Duane's retraction syndrome

SURVEY OF OPHTHALMOLOGY

MAJOR

VOLUME 38. NUMBER 3 * NOVEMBER-DECEMBER

1993

REVIEW

Duane’s Retraction Syndrome PATRICK A. DERESPINIS, MD, ANTHONY RUDOLPH S. WAGNER, MD, AND SUQIN

R. CAPUTO, GUO, MD

MD,

Abstract.Duane’s

retraction syndrome (DRS) has been a recognized clinical entity for nearly a century. It is a clinically well described ocular disorder consisting of retraction of‘the globe with narrowing of the lid fissure in attempted adduction, frequent abduction deficiency with variablt limitation to adduction, and upshoot and/or downshoot ofthe affected eye on adduction. Among strabismus patients the incidence of DRS is probably not more than 5%. Most cases are sporadic, but familial cases have been estimated at 10% by most authors. Numerous theories concerning the etiology and pathogenesis of DRS have been proposed. including agenesis of‘ the abducens nucleus, but the majority of investigators concur that the characteristic findings are best esplained by a paradoxical innervation of the lateral rectus muscle, which subsequently causes a cocontraction ofthe horizontal rectus muscles. The frequent association of DRS with other congenital anomalies suggests a teratogenic event occurring between the filurth to eighth week ot gestation as an etiological factor. In this review historical aspects and theories of the syndrome are studied and statistical data are compiled and analyzed. Clinical features, differential diagnoses and variants of the syndrome are examined. Testing and treatment objectives are tliscussed. (SW-V Ophthalmol 38:257-288, 1993)

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Surv Ophthalmol

38 (3) November-December

cause of the retraction syndrome over the next 75 years will consist of mostly mechanical theories. Not until the advent of electromyography in the 1950s will innervational theories be introduced.”

I. Historical Aspects A. PATHOGENESIS THEORIES 1. Mechanical Anomalies

and

AND ETIOLOGICAL Peripheral

Structural

In 1899 Turk wrote that the retraction syndrome occurs because the lateral rectus behaves In 1900 Wolffe reported as “an inelastic band.““” five cases of the syndrome and proposed a congenital origin.““’ His complete account described many of the elements which today we would recognize as classic Duane’s retraction syndrome (DRS). W01ff-‘“~ also felt that the lateral rectus was connective tissue replaced by “an unyielding strand.” Both Wolffe”‘!’ and Varese’!‘” in separate papers in 1901 felt that there were hereditary tendencies in patients with the condition. That same year Axenfeld and Schurenberg” claimed that an abnormally posterior insertion of the medial rectus caused the muscle to behave as a retractor bulbi. Earlier Bahr”’ had observed that the medial rectus inserted in two portions, one 12 millimeters behind the limbus and the other even further behind. In 1903 Bietti’” published a paper consisting of 3 1 cases of the retraction syndrome. Duane’s landmark paper of 54 cases in 1905 included the unique features of the syndrome of which the most prominent was retraction of the globe. He reported that the complete syndrome consisted of the following components: 1. Comn$ete, OTless often pa&l, absence of out714wl mozlement of the clffected eye. 2. Purtial, or rarely comfilete, deficiency of mouemrnt inward of the affected eye. 3. Ketwction of the affected eye into the orbit when it is (ld~ucted. 4. A sher@y oblique mozlement of the uffected eye either- up nud in or dour and in when its is udducted. 5. Partial closure of the eyelids (pseudo-ptosis) of the affected eye ulhen it is adducted. 6. Paresis, or clt leust marked deficiency of COIIZIPIgence, the (Iffected eye remuini?lg fixed in the primtlr;y positiojl zuhile the sound eye is co~lz~ergi~lg.” Duane felt that some of the characteristics of the syndrome were in part caused by a double insertion of the medial rectus, with one component rotating the globe and the other retracting

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DERESPINIS

ET AL

it. This belief was shared with a number of other authors,‘.“‘.‘~.‘“: Over the years a great many authors have published papers on the retraction syndrome, exploring every aspect of the condition. What has still remained a relative uncertainty throughout the years is the etiology and pathogenesis of the disorder. The double insertion theory of Duane’s was for some time a popular way of explaining the unusual eye movements in the syndrome. Unfortunately, even by Duane’s own admission, it was flawed. If in DRS the more posteriorally inserted half of the medial rectus truly causes the retraction of the globe on adduction, then why is this not always the case when posterior insertions are found. In 1974 Gobin reported that in 67 cases of DRS on which he performed surgery, only 23 cases were found to have anomalous insertions of the medial rectus.“” Muscle surgeons will attest to the fact that on frequent occasions anomalous insertions and strands are found during routine strabismus cases. Anomalous or multiple insertions could not sufficiently explain the retraction syndrome. From as early as 1900”” to as late as 1934,“” authors described an inelastic, fibrous lateral rectus muscle as being the cause of the retraction syndrome. Unfortunately for the proponents of these theories, normal medial and lateral recti muscles have been found by various other authors during surgery.““.“” In 1921 Gallus hypothesized that a fibrous transformation of the lateral rectus muscle could take place as a developmental anomaly caused by nuclear aplasia or may occur during a hemorrhage into the muscle or damage to the tendon sheath during birth trauma.“’ Clifford in 1926 also proposed this theory, describing how the injury to the lateral rectus muscle could occur as the infant’s head passed through the birth canal.“‘C;ifford had his doubts concerning this theory, since DRS was found in a baby born of a normal Caesarean section. A case was even made for the etiology of DRS being due to the findings of dense adhesions between the medial rectus muscle and the wall of the orbit in eight surgical cases as late as 1956.“’ In the 1930s and 1940s many notable authors were to publish numerous papers on the subject of DRS. The majority of these papers basically reiterated what was already reported in previous papers. It was not until 1957 that Breinin, utilizing electromyography, reported one of two patients with typical retraction syndrome who had evidence of co-contraction of the medial and lateral rectus muscles on adduction.“”

DUANE’S RETRACTION 2. Innervational

SYNDROME

Anomalies

Rreinin described the absence of electrical potentials ofthe lateral rectus muscle on abduction. hut the presence of action potentials on adductiott. Ele felt this phenomenon of co-contraction wab responsible for the globe retraction commonly seen during aclduction in the syndrome. Ilk explanation that co-contraction ~vas due to the abt~ortnall~ sensitized stretch reflex of the 1;rteral rcctus muscle was disprovecl in a subsequent study in \vhiclt co-contraction occurred in ;I lateral r&us muscle that was detached from ttte y$~be cluritig surgery.- “” There were mart) ~le~trotri~o~laphi~ studies of the retraction syt1drome lo ti)llow. In l!NiO Sat0 studiecl 14 patientr with DKS.. ,I,->’1welvc of‘the patients wet-e smx esshill\, \ruclietl, reve;ilitig various degrees 01’ ;ttiottialous ititierv;itioti of the lateral rectwi tntisc,lc- during aclductiott. ~l‘lie action potentials of the lattwl tutus niusclcs ranged from no acTivit\ tluring abcluction to ~ssetttially equal activity in :t(lduction versus abcluction. Medial rcctus acti\,itv XI\; ~tppawtttl~ ~tortttal in the patients studicd. :\Iso th;t( yar. l’apst and E:sslen studied tiw c~litiicallY t\,pical casts of DKS and fi~iincl it)c~e;~setl intitwWion of the lateral rectus only in ;ttltluc~rioti.’ “’ (:o-cotitr~ic.tiot~ also occurrecl bcI~vc‘c’n the 1;11c~al rec~tu~ and either the sitperiot or itifi~riot- wctits in these c‘;tsc’s. Iii I Wi’l Orlo\~sl;i ;itt(l \Vc?jtowicz studied timr patients ;incl detern~itied tltat the lateral recti sItowwl it:cotnplcte inhibition on aclduction and I~oot~ i~rc.t~uittitet~t iit abductioti as comparecl 10 r hc 1101~111;11 responsc~. ’ “I ~i‘hev also postulated coittti~r\~~ition betM.eett the superior, inferior and I;tteral iwttrs muscles. lit 19% Wojtowicz exat1iin4 six paticttts ancl fi)uncl IOM.degrees ofactivitv c)f‘the Iatcral rectits in all fielcls ofgaze with the highest degree of‘acri\ity occurring in downgaze itt ti)ur of‘ the pttiettts.? Iii 1Sci’i %aul~et~tiiati et al reported 10 cases of clinically rypical DKS in which the lateral retttts ni~rsclr shotvecl various degrees of electrical xtivit\, on k:MC; testing, ranging from firing paratlosk~lly OII xlduction to not firing at all. The tiiecli;tl rectus muscles were normal.“” K\. this poinr it W;IS evident that the abnormalitv ii1 DKS was primarily related to paradoxic;tl it;tiet-\atic,tt of’ the l,rtet~al rectus tnuscle. A witle \,:tt-iet\, of’intter\-atiotial abtiortnalities of the latcra1 recti have been reportecl in these studies itlclucling c-o-firing in adduction, reduced activattempted abduction, inappropriate relty OII sp~ttses on upgaze ;IIIC~ downgaze and abnormal obticltte ttto\w11en~s. <;lobe retraction xld nar-

“.X

t-owing of the palpebral fissure in aclcluctioti was also thought to be related to this primat.\ innerwtional anotiial~ of co-ccmtraction of the horizontal rectus muscles plus iti\~ol\~etnetit ot‘the ittferior atnd superior rt’ctus muscks as well. ~l‘hese findings are further supportecl b\- ;t stud! performed b\, Strachan and Ktxnvn on ;I series ot‘ patients 4th DKS.“’ _1_hex t\venty su+Ts M’t’l‘t’ nie;~~urc~l rlectt-otti,ogr~tpl~i~~tll~ iii ;I clu;tntitative tii;inner using both spike dur;ttion attd ;tvt‘r;igv ti~ecluenc~~. Lateral rfxtus firittg \vas pal-aclosic to subtiorttial clepetiditig on the g_;t/e. l‘ltr medial rt‘c‘tus firing was ttot-m;il. ‘l‘her~ \v;ts i~tioni;~lo~~s synergistic ititter\~atioti betwet‘tt thrmedial rectus and the sulwriot. ;tntl inkriot. WCtus alotig \vith tlit. oblic~ti~ tntt~.lcs at tin1c.s. 3. Central Nervous

System Anomalies

E~le~trotlt~~ograpltic studies h;tvv bc*eti \.ery helpful in educating us a5 to the ctiolog!, of DKS, but it Itas basically given us the et’kct and not the c;iuse. Since itiwstigati\.e lintlings ha\ c clctermined that innervational ;Il,not.ttt;tlities in IfKS are the likeI!. C;IIISC fi)r the clisotxlcr, the cluesfioti has ariseit as to w~hether it i5 01‘ n~~c~lcat~or sttpr;itiuclear origin. The sitnultattc.oii5 cotiltxctioti in 1)KS ~ottlcl be esplaincd b!, A prc~rto~or brainstein abnortnalit~, such as ;I dekt in llic~ \wtibutar ttuclci, medial longitudinal ktsiwlus or the paraniecliati pontine reticular fijt-ni;t~ioti. or tttav sitiipl!, be more peripheral, suc.li as an ;uiomiilous ititier~~ation of the I;iteral twtus riiiiscle b) tile ociilotnotor nerve. It may ver\ \vell bc ;I \wiation of‘both possibilities. Pi-oblems at-kc in clv tcrtnining the causes of‘ DKS bccattse it ih ;I brtiign clisorder and the niajorit\~ of I how Akctc-cl are he;ilthv young children. Aufops~ \ubjccTs art’ rarel~~ a\Aable fi)r stud!,. In I!)46 Matteuci reported thr findings of’ an autopsy perfi)rmecl on ;i patient with a cIocunientetl unilateral DKS.“” I‘he abduccns nucleus \V;IS livpoplastic ant1 the 6th nt‘rvt’ \t’;ts ;tbscnt on the affkted side. Innet~v;ttion~tl infi~rtnation was not provided. Kotli Sat0 in I CM)“” antI Klocli c’t al in 1964.“” atier perti~rnting k>l(; stuclics on their patients, clec~idecl that the retrac~tioit syttclromc wx best esplainecl 1)) ;I detkcr at the supr;uiu~~lear level. Hoyt and Nat-htigallrt. believed that the close proximity of‘ Ihe third ;tttd sixth nerves ;IS they pass through thy wvt~t~ous sinus and enter the orbit provide ~I;III~ 1hwt-eticxl opportunities for im intermingling ot‘tli~ir ilsotts.7!’ whey t&It that ;m absettt sixth nc‘r~c cx~ntbinecl with anotll;tlies ol‘clistril~~ttion of‘thc oc~ulotIlotot~ ttervc’ was the most likely par hogenesis of’ tttc-

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syndrome, although brain stem defects could not be ruled out as a cause. Absent sixth nerves and sixth nerve nuclei were reported in a bilateral Duane’s Type 1117’ followed by a unilateral case with an absent sixth nerve on the affected side.“’ In the latter case the abducens nucleus contained no motor neuron cell bodies, but did contain several small cell bodies compatible with internuclear neurons. This was thought to explain the preservation ofconjugate eye movements through the medial longitudinal fasiculus, even though the sixth nerve nucleus was absent. The observation of sgccade movements have also given valuable information as to the etiology of DRS. Metz and associates used electro-oculography on 28 patients with DRS and observed frequent slowing of adduction saccades in patients with limited abduction.“” Decreased abduction saccades were explained by an absence of lateral rectus recruitment, while slow adduction saccades were explained by paradoxical innervation of the lateral rectus. This differentiated Duane’s from a lateral rectus palsy. Unfortunately, the authors tested only the affected eyes. Nemet and Ron three years later performed bilateral EOGs on 10 patients with Duane’s syndrome and found that the saccades of the normal eyes were affected.“” Co-contraction of the medial and lateral rectus muscle in the affected eye contradicts Sherrington’s law, which states that for every impulse to contract that an agonist muscle receives, an equivalent inhibitory impulse is sent to its antagonist in the same eye. Thus, DRS is a distinct exception to Sherrington’s law of reciprocal innervation.‘“” The law that concerns itself with the concerted effort between both eyes to achieve a desired direction of gaze is Hering’s law of motor correspondence. It states that equal and simultaneous innervation flows to synergistic muscles. According to Hering’s law, anomalous innervation might be reflected in the unaffected eye of a patient with DRS.” The authors found that when adduction of the aff‘ected eye was present, Hering’s law held, but when adduction was limited, then the law could not be demonstrated. This may represent a more supranuclear explanation of the syndrome. In 1974 Schmidt suggested that unilateral abducting nystagmus in the normal eye of a patient with unilateral DRS pointed to a central def-ect, particularly of the premotor structures in the area of the brainstem.“” In 1980 Ramsay and Taylor linked the association of DRS with croco-

1993

DERESPINIS ET AL

dile tears as being due to a teratogenic effect during embryogenesis.“” They further postulated that the area controlling both lacrimation and the eye movement disorder in DRS lies in close proximity to the abducens nucleus. In 1981 Gourdeau et al studied five patients with unilateral Type I DRS.“’ Using vestibulo-ocular reflexes, optokinetic nystagmus and after-nystagmus, they also noted abnormal saccadic velocities in both eyes. They propounded a primary brainstem abnormality involving the premotor structures as the cause. Various other modes of testing have been recruited in attempts of better understanding Duane’s syndrome. The use of infrared oculogra,phy”” and auditory evoked responses”‘,‘H’i have been reported. Taylor and Polomeno performed auditory brainstem responses on 16 patients with Duane’s syndrome and found no differences between the patients and the normal controls.“” They felt that the defect could not be in the area of the pons, but they could not discount the possibility that the lesion was in the area of the abducens nucleus. This contradicted the earlier findings of Jay and Hoyt, who three years earlier performed auditory evoked responses on 14 patients with unilateral Type I DRS and found the responses to be abnormal on the side of the presumed lesion in 9 out of 14 patients.x’ They thought that these were suggestive of a primary brainstem anomaly, possibly pontine in origin. The other five patients could be explained only by nonbrainstem mechanisms. Perhaps there are then several causal factors involved in the pathogenesis of DRS”” and it is a disorder composed of a spectrum of various developmental defects which give a clinical picture composed of many of the same essential elements.‘!’ B. ATTEMPTS AT CLASSIFICATION Papst14”,142proposed different clinical

varieties of DRS using the information gathered by EMG studies and based his categories on abnormal cocontraction of 1) the medial and lateral rectus, 2) the superior and lateral rectus, 3) inferior and lateral rectus, and 4) lateral rectus and several other muscles. By 1974 Alfred Huber felt that enough electrophysiologic information had been gathered on the syndrome to disprove the theories of the majority of earlier authors. “The explanations based on mechanical concepts (birth injury, congenital or acquired musculofacial anomaly, etc.) are either insufficient or at variance with the facts.““”

