Duane’s SyndromeDuane’s Syndrome

Violent Violation of Violent Violation of

Sherrington’s LawSherrington’s Law

DefinitionDefinition

Disturbance of ocular movement Disturbance of ocular movement characterized by simultaneous characterized by simultaneous

contraction of the medial and lateral contraction of the medial and lateral rectus muscles in adductionrectus muscles in adduction

HistoryHistory

1879 - Heuck describes a case of retraction in 1879 - Heuck describes a case of retraction in adductionadduction

1887 - Stilling1887 - Stilling

1895 - Sinclair1895 - Sinclair

1896 - Bahr1896 - Bahr

1899 - Turk1899 - Turk

1900 – Wolff1900 – Wolff

1905 - Duane presents 54 collected cases 1905 - Duane presents 54 collected cases

Duane's known as Stilling-Turk-Duane Syndrome in Duane's known as Stilling-Turk-Duane Syndrome in EuropeEurope

PrevalencePrevalence

Types I, II, and IIITypes I, II, and III Incidence 1-4 percent of all strabismusIncidence 1-4 percent of all strabismus Female 54-62%Female 54-62% Left eye 60-75% where unilateralLeft eye 60-75% where unilateral Bilateral 18-22%Bilateral 18-22% Many associated congenital anomaliesMany associated congenital anomalies Occasionally familialOccasionally familial

Diagnostic FeaturesDiagnostic Features

Reduced abductionReduced abduction Retraction of the globe on adductionRetraction of the globe on adduction Co-contraction of the lateral and Co-contraction of the lateral and

medial recti on adductionmedial recti on adduction

Associated FeaturesAssociated Features

Upshoot or downshoot in adductionUpshoot or downshoot in adduction Narrowing of palpebral fissure - Narrowing of palpebral fissure -

minimal in some casesminimal in some cases Low angle esotropia or exotropiaLow angle esotropia or exotropia Head turn for fusionHead turn for fusion "Y" or "V" pattern"Y" or "V" pattern Synergistic divergenceSynergistic divergence

Differential DiagnosisDifferential Diagnosis

Abducens palsy - usually larger angle Abducens palsy - usually larger angle esotropia in primary gaze esotropia in primary gaze

Ocular myastheniaOcular myasthenia Spasm of the near reflexSpasm of the near reflex Medial rectus entrapment with medial orbit Medial rectus entrapment with medial orbit

wall fracturewall fracture Strabismus fixusStrabismus fixus Ocular neuromyotoniaOcular neuromyotonia Graves ophthalmopathyGraves ophthalmopathy

Duane’s-Associated SyndromesDuane’s-Associated Syndromes33% of All Duane’s33% of All Duane’s

Klippel-Feil Anomaly 3-4%Klippel-Feil Anomaly 3-4% Labyrinthine deafness 8-16 %Labyrinthine deafness 8-16 % Wildervanck Syndrome both of aboveWildervanck Syndrome both of above Goldenhar SyndromeGoldenhar Syndrome Crocodile tears Crocodile tears Arthrogryposis multiplex congenitaArthrogryposis multiplex congenita Marcus-Gunn Jaw Winking SyndromeMarcus-Gunn Jaw Winking Syndrome Many othersMany others

EtiologyEtiology

NeuroanatomyNeuroanatomy 1. Deficient innervation of lateral rectus1. Deficient innervation of lateral rectus 2. Innervation of lateral rectus by 2. Innervation of lateral rectus by

anomalous branch of 3rd nerve anomalous branch of 3rd nerve 3. Brainstem origin3. Brainstem origin

EmbryologyEmbryology 1. Teratogenesis at 8 weeks gestation1. Teratogenesis at 8 weeks gestation 2. Absence of abducens motor neurons2. Absence of abducens motor neurons

