONSISTS OF CON-

genital limitation or absence of abduction, limitationof adduction, and retraction of the globe with narrowing of

Accepted for publication Jul 24, 2000.From the Jules Stein Eye Institute, University of California, Los

Angeles.Inquiries to Joseph Demer, MD, PhD, 100 Stein Plaza, Box 957002,

UCLA Jules Stein Eye Institute, Los Angeles, CA 90095; fax: (310)206-7826; e-mail: jld@jsei.ucla.edu

Supported by Grant EY-08313 and Core Grant EY-00331 from theU.S. Public Health Service, National Eye Institute. Dr. J. Demer receiveda Research to Prevent Blindness Lew R. Wasserman merit award and isDavid and Laraine Gerber Professor of Ophthalmology.

FIGURE 1. (Top) Preoperative photos show esotropia and marked limitation of abduction of the right eye. Alignment in the primaryposition and abduction are improved after transposition surgery with posterior augmentation sutures. Narrowing of the right palpebralfissure was present on adduction. (Bottom) Magnetic resonance image scan shows atrophy of right lateral rectus (LR) muscle. SR 5superior rectus muscle; ON 5 optic nerve; SO 5 superior oblique muscle; MR 5 medial rectus muscle; IR 5 inferior rectus muscle.

AMERICAN JOURNAL OF OPHTHALMOLOGY146 JANUARY 2001

the palpebral fissure on adduction.1 The pathophysiologyof this disorder involves absence of the sixth nervenucleus, with innervation of the lateral rectus frombranches of the third cranial nerve.2 This anomalousinnervation results in a retraction of the globe fromcocontraction of the medial and lateral rectus muscles.Through the use of magnetic resonance imaging (MRI),we had the unexpected finding of a large meningioma in apatient with Duane’s syndrome.

● CASE: A 56-year-old woman presented in January 1994with a chief complaint of binocular diplopia. Her medicaland surgical histories were unremarkable. She described alifelong history of strabismus, with patching of 1 eye at age5. She had noted an inability to rotate the right eyeoutward since childhood.

Visual acuity was 20/25 in the right eye (13.00 10.75 3 75), and 20/20 in the left eye (13.00 1 1.00 3100). No torticollis existed. Pupils were equal and nor-mally reactive. Visual fields were full to confrontation.Slit-lamp and fundus examinations were within normallimits. The optic nerves were unremarkable.

Marked limitation of abduction in the right eye existedto less than 10° beyond midline. A 35Œ esotropia existed inprimary gaze that decreased to 30Œ in left gaze, but was toolarge to measure in right gaze. At near the esotropiameasured 45Œ. The left eye was preferred for fixation. Novertical deviation was noted. Abducting saccades for theright eye were terminally slow. Narrowing of the rightpalpebral fissure and globe retraction on adduction oc-curred, with widening of the palpebral fissure on abduc-tion.

In February 1995, the patient underwent a 7-mm rightmedial rectus recession, resulting in significant improve-ment in her esotropia. In March 1999, she returned with acomplaint of increasing right esotropia (Figure 1, top). Herexamination was nearly identical to the initial examina-tion with globe retraction, and a 35Œ esotropia. As part ofa prospective study, a high-resolution coronal MRI scan ofthe orbits was performed,3 demonstrating uncharacteristicatrophy of the right lateral rectus muscle.4 This findingseemed more consistent with abducens palsy rather thanwith Duane’s syndrome.

An MRI scan of the brain was then obtained (Figure 2)that demonstrated an extensive mass in the right cavern-ous sinus most consistent with a meningioma. A neurosur-gical consultant found no other neurologic involvement.

In September 1999, the patient underwent transpositionof the superior and inferior rectus muscles to the lateralrectus insertion, with posterior augmentation sutures.5Postoperatively, she had only a 6Œ esotropia, and she wasable to abduct 15° past midline with resolution of herdiplopia (Figure 1, bottom). Repeat MRI scans showed nochange in the tumor that the neurosurgeon elected toobserve.

We are unaware of previous reports of a meningioma inassociation with Duane’s syndrome and could find noreference to it in a computer search using Medline. Thiscase is especially unusual in the manner that the tumor wasdiscovered. The patient had lifelong Duane’s syndrome.However, MRI scans of the orbits showed lateral rectusatrophy, a distinctly uncharacteristic finding. Thisprompted further neurologic imaging and the discovery ofthe tumor.

FIGURE 2. Magnetic resonance image scan of the brain demonstrates a homogenously enhancing mass in the right cavernous sinus,involving Meckel’s cave, the clivus, petrous ridge, and the cerebello-pontine angle.