DUANE’S RETRACTION

SYNDROME

I i~11x~~ did not exclude secondary anatomical altcrations ol‘the n~uscx~lal- tissue brvught about b) primary iunervatioual disturbances; he situpi) did uot corisider these chauges to be the primat ~tl~uot~iiiality in DKS. Huher analyzed the classification systems proposed by both Malbran ““’ and Lyle and Bridgenla11.“~‘~ Lyle and Hridgeman divided DRS into rlllu!

sul,types.‘”

A: Abduc-tiotl more deficient than adduction hut both are deficient. Adduction causes glolw wtraction and pIpebra tissue narrowing; F~‘vpe H: Abductiotl is deficient hut adduction is pl‘qx

alld ~l‘\pc (1: .Ibduction is less deficieiil Ili;tu atlcluc~tiotl hut both are deficient. :\tlductiotl (;IIISC’S globe retraction and palpehtxl tisstlv Iurro~ving. Malbrm divided the s~udrou~t iuto t tlr ti)llowins three subtypes: ““’ -l‘ype I: Palsy of’ ahductioii: I‘J~ 1I: Palsy of adductiou; and -I‘ype I I I: Limitation of deprrbsioti 2nd &\;Ilion without inipirnient of‘ horizorit;~l moveIIO~;

111rn

ts

using the above clasGfic_ations togethwith the electt.om~ogl.~~phic itlfi)riiiatioti theii

k1 IllXT,“‘~ t-1‘

262 available

Surv Ophthalmol presented

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38 (3) November-December

1993

following

possible motility problem that could be seen in DRS. Unfortunately, the system was much too complicated to ever be of any practical use. The classification system suggested by Huber is still the most utilized today, although the modification by Ahluwalia et al has many practical clinical aspects. No one would argue that the retraction syndrome is a much more complicated entity than what was proposed by the early describers of the syndrome at the turn of the century. Huber’s system is a simple description of the major clinical findings in each eye. It does not distinguish between the many innervational variants of the syndrome. It does not encompass vertical deviations, anomalous head positions or misleading bilateral presentations. Certain authors advise against the use of the system because it limits one’s thinking in deciding upon an appropriate surgical plan.“” Huber’s classification system has remained popular because it used clinical descriptions to simplify a complex syndrome. As long as the clinician has a working understanding of the pathogenesis of DRS and its many overlapping presentations, the Huber classification system remains a simplistic description of a complex group of findings. Its use should be accompained by detailed descriptions of other associated ocular findings (e.g., head position, overshoots, deviation in primary gaze).

classification

system which is most commonly used up to this day: Type I: Marked limitation of abduction with minimally defective or normal adduction, retraction of globe and narrowing of the palpebral fissure in adduction, widening of the fissure on abduction (Fig. 1). Simultaneous EMG recordings of the lateral and medial rectus muscles showed paradoxical innervation of the lateral rectus, with peak impulses on adduction and defective impulses on attempted abduction. There was normal electrical behavior of the medial rectus. Type II: Marked limitation of adduction with exotropia of the affected eye, abduction normal or slightly limited, retraction of the globe and narrowing of the fissure on attempted adduction (Fig. 2). On EMG, the lateral rectus showed peak impulses on abduction and a second paradoxical peak on attempted adduction. There was normal behavior of the medial rectus. Type III: Combined limitation or absence of both abduction and adduction, retraction of the globe and narrowing of the palpebral fissure on attempted adduction. (Fig. 3). EMG findings showed intense innervation of both the lateral and medial rectus muscles, whether in primary gaze, abduction or adduction. Huber also recognized the presence of the additional vertical motor anomalies, and A-, V-, and X-patterns that frequently accompany the retraction syndrome. He felt that “the paradoxical innervation of the lateral rectus muscle comprises variable synergistic innervations not only with the medial rectus, but also with the superior or inferior rectus or both.” A modification of Huber’s classification was proposed by Ahluwalia et al in 1988.” It expanded on Huber’s system by including three subgroups in each of the three types based on the deviation noted in the primary position of gaze. All of the other features found in Huber’s description otherwise remained the same. Therefore, Type lA, 1B and 1C DRS would be an abduction deficiency with the deviation in primary gaze being an esotropia, exotropia and orthophoria, respectively. In the same study Type 1A (25%) and Type 3C (21%~) were found to be the most frequent subgroups. Reny and Brichet”” also used Malbran’s scheme and electrophysiologic information to devise a classification system for DRS. It encompassed many of the descriptions in the other classification systems and actually described every

II. Current Concepts A. PATHOGENESIS Mechanical, innervational and central nervous system abnormalities have all been implicated in patients with DRS. Is one concept of pathogenesis the correct one or can a combination of these defects account for the clinical findings in DRS? Numerous investigators have found a spectrum of anatomical findings when operating on the extraocular muscles. The lateral rectus muscle has been repeatedly described as fibrotic, inelastic and unyielding by some authors.“‘,“.““.‘)“!’ Retraction of the globe on adduction, a major component of the retraction syndrome, was originally felt to be due to this phenomenon. It was also thought to occur because an either posteriorally or multiply inserted medial rectus muscle exerted a retracting force on an adducting Although numerous authors have reeye.‘“.“” ported these surgical findings, other-s have fimnd the muscle to be completely normal. Co-contraction of the medial and lateral rectus muscle, although considered to be one of the rnajor features, has at times not been confirmed

DUANE’S RETRACTION

SYNDROME

elcctromyographically in patients with DKS.‘,“’ h:ventually the retraction of the globe was credited to co-contraction although, according to a study by Scott in 1971, “” in which he measured the rearward force needed to generate globe retraction, both the co-contraction theory and the lateral rectus fibrosis theory were capable of‘ explaining the phenomenon. Perhaps the fibrotic cliatigcs which may occur in the lateral rectus muscle are clue to the longstanding innerva tional abnormalities in the muscle. Further stuclits were perfi~t-med in which the lateral rectus musclt was renderetl ineffective by the injection of’ anesthetics. In one study retraction persisted.“i while in the other it disappeared.“’ LIpshoots and downshoots of’ the affected eye in DKS has in thy past been attributed to an ~tbnortnally high or low insertion of‘ the medial rectris muscle.’ Srit~secluetttly, a~~oniiilous it)ttcrvations of‘ the vertical muscles were proexplanations l,l_lve p’W.Xl. “.I “‘.‘.“‘.““.‘S’ l‘hese sitice been strongly tlisputed. Eliminating these abnc~rtnal vertical movements has been attemptrtl surgically bv weakening procedures on the \ t’rt ical rectus atid oblique muscles without mrtc~lt success. Van Noorden and Murray reportccl five pa’ients with vertical upshoots or downshoots who underwettt horizontal posterior fixaton procedures.“‘” ;\I1 five patients were cured ot‘ their abnormal excursions. Magoon and coworkers injected xylocaitte into an inferior oblique tttuscle of‘an eye with an upshoot arid enophthal11~0s.“‘~’E:M(; recordings of‘ the muscle were absent. yet there was no alteration in the pattern of o\,et-shoot and retraction. When the same procetturc \v;ts repeated on the lateral rectus muscle of‘ the satttc’ rye, the retraction and overshoot were rlimin;tted. Roth these studies seem to confirm that co-contraction is responsible for the enc~l~htl~;tlt~~os and possibly the vertical overshoots fihuttct in DRS. Apparently there is a “bridle” or “lethet-ing” elf&t in the horizontal muscles and there is a certain atitouttt of slippage which ocWith atly amount c~felm_ (111‘s.I”i,lIT li;.ll,‘l I~‘~.l~-l,l’~x ~;ttion or clcpressiotr outside the primary position there is a change in the horizontal rectus in rrference to the center of rotation of the globe, causing these muscles to becotne elevators and depressors in addition to their horizontal a(‘ti,,,,,~~ IO”.IO”’ fhis tethering effect does not completely dispel the anomalous innervation ofverti(a1 tnuscles in DKS, but tnerely suggests it cannot ac.t alone in producing the clinical picture. Rec(‘ttt studies using magnetic resonance imaging have attetnp’ed to determine if’ any vertical dis-

placement of’ the horizontal rectus muscles occurs during overshoots. Miller et al tijund no vertical displacement ofthe later-at rectus muscle in an upshoot patient, but fi)und a 2 mnt displacement on a clownshoot patient.“” Rloom and associates recently found a l-2 mm vertical shift ofthe lateral rectus muscle on upshoots or downshoots.” No obvious slippage of the muscle was noted in either study, as would be expected b\ the bridle effect theorv, but, as one ofthr autliot.5 pointed out. maiti&ing it constant fixatiott in ;I clifficult gaze for a prolonged period of‘time ma) 1”)s~ a significant problent in imaging the abnortiiatitv. Surgical findings, cadaver studies and electrop 11y. 40 10 $’ Tic‘2t 1 studies : all seem to point out that the retraction svttdronte, althouglt ~\.ell dcscribed in the literature, is im etititl thal cttcot~ipasses aspects of’ all three theories ot‘ pat hogcnesis. Spcculatiott dictates that patiettts who present with the clinical picture of’onc particular subgroup of‘tlte syndrome (e.g., Hrtbcr’s .l‘ype I) may in titct have various anatomical defects that cause the same clinical picture. DRS is most pi-obably ;I spectrum of mechanical. anatomical and inttervational disorders which WV ttavc classified 2s ;t single clinical entity. B. EMBRYOGENESIS .l‘he t&t that the literature points to l>KS as having frequently associated congenital nnalfi)rmationa ((;oldenhat-‘s syndrome. neurosettsor) deafLess, etc.) may link the disorder to ;m event at ii critical point in etnhryogetiic development. As. discussed by Smith, Iii in 1972 Pfaffenbach et al collected 1X6 cases attd showed that patients with the sporadic fi)rm of DRS wet-e at ;I IO-20 times greater risk for having other congenital malformations.” That same );rar (:ross and Pf%fenbach studied four cases and decided the v;isf ntajority of anomalies associated with Duane’s syndrome could be divided into titul categories: skeletal, auric-ular, ocular and neu1-d. is The primary skeletal abnormalities usuall) involved the palate and vertebral cc)lumn. ‘l-he auricul;tr abnormalities included the external ear, external auditory meatus and semicircular canals. Ocular defects involved the ex;~raocttla~muscles and the eyelids and ittclucied ocular dermaids. Neural abnormalities ittvol~~ed tltr ttuclei of the third, fourth and sixth cranial nerves. It seemed evident that a disturbance betweett the fourth to tenth week of embryogtznesis (.ould cxat~tiortttaliti~s t-eplain the various notiociilat pot-ted in combination with lhtartr’s ‘I!ttdmtrte.

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Surv Ophthalmol

38 (3) November-December

These conclusions were further corroborated by studies on children with thalidomide embryopathy,‘:“,‘:‘H,‘!“’ in which two separate authors reported a total of seven patients with DRS and other associated congenital abnormalities. The teratogenic effect of thalidomide was determined to be between 34 and 39 days after the mother’s last menstrual period. Numerous other authors have agreed with these conclusions in their studies.i:S.iH

Apparently in the patients reported by Pfaffenbach et al”” and others, DRS was only one of many congenital anomalies. Many of the patients in these retrospective studies were being seen for other clinical problems. The studies may be biased in this regard, and the incidence of congenital malformations may not be as significant as stated in the published literature. As most experienced practitioners will attest, as well as the authors of this review, most DRS patients appear to be relatively healthy and free of other congenital defects. Yet these studies are important because they give us a better insight into the syndrome’s embryogenesis. C. HEREDITARY

FACTORS

Genetics as a factor in the development of DRS was postulated as early as 1879 (Heuck),” 1896 (Turk)‘“’ and 190 1 (Varese, Wolff),‘““.“‘X although there was no substantial scientific evidence to support the premise. Turk’s report included three generations of involvement (a father, three daughters, and a grandchild). Familial cases of the syndrome have been reported by many authors in the past century. This incidence has been approximated at 10% by most authors.X’.“” but has been as high as 19%“” and 23%?” and as low as 5%“” It has been postulated that the incidence of familial DRS is probably underreported due to the likelihood that the gene has reduced penetrance and mild or subclinical forms of the syndrome may be missed.‘“’ In a 1969 study Kirkham first noted a genetic link between patients with Duane’s syndrome and deafness.“” The study involved five generations of a family with dominantly inherited perceptive deafness. DRS was found in a girl and her aunt. In 1970 he reported on a series of 112 patients with Duane’s syndrome!“’ Twelve had perceptive deafness and five had Klippel-Feil anomaly (a syndrome consisting of bony deformities of the cervical spine). Evidence of all three disorders were found in the patients’ families. Although these studies did not emphatically prove that there was a high incidence of deafness

1993

DERESPINIS ET AL

in patients with DRS, the findings were interpreted to suggest that all three anomalies could be found in a single gene trait manifested in the heterozygote. The gene was felt to be incompletely penetrant with variable expressivity inherited in an irregularly dominant manner. The study went on to say that the gene was partly sexlimited in such a way that females were more susceptible to the action of the gene. Unidentified environmental factors (e.g., thalidomide)““.“‘X~‘“” were also possibly important in modifying the gene’s effect. Later that same year Kirkham reported on four patients with cleft palate associated with the retraction syndrome and from the study of those patients and their f&nilies concluded that cleft palate, Klippel-Feil anomaly, perceptive deafness and DRS were all manifestations of a pleiotropic gene inherited in an irregularly dominant manner.!‘” Pfdffenbach et al found most cases of DRS to occur sporadically, but in approximately 5%~of the 186 patients studied in his series an autosomal dominant pattern of inheritance was seen.““’ These familial cases had a virtual absence of other anomalies, suggesting a unique form of the disorder, whereas the sporadic cases were frequently associated with other congenital malformations. He postulated that DRS is a heterogeneous disorder with multiple causes. It is interesting to note that the authors of this review have not seen a significant incidence of congenital anomalies in their patients with DRS. Most practitioners will agree that their DRS patients are usually healthy with no significant preponderance of malformations. Congenital hypoplasia of the thenar eminence and DRS (Okihiro syndrome) have been shown in a pedigree over four generations and their association also points to pleiotropic effects of a single gene inherited as an autosomal dominant trait.“” Over the last twenty years numerous studies have indicated a strong argument for the autosomal dominant mode of transmission.“.“O. f~0.65.Y6,‘7?.17R.‘R9

Studies of monozygotic twins have added some confusion to the genetics of DRS. A number of authors have reported twins concordant for DRS,“7.“‘.i“ while others have reported discordant cases.!‘I’.‘.~!‘.L’~~’ Still another study reported monozygotic twins with unilateral DRS, each with the opposite eye affected-or otherwise “mirror images. ““” In the latter case the authors hypothesized the existence of a gene responsible for the retraction syndrome and bilateral involvement. They felt that various internal stimuli during embryogenesis determined uni- or bilat-

DUANE’S RETRACTION

SYNDROME

C‘ifortl ( 1Y”(j)“” I&-khxn [ <970)‘“’ Pfillfelltdl

Ct al

Iscmlmg

et

al

(

(1972)““’

1977y

o’Mallc~ et al (198”)‘:‘-’ Trcdiri Kxll,

( 19tY1)‘~”

85 van Nom-den (IS-Hi)“‘!’

40

23(58%)

126 186” 10 1 97 70 70

82(65%) 106(57%) 58(57%) 60(62(X) “8(40%) 45(64% )

.-\hluwalia et al (19)xX)” l&l et ‘II ( lw~))‘~i” . . IotAi’

“0

1 I(Srk)

71

37(5?%)

835“

48 l(3w)

li(-l2%) 34(35%) HO(43% )

26(65% 76(60% 107(58% 56(55% 54(55(X 36(52% .47(67% 11(55%) x3(54%)

43(33% ) 37(38%) 4360% ) 2.5(:36’%) 9(45%) 34(48’% ) 35 1(42% )

Y+lri; i “2(lXci) 34( 18% ) I 6( 16% ) 17(1#‘%) 17(2+x) 7(10%)

5(1!?%)

“tw?X) 37(“0’%) 29(29c/;) 96(“7% ) 17(2.l%) l(i(“Y’/: ) j(25V ) 17(“4%)

~(“0%) 16fP% ) 147( 18%)

191(23%)

389(59% )

,‘Inti)l-nution

concerning the patient’s sex not available in three patients (6%). laterality was not indicated. II only the unilateral cases are tallied the percentage of’left e\e involven~ent >vould he “Y? /(4H9/6XO c;~scs) and right c! e in~olvcrncnt would he 287~ (1911680 cases). “111 8 c;lscs

et-alit!.

(3%)

sicletliiess

Kii-lJi;~ni

proposed

ii~conipletely sivit\..“”

the gene

pelietrmt with

“Mii-ror

image

of‘ expression. in question

with variable nioiioqyHic

in

eit1ic.r

iroiiniental

(‘ii\

tioii.

degree

that

l>iscx~rtlaiic~

coii~patible ;iIi

a11d

of these

genetic

c;~iise or ;I postzygotic twins”

are txst

wxi

exprestbvins is

close

models.

I‘lie only

muta-

esplainetl

h

the t hol-!~ of’Mrhdorn

and pommel-ell.““~I‘~lel~t i4 also ;I report of :I fimiily With pres~1mabl~ auto~bnial vxcx4ive type retixction syndrotne.” Two 4stc.l.s were affixted with no other fknily n16’111I)~‘13 h;i\,iiig the disoi-der ovei- a period of two represelli gc.iirixtions. J‘liese Iii;iv, of coui-se, 5I)oixlic.

coiiicitlentd

occurrences.

A. INCIDENCE ‘l‘he ti-rc~u~~~!; ot hlane’s syndrome in the gelletA popttlatiotl of strabismus patients has heen estimated to hr I-4%. In a study of 1062 lxitients with motility clisorders in 1939, 12 patients or I .I’% were ti)und to have DRS.“” BeI \~~TII 1949 ;111d 1WiH Kirkham reported 0.84’% of‘ sonic 14,ClOOlxitieiits with stmbisrnus as haviiig I>uaii~‘a syntlrotne.“~’ Ahluwalia and associ:rtes ti)uiid 20 out of‘.WO patients (4%) with nwtilit! p~ohlrms to ha\e DIG.

.\s

tar

hack

DISTRIBUTION ~1s Ihai~e’s

original

of‘ the retraction iioted. ‘l‘he majorit\ of‘ studies l)i.~~loriiiiiaiice ;,t’th;* syndrome

~)~‘~po~l~lei~;~iic~

a female syndr-oine was point to 2%(i()‘A amorig females. article.

together Duane’s

to ;I century

more

ago.

study

topical

tcii major studies trt origirial article fixm

Xmong

38 1 (34%)

tht. disoixlcl-,

wei-e

that

appeal-ccl

finding

was that

Nooderl

in which only 30%

fen1;1le.‘~”

Kirkham

cal way to explain a1n011g

fernales

spoiisihle,

postulated the

M’as

twsicles

\vitIi incoiiiplete limited, cffiYI4

III. Demographic and Epidemiologic Factors

B. SEXUAL

\t’v have grouped I)KS including

the

females

to tlitter

I ).

ti-otn

0f”l‘redici

the

ant1 vote

of‘ the patients

were

that the moat logitht

svidroInc’ the

autosoinal

being

with

(‘l‘;lld~~

of’tlw

suggest

peneti-ance.

making ot.

tiequenq to

X~?J patients female

gt31c

I‘C-

tlomiiimt

walr ~dso partl)~ ses-

mow

su4cq)titdr

;o the

gme.“”

C. LATERALITY ‘l-lie predilection ti)r leti ryv iilvolverrtent has hem cited ill practically all the studies ot’ l)KS owr the last century, J‘he ixmoii for this has not lxvn xlequately t3plained. Xs show~l in ‘I‘;lble I. 489 of’ X35 (59%) retraction s~~id~w~~i~ patients had

lef‘t eye involvement.

~l’\veiltb-thwc

ptTc‘t9~l

~llld IX% 01’I hc ( ;1st’s L FVCI-c bilatel-al. It‘ we elimiiiate lllr l)ilatcxll (at’s and observe only the unilateral cxeh, r\‘c’disco\.c’r that G-49 of‘ 680 (i2%) of‘ the paticilts with uiiilatcral DKS have lefi c’vc iii\~olvcn~rllt. ~I‘tiia 3: 1 ratio shobvs ;I sigllif&mt tenttellc-!, whic~ll should he kept in mind.

had

right

eye involverncnt,

D. RACE Studies 0f‘DKS have been put~lishtd iii vii-tual1~ all areas ofthe worlcl. and 110 particular race 01

266

Surv Ophthalmol

38 (3) November-December

DERESPINIS ET AL

1993 TABLE 3

TABLE 2 Recent Majos Studies of Duane’s Syndrome (1982-l 989) Huher l@es 011Presentation

Investigators/ year O’Malley et al (1g82)l.i” Treclici & von Noorden (1985)“+” Raab ( 1986)14” Ahluwalia et al (1988)” Ro et al (1989)‘“” Totals

No. of Eyes 97

Type I

Type II

84(87%)

1 l( 11%)

Recent Major Studies of Duane’s Syndrome (1977-l 989) Ocular Deviation in PrimaT Gaze (Unilateral and Bilateral Cases) No.

Type III 2(2%)

9(13R) 20(26%)

70 77

56(80R) 56(73%)

5(7’%) l( 1%)

24

11(46%)

5(21%)

72

58f81%)

l(l%)

13(18’%)

340

265(78%)

23(7%‘)

52(15W)

8(33’%)

Investigator/ year Isenberg et al ( 1977)X9 O’Malley et al (1982)‘“” Raab (1985)‘“” Ahluwalia et al (1988)” Ro et al (1989)‘“‘; Totals

ethnic group seems the syndrome.

to have a predisposition

for

E. TYPES OF PRESENTATION Duane’s original description of the syndrome was that of severe limitation of abduction and fairly normal adduction accompanied by retraction. It is not surprising that the most common form of the syndrome is Type I of the Huber classification system. A review of live separate studies over the last decade reveals a three to one preponderance of Type I over the other forms of DRS (Table 2). In four of five of the studies Type II was the least common form of the syndrome. The average occurrence of Type II was 7% among all investigators. F. OCULAR DEVIATION POSITION

IN PRIMARY

Most patients with DRS appear to have strabismus in primary position. Patients may adopt small, relatively unnoticed head positions which may obscure a small angle strabismus in primary gaze. Most skilled clinicians will take this into account when examining a patient with DRS. Esotropia was the most common presenting deviation in primary gaze in the majority of reviewed studies -IO.-17,Xo.“7.l’X.‘:ii.‘Sti.“‘o and it was more common in Duane’s Type 1. Orthotropia was the second most common finding in primary position and in some instances was more frequent than esotropia”.“’ (Table 3). In one study 11 of the 16 exotropias occurred in Type II,‘“” yet in another study nine of the 12 recorded exotropias were in Type III.“” The deviation in primary position when bilateral DRS was present

of Patients

Esotropia

Exotropia

Orthotropia

85”

24(28%)

25(29%)

36(43%)

97h 61’

51(53%) 16(16%) 24(39%/o 13(22%)

30(31%) 24(39%)

24 71d 338

7(29%)

7(29%)

10(42%)

37(52%)

8(11%)

25(35%)

143(42%)

69(20%)

125(37%)

“Bilateral cases (16) were not included in this group. All Type I patients were esotropic. hAll Type I patients were esotropic. 11116 Type II patients were exotropic. ‘Information available on 61 of 70 patients. 50% of unilateral Type I patients were orthophoric and 43%~ were esotropic ‘lone patient (2%) had a hyperdeviation in primary gaze.

was variable and severity

and dependent on the combination of the types involved.

G. REFRACTIVE

ERRORS

Reviews of DRS patients have shown hypermetropia of greater than + 1.50 in 7 1% of the patients (Table 4). Kirkham reported 82% hyperopia among his 90 patients including 26 with + 4.00 to X.00 diopters of correction.‘” Myopia and emmetropia were seen in relatively equal amounts in the studies reviewed ( 15 and 14%, respectively). In the author’s experience the majority of children who undergo retinoscopy with cycloplegics before the age of three will show various degrees of hypermetropia. It is therefore unsurprising that the majority of patients in this age group who are diagnosed with DRS will probably be hypermetropic. It would be interesting to know the ages of the patients diagnosed with DRS in these studies so that we could correlate their refractive errors according to age. Unfortunately, this information is not available. H. AMBLYOPIA Anisometropia of greater than 1 diopter was found in 40%) of the patients in Kirkham’s group in 1970.!” Over half of these patients were amb-

DUANE’S RETRACTION

SYNDROME

No. Patients

Invcstigators/yeat Kirkh,tm L Isenberg O’Malley

( 1970)” et -aI ( 1977)“’ et al (1989)’ ”

~lixxlici & van Noorden ( 1985)‘x” .~ltlitwalia et al (1988)” Ko et L 31 (1489)‘“” . lijtals

(average

%)**

267

Hyperopia No. (‘7)

110 101 97

90(82%),’ 67(66%) N/i\*

72 “0

52(72%) 8(40%)

71

47(66%)

471

“64(71%)

Myopia No. ((2)

Etnmetropia No. (%)

14(13’%) IX(IH’/i) N/A* l.i(?l%)

6(Scic) 16(16%) N/.1*

“r,‘;;

WI,”

10’;:

N: ;\*

IT/;

I fi’?c (’

:v,i

~(“5%)

.‘,(7’:0 7(Wz)

1o’j;

S(S%)

19(17%)

I 7’;;

11’4

14%

L’:)‘&

Si( 15%)

.X(14%)

*Infinmation not available. **\Giyhtcd averages taking into account total number of’ p,itiettta in particular ~‘26 p;;tients were between 4.00 and 8.00 diopters of hyperopia. “.~riisotnctropia of greater than + 1 .OO diopters in sphere, cylindct- or both. 'pauses of atnblvopia were strabismus in 1 1 and anisometropia in 3. “‘I~wel\~e of the &isomett-opes were due to astigmatism. ‘Sis of’ the anisometr-opts were spherical and -4 were astigmatic.

tyopic. ‘t‘he average incidence of anisot~~etropia itt major stttcties rc\+wecl over the last tW0 decactcs M.as 23(/c, although lyreciici and van Noorden ti)uticl this nnmher to be as tow as ?!f .“:‘I O’Mattev et at reported I ‘L of I6 patients to have asti
Ambl~opia ri

group

Anisometropia ‘1

I j’d” Ui.\*

(371).

et]findings into grottps. “’ l‘tie most fiqitentt~~ cx~untered ocular abnormatit~~ \v;ts n\~stagmtts (four cases). fitltowecl b) epibutbar dermoid, ansocot% and ptosis (three cases each). N~stagmtts was also a notable finding in other studies, ‘.“‘.““’ 2s

\Vffl.C

itniscoria;

‘il‘Il.Ii, iii’ ~,tosis

‘~.“‘.111I“I’I;I

epibttlhar clelmoids:“-“,I’” ’ ” “l’.““.“” man) of t tie epihttthar dermoids were acsoc.i;tted with (~otctet~t~at-‘s sylctrome. O’Matley et at fitttntt three ti~ndus cotohomas among his 9’7 patients \\.ith DRS; one of these patients had (~oldetthar’s and

IV. Special Considerations of the Retraction Syndrome A. ASSOCIATED CONGENITAL AND SYNDROMES l‘tte

ANOMALIES

points to frequent associations congenital matformatiot~s and DRs. .\ ntttnher ot‘these studies may be biased, dehttt in cornpat-in, u the stages of etnbtyogenic \~vtopment of‘ vat-ions ocular and t~onocuta~ 4tructttres. it is evidrttt that the majority of’dystttorphogenesis seett in both DRS and Duane’s tx.tatect attot~~alics occur between four and eigltt weeks of gestation. (~l‘abte 5).

;~tnotifi

literature v;trious

1. Associated I%ff&ibach

Ocular and

of’ f)KS and di\ictect

Findings

associates collected their ocular and

1% cases t1ot10CLtt~lt~

Cranial nerves and Nuclei;(kII, IV &T\‘I) Extraocular muscle innervation Pinna and external auditory canal Auditory ossicles and semicircular canals Palate Oral fissure Vertebrae Upper extremity Enteric neuroblast migration Epibular dermoids Renal agenesis Ventricular septal defect *Data from Smith,“’ .+ey,” Mann.“” der.” Jones, “” and Okamoto’~~~~

.’ to 8 4 to 6 6 to x fi to 8

7 to i to -1 to 3 to 7 to 7 4 to 5 to

IO H 8 7 12 :i 6

Duke-El-

268

Surv Ophthalmol

38 (3) November-December

Regenbogen and Stein reported an syndrome.“” optic nerve coloboma in an unusual case of a male with cervico-oculo-acoustic syndrome (Wildervanck’s syndrome).“’ Other findings include Marcus_Gunn jaw_wink,').X'l,'i'i."'~ congenital cata_ ract,:i..~~'.l""

heterochromia,:',"",I.'~'

and

optic

nel-ve

hypoplasia.‘,x,“’ An association of DRS and crocodile tears, or paradoxical-gustatory-lacrimal reflex, has also been reported. It was recognized as far back as 1949 by D’Ermo,“’ and has since been reported “2:5,:17. 127. lil) Crocodile tear by numerous authors. syndrome is frequently due to a misdirection of regenerating nerve fibers during the recovery stage of a seventh nerve palsy. This may be due to an inflammatory or traumatic condition. When it is associated with DRS, central and not peripheral factors have been implicated. Ramsay and Taylor’“” felt the most logical explanation would be a lesion causing a nuclear degeneration or dysgenesis in the immediate vicinity of the sixth nerve nucleus, with the retraction syndrome and crocodile tearing being the result of substitute innervation of the lateral rectus by fibers of the third nerve and the lacrimal gland by fibers subserving salivation. Marcus-Gunn jaw wink, another miswiring syndrome, is probably similar in its pathogenesis and has also been reported with DRS.“,X~.l’“.‘“” Table 6 summarizes ocular DRS.

anomalies

2. Associated

that have been Nonocular

associated

with

Findings

Wildervanck syndrome, also known as the cervice-oculo-acoustic syndrome consists of DRS, the Klippel-Feil anomaly and congenital sensorineural deafness.““’ The Klippel-Feil anomaly consists of a malformation of the cervical vertebrae with possible webbed neck, torticollis and facial asymmetry. The frequency is about 1:42,000 births and 657~ of the patients are female. Deafness occurs in 30%. of the patients.HH Klippel-Feil anomaly and deafness in association with DRS has a male to female ratio of occurrence of 7:75.‘“’ This raises the possibility of a sex-linked dominant mode of transmission with lethality in the homozygous male;“” however, most authors consider this unlikely since a number of affected male patients do exist.““.‘” Klippel-Feil anomaly and perceptive deafness also occur separately with DRS. In a study by Kirkham of 112 DRS patients, 12 had perceptive deafness and five had the Klippel-Feil anomaly.“” Only two patients demonstrated the complete triad known as Wildervanck’s syndrome. There

1993

DERESPINIS ET AL

appears to be a significant overlap among different entities occurring with DRS. Okihiro syndrome is the association of DRS with congenital thenar hypoplasia. This upper limb anomaly is more accurately a defect of the radial ray (radius, scaphoid, trapezium, 1st metacarpal and the two phalanges ofthe thumb), with the thumb and thenar eminence more commonly affected than the proximal structures.“‘” In 1975 Tetamy et al described DRS associated with radial ray dysplasia as an autosomal dominant disorder.“’ In 1977 Okihiro and associates reported a family in which three generations were afflicted with DRS.““’ Four out of five of the family members also had hypoplasia of the thenar eminence. Some family members also had deafness and showed features similar to Wildervanck’s syndrome and the Holt-Oram syndrome. Again an overlap of syndromes and associated congenital malformations appear to be common in patient’s with DKS. In 1985 Hayes and associates reported a child with hypoplasia of the thenar eminence, DRS, deafness and cervical spine abnormalities.“” A number of relatives had a combination of similar findings plus and array of other congenital anomalies. They proposed that the disorder be named the Okihiro syndrome after the earlier describer. -l-he eponym was subsequently used in the literature. ‘x” The other associated anomalies were somehow disregarded and since that time the only two consitutents of the syndrome that remain are DRS and thenar emineJ!ce hypoplasia. Goldenhar’s syndrome (oculo-auriculo-vertebra1 dysplasia) is due to an abnormal morphogenesis of the first and second branchial arches and is classically accompanied by vertebral anomalies, epibulbar dermoids, facial hypoplasia and preauricular skin-tags.xx It has been associated with DRS in the literature.“x.“‘.‘~~~‘~‘.‘!” Goldenhar’s syndrome and the cervico-oculo-acc,ustic syndrome overlap, as can be seen in multiple cases of DRS associated with these entities.“‘.” A number of nonocular anomalies are seen separately or together in a variety of recognized syndromes, including DRS. The Holt-Oram syndrome consists of congenital cardiac anomalies and musculoskeletal anomalies of the upper limb, in particular, hypoplasia of the thumb. A familial occurrence of DRS and the Holt-Oram syndrome was reported by Ferrel et al.” Hypoplasia of the thumb is also one ofthe constituents of the Okihiro syndrome. Awan reported a patient with DRS in association with Klippel-Feil anomaly who also had cardiac malformations

DUANE’S RETRACTION

Associutrd

SYNDROME

Ocular

269

24nomalie.s in Duanr’s

Syndrome (Not Including

Fundus Frequently

(Aobe

reportttf”

Less frequent”

Optic

Infrequent’

Morning glory disc” Situs inversus disc’l” Myelinated nerves14” Staphyloma”’ Persistent hyaloid arterylq4

nerve

Kefractw

hypoplasia

,‘Multiple cases (greater than five) reported “More than one case reported in literature.

Er~-or\)

Ocular

Adnexa

Nystagmus Epibulbar dermoid Anisocoria <:oloboma:“,“:

Ptosis (:rocodilc

tear\

Cataract Heteroc-hromia

M-C; jaw-wink Abnormal lacrimal apparatus

Microphthalmosi44 Keratoconus Spasmus nutans Microcornea”‘s Brown’s syndrome”“” Familial external ophthalmoplegia”

Nevus

of

ora7-z

Ectropion’4’ L~agophthalmos’“’ Homer’s synd.‘“” Prom. cpicanthus Abnormal caruncle’x

in literature.

‘L sually isolated case reports.

reminiscent c’t al reported

ofthe the

Holt-Oram case

syndrome.’

of‘ a deaf

patient

Hayes with

syndrome with elements of Klippel-Feil anomaly.“” Rrik and Athayde reported a case of bilateral DKS associated with the Khppel-Feil anomaly and crocodile tearing.‘” From his studies Kirkham concluded that Klippel-Feil anomaly, cleft palate, DRS and perceptive deafness a-e all manifestations of’ a pleiotropic gene.“” ‘This “o\,erlap phcnomeoli” is reported with more frcquencv as our recognition of DRS and its associ:ited nialfi)rmations has increased. l’able 7 provicles a list of nonocular findings associated with DKS. Sonle findings are much ni0i.e fi-ecluent rhan others and some represent single case reports. Pfdfftnbach studied 1X6 pat ients with DKS and fi)uncl roentgenographic ab~lormalities of the bo11y skeleton in 18%. Vertebral abnormalities (cervical, thoracic, lumbar and sacral) on x-ray were seen in 28 patients, including thoracic scoliosis that was clinicall\ present in seven. 01 her skeletal deformities incl~~tlecl: head deti)rmities (three); facies, including palate (13); extremities and digits, including extremity hypoplasia ( 13); and trunk deformities ill addition to scoliosis.” He also reported congenital central ner\‘ous system disorders (10). congenital skin lesions (13). genitourinar\;tnom;ilies (eight), and cal-diopulmonal~~ aiiomalie4 (three). l’fdk~~lx~l~ also reported congenital ear and atldiometric findings: sensorineural deafness ( 1L’ lxitients); preauricular skin tags (fimr); malOkihiro

finmed pinna (three); hypoplastic external canal (two); and ~IIOII~OUS ossicles (one).“’ I’e~xq~tive deafiless was reported in 1 1’2 of liirkham’s DKS patients”” and in 4% of Ro and asxociates‘.“” Pfaffenbach compared the rates of the various congenital malformations found in his study to those fijund in the general population. ‘l‘he rate5 of musculoskeletal. fi)ot and ear anomalies ill DRS were all 10 to 20 times higher than those fi)uncl in the general population. Among Pfaffenhach’s 1 X6 patients. 62 (33%) had OIW or more anomalies in addition to DRS; 32 (17’% ) had two or more; and 15 (8%) had three 01‘ mar-c congeniIn 67 patients with DRS, Ko tal rnalfilr-mations.““ and associates found the incidence ot’associated c-ongenital malformations to be 1.3(X.“” Pftitftnbath discovered that the more extensive his evauations of DKS patients became. the higher was the incidence (50% ) of associated anomalies. In the general population, Marden et al”‘” and McKeowen and Record “-I found thr incidence of major congenital anomalies to be 2% and 2.3%. rer;ectively. Even with minor anomalies inc.ludrd in the statistics the incidence has Ixw~ fi)und to be between 7.8% and 14.7%.“‘“,“’ It would apa disorder in wtiictl the pear that DRS 1.epresents chance of’ finding other Inalfi)l.m;~tiotl~ may be significant. It seems reasonable to suggest that pleiotropic ef‘tects are being demonstrated in these patients and it may represent ;I sin&l gcnc defect. Unknown environmental factors may also be partly responsible in modifying these effects. Pfaffenbach’s study was important in that it

270

Surv

Ophthalmol

TABLE Non-ocular Affected Central

7

Less Frequent

Frequent system

ET AL

Anomalies Associated with Duane’s Syndrome

Areas

nervous

DERESPINIS

1993

38 (3) November-December

Perceptive

deafness*

Cerebral A-V malformation’@ 7th Nerve palsyltr4 Seizures Retardation

Ext. ear/pinna defects Preauricular tags Cleft palate Asymmetry of facies

Cranial synostosis Microcephaly Microstomia/microsomia Abnormal dentition Hemangiomas of skin High arched palate Iniencephaly

Extremities/musculoskeletal

Vertebral anomalies Thenar hypoplasiat Rib anomalies Foot anomalies

Spina bifida Phocomelia Sprengel deformity Severe radial ray anomalies Muscular dystrophy77 Finger deformities

Viscera

Cardiac

Renal dysplasia Vesiculo-ureteral reflux Hirschsprung’s disease Segmental colon dilation Focal segmental glomerulonephritis’@ Imperforate anus Umbilical hernia Genito-urinary anomalies

Syndromes

Klippel-Feil* Goldenhar’s

Head

and facies

*Components iComponent

anomalies

Holt-Oram Fetal ETOH7” Moebius Marfan’s/Ehler’s

Danlos’“O

of Wildervanck’s syndrome of the Okihiro syndrome

made us aware of the necessity of looking for additional malformations in DRS patients, although from personal experience we usually find DRS patients to be no different from the general populace and relatively free of other congenital anomalies. To the ophthalmologist the designation “Duane’s syndrome” is appropriate in describing the spectrum of eye findings in the disorder. To reduce the syndrome to an anomaly, as was suggested by Temtamy, represents a subjective point of view.lxx It would be more practical to describe the combination of DRS and more than one nonocular malformation as “Duane’s complex.” This would not only serve to describe the complexity of the overlapping malformations but would also indicate the presence ofassociated nonocular components.

B. VARIANTS OF DUANE’S SYNDROME Although exceedingly rare compared to its horizontal counterpart, vertical retraction syndrome has been reported in the literature. Patients exhibit some degree of limitation of the affected eye on elevation or depression associated with globe retraction and narrowing of the palpebral fissure. The eye may be either orthophoric, hypotropic or hypertropic in the primary position. Two cases of this clinical variant first appeared in the Chinese literature in 1935.“” One case was thought to be traumatic and the other to be congenital in origin. Fourteen cases have since been reported, nearly all congenital. Four cases were bilateral, and in unilateral cases the left eye was more frequently involved (7:3). Sexual distribution was equaI.‘x”,“4 Combined horizontal and vertical retraction syndrome has

also hrhen reported iti \vttich the classical horizontal restrictions and retraction plus the vertical c’oll1po1lellts c011ld he ti)und concurrentI\. I IO,,rl’.i’,-c \‘ertial retraction syndrome. like DRS, has been found to o(.cur spontaneously and has also bveti li)und in siblings. Khodadoust and van hoorden described bilateral vertical retraction 5) ndrome in two sisters. I 1 and 13 years of‘age.‘“’ ‘l~hree other siblings were normal. The syttdt-omr has also been linked to both peripheral ;~ttd itinet~vatioiial anomalies. One surgical esploration has revealed an anotnalous superior txactus muscle divided into two muscular bands witlt different scleral insertion sites,“” yet other reports have not revealed any abnolmalities.‘~~ ~\bnornial vertical nio\‘enit’nts have also been linked to slippage c;~used hy the “tethering” vtfeet of’ the horizontal muscles, fibrosis of‘the vertic.al muscles and innervational anomalies fi-om tither supt~attucleat~ or more peripheral origins. 121, all accounts the pathogenesis of both I)RS aittl its vertical counterpart appear to be similar. c\nctt hrt- rare variant of‘ DRS is congenital adduction tleficit with synergistic divergence. vt’~velve C;ISC’Shave been reported in the literaI” 2; OX.‘(‘I ,SII,L’III,.L’,I, ?, ,.l’,i to be a \ariIt appears t Ill-(‘. attt of‘ Huber’s classification Type II. ~l’he erttit! consists of unilateral aclduction deficits and sitiiult;uteoiis abduction of‘the eyes on attempted lateral gaze into the field of action of the apparctttly paretic medial rectus muscle (Fig. 4). Othct fcaturcas that have hrten reported at-e relativeI) large angle esotropias (60 prism diopters ot greater), horizontal face turns away from the in\.oI\~l eve. simultaneous abduction nystagmus oii attenipted adduction (inverse nystagntus), atld tiarrowiti~g of the palebt-al fissure in the afltict~tt eye on attynil)tcd adduction. \\‘ilcos and

associates prrfi)rmrd elc~trotrl~oRt-al,hic studies oit ;I patient \). This tiiay be attata~ous to the rt4ationship of’l‘ype II I to l‘\,lx 1, where ‘I‘ype I I I nia\ bv ;I vari;mt of’ I’yp~~ I (Figs. 3.4 and C).’ “’ I~it’rogetiic- simultaneous ;tbduction. termetl the “0cuL1r splits.” has lq”“‘ttY1 after il lqy Iatel~al IYXY 115 ;tIsc 1 twen rc.st.ctioti in ;i patimt with I)RS :itttl t ttercfi)rc iwec~tiotis arc discor~t~;tgett.”

C. ACQUIRED

RETRACTION

SYNDROME

In 1976 Thomas Duane (not to tw co~itiised ~+iith ;\tesander I)uane), Schatz ai~tl (:;tputo rc‘ported a series of five cases in which the patients had \vhat was termed “pseudo-I)tianr’s ret ix‘tiott ~~tidt-otne.~“~ In all of‘ t!lcW 1‘ascs but one, there \V;IS a historv of orbital trarima with suhscqueti’ tiietiial wall blow-out fracture and entrapment of the medial rectus muscle. 1)iptopia JV;IS also present in lateral gaze. The fifth patient of’ the group had entrapment due to orbital nit’t;istats from ;I breast carcinoma. iVh;tt difkrctttiated these patients fiiom true DRS 14v1.e the historie4 of ot~bit;tl tt-aitm;i, cliplopi;i ;tt1tl the rt’triictiott of globe (rewrse rett2c.tiot~) 011 attcmptcxl abduction. The condition has also becil txqxxted after prostatic (.;trcinoni;i” and after ;I Literal orbitototny (Krottlein pi-ocedurc) due to fibrosis and scat-ring in the \icittit!. of‘ the lateral rect IIS n1us&. ‘7XOther etiologies 0t‘;icquired retrac tioti 5vndrotite at-e listed in ‘l‘;iblc X.

272 DUANE’S

Surv Ophthalmol

38 (3) November-December DUANE’S TYPE

TYPE I

1993 DUANE’S

I I

Fig, 5. Possible peripheral innervatic& variations to explain the rotationa! deviations seen in DKS. Excessive innervation may determine whether ‘I‘ype II presents as usual Type 11 or as synergistic divergence. Type I and Type III may be similarly related. .I‘he medial rectus mu& (MK) and the lateral rectus muscle (LK) of the right eye arc represented. The subnucleus of the third nerve (I I I) and the sixth nerve nucleus (VI) are also shown. The thickness of the arrows represent the amount of innervation to the muscle. Dashed arrows indicate either hypoplasia or absence of the nerve or nucleus. (Adapted and modified from Wilcox LM et al”“’ with permission of the authors and ‘I‘he American Journal of Ophthalmology, copyright The Ophthalmic Publishing Company)

V. Clinical A. GENERAL

DERESPINIS TYPE

SYNERGISTIC

ET AL

111

DIVERGENCE

Features and Diagnosis

APPROACH

TO THE

PATIENT

Duane’s retraction syndrome is characterized by abduction deficiency, a variable limitation of adduction, globe retraction and narrowing of the palpebral fissure on attempted adduction, and oblique elevation or depression on adduction.47 Although these elements are present to some degree in most cases, the clinical presentation may vary,“.““’ and it may be difficult to confirm the diagnosis of DRS in some cases, especially in infancy. A parent or pediatrician might become aware of a strabismus or a limitation of abduction in one or both eyes in early childhood. Strabismus, in general, and, in particular, esotropia, is usually of a small angle in DRS and less easily recognized than the large esotropia typically found in children with infantile esotropia.““’ Sometimes the deficiency in abduction presents as an esotropia

of the contralateral noninvolved eye when the child’s gaze is directed toward the side of the involved eye. Hering’s law of motor correspondence states that whenever an impulse for the performance of an eye movement is sent out, corresponding or “yoke” muscles in each eye receive equal innervation to contract.” For example, on attempted left gaze in a patient with an abduction deficit in the left eye, the right eye adducts excessively and produces an apparent right esotropia. The clinician must demonstrate this finding to the parents and skillfully explain that the problem is not in the eye that they thought.

DUANE’S RETRACTION SYNDROME Parents may notice \xrying degree, when

a horizontal face turn of the child attempts to view

object in what would normally be considered the straight-ahead position. The lid fissure changes may be the sign that indicates to the parents that something is wrong. A pseudo-ptosis may be present with attempted adduction, and the itI\,olved eye may be thought to be smaller because of‘ the enophthalmos caused by the globe retraction. In contrast, the involved eye in abductioII, or the noninvolved eye. may appear to be large1 or proptotic. The cosmetically unacceptable upshoot or downshoot in adduction may also cause the family to seek medical attention. Although DRS is present from birth, it may not be recognizable in the fullest form during intanq;. It is our clinical impression that many infants ~111 present only with an abduction deficit and later on develop the diagnostic signs of adcluc.tion deficit, fissure narrowing and retraction of‘ the globe on adduction.“! We could theorize that these findings become more obvious as the lateral rectus contracts with time and becomes more inelastic or fibrotic. It may also be more difficult to recogni;le siInply because of the problems inherent in examining young children. Photographs of the individual in early childhood may help to confirm the diagnosis when there is contiIsion as to the age of onset of the condition. It is important to discuss with the family and the patient, it‘ he or she is old enough, the congenital, nonthreatening nature of this condition. Occasionally patients with DRS appear in an emergency room following trauma or present to a new, unknowing physician with what appears to be a sixth nerve palsy. A well-informed patient or parent can prevent an extensive neurological work-up by identifying the condition. It is. of’ course. important to distinguish DRS from a new OIlset abducens ner\‘e palsy which may carI-); a mox serious prognosis. B. USE OF CLASSIFICATION

SYSTEMS

4s Inentioned previously, Huber’s classification is most frequently utilized by clinicians and aIItbors in their descriptions of patients with 1)RS.“” We prefer this classification, as it provides ;I mt’ans OF common communication among clinicians. It is importatlt to recognize, as Raab has emphasized, that assignment to one or another subtype of’ DRS is often difficult.“” In particular, the hr~ndary between ‘I‘ypes I and III involves a vnriable subjective decision by the examiner as to the clegree‘of’ limitation of’ adduction. He be1icvcs iI may be pref&Ible to regard J‘ypes I aIl(l

.I‘ABLE 8 I:‘tiologin

of

z4rqztir(TdRetraction

(P~r/cno-I)rtrrlcc’;)

S?;Ml,OW*

Mechanical

(orbit)

Fractures (medial \vall) Bone nletastases (breast and prost;ite) Soft tissue tumors Inflarrimation Surgery

MLlS&

.Thyroid myopathy CoIlgenital fibrous txintls

Conjunctiva

Surgrr)

Neurogenic

Head trauma Surgery on (:~ini;Il ncl vc’ V Brain stem tumor Aberrant regeneration of’ the III nervt’ Vasculitis

*Adapted gan’”

and Inodified

from IiivliII ;md Lunder-

III as reflecting differences in severity rat her than flmdamentally different anomalies. Wagner and associates made a similar argument fi)r patients with simultaneous abduction (synrrgistic divergence) being a form of3‘ype I I 1)KS \vitlI maximal lateral rectus firing on attt9~Ipted adductioII.““’ C. TYPICAL FINDINGS 1. Defects

in Abduction

and Adduction

The abduction deficit in DRS is ust~ally the most prominent finding. The proposed mechaIIisIn for the horizontal abnormal eye n~ovement disorders has been discussed at length iII another section. Frequently the degree of‘ the ;rl~tluctiorI deficit seems out of’ proportion to thus I.ecognizable strabismus. This mav be helpfI11 in tlift&xxItiating DRS fi-om an abducena nc’I ye lxIl\y. in which there is usually a large esotropia. \l’cmg and associates found that esotropic p;ItielIIs with DRS had a primary deviation ot’ 30 prism and Lirist in IheiIdiopters or less.““’ Isenberg stucly of’ 101 patients with DRS fi)u~~d the greatest deviation in any of‘their esotropic. paticlIts to be 1ti degrees by Hirschberg test .” ~l‘h~ Ixxon for the smaller angle of strabismus is most likei), related to the adduction deficit w1Iic.h is fi)uIIcl in all cases of DKS, although in \aI-\ing degrees. The adduction deficit is best clc~IIonstI.atc’cI 11) attempting to measure the ne;iI. poiIIl ot’cx)rlveI~gcnce. .,\I1 DRS patients stem to II:Ivtb ;I rcmotc or Ix-cc_ssecl near poiilt of‘coI~\‘cr~~II~~t’, It III;i\’ ;Ilso

274

Surv Ophthalmol

be demonstrated the gaze opposite adduction. 2. Esotropia

38 (3) November-December

by measuring the affected

the exotropia in eye or toward the

and Exotropia

Although many patients phoric in primary position,

with DRS are orthoexotropia in straight-

ahead position also occurs. Esotropia, however, is the most commonly diagnosed type of strabismus. (Table 3). This reflects the increased frequency of DRS Type I, in which esotropia would be expected to be most common. In order to accurately measure the angle of the deviation in patients with DRS, it is imperative to control the fixating eye during alternate cover The secondary deviation is measurements. greater than the primary deviation, as in other paretic muscle disorders. It is also important to control the head position. The drive for maintaining binocularity is strong and patients will seek their preferred head position during the examination, often resulting in positional variability of strabismus measurements. The head must be stabilized in “forced” straight-ahead gaze in order to measure the strabismus in primary position. When measured using this technique, the deviation in primary position will usually be larger than originally thought by the examiner. Perhaps the most useful maneuver in diagnosing patients with DRS is the measurement of the strabismus in lateral gaze to the opposite side. Since these patients have an adduction deficit, an exotropia will be elicited when alternate cover measurement is performed in this position. In contrast there will be a large esotropia in gaze directed to the involved side in patients classified as Type I. There will therefore be a position where no strabismus is elicited. In those patients where the adduction deficit balances the abduction deficit, there may be orthophoriiin primary position. Usually the deficits are not in balance, however, and the predominant deficiency, abduction or adduction, will determine not only the primary deviation but also the type of DRS classified. 3. Upshoots

DERESPINIS ET AL

1993

and

Downshoots

The characteristic vertical deviations seen in DRS include the overelevation and depression seen in the adducted position. In contrast to typical overaction of the superior oblique and inferior oblique muscles, these ductions are often exaggerated and, as von Noorden points out, are of almost grotesque cosmetic proportion, often

Fig. 6. Significant

globe retraclid fissure narrowing, tion and upshoot of the right eye. This is the same patient shown in Fig. 2 with Type II DKS.

causing the cornea to disappear from view (Fig. 6).‘!” The associated lid changes and retraction of the globe help to distinguish these vertical movements from true oblique muscle overaction. An individual patient may have an upshoot, downshoot, or both. In fact, the position of the eye in primary position may determine which occurs in an individual patient. The eye in DRS does not usually upshoot or downshoot when the eye is adducted in the horizontal position. It occurs when the eye begins to move up or down in the adducted position, and then a “flipping” up or down movement is noted.‘“’ This abnormal movement had been postulated to be related to a slipping of the lateral rectus muscle over the globe, as has been previously discussed.‘“’ In those instances where the affected eye does upshoot or downshoot in the horizontal position it may be possibly due to the co-innervation of the vertical rectus muscles with the lateral rectus. It is not uncommon to find cases where the affected eye is significantly hypertropic or hypotropic in primary position, even before the eye reaches the adducted position. It can also occur due to the lateral rectus side-slip along.““,“‘” According to this side-slip theory, if the eye in primary position is slightly elevated, an upshoot will usually result when adduction is attempted. In contrast, a downshoot can occur if the eye is already gazing inferiorly when adduction is attempted.“” This clinical finding would lend credence to the previously discussed bridle or tethering theory for vertical movements. 4. A and V Patterns Various

types

of A and

V patterns

may

be

DUANE’S RETRACTION

SYNDROME

fi)uncl in DRS. The L’ pattern is more common. but A patterns also o(xur. These most frequentI>. manifest as an exotropia in up or down gaze. Some patients will have an X pattern if they have both an upshoot and downshoot. Interestingly, patients with bilateral DKS tend to have A patterns more frequently then V.“” It has been suggested that ifone finds a patient with DRS and aI A pattern, one should look carefully for findings of’ this syndrome in the other eye. 5. Eyelid Changes

and Globe Retraction

fhe eyelid changes fi)und in DRS in adduction related to a dropping of’ the upper lid and elevation ofthe lower lid in nlany cases.” In lsenberg and Lirist’s’ study, 52% of’ patients showed changes in both lids. while 18% had a dropped upper lid only and 2 I% had an elevated upper lid 0111~~.~” In 7% of their patients there were no eyelid changes in adduction, and therefore no narrowing of the palpebral fissure. The lower lid c.haiiges are associated with a change in the corb tour of’ the eyelid fi-om concave to horizontall) straight with’ the lacrimal puncta prominentl~~ pointing superiorly. ‘I‘he rayelid changes seem to involve more tllan just a passive narrowing horn the retraction of. thr. globe. which results from the cocontracting estraocular ~nuscles. 130th the lid narrowing antl glolw retraction :11-e clfien the most pronounc.ed componentb ot DKS 1l;ig. fi). we

attributed to maintenance of’ straight e\‘es and fusion in some position other than primal’y, Kaab found that 35% of primary position orthotropic patients nevertheless had face turns.“” lie suggested that another cause of‘face turns is to avoid the visual amloyance of’ an anon~;~lous vertical mo\~ement. However, anomalous vertical movements occurred with similar fi-equencv in the orttlotropic patients who either did or did not have a face turn. While face turns are most ofiell related to the maintenance of’ fusion, other les:, obvious influences on the patient’s orclinCir!, visual conifi)rt ma); play :I role in their development. 7. Bilateral Cases Patients with signs of’ DRS in both e\‘es are less fixquent than unilateral cases.‘~*‘~~“” Four of’ Kaab’s seven bilateral cases showed the same type of l)KS (I and I) and three showed difkrcnt tylxs in each eye (I and II I).“” Isenhtrg and Urist had 16 bilateral cases out of’ 101:” sowi had .4 patterns and six had 1’ patterns. 111 Duane’s original report, five of hi4 34 casts were bilateral.” In our experience. l&v of these bilateral c-aaes require strabismus surgeI-) li)r ;I hori0)ntal deviation, although a significant head posture may necessitate strahiamus su~ym. OII 0’L.I “l’.,.hC the lixating eye to correct the torticollis. fissurt cosnretic defect of the globe retrac-lion. narrowing and xcrtical c’vc mo\‘c’mc’nt~ ~‘811be quite significant in these bilateral c;l\c ;111tlslurgical ilitcrvention Ina!. Ix n~ct’5s;In (Fig. 7).

6. Torticollis 1;ac.eturn

is a major characteristic of DRS. Almost all patients with esotropia or esotropia ill DRS were fimnd to have a horizontal head turn in Isenberg and Urist’s’ series.“” Most patients in Kaah’s series had face turns als~.‘~“The f>ice position is ;I mechanism of’ maintaining single binocular \Gion and even bifoveal fixation in man\ cases. “” The face turn is useful ti>r neutralization of.1 hr efftct of the underacting muscle. Patients turn thc4r heads to align the eyes while viewing objects directly ill ti.ont of‘ them. The face is turned in the direction of’the horizontal muscle kt,ith thcx greatest deficit. This keeps the eve farthest fi-om the field of‘action of‘the “paretic” mmclr. For esa~nple, an esotropic patient with DRS who has a left medial rectus muscle underacting 11101x- ttlan the left lateral rectus would turn his head to the right to look straight ahead. A patient with the more common Type I DRS in the left eye would utilize a face turn toward the left if an esotropia is present in primary position. .Althollgh head positions in DRS are usually

D. SENSORY

FINDINGS

As previousl!. mentioned, maIiy patients with DKS adopt ;I heacl turn to niaint;Gn single billocular \,ision. C:omplaints of diplopia arc lxre, and

it has been

suggested

that

thy wco11(1 iIn+y

is ignored rather than suppressed. iI1 \iew ofthe difficulties encountered in plotting ~upprtxioti scotomas in such patients. “” I”‘,’ It is 0111 r~linic;il experience that patients (x1 be Inad~ ;i\vare of diplopia bvlieli questiolled, but do tclld to ignore this serlsation. .-\s expected, ma11!. of tlresc. IXtients ha\~ full stereopsis ivhen tested. E. AMBLYOPIA 111the past

much

the high prevalence tropia in patients tend to contradict cowc~I4w~s

has ken

plxwt OII

of anibl?-opia ;rlld anisomewith DIG.” Kecent studies this. Of ‘Ififi cases hlauro mtl

11ad 0111~ two an1l,lyopcs

aliisornetl.opes. prosiniates 1‘redic.i

emphasis

“I The

plx%ileIlcY

Alld

0111~

of‘ these

that in the Iio~mial p)opiIliitiotI. and VOII Noo~de~l found ;I I W

1”

ap-

inci-

276

Surv Opttthalmol

38 (3) November-December

Fig. 7. Bilateral

downshoots

DKS. Rilateral lid fissure narrowing can also be seen.

dencc of anisometropia in 72 patients and only per3%~ with amblyopia (Table 4). “” Fifty-eight cent of their patients were strabismic in primary position, but almost all of these compensated for their misalignment by the use of a head turn. In this manner, they were able to achieve single binocular vision. Therefore, in the absence of a significant anisometropia, it is not surprising that only one of these patients developed strabismic amblyopia.

VI. Diagnostic A. DIFFERENTIAL 1. Abducens

Dilemmas

DIAGNOSIS

Nerve Palsy

In contrast to DRS, patients with abducens nerve palsy have normal adduction and preservation of convergence. A definite onset may be documented by a new esotropia with diplopia. The esotropia is not associated with globe retraction, vertical upshoots or downshoots. In infancy it may be quite difficult to distinguish DRS from a sixth nerve palsy. “!I An entity known as benign

DERESPINIS ET AL

1993

and globe retraction

are present.

Upshoots and significant

transient sixth nerve palsy may occur in childhood. These children also present with a new onset esotropia, diplopia and a face turn toward the side of the abduction deficit. The acute onset and resolution in approximately six weeks following a viral illness help to differentiate this condition from other more serious conditions of sixth nerve palsy.“” It is important to consider a prompt neurologic work-up when the etiology of the abduction deficit is uncertain. 2. Moebius

Syndrome

Moebius syndrome or congenital diplegia is characterized by sixth and seventh crania1 nerve palsies.‘!’ Motility disturbances reported in Moebius syndrome may include a unilateral or bilateral limitation of abduction, adduction and convergence. The mask-like facies, and associated abnormalities of the limb chest and tongue help to distinguish this condition from DRS. Furthermore, patients with Moebius syndrome would not be expected to have the retraction of

DUANE’S RETRACTION

“77

SYNDROME

the globe and vertical upshoots and downshoots typical of DRS. Differentiation from cases of bilateral DRS may be diffLzult, however. 3. Congenital

Oculomotor

Apraxia

(:hildren with congenital oculomotor apraxia have an impairment of voluntary horizontal game, alld may mimic a bilateral DKS.““They can bc distinguished by their characteristic jerk) horizontal headthrusts. These children have ~~~rnlal vertical eye movements and full retcntion of random eye movements. Gradual improvement occurs with time..” 4. Congenital (:hildren

or Infantile

with congenital

esotropia

may cross-fisate and appear lo have a clinical abduc.tion deficit. Patching one eye for a brief’ period of‘ time should help the examiner to determine if’ a true abduction deficit exists. With the patch on. the child with congenital esotropia will abduct ti~lly in time. A skillful examiner can rotate the child in tlica horizontal plane to elicit filth rotations ill infancy in most cases. (:hildren wirh congenital esotropia generally have much larger deviarions than are found in patients with 1)KS. ’ ‘I’ B. MODES

OF TESTING

In most classic presentations of DRS observatioll is the only requirement in making the diagnosis. Since many patients with DRS present with orthophoria in the primary position it is important IO pertimn due-Cons, versions and cover testing ill dillerent gazes. (Jobe retraction may be acc.tlratc*l\. measured using an exophthalmometer. although this is tliffcult in young children. FOI those cases which tend to be less routine, other useful diagnostic tests exist. 1. Forced Ductions or Force Generation

testing.

duction testing can frequently distinguish between ii loligstanding process in which fibrosis or tightness of’ the muscle has occurred vers~ls a recent disorder (e.g.. sixth nerve palsy, In a recent sixth nerve palmvasthenia gravis).“” sy’thc globe cannoT be voluntarily rotated in abduc,tion. although it can be easily rotated with a ti)rc~ps under topical anesthesia. In typical DRS, the involved medial rectus muscle is usually ti~~ntl to by tight anti this is evident on lateral rotation during tijrced ductions. Restrictive processcks such as thyroid ophthalmopathy and orbital ti-actures ills0 give positive forced duction te41\. FOIW~L

syndrome”) the lateral rectus muscle generates a normal amount of force. Unfortunately, these two testing methods are difficult, if not impossible, to perform in young children. 2. Saccadic Velocity

Esotropia

or infantile

Topical anesthesia is also used in ii)rce getleration testing. The examiner assesses the amount offorce the patient creates to rotate the eye latet-ally as the lateral limbus is grasped with ;I toothed hjrceps. In VI‘ypes I and I11 DRS and sixth nerve palsies the lateral rcctus muscle dots not generate a normal aniount ot’ fi~rcc. In a rcstrictive process such as a medial wall fracture (as is alluded to in the discussion of’~~sc~~~to-l>r~~~r~e’s

Testing

Saccadic eye movement velocity apptm3 IO tx directly proportional to the ac-live tijrce ot‘a I ectub musc~le.‘“” It may be determined quillilatively, or quantitatively using electro-oculogra~hy with carefully placed skin electrodes to record sacc;idie amplitudes. A normally functioning rc’ct us muscle will record rapid saccatlrs, whereas a weakened muscle will record reduc.ed saccatlic. velocity. DKS Types I and I I I rectl retlucctl abductIon speeds. ‘I!’ ‘I-he cause ti,r rctl~~crcl adduc.tion saccadic speecls in I>KS is paradoxical illner\,ation of‘ the lateral reel IIS nlusc.lt* on aAlthough tempted adduction.“‘” ing, an EO(G can be performed, dren.

time-consumeven on chil-

3. Electromyography ‘l‘he historical importance of’ E:M(; ill tlctcrmining the innervational anomalies of DKS should be underscored. It can bc’ ;I usefill diagnostic test in difficult cases. Because it requires the t)lacenlellt of electrodes and tile use of’ local anesthesia. elccrt.orn~ogt-aloha can he ust%tl 0111) in estrcmelv cooperative children and adults. 4. Hess-Lancaster

Screen

-[‘tic 11seof tlw I-less-LAIIGIS~~I- SCWC’IIhas decreased in tiequency in recent yrara. TI‘his is unftirtunatt., as it is an excellent test to dctineate difficult restrictive processes, in particular-. alypical and bilateral DKS cases. In DKS ttlcre :~IY often subtle limitations of ocular motilit\. which at-e superimposed on the obvious ones. XIan\ authors have been stronXg proponents of the use of’ the Hess-I.ancaster screen in their t%valrlation <,f- I)KS.5l.l”’ IIS C;f~\f’S/,(C/~: .\ seven-vear-old fenlalc prc5etItecl with W/Z5 Snrllen acu’ity in each eye with 10 WCS of’ stereoacuitv. A moderate right tiice turn was noted along with limited abdriction in her leti

278

Surv Ophthalmol

Fig. 8. 7‘0j: test revealed left lateral performed. tion of the

38 (3) November-December

1993

DERESPINIS

ET AL

Pre-operative Hess-Lancaster screens in ;I child with an apparent abduction deficit of the left eye. The a left adduction deficit instead with a conlpcnsatory overaction of the right lateral rccms mmde. ~I‘hc rectus muscle was thought to be excessively innervated. A 7 mm left lateral r-ectus recession was Bottom: Post-operative screen with improved adduction in the left cyc with less compensatory ovcracright lateral rectus. The child was orthophoric in primary position.

eye. Her deviation measured 35 prism diopters (pd) of left exotropia (LX?‘) in primary position, 35 pd of LXT in right gaze and 8 pd of exophoria in left gaze. There were upshoots and down-

amount of abduction deficit in the affected The Hess-Lancaster screen clearly revealed

shoots

the

present

in the

left eye

in adduction.

A

Hess-Lancaster screen was performed while the child’s head was held firmly in primary position. It revealed marked limitation to adduction in the left eye (Fig. 8, top). The test clearly showed that clinical evaluation alone can be misleading in the evaluation of DRS.

In this case have

been

adduction

an inexperienced

misled

deficit

by

the

was

clinician

may

disproportionate

more

eye. that

pronounced.

Since a LXT was manifest, we can presume that the overacting muscle was the left lateral rectus muscle. The patient subsequently underwent a 7 mm left lateral rectus muscle recession and postoperatively was orthophoric in primary position. A repeat Hess-Lancaster screen revealed im-

DUANE’S RETRACTION SYNDROME

“79

proved adduction in the left eye with more limitation in abduction. The right eye revealed overaction of the medial rectus muscle, but less compensatory overaction of the lateral rectus (Fig. 8, bottom). ~I‘he Hess-Lancaster screen can be a useful adjunct in the evaluation of paralytic strabismus, but it should he borne in mind that it only indicates the subjective angle of strabismus and cmnot he used in patients with abnormal retinal coi-i-espondrnce.

VII. Therapeutic Modalities A. GOALS OF TREATMENT

It has been accepted by most pediatric ophthalmologists that the positive indications of surgery in DRS are as firllows: 1) a noticeable horizontal ocular-deviation; and 2) an abnormal head l~oSitjoll~i”~~C~c’~ll’~.i~~~~l~i “I” Relative indications for surgery. in DRS are: 1) marked retraction of the globe on attempting adduction; and 2) a cosmetically unacceptable downshoot and/or upshoot of the eye in ilcldllctioil.i”.(lll Besides reducing or eliminating the face turn and tropia, Molarte proposed that additional important goals of surg-c~y. in patients with DRS sl~oulcl be to center a~icl enlarge the binocular diplopia-free held and to iucrease the rotational movement of the abduction in the atfectecl eye.‘“’ For this reason, v,erticxl rectus muscle transposition surgery foi DKS m;~\’ be useful.“” “’ B. SURGICAL

PROCEDURES

1. Horizontal

Surgery

-

Recession

Versus

Resection

V’arious surgical approaches have been advoeliminate face turn and tropia. LJnilateral ;iiid bilateral medial rectus muscle recessions arc perli~rmetl for DKS with esotropia.““,““.“‘!‘,‘~’ Rer.essiou of the lateral rectus muscle”“~“” and E‘atlen posterior fixation sut~~re”~““~““~‘!” have becsn used for DKS with esotropia.“‘.“” Vertical ret 111s ~nuscle transposition alone‘” and combiued with a medial rec‘tus recession for the management of‘ DRS has been reported.““,“.‘.“~‘,‘~~ .I‘he muscle recession was first proposed by In his paper of 1905 and has been used Ihlall~‘~ IL’ extensi\,ely with success. I.liLY’I.I!%.I:~‘?.? Recession of the tight and relatively overacting muscle is a simple and effective operation with minimal I 3,..ll’.li.l,l~.ll’ll.l!ll~ Although simple re
c;~tetl to

risking reduced vertical tropias (breaking fitsional ability) and rarely o\‘erc(~rrects.‘L’,‘~i l‘he appropriate muscle to recess is indicated by the eye with the most marked limitation in adduction or abduction and by whether an esotropia or esotropia is present in primary position. If’ au esotropia is present, the medial rertus of the eve the limitation of‘ abduction is rewith cesscd. “.fi:‘.Iz”.l-I’ If‘ the*-e is ;i limitation of‘ aciduc._ tion and an exotropia present, the lateral rectus of’the eve \vith limitation of the adduction is recessed. In many cases the unilateral niecIial rectus inusc~le recession can eliminate esotropia in primary, position. ‘-IT It has been suggested that a recession of the contralateral medial rect us muscle should also he recessed in certain situations.“” a biIf the deviation is ovei 20 prism diopters. lateral medial rectus recession should he c-onsideretl. A bilateral medial rectus ~nusck rccessiou should also be performed in DRS with esotropia over 20 prism tliopters and limited aclduction and \:er\. slow saccadic velocitv of‘ adduction in the affected eye. Large medial rectus muscle recessions, up to 6 or 7 mm, on the affected eyes have been recommended fix those patients.“‘.’ ” Hovvever, the above procedure ma!’ C;IIISC an overcorrection with synergistic divergence.“” it was also reported that a number of such patients showed markedly reduced saccadic velocities in adduction caused by marked lateral rectus cotiring on addriction.““,“” According to I lcring’s lava, the recession of the medial rec‘tus of the fellow eye by 6 mm or more has heen suggested to help stabilize the surgical result. “.‘I” It‘thc esotropit deviation is in the 20 to 30 prism diopter range and a marked retraction is present, bilateral medial rectus and ipsilateral lateral rectus muscles should be recessed to correct hot11 the esotropia and the retraction.“” (;obin”,’ preferred a recession of’medial rect us to a resection of‘the lateral rectus of‘the eye with DRS. because a recession creates less risk of an enophthalmos and it is less traumatic than a resection, thus decreasing the risk of‘ adhesions. &bin operated on 67 cases and found that onl! eight cases showed limitation in extreme deprcssion. In 4-1of 67 cases the ahductiou was limited (forced duction = positive), but became normal after the recession of the medial rertus. ‘l-his illdicated that the elasticitv of the medial rcctrts is restricted in most cases and this is a major reason for recessing the muscle. They assumed that the fibrosis of the media rectus may be ;I primary anatomical anomaly. I‘he medial rectus muscle should be reattached at the place where it comes

280

Surv Ophthalmol

38 (3) November-December

to rest when the eye is fully abducted.’ Also in Gobin’s study, “” abnormal bands or insertions that had an unusual shape and localization or fibrotic check-ligaments were found in 23 of their 67 cases. When fibrous, episcleral bands are present, they can function as leashes and cause limitation of adduction and marked retraction. Studies have shown the importance of combining the release of a tight lateral rectus muscle with dissection of fascial bands to achieve favorable surgical results.““.‘“‘” Pressman and Scott”’ reported 19 patients who had DRS and underwent surgical treatment. Those patients were treated with appropriate horizontal muscle recession in order to relieve an abnormal head position or a significant tropia in primary position. The success rate in eliminating the abnormal head position was 79%, while 100% were significantly improved. No overcorrections occurred, despite large recessions. Retraction in adduction was reduced in each case. Motility of the eye was not increased following recession and in no case was fusion ability or stereopsis affected. The elimination of abnormal head positions appears to be stable for periods up to 8.75 years. postoperatively, patients obtained approximately 30 degrees of binocular visual field including primary position.“’ One of us (ARC) has performed approximately 120 recessions of the horizontal recti in patients with DRS (Types I and II). Although the motility of those eyes was not normalized, abnormal head positions and tropias were relieved in the majority of the patients. Retraction and overshoots in adduction were significantly improved in most of his patients after the recession of the horizontal rectus muscles. For those patients with exotropia, a weakening of the ipsilateral lateral rectus should be performed.““~‘“‘~“‘7~‘7” This procedure should relieve exotropia, retraction, and abnormal head position. If the exotropia is over 25 prism diopters, consideration should be given to including a recession of the contralateral lateral rectus muscle.“!’ A resection of a fibrotic lateral rectus in DRS would increase the retraction of the eye and result in a gross limitation on adduction or even in a fixation of the eye in abuction.“” Kaufman et al’” reported six patients who underwent recession and resection operations. Two of those had good results and four had inadequate results.‘” Recession-resection procedures may also correct the deviation in primary position; however, two studies have shown that this method improves

1993

DERESPINIS

ET AL

abduction to a lesser degree and limits adduction to a greater extent.“x.‘x’ Resection of either the medial or lateral rectus will not be of a significant benefit in aligning the eyes in DRS and will worsen its features. With lateral rectus resection, the retraction is often increased and marked limitation of adduction is produced with little gain in abduction. Resection in DRS produces little improvement in the abnormal head position and markedly reduces the motility, while increasing upshoots and co-contraction. They should be ~vo~~~~~‘“~~~~~‘“O~“”

2.

Transposition

Procedures

Transposition of the superior and inferior rectus muscles toward the lateral rectus has been advocated and used for a number of years.17,““, “‘Lo’.“’ This procedure has been reported to increase abduction, although adduction is usually sacrificed to some degree.““,” Transposition procedures will theoretically increase the restriction on adduction and increase the tendency for upshoots and worsen the co-contraction.“‘!‘Another problem with this procedure is that it causes an inducement of vertical tropia in up to 10% of the patients, producing diplopia and a poor reSult,:ll.li’ There is also increased risk of anterior segment ischemia when three or four muscles are operated on in one eye. It has been reported that results of transposition procedures can be disappointing and sometimes disastrous, because DRS is essentially of an innervational nature with co-contraction of the lateral and medial rectus muscles.“‘” Success with transposition procedures has varied between 50 and 75% in eliminating the abnormal head position. Ii’ Failures and overcorrections of approximately 30% have been reported using different transposition procedures.‘7 Transposition surgery has been reported in patients with DRS, resulting in large amounts of abduction postoperatively.“’ Full abduction is usually not obtained, but lo-15 degrees of abduction may be achieved.“” deDecker reported a Kestenbaum transposition operation for treatment of the DRS Type 1 retraction syndrome.“” Twelve cases were treated with the Kestenbaum procedure. Three cases showed fair results, and another nine patients were not able to abduct postoperatively. He suggested that one should operate 4 x 6 mm on a 20 degree head turn and 4 x 4 mm on a 10 degree head turn. Surgery should be limited to recession of the medial rectus muscle on a turn of less than 10 degrees.”

DUANE’S

RETRACTION

SYNDROME

2x1

Molarte arid Kosenhaum reported 13 patients wirh ?‘~,pe 1 DRS who all underwent full vertical wctus muscle transpositions.“” Prior to surgery, all patients had esotropia in the primary position and I 1 patients had ;i face turn. Postopertively. esotropia was improved in ten. The face turn was improved in all of’the patients arid eliminated in 73’% (8/ 1 1). Abduction ability was increased and the. binocular diplopia-free field size enlarged to a mean of 60 degrees. Six patients (WX) with ;I me;111 J)reoJ~erative deviation ot‘ 30 prism diopters required an additional medial rectus rect5sion~several months later. Two patients (15% ) cl~~elopecl ii \~ertical deviation following vertical rt7t1is ~nuscle ti.ansJ)c,sitions.‘~’ I:t’rctis et al reportrd l-1 patients with esotroJli;r who under\zent recession of‘ the medial rettus mu&2 and transpositions of‘ both vertical rt’c trls muscles to the insertion of the lateral rettlls lllu\cles.~~’ .A corrt’ction of nearly 16 prism dioJ>ters in the primai-v position was achieved h, rcc,essinq < the medial rectus muscles ant1 transposing [lie vertical recliis muscles to the insertion Of the 1,1ter:11 rectus miiscleh. In all their cases, thv!, \ucvzetletl in improving ahtluction by using tllc Iransl~osition J~ro1~edure. especially duriiig ahtluction VF’Cniow~iimt.~‘~ 3. Posterior

Fixation Sutures

I’osterior lisation suture (Faclen) procedure has also been ;1dvocatt~d for treatment of‘ DRS in conjiinc.tion \vitli recession of‘ the appropriitte lic~*‘i7c,ntal rectus.““’ Experience with this technicfuv is limited.“’ Faderi operation on the contr;daterd medial recttls muscle in order to correct the latrral incomitalce did not ~111)~ impI-cssi1.e results. Eis~nhaum and Parks rcportcd perti)rniing the Jx)sterior fixation suture on vertical ;md liorizontal muscles in patients with DRS.” I’os(crior fixarion suture on the lateral reccl15 niuscle l~rodiiccd satisfactory results with elimiiia~ion of‘ the upshoot. flcn~w~er-, posterior fixation sutllrv on the \w$cal rcctus n~uscle did Iiot c-liaiigc- [lie al~normal e1.e movement. 4. Approach

to Upshoots,

Downshoots,

A and V

Patterns I\losr surgical ~~~~occdur-es toda). are designed to caliniinate the esoCropia and face turn and do not necessarily acldrtss the upshoot or clockssh(,(,t.“” The upshoot or clownshoot in DRS can TV ;1s cc~snierically distracting as the abnormal head posture. ‘I‘he Inechanical cause li)r upshoots ~ntl downshoots has been attrihutec! to the xitlc-sliJ, of‘the tight lateral rectus mllsclr 21s

the adducted globe moves ahovc or hcIow the .I‘his ll;1s t,ec*l te,.nletl horizontal plane. ‘ii.\iT.“l”.I!‘X a “l,l.idlc” ef‘fect,“‘.“” ElC(_lroniyogi-;il)hi(. studies in Duane’s syndrome have denionstr:itcd the paradoxical innervation of‘ the latr~rxl rcct11s on adduction. .I’his ahnormal innervation prodt~c~s ;i taut lateral rectus niusclc when it slioultl tx relaxing. As the coritracture incrc;isc3, the’ latc.ral rectus c;m slip over the glohc NICI protlucx~ IIJF shoots 2nd downslloots on adduc~lion.“~ ‘1 hi-w diff~reiit owrshoots can occur I ) iii straight xlduct ion ;uid in atld1iction-a~icl-~l~~~~itioii. 1 ausilig an upshoot with ;I V Jxirtern; 2) iti acIdu~~tio~i 2nd in atl~lu~tiori-ariCl-~l~J~~~~ssio~i, causilig ;I do~.nshoot \vith ii A JxitMx; antI 3) ;I c~orli~~iri;ilio~1 of’ tlir two, resulting iii an X f)attc.rtl.“” ~l‘he iipslioots alid clownshoots c;iu4~d by sideslip of tlie lateral rectiis an tx. iniJ)ro\,ecl in SC\eral wa!‘s. (hlv approach is to i‘t~c’ss rhc latcr;il rectus muscle. ~I‘lie amount of’ia e\sioti is tlecermined I)) tllc stiffiiess 01 (lit lillCl~~lI I‘t’CtIlS 011 fi)rcecl clric.tion test and whether or iiot tlic. muscle is fbund to he fibrotic on ex1minatio~l d1iring surgcr)-. Recessing ;I \‘t‘ry stiff’. fihro(ic, innxle 7 to X mm 1x11 significantl!. I-~1111~~[lit 0~~e13hoc~ts. A nonfil~rotic lateral rcctiis with rililtll!~ J,o5iti\x fi)rc~l tlric tioiis wo111d he recessetl ii~orc’. at Icasl 1O-1 2 mm, to ;rcliie\,e the wiic‘ goal.“” :\I) ;iltt3n;iti\,c siirgical ap~~ro;icli fi)r 11~~slioots itic~liitlcs JxMerior fixation (I~aclen) sutures 0i1 [tic* IatUi-al rectus miiscle, with or without sinliI;ri- siitrircs on tlic ipsil;iteral mrcli~il rec‘tus rii~id~~, ;~lorig wit11 appropriate recx3sion of‘ 1lie niusc~le. ““’ I”’ .A \veakenillg of’ ;I vertical rectum:, nlu~.lc. ~1n relieve an upshoot if‘there is ;I primaril\. innt*rv;1tional miise.“” I‘lie latter is intlicatccl I,),‘:1 gr;idii~iI mo\‘enit’nt of‘ ~lcvatiori or tlepi~c5sioii ;1iitl/or- 2 Iat-gc \.ertical tropi;r in the priniar! l~osition. 011~ cae was reported in which ;I vc~rlic.;il l-t’1tris ixxc’ssion iml~roved retrxtioii ivitti ;11i ~~pslioot.‘~” .Ilie ;iiithors suhsecluently frlt tlli. pi-0~~~1lrllY c~~iltl Jfo5siblv ind1ic.e ve1-ticxl irnft;ii;iticc* in the c~ollltl llOl primarv Jx~sit’ic~n. ancl tlle\~, tllcwfcbrc.. ~w~o~iiniend it. Eiaenl)aum ailcl Parks reporrt~l lxrlorlning the postr*rior fisaGon SLI~LIWon t hc \.crticxl and hori/on~;tl muscle in rlleir p;lfirllls \vith DRS.” Postc.rioi fixation suture on thrb Ialei2l rcx‘lus niusclc l~n~lu~cd satisfacton rc511lt5 \\.it Ii ~limination of‘ the upshoot. Ho\~e\,el-, J)oytel ior fisatioii S~I~IIW on the \xWical rectus mii5c~le tlitl Ilot c11;111gc

the

a~,llolIll;ll

oveldloot\.“-

13crndt fi)und ;I tlec~casc~ in the iipshcx)t ii1 thr ;dtluctecl position in DRS ~vllen 11~ lo~.~red tht* insertioil of rhc 1;iter;il rectiis nl11sc~le.” IllccXlXb

282

Surv Ophthalmol

3X (3) November-December

myographic studies in DKS have demonstrated paradoxical innervation of the lateral rectus on adduction,“’ which was thought to produce the upshoot or downshoot on adduction. Feretis et al noted a decrease in the upshoot ofthese patients after a recession of the lateral rectus muscles.“” The splitting of the ends of the lateral rectus into a Y configuration is a unique idea that was first advocated by Jampolsky.“” 1t is easily pertinmed with no special skills required. It has not produced a decrease in abduction in any of the reported cases, nor has it produced a change in elevation or depression of the eye. Jampolsky et al’” suggested if a marked globe retraction on a moderate recession as adduction is present, well as the splitting procedure should be performed.“,‘” Rogers and Kremer reported five patients with Type 1 DRS who underwent the surgery of splitting the lateral rectus muscle.“’ The lateral rectus muscle was split into two halves to a point 10 mm posterior to the insertion. The two halves of the lateral rectus muscle were reattached to the sclera, forming the Y pattern. All their patients showed an improvement of head turn, esotropia and upshoot or downshoot after Y splitting of the lateral rectus and recession of the medial rectus muscle. Magoon and colleagues injected xylocaine into the eye muscle while the eye was anesthetized using topical proparacaine.“‘” Paralyzing the vertical recti and inferior oblique muscle was not effective in eliminating the upshoots. When the lateral rectus muscle was injected and relaxed, the upshoot was stopped.‘“’ Weakening of either the inferior or superior oblique muscle did not eliminate the upshoot, indicating that this abnormal movement is not related to oblique dysfunction.‘4”

1993

DERESPINIS

ET AL

attempt to avoid the need for a medial rectus recession.‘,” In both cases the injections were ineffective in decreasing the angle of esotropia 01 in increasing the abduction rotational movement.“”

C. SURGICAL

COMPLICATIONS

The possible complication ofanterior segment ischemia due to the vertical rectus muscle transposition with a recession of the medial rectus of the affected eye has been mentioned. Molarte and Kosenbaum recommended that a transposition procedure be performed as a primary procedure.‘“’ Ifnecessary, a medial rectus recession is performed as a secondary procedure no sooner than four months following the transposition. There is a risk of severely compromising adduction and causing an exotropia postoperatively after large medial rectus recessions in patients with DRS.““,“” The marked co-contraction of the lateral rectus on adduction augments the effect of the weakening of the medial rectus, leading to an overcorrection in the primary position.xs Therefore, it was suggested that if adduction saccadic velocities are very slow and limitation of adduction is present, one should consider limiting the recession of the medial rectus of the affected eye to no more than 5 mm, and adding a recession of the opposite medial rectus to fully correct the esotropia.“‘,‘“” Nelson has reported two patients who had the unusual complication of severe limitation of adduction after having a large medial rectus recession for a cosmetically unacceptable face turn and esotropia in primary position. The operated medial rectus muscles needed to be advanced postoperatively.“‘!’

5. Adjustable Suture Technique Adjustable recession technique for DRS has been recommended by Pressman and Scott.‘4’ They performed a left lateral rectus recession with an adjustable suture technique on one of their patients with DRS and reported a good result. They recommended that recessions should be done by the adjustable technique for DRS whenever possible. Adjustable recession procedures have also been performed on patients with DRS by Franklin and Hiatt, and Molarte and Rosenbaum. However, the results were not favorab,e..i”,‘“” 6. Botulinurn Injection Botulinurn injection was used on the ipsilatera1 medial rectus muscles in two patients in an

VIII. Summary The literature on Duane’s retraction syndrome suggests that the syndrome involves a complex set of clinical ocular findings which are caused by a spectrum of mechanical, innervational and central nervous system anomalies that occur together or separately. It is most frequently sporadic, but approximately 10% of cases are familial. The frequent association of DRS with nonocular anomalies, such as Wildervanck’s syndrome, point to a teratogenic event occurring between the fourth and eighth week ofgestation. In the literature there is a frequent overlapping of DRS with nonocular malformations, although this may not be clinically apparent to most practitioners. In unilateral cases of the syndrome the

DUANE’S RETRACTION

SYNDROME

trccluc~icy oflefi side involvement

averages 72%.

No rcasonablc explanation fi)r this phenomenon ha4 bwti hypotliesi&. Female prepotideratice Ita4 been postulated as being due to inheritattce itt an autosomal dot~iitiatit fashion with the pleiotropic gene responsible also being partially sexlitnitcd.““A cotnpilatic~n ofthe data available suggcsts that the tvpical DRS pafient is most likel\~ to by ;I h~pcrc,pi;~ fetitalc with utiilateral ‘l‘ype i atfee ring the left eye with an esotropia in i>rim;tt-) po4itiott. It has beet! suggested that ?l’ype 1 DRS is a \ ariatit of‘ Fl‘vlw I1 I and that the differences betwcett 11te two types involves ;I subjective decision b> the rsatitittet~ as to tltc degrw of lit~~itation 01’ ;i~l~lrtc~tioti.“” ’ l‘his smle line of’ thinking can bc usd (0 drgw that I‘yw II r)m with its tiianitnal l;tl~ul rectiis tiring ‘on attempted adtluction is ac-tuall) ;I \xri;inl 01’ simultatirous abduction (synergisticcti~ei.S‘etic,e).“” *l’he classification types pwposetl hv I lutw- alo~ig with syttergistk cii\ergettrx2 at-e only clitiical deccriptiotis. l‘hc authors agtw that there is a close relatiotishil~ tw t\+ (bcti ‘1 !pes I atltl I I I and ‘l‘ype II with synergialvhiclt probably represents att tic di\ergeticc. it~net~vatiot~al c~otititiutttii of‘ Ihe same iiti01ii;iIv. (hit. rttttlet.st~tt~clitlg 01’ the ;tfi,remeiitic,ticc( ntatcrial will help IIS in our ;rl~pi~oach to the ])atit,ttt. Most patients with DRS \vil1 tttaintain not’tti,il slct.copsis, but ntCiv assume it barely ttoticcable ;~ttottialous hcwl position. l‘hc practitiottet 1tius1 ht. ;~w;~t-e01‘ the ow.1rtx3ice of atiiblyol~ia. atiisot~ic~trol~i;I and as~mtttett-ic bilateralil~ ol’tttc. sv~idro~it~‘. k-or the most dillicwlt cases, a dt;igtto+ tic, lest such ii5 rlie I less-I.atic;istcr scrwti shoitld ht. considerrd. If there is a positive indication for surgery, sue h as a sigttificantly abnormal head position ot a ttotiwable ocular deviation, the practitionet should be aware of the therapeutic options. 11 is essential that the proper diagnosis is made and if DRS is present, the appropriate muscle(s) be operated on. Ck~otl clinical acumen and an understanding of‘ the pathology of the retraction syndrome will enhattce the surgical outcome. I)uane originally described the retraction syndrome nearly 100 years ago, yet no truly definiti1.c. explanation of the etiology of the syndrome haa been suppositioned. Recently MRI scanning has been utilized to determine if there is an) vertical displacement of the horizontal recti ntusclcs. Perhaps improved scanning and electrophvsiologic techniques combined with more autol)4\ stttdics will lend us a better insight into the cs;tct tt;tture of the wtraction syndrome.

284

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38 (3) November-December

paralysis of the me27. Burian HM, Cahill JE: Congenital dial rectus muscle with unusual synergism of the horiLontal muscles. Trorr~ .dat Ophtfudmol SOC 50:X7-102, 1952 HM, Van Allen MW. Sexton RR, Bailer KS: 28. Burian Substitution phenomena in congenital and acquired supranuclear disorders of eye movement. (~~/)llrllrtrl~~/~~~~~~~ 69:1105-l 114, 1965 29. Burian HM, van Noorden C;K: Bi,roc~r/rrt. l’i.\rorr tr& &I(In,- MoG/i!y. St Louis, CV Mosby, 19X.5, pp 371-377 30. Calmettes L, Deodati F, Delfilur G, Bechac G: Le S~IIdrome de Stilling-Duane. Line observation familiale. Hltll Sor Of,l/lril,,~~l Fr 6X.364-368, 1968 31. Carlson MR, Jampolsky A: An adjustable transposition procedure for abduction deficiencies. .i~/ ,/ O~/,lr~/r~r/t~~o/ X7;3XP-3x7, 1979 32. Cibis GW: Congenital familial extet-nal ophthalmoplcgia with co-contraction. ~~~/~~/~~//r~~/~, f>orciic~(rr/~ci (;P~IPO
33.

3-i.

._

Cogan DG: Congenital ocular motor apraxia. (,‘ot/ ,/ O~/r/h//lrt~n/ I:“.‘,:%-260, 1966 8). ,$IP~,/ 2:X46-X5 1, 1X99 35. &llins El‘: Enophthahnos. 36. Cremers WRJ, Hoogland GA, Kuypers it’: Hearing loss in the ret-vice-oculo-acoustic (Wildervanck) svndrome. .-It,& Olo/,r,?‘,rgo/ I11):54-57, 19x-l 37. Cricchi M: Su di un nuovo taso di syndrome di lacrime di coccodt-illo di natu1.r congrnira associata alla s) ndrome di Stilling-‘l‘urk-I)ti~lli~. Ho// Orrcl J/:587, 1962 DD: Duane.5 rerraction syn3X. Cross HE, Pfaffenbach drome and associated congenital n~alfi)rln;ltions. .-1,t/,/ O~/l//l///rrro/ 73:449-450. 197” JRM, Mtanda .%I‘. I~Uiiikerke-Eet-ala KU, :59. Cru)sberg Huygen PLM: (:ongenital &lucrion pals) and synel-gistic divergence: a clinical and electr[)-ocuIog~Iphic sllldy. HrJ op/Icr/lllo/ 73:68-75, I989 de la motilite 10. Danis P: Sur les anomalies congenitalrs oculail-e d’origine musrulaire et en particulier sur le syndrome de Stilling-.l‘tlrk-Duane. .-I,,,/ Orrtl (f’tr~\) IXI:I4X-IX], 1918 11. Dastur YE;, I‘rivedi H,‘l‘apaswi N, Shah N: Goldenhar’a syndrome with unilateral Duane’s retraction syndrome and “Butterfly” Vel-tehl-a. In//,/ f~~/f~/~f~~r/~~)/ 3 3: 187-l X9. 1985 -I:! tIeDecker it’: Kestenbaum transposition operation fix treatment of the Duane I retraction syndrome. ‘IS-N,~.~ O~/ll//rr/,~/o/ Sot 1 !K 100:47%4X?, 1980 43 Denslow (:I‘, Sims M: Duane’s retraction svndrome associated with optic nerve hypoplasia. ,/ Pm&r/r O~/~/hcrlrr/o/ S/rrrh;.\,nrc.\ f7:L’6-28, 1980 44 D’Ermo F: Su du casi di syndrome delle lacrime di coccodrillo di natura congenita associata a syndrome di Turk. Hoi/ Or/cl 2X:274, 1949 45 Discepola MJ, Polomeno KC. Zeesman S. et al: Autosomal recessive Duane’s retraction syndrome. (:u)I,/ Oph//r/l/ U/O/22;384-386. 1987 46. Dodds: H, J O]~/~//~rr/~no/iO:ti-lS, 19% 47. Duane A: Congenital deficiency ofabduction associated with impairment of adduction, retraction movements, contraction of the palpebral fissure and oblique movements of the eye. Arch Ophthalmol ?J: 133-l 59, 1905 -Ix. Duane I‘D. SchatL NJ. Caputo AR: Pseudo-Duane’s retraction syndrome. 7‘r(of,\ .-IuI O@/hn/>~o/ soc 74: I L’2129, 1976 49. Duke-Elder WS: Noncomitane squint, in Sy\/r,rr ~/O/I/~thrtlmology I’ol II’. St Louis: CV Mosby. 1949, pp 4077-4080 .50. Duke-Elder S: Normal and abnormal development, Congenital deformities, in S$lrm of O~htf~rtholog~. I’ol. 3, pl. 20. St Louis, CV Mosby, 1964, pp 991-995 5 I Du ke-Eldet- S: S+/PI~ I/ ~~~f~th~J/?/l~l~g~.Emhyl~p, VOI