Type I Duane’sType I Duane’s

Most Common – 78%Most Common – 78% Very reduced abductionVery reduced abduction Globe retraction with attempted Globe retraction with attempted

adductionadduction Narrowing of palpebral fissure with Narrowing of palpebral fissure with

adductionadduction Typically esotropicTypically esotropic Absent sixth nerve nucleusAbsent sixth nerve nucleus

Duane’s Retraction Duane’s Retraction SyndromeSyndrome

Type I Duane’s EMGType I Duane’s EMG

MR – AdductionMR – Adduction

++MR- AbductionMR- Abduction

--

LR – AdductionLR – Adduction

++LR – AbductionLR – Abduction

--

Type II Duane’sType II Duane’s

Least common -7%Least common -7% Fair abductionFair abduction Reduced adductionReduced adduction Globe retraction and narrowing of Globe retraction and narrowing of

palpebral fissure with adductionpalpebral fissure with adduction Often ExotropicOften Exotropic

Type II Duane’s EMGType II Duane’s EMG

MR – AdductionMR – Adduction

++MR- AbductionMR- Abduction

--

LR – AdductionLR – Adduction

++LR – AbductionLR – Abduction

++

Type III Duane’s SyndromeType III Duane’s Syndrome

Incidence about 15%Incidence about 15% Poor abduction and adductionPoor abduction and adduction Globe retraction and narrowing Globe retraction and narrowing

fissure in adductionfissure in adduction Minimal deviation in primary gazeMinimal deviation in primary gaze Tonic firing of horizontal rectus Tonic firing of horizontal rectus

musclesmuscles

Type III Duane’s EMGType III Duane’s EMG

MR – AdductionMR – Adduction

++MR- AbductionMR- Abduction

++

LR – AdductionLR – Adduction

++LR – AbductionLR – Abduction

++

Secondary Effects of Secondary Effects of Duane’sDuane’s

Pseudo-overaction of inferior obliquePseudo-overaction of inferior oblique Due to leash effect of contracting LRDue to leash effect of contracting LR

V, Y and X patternsV, Y and X patterns Face turnFace turn

Treatment of Duane’sTreatment of Duane’s

Rationale for treatmentRationale for treatment Disruptive head turnDisruptive head turn Diplopia (rare)Diplopia (rare) Suppression and amblyopia (uncommon)Suppression and amblyopia (uncommon) Large angle deviation in primary gazeLarge angle deviation in primary gaze Deviation in up or downgazeDeviation in up or downgaze

Treatment modalitiesTreatment modalities Many cases require no interventionMany cases require no intervention Prism in spectaclesPrism in spectacles SurgerySurgery

Surgery-Type ISurgery-Type I

For minimal co-contraction do large For minimal co-contraction do large ipsilateral MR recessionipsilateral MR recession

For severe co-contraction-small For severe co-contraction-small ipsilateral MR recession and large ipsilateral MR recession and large contralateral MR recessioncontralateral MR recession

Avoid lateral rectus resectionAvoid lateral rectus resection Approach transposition with caution Approach transposition with caution

because of vertical deviationsbecause of vertical deviations

RecessionRecession

MEDIAL RECTUS

TranspositionTransposition

Surgery for Type IISurgery for Type II

Ipsilateral lateral rectus recessionIpsilateral lateral rectus recession Contralateral medial rectus resectionContralateral medial rectus resection

Surgery for Type IIISurgery for Type III

Fadenoperation on Contralateral Fadenoperation on Contralateral medial rectus and lateral rectusmedial rectus and lateral rectus

Surgery for Upshoot or Y-Surgery for Upshoot or Y-patternpattern

Y-splitting of lateral rectusY-splitting of lateral rectus Fadenoperation of lateral rectusFadenoperation of lateral rectus

Bilateral Duane’sBilateral Duane’sDanger of consecutive XTDanger of consecutive XT

Simultaneous recession of medial Simultaneous recession of medial and lateral rectusand lateral rectus

M.R

FINFIN