BRIEF REPORTSVOL. 131, NO. 1 147

How do we explain the lateral rectus muscle atrophy?Recalling the pathophysiology of Duane’s syndrome, mis-innervation exists, not denervation of the lateral rectus.The innervation of the lateral rectus muscle comes fromanomalous third-nerve branches. We speculate that thisanomalous innervation is tenuous compared with normalinnervation. In this case, the atrophy of the lateral rectuswas presumably induced by tumor damage to anomalousbranches of the third nerve. Perhaps when the anomalousoculomotor nerve fibers were compressed by the tumor,insufficient axonal redundancy existed to maintain musclefunction, although normal target muscles of the oculomo-tor nerve may have been spared. Curiously, despite thelarge size of the tumor, no other cranial nerves were alsoinvolved. In retrospect, the initial presentation of a 35Œ

esotropia was relatively large for Duane’s syndrome, whichnormally has less than 30Œ deviation in primary gaze.2

In conclusion, this is a case of Duane’s syndrome withthe confounding presentation of a recurring, relativelylarge-angle esotropia that was induced by a large skull basemeningioma. The ophthalmologist should be mindful ofthe differential diagnosis of an abduction deficit, especiallyin cases of Duane’s syndrome when the deviation is largerthan expected. Imaging may be clinically useful in atypicalcases.

REFERENCES

1. Duane A. Congenital deficiency of abduction associated withimpairment of adduction, retraction movements, contractionof the palpebral fissure and oblique movements of the eye.Arch Ophthalmol 1905;34:133.

2. Jampolsky A. Duane Syndrome. In: Rosenbaum A, SantiagoA, editors. Clinical strabismus management, principles andsurgical techniques. Philadelphia: W.B. Saunders. 1999:325–346.

3. Demer J, Miller J. Orbital imaging in strabismus surgery. In:Rosenbaum A, Santiago A, editors. Clinical strabismus man-agement, principles and surgical techniques. Philadelphia:W.B. Saunders. 1999:84–98.

4. Miller J, Demer J, Rosenbaum A. Two mechanisms of up-shoots and down-shoots in Duane’s syndrome revealed by anew MRI technique. In: Campos E. C., editor. Strabismus andocular motility disorders. New York: MacMillan, 1990:229–232.

5. Foster RS. Vertical muscle transposition augmented withlateral fixation. J Am Assoc Pediatr Ophthalmol Strabismus1997;1:20–30.

A Case of Marcus Gunn Jaw Winkingand Pseudo Inferior ObliqueOveractionJeong-Min Hwang, MD, and Seong-Ho Park, MD

PURPOSE: To report the association of Marcus Gunnjaw-winking phenomenon and pseudo inferior obliqueoveraction.

METHODS: Case report.RESULTS: A 21-year-old woman presented with righteyelid elevation on the chewing movement since infancy.Examination showed that both Marcus Gunn jaw-wink-ing phenomenon and pseudo inferior oblique overactioncoexist in this patient.CONCLUSION: The association of Marcus Gunn jawwinking and pseudo inferior oblique overaction in onepatient is reported. This coexistence in our case mayprovide some support to the existing concept that bothphenomena are caused by an ocular aberrant innervation.(Am J Ophthalmol 2001;131:148–150. © 2001 byElsevier Science Inc. All rights reserved.)

LEVATOR CONTRACTION WITH CONTRACTION OF THE

external pterygoid muscle is the most common form oftrigemino-oculomotor synkinesis.1 Kushner first describedpseudo inferior oblique overaction, a Y-or V-pattern exo-tropia caused by cofiring of the lateral rectus with thesuperior rectus in upgaze.2

This report describes a patient with unilateral MarcusGunn jaw-winking phenomenon and pseudo inferioroblique overaction. This association has not been previ-ously reported.

● CASE: A 21-year-old woman presented with right eyelidelevation on the chewing movement since infancy. Thecondition had remained unchanged since then. The pa-tient was born after an uneventful pregnancy and delivery.She did not complain of diplopia and had no history ofhead trauma, use of an eye patch, strabismus surgery, orunusual febrile illness. Medical history and family historywere unremarkable.

Ophthalmic examination showed visual acuity of RE:20/25 and LE: 20/20. Pupillary examination was normalwithout any change according the directions of gaze.Slit-lamp examinations, including intraocular pressures,were normal. No excyclotorsion of the fundus occurred ineither eye. She was orthophoric in the primary positionand downgaze, and 45D exotropia in upgaze in the distanceand at near (Figure 1). On side gaze, no hypertropia ofeither eye occurred. As the eyes elevated further andreached maximum upgaze, a marked abduction of the righteye occurred (Figure 1).

The eyelid positions and levator functions were normalbilaterally in the primary position (Figure 2, left). Whenchewing gum, the right upper lid was maximally retracted7 mm (Figure 2, right). Eye movement or neck movementdid not affect the eyelid positions. The complete neuro-

Accepted for publication Jul 21, 2000.From the Department of Ophthalmology (J.-M.H.), and the Depart-

ment of Neurology (S.-H.P.), Seoul Municipal Boramae Hospital, Col-lege of Medicine, Seoul National University, Seoul, Korea.Inquiries toSeong-Ho Park, MD, Department of Neurology, Seoul Municipal Bora-mae Hospital, 395 Sindaebang-2-dong, Dongjak-ku, Seoul 156-012,Korea; fax: 182-2-849-3689; e-mail : nrpsh@brm.co.kr

AMERICAN JOURNAL OF OPHTHALMOLOGY148 JANUARY 2001