1993

DERESPINIS ET AL,

3, Pt 1, St Louis, CV Mosby, 1963 AM, Parks MM: .A study of various surgical 52. Eisenbautn approaches for the leash effect in Duane’s, Presented at the joint session ofthe American Association for- Pediarric Ophthalmology and Strabismus and the American Academy of Ophthalmology. Chicago, 1980 B. ‘I‘samparlakis J: Planning 53. Feretis D, Papastratigakis surgery in Duane’s syndt-ome. O~/lt/rn/t~ro/og/rrr~ (Hn\rl) IX3:148-15’3 . . 19x1 occur2. Ferrel RL, Jones B. Lucas RV: Simultaneous I-c‘nce of the Holt-Oram and the Duane syndrome. ,/ PP~/NI~. h9:630-634. 1985 Franyois J: Hrtrtli!\ I), O~/t//~n/trrolog~. St Louis, <:V Mosby. 1961. pp 247-2.51 Franklin SK. Hiatt RL: Adjustable sutures in strabismus surgery. .-l~,r O~frtf/cr/mo/ Z/:285-289, 1989 Framtto D, Dellet- M: Jumelles Monorygotes et syndl-omr de Duane. Hit/l Sot Fr ~~f~t~~/~t~ff/K-11580, 1971 Fricdenwald: Hit/I,/ Ho@r, Hn$ TV,,: I35 and 202. 1896 (;allus E: Sinddlesog angeborener: Retraktionsberegungen des Auges dir Folge einer Gebutsverletlurg. .I ,cI/ .iugwhrr/kd X7:3.5-45. I92 I 60. (;arrec A, Dal-y J, Chesnais A. Delattre A: Faut-il operer les syndromes de Stilling~l‘urk-Dualle? Hull Sor (@lo/tt/tr/ F,, 70:~~9-G2, 1970 61. Gedda L, Magistretti S: Paracinesia addutorio-enoftalmica gemello-familial--e e albinismo oculare in altra familgia. .-I& (;rrrr/ lCfrrl Grnir//o/ 5:291, 1956 6L’. (iifford H: Congenital defects of abduction and other ocular movements and their relation to birth injuries, .-la ,) v~hlh/rl,nol 9:3-I 7, I 926 63 (Robin MH: Surgical management of Duane’s syndt-ome. H,-J O~frtfwlmol ia’: l-306, 1974 64. Gobin MH: Aspects of horizontal muscle surgery. Particularly in Duane’s syndrome. Trans Ophthalmol Sot UK 92t685-693, 1972 65. Goldfarb C, Gannon FL: Familial congenital lateral rectus palsy with retraction. Dis Nerv .S@ 25:17, 1964 66. Goldstein JH, Sacks DB: Bilateral Duane’s syndr0me.J Pediatr Ophthalmol 14: 12-17, 1977 67. Gourdeau A, Miller N, Zee D, Morris J: Central ocular motor abnormalities in Duane’s retraction syndrome. Arch Ophthalmol 99: 1809, 198 I 68. Gundersen T, Zeavin B: Observations on the retraction syndrome of Duane. Arch Opht~~rno~ 55:576-580, 1956 69 Hayes A, Costa .I‘, Polomeno KC: The Okihiro syndl-ome ofDuaneanomaly, radial ray abnormalities, and deafness. .d~nJ &f~rl GP?IP~22:273-280, 1985 70. Henderson JC: The congenital facial diplegia syndrome: clinical features, pathology and aetiology. A review of sixty one cases. H~U~II’N 62:381-403. 1939 71. Hering E: dir Lrhrr lrom Binoculren Sehen, Leipzig, Wilhelm Englemann, 1868 72. Heuck G: Uber angeborenenvererbten Beweglichkeitsdefect der Augen. K/in Monntsbl Augenhedkd 17:253, 1x79 73. Hickey WF, Wagoner MD: Bilateral congenital absence of the abducens nerve. I’urhouo rlrch [Path Anal] 402: 91-98, 1983 73. Hofrnann JR: Monorygotic twins concordant for Duane’s retraction syndrome. Am J Ophthalmol 99: .563-566, 1985 75. Holtz SJ: Congenital ocular anomalies associated with Duane’s retraction syndrome, the nevus of Ota and axial anisometropia. .4m / Ophthalmol77:729-73 1, 1974 76. Holrman AE. Chrousos GA. Kozma C. et al: Duane’s retraction syndrome in the fetal alcohol syndrome (letter). ,4m J Ophthnlmol 110:565-566, 1990 77. Honda Y. Yoshioka M: Duane’s retraction syndrome associated with muscular dystrophy. J Pedfatr Ophthalmo/ Strabismus 15: 157-159, 1978 78. Hotchkiss MG, Miller NR, Clark AW, Green WR: Bilateral Duane’s syndrome. Arrh O~hthff/rnof 98:870-874,

286

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38 (3) November-December

Nail- KK: Bilateral Duane’s retraction syndrome associated with crocodile tears s) ndl-ome. ~V~~ro/r~gv /&rtc -3h:l44-146, 197X IX Nast- AM, Klawwam E: Duane’s syndrome: a case t-eport. ,J Ocltltr,, T/trr stctg 2:24x-250, 19H3 129. Nelson LB: Sever adduction deficiency following a large medial rcctus recession in Duane‘s rrtl-action syl,drome. .-1,-/A q///l//rr/,,/o/ /11~:85%+62. I986 I XI. Nelson LB, Wagner KS, Simon jib’. et al: C:ongenital esotropia. Srct.71O~~hlhtrl~rcol3 /:36%3X:!. 19X7 13 I. Nemrt P, Ron S: Oc~tdat- saccadex in Duane’s 5~1~

1993

12i.

dromr.

H,

/ Of,h//rrr/d

h-7:3X,

1.55. 156.

157.

1.58.

I oix

and surgical treatment of I :IL’. Nutt AB: ~i’he significance congenital ocular pal&s. .-I,/,/ K CA/ Slog Et/g IhtJO-59, 1955 133. Okamoto E, Lcda’l‘: Emhi-yogenrsis ofintramural ganglia of the gut and its relation to Hirschsprung’s discast’. / Policrlr s/c,g _7:437-443. I967 HE;: 134. Okihiro MM. .l‘asaki ‘I‘, Nahallo KK, Bc1111ett Ihlalne syndromr and congenital upper-limb anomitlies: a l~niilial occ~~~‘~‘c’llc~. .-lwh ~VP~O~O/ 34: 174-l 79, l97i

1.59.

160.

161. 162.

163. 1%.

137.

1%. 139.

140. I4 I.

142. 143.

144.

14.5,.

146.

147. 14%

I-19. 150.

1.5I. 1.52. 153.

154.

Orlowski i4’J, ~li(!jtowic/. S: Is the Stilling-~l‘ttrk-I)uanc s) ndi-ome an independent pat hological tzntity? Ol,/~t/rc~/nrologicx I -If: 19%220, I962 Papst Lb’: Rundgesprach ttber’l‘halitlotlitl und angeborene fehlhildungen der Augetj. fI/\// O~~//,(I, (;I,\ h5;209214. 196-L Papst W: ‘l‘halidomid und kongenitale Anomalien der Augen. L)l\r/f O+/~lo/t& C;P.\ h5:209-2 1.5, I963 Papst W. Esslen E: Ztu- Atiologie der Angehorenen ahdu/r~~slahmlt~~g. k//r/ nltr,/tr/\h/ .itrgA~//kt/ /37:3Oti:\27. I960 Papsr W: k//r/ ilfr,t/rrl.\h/ .4 ctgf&v/kd 1 -I 1: 199, I Yti2 Papst LV, Esskn E: Symptc,mal~~ll~gy and therapy 01 ocular motility disturbances. .-II)/ / O+h/htr/~~)/ 5X:275291, 1964 Papst LV: Paralytischer Strabismus int;)lge paradoxes innervation, Hr;. D.rtc+ OI/jhlhn/t~ro/ (;r\ 67:X4, 1966 Parks MM: Ophthalnloplegic syndromes and trauma, in Duane ‘I‘D (ed): C/r~icn/ O~~/~//lcr/,,rr,/f?, Vol I. Philadelphia, Harpet- ancl Row, 1982. (A 20 Ptaffenhach DD. Cross HE, Krarns ‘1’1’: Congenital anomalies in Duane’s retraction syndrome. .-lr~h Of,ht/~N/~tro/X8:635-639, 1972 Pieroni 0: Goldenhar’s syndt-ome associated with hilatrt-al Duane’s retraction ~ynclronw.,/ Prdrrdr O/drlhcritw/ S1,_crhi\,aK\ 6: 16-l Y, 1969 Prakash P, Menon V: Combinrcl hori/.ontal and vertical retraction syndrome. fttbtr J O/~~/~lho/v~o/29: 1 13-l 14. 19x1 Pt-essman SH. Scott i%‘E: Surgical treatment of Duane’s syndrome. Ol/r//rrc/,rro/~?~?~~~ 93:29-38, I986 Prieto-DiaL J: Duane’s syndrome: electro-oculograph) pattern. J Pr~/tn/~, Uj~/rl/ict/~rro/ .S/t~/v\t~rx\ -32:243-245, 19x5 Raah EL: Clinical features of Duane’s syndr-omr. j Pr1986 /l/n/l oplll/lfil~no/ S/rclhift/U\ _33.64-68, Kamsay~j,‘L‘aylor D: Congenital crocodile tears: a key to the etiology of Duane’s syndrome. Rr J fIfihlhn/mo/ hl:518-522, 1980 Rashad F, Keith MW, Shields R, et al: Congenital vascular abnormalities in Okihiro’s syndrome - a case report. Angio/f~g~ 38:642-646, 1987 Kegenbogen L. Godel V: C~r~ico-oculo-acoustic syndrome. Ophthalmic Paediat &net 6:183-187, 1985 Regenhogen L, Stein R: Crocodile tears associated with hrnnolateral Duane’s syndrome. O,!Ahn/rt&ogrra 156: 3.53-360, 1968 Remenar L, Marek P: Electromyogram of Duane’s syn-

164.

165. 166.

167.

16X. 169.

170. I7 I.

172.

172a. 173.

174.

175. 176. 177. 178.

179.

DERESPINIS

ET AL

drome. Szrmrs:r! /O/:49, 3964 (abstracted in the Am J O~ph/hcr/m/ i8:720, 1964) Reny A, Brichet B: Le syndrome de Stilling-TurkDuane. Arrrt 0~~1 (Porrs) 205: 1063-1083, I972 Ro A, Gummeson B. Orton RB, Cadera W: Duane’s retraction syndrome: Southwestern Ontario experience. Con / Ophlhn/d -34:200-203, 19x9 Roget-s Gi. Bremer DL: Surgical treatment of the upshoot and downshoot in Duane’s retraction syndrome. O~h~htrlmolog?, L)/:l380-1382. 1984 Rosenbaum AL, Scott AB: Botulinurn toxin therapy in the management of strabismusand lid disorders. W?.\1] iv/v1 I G:446-457, I986 Rosenhaum AL, M’eiss SJ: MonoLygotic twins discordant for Duane’s retraction ryndrome. J P~rirnlr Opht/ff~/& S/~C&\~II~~ /5:359, 1978 Rosen S. Ro/enman Y, Arnon N, et al: Marfanoid hypermobility syndt-ome associated with Duane’s retraction s) ndrome. ,4n>r Oph~hm/mo/ 15:X62-864, 1983 Ruhin SE, Wagner KS: Ocular torticollis. s‘1~1.gOphthdm/ 30:366-:%X7, I986 Sachdev JS, Harrington J I.: Duane’s retraction syndrome associated with cerebral artcriovenous malformation. .%rtl/ret ,i lMfT/,/ 7’-),62%-625, 1986 Salus R: Lleher die angelxxenr Rrtraktion des Augapfrls. k/r,, Mot//r/h/ .-ficgrnhri/kt/ ‘)2:79, 1934 Saraux H, Laroche L, Lacombe H: Congenital horiomtal gale pat&G ancl ear clysplasia in a boy with Duane’s retraciicjn syndrome and seventh nerve palsy. Op/rltrtltnro/~~~;~/~ /XK:WX-2 I I, 19x4 Sate S: Elcctronlyographic study on retraction syndt-otnr. ,/p,t ,/ Ol,ht/~cr/t~~~/j:i’i-66. 1960 Sato H, Saito ~1‘.Ootaka ‘1‘. Yamakage K, et al: Duane’s syndrome and focal segmrtltal glonlerulosclet-osis. Srph,a~~ i/:274-273, 1989 Stassellati-Sforzolini (b: L’na sindrcmie molto ral-a: diletto congenito monolateralr della clrvaAone con t-eIIraAne del glolx~. K/i) O/o-Nr/r,-,,-O/(lr/mo/ 3 3~43 l-439. 195X Schmidt D: Congrnitale Augenmttsk~lpar~s~~~. ,i//vrrh 1’ C;).CIP/z\.4,uh o~hlh,l/vU~/ I ‘)2:2x5, 1974 Scott AB: LIpshoots ant1 downshoots. in Sousa-DiaL C (cd): 1’Cottg1~u\\ o/ f:./...4.D.E. (Cottdho LnliNo-,l,nr,r,cl rlr E\/rcrbiwm). Oc/o/wr Ih-/ 7, I Yi’h. (;uaruja-Brasil Sao, 197X Scott AB. \L’ong (;P’: Duane’s syndrome: An electrom)ographir study. ,.l,(.h O/,/~//!o/v~o/ X7:130-147. 1972 Sen DK. Harimohan: Duane’s retraction syndrome associated with congenital ptosis. /n&n f f~~~t~ft//~/~~~f~/ 20: I G-186. 1972 Sevel D. Kassar BS: Bilateral Duane Synclromc. Occurrence in three successike generations. Ardt O~ptrlt~rr/vro/ 9/:492-494. 1974 Sherrington <:S: Experimental note on two movements of the eyes. J Phv.ciot (London)f7:27. I894 Simons/ HJ: Investiga&ms of ocular counterrolling and Bielchowsky head-tilt te5t. stiffness in passive ecular rolling and displacement of recti eye muscles.Doc/OU// It/r.\/\, L’Vi7l c)~.i,n\lr~dnr, Aug 30. 19x4 Sinions/: HJ, Hat-ting F, deWaal BJ. Verheeten B\vJM: Sideway displacement and curved path of recti eye tllusclcs. Awh ~~~ll~(I~l~~~i 103: 124, 1985 Sinclair WW: Abnormal associated movements of the eyelids. Oph/hn/nm/ Karl I -1:307-3 19, I X95 Singh P, Patnaik B: Heredity in Duane’s Syndrome. .+~a Oph~hdmologim 4’): I OS- 1 IO, 197 I Smith AC;: Duane’s svndrotne. ObhU~n/m~r Srmimrr 2: 33-72, 1977 Sood (X, Srinath BS, Krishnamurthy G: Acquired Duane’s retraction syndrome following Kronlein‘s operation. Evr Ear No\r 7%ronl Mott/h!y 5J:20-23, 1975 Souza-Di& C: Congenital VI nerve palsy is Duane’s syndrome until disproven (letter). B~nocttlor- I’oiorr Orw

DUANE’S RETRACTION SYNDROME

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18-l

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“II

2 I2 186 ‘1 I :i IX7 L’II 2 I .rl IXX

I XII I90 191 I1)L’

Outline

I!):$ I94

I !I’,

I. Historical ,I.

anotnalies

\ c~,!!,g~!tx!l 5, (:I!.,,-,) I\: St~g,!!e,!tal clilati,~!! 01 tlw Cohn!! .!,!(I It,<, Stilli!!a-‘l‘,,!.k-l)!,.,,,v !~t.t!~;!~tio!! b\,!cl,-o,,!e. IE’

2.

B.

i!Hi

II. I97

I90 L’oo

20 I

L’W

2o:i 20-l.

“05

Innervational anomalies 3. Central nervous svsteni Mernpts at classitica~ion

~:l,lTent A.

K. C. Iw

aspect> I’athogenesis and etiological theol ies 1. Mechanical and periphcwl stru( tural

anomalies

CollCepth

Pathogenesis Embr!~ogenesis Hereditar) tartars

111, Demographic

and epidemiologic A. Incidence B. Sexual distribution C. Laterality D. Race E. Types of presentation F. Ocular deviation in primary C;. Refractive errors H. Amblyopia

factors

position

IV. Special considerations of the retraction syndrome A. Associated congenital anomalies and syndromes I. Associated ocular findings 2. Associated non-ocular findings B. Variants of Duane’s syndrome C. Acquired retraction syndrome

Surv

288

Ophthalmol

38 (3) November-December

V. Clinical features and diagnosis A. General approach to the patient B. Use of classification systems C. Tyoical findings and adduction 1.’ ‘Defects in ibduction 3_. Fsotropia and exotropia and downshoots 3. Upshoots 4. A and V patterns 5 Eyelid changes and globe retraction 6: Torticollis 7. Bilateral cases D. Sensory findings E. Amblyopia \‘I.

Diagnostic dilemmas A. Differential diagnosis 1. Abducens nerve palsy 2. Moebius syndrome 3. Congenital oculomotor apraxia 4. Congenital or infantile esotropia B. Modes of testing 1. Forced ductions or force generation testing 2. Saccadic velocity testing

DERESPINIS

1993

ET AL

3. Electromyography 4. Hess-Lancaster screen VII.

VIII.

Therapeutic modalities A. Goals of treatment B Surgical procedures 1. Horizontal surgery - recession versus resection 2. Transposition procedures 3. Posterior fixation sutures 4. Approach to upshoots, downshoots and A or V Patterns 5. Adjustable suture technique 6. Botulinum injection C. Surgical complications Summary

Supported in part by the Children’s Eyecare Center of New Jersey, Research to Prevent Blindness and Fight for Sight, Inc., New York. Reprint address: Patrick A. DeRespinis, M.D., Children’s Eyecare Center of New Jersey, 1.5 South Ninth Street, Newark. New Jersey 07107.