CASE PRESENTATION:

DUANE’S SYNDROME

Prepared by:

Anis Suzanna Binti Mohamad

Optometrist

Hospital Langkawi

CONTENT

Introduction Aetiologies

Clinical features Classifications

Case study Discussion ConclusionReferences

INTRODUCTION

Binocular vision

Non strabismus Strabismus

IncomitantNeurogenic

palsy

Myogenic

palsy

Mechanical palsy

Duane’s Syndrome

Vertical Retraction Syndrome

Brown’s Syndrome

Marcus Gunn Jaw Winking

Orbital Injuries

Comitant

WHAT IS DUANE’S SYNDROME? aka Duane’s retraction syndrome or

Stilting-Turk Syndrome a congenital eye movement disorder

due to misdirection of the nerve fiber on eye musclecausing some eye muscle to contract when

they shouldn’t, vice versa can be unilateral or bilateral Usually congenital but may be acquired Affects females to males in ratio of 3:2 May be associated with other defects

such as deafness, anisometropia etc.

AETIOLOGIES1. Mechanical factors

fibrosed LR abnormally insertion of MR binding of MR sheath to wall

2. Embryonic factors Disturbance in normal embryonic development

during 2nd month of gestation Development of 3rd, 4th, and 6th cranial nerve occur

3. Paradoxical innervation Increase innervation to both MR & LR during

ADD and relaxation of both MR & LR during ABD

4. Trauma

CLINICAL FEATURES limitation of abduction with or without

limitation of adduction attempt adduction: retraction of the globe

with narrowing of fissure Protrusion & widening of the palpebral

fissure on abduction May or may not have AHP Possible defect of convergence Strabismus

May have updrift or downdrift on adduction with A or V pattern

Amblyopia if not adopted AHP Generally positive forced duction test

(done by ophthalmologist)

CLASSIFICATIONS

Clinically, it is often subdivided into 4 types with associated symptoms: 1. Type 1 2. Type 2 3. Type 3 4. Type 4

Each of the group can be further classified into 3 subgroups depends on where the eyes are when on the primary gaze: Subgroup A (Effected eye looks esotropia) Subgroup B (Effected eye looks exotropia) Subgroup C (Effected eye looks almost orthophoria)

TYPE 1

-Poor abduction, good adduction

-agenesis of 6th nerve-3rd nerve split innervate LR, MR-adduction intact as most nerve goes to MR

TYPE 2

-Poor adduction, good abduction

-6th nerve intact-3rd nerve split innervate LR, MR-Poor adduction as LR contract against MR

TYPE 3

-Poor adduction, poor abduction

-6th nerve agenesis-3rd nerve split innervate LR, MR-The split is equal-Eye not moves in/out

TYPE 4

-Paradoxical abduction on attempt adduction

-6th nerve agenesis-3rd nerve split innervate LR, MR-most innervate LR-when ADD it ABD

TYPE 1(70-80%) ie: LE

TYPE 2(~7%) ie: LE

TYPE 3(~15%) ie: LE

TYPE 4 ie: LE

LE Esotropia with head straight

Face turn to affected side

Limited abduction left eye

-Normal or less adduction-Narrowing of fissure-Globe retraction

LE Exotropia with head straight

Face turn to non-affected side

Normal or less abduction

-Limited adduction-Narrowing of fissure-Globe retraction

-Marked upshoot and sometimes downshoot on adduction

Eyes are aligned in primary position with head straight

Limited abduction left eye

-Limited adduction-Narrowing of fissure-Globe retraction-Upshoot/ down shoot

Large LE Exotropia

Limited adduction RE

Simultaneous abduction when looking toward uninvolved side

-violating Hering’s law

CASE STUDYPATIENT: MALE/8/MALAYID NO: AS00022278 Date: 1st February 2012 (GEN

clinic)

CHIEF COMPLAINT-Referral from optometrist Kulim Hospital.

OCULAR HISTORY-LE Duane’s retraction syndrome type A.- Incommittant angle exophoria at primary gaze.- No diplopia.

GENERAL -Under peads SOPD follow-up for omphalocele at birth and bilateral

undescended testis.- Curently patient in primary school and sit in second row in class.

Able to read blackboard without glasses.

FAMILY HISTORY-Unremakable

RE LE

VA ( unaided) 6/24, Pinhole: 6/12-2 6/12, Pinhole: 6/9

Refraction (done in Kulim Hospital

Pl/-1.00x10 (6/9) Pl/-1.00x175(6/9+2)

Diagnosis: BE astigmatism. LE Duane’s retraction Syndrome Type A.

Management and plan: Cyclo RA appointment and review by General Follow-up

Clinic on 1/12. KIV prescribed if needed.

CLINICAL FINDINGS

FIRST VISIT Date: 23rd April 2012 (OPTOM clinic)

CHIEF COMPLAINT-Father claimed that patient not aware of

vehicles while cycling. - Father claimed that sometimes when

patient want to focus on something, he will chin down.

OCULAR HISTORY- Never wear glasses before.

CLINICAL FINDINGSRE LE

VA (unaided) 6/12, N5 6/7.5, N5

K-reading 38.25@1140.00@101

38.50@536.50@95

Refraction +0.25/-1.75x10 (6/6-1)

Pl/-1.25x180(6/6)

AHP Sometimes chin down

Hirschberg Intermittent LE small XT < degree.

Cover test (33cm): Small XP with fast recovery(6m): Small XP with fast recovery

OMT

SR u/a -1LR u/a -3MR u/a -1SO u/a -1

NPC To measure on next visit.

Impression: Bilateral astigmatism. Vision improves with spectacles

Rx. Intermittent small XT on LE. Patient able to control.

Management and plan: Prescribe glasses. TCA 3/12 to monitor binocular assessment on next visit

(NPC, CT, stereopsis, monitor squint) and to review on general clinic.

SECOND FOLLOW-UP VISIT Date: 9th July 2012 (OPTOM clinic)

CHIEF COMPLAINT-Come today for RA and BV assessment.

OCULAR HISTORY- No active complaint with current

glasses.- Patient comply with glasses.

CLINICAL FINDINGSRE LE

VA (aided) 6/9-2 6/9+3

Current glasses +0.25/-1.75x11 Pl/-1.25x1

Refraction +0.25/-1.75x15 Pl/-1.25x175

NPC Reduced compared to patient’s age

Impression: New spectacles Rx almost the same with current glasses. BE mod astigmatism. LE Intermittent small XT with convergence insufficiency. (NPC reduced

compare to pt’s age).

Management and plan: Continue wearing glasses constantly. To start pen-to-nose visual therapy ( 5session, 5x per day). TCA 3/12 to monitor binocular assessment on next visit (NPC, CT,

stereopsis, monitor squint) and to review on general clinic.

THIRD FOLLOW-UP VISIT Date: 25th October 2012 (OPTOM clinic)

CHIEF COMPLAINT-Come today for RA and BV assessment.

OCULAR HISTORY- No active complaint with current

glasses.

CLINICAL FINDINGS

RE LE

VA (aided) 6/7.5+3 6/7.5-1

Current glasses +0.25/-1.75x10 Pl/-1.25x175

Refraction +0.25/-1.75x15 Pl/-1.25x170

AHP Absent

Hirschberg Symmetry

Cover test(33cm) : Orthophoria

(6m) : Small exophoria with fast recovery

OMT RMR o/a.

NPC Reduced compared to patient’s age

Impression: No significant changes in Rx. BE mod astigmatism. LE Intermittent small XT with convergence insufficiency.

(NPC reduced compare to pt’s age). RE medial rectus overaction. No squint noted at primary gaze.

Management and plan: Continue wearing glasses constantly. To start pen-to-nose visual therapy ( 5session, 5x per day)

to improve NPC. TCA 3/12 to monitor binocular assessment on next visit

(To review on VA, RA and squint assessment).

FOLLOW UP VISIT IMPRESSION MANAAGEMENT & PLAN

4TH FOLLOW UP VISIT(22/4/2013)

BE astigmatismLE slightly exotropia with BV good with Rx

• Continue wearing current glasses• Continue pen-to-nose exercise (5session/5x/day)•KIV dot card exercise @ Brock’s string if convergence still reduced.• TCA 3/12 to review RA and NPC.

5TH FOLLOW UP VISIT(24/7/2013)

RE hyperope and astigmatism. Flat cornea from k-reading?Poor convergence, poor prognosis as suppress RE during previous therapy.

•Continue wearing current glasses• Continue pen-to-nose exercise coupled with physiological diplopia awareness.• TCA 3/12 to review RA and NPC.

6TH FOLLOW UP VISIT(3/11/2013)

BE low refractive error.NPC improves slightly.

•Continue wearing current glasses• Continue pen-to-nose exercise coupled with physiological diplopia awareness.• TCA 4/12 to review RA and NPC.

VISIT SUMMARY

DISCUSSION**POINT TO PONDER

Clinical investigations:-1) Age of presentation

2) Visual acuity

3) Abnormal head posture (AHP)

4) Cover test

5) Ocular motility

6) Hess chart

DISCUSSION History taking

During history taking, we need to ask about the age of presentation.

Because this syndrome may be occurs during first year of life, but occasionally later.

Treatment need to give as early as possible for better prognosis of vision and patient’s condition.

DISCUSSION Visual acuity

Visual acutiy is good if BSV maintained and no anisometropia and amblyopia occur.

Important to detect and treat the underlying causes within plastic ages to avoid development of amblyopic eyes.

VisitsVisual acuity

RE LE

2/2/2012 6/24 6/12

23/4/2012 6/12 6/7.5

9/7/2013 6/9-3 6/9+3

25/10/2013 6/7.5+3 6/7.5-1

22/4/2013 6/6-2 6/6-1

24/7/2013 6/6-1 6/6-1

3/11/2013 6/6-1 6/6-1

* This patient good vision prognosis because he not developed amblyopic eyes.

DISCUSSION Abnormal Head

Posture (AHP)Face turn to affected

side in types A and B. Face turn to unaffected side in type C.

For this patient, the examiner detected patient had face turn to the left (affected side).

Visits AHP

23/4/2012 Sometimes chin down

3/11/2013 Face turn to the left and chin up in primary position slightly.

DISCUSSION Cover test

BSV often maintained with AHP. Without AHP, types A and B – esotropia. Type C –

exotropia. In this patient all clinical findings are showed patient

had slightly exo deviation. Probably had type 3? Confused and indistinct

classification.

DISCUSSION Ocular motility

limitation of abduction with or without limitation of adduction

attempt adduction: retraction of the globe with narrowing of fissure

Protrusion & widening of the palpebral fissure on abduction

May or may not have AHP Possible defect of convergence Strabismus

May have updrift or downdrift on adduction with A or V pattern

Amblyopia if not adopted AHP

DISCUSSION Hess screen test

Additional test- Hess chart: to investigate incomitant strabismus in order to asses paretic element .

May show a large restriction for a small deviation in primary position. Alternative may use binocular Bjerrum visual field.

Examples of Hess screen chart result among Duane’s Syndrome Type 1.

DISCUSSION Optometric

impressionOptometric impression

follows Lyle’s classification.

Patient had LE Duane’s retraction syndrome Type A.

Type A Abduction and adduction are both defective but abduction is more defective than adduction.

Type B Defective abduction only, adduction is normal.

Type C Abduction and adduction are both defective, but adduction is more defective than abduction.

DISCUSSION Optometric

management and planManagement in this

case: Correction of refractive error observation BV therapy.

3. Observation

4. Surgery indicated if:• M

arked AHP

• Decompensating

• Cosmetically poor deviation

• Diplopia occuring more frequent

CONCLUSION Duane’s syndrome is a congenital eye movement

disorder in which there is miswiring of the eye muscles that typically can be recognized through a few ocular signs and symptoms.

As an optometrist, we should smartly recognized this syndrome according to the history taking and clinical findings in order to make an accurate diagnosis.

Although the syndrome is permanent, further managements is crucial in order to solve patient’s problems such as marked AHP and also on.

REFERENCES Kim JH, Hwang JM. Presence of the abducens

nerve according to the type of Duane's retraction syndrome. Ophthalmology. 2005 Jan;112(1):109-13. PubMed PMID: 15629829.

National Human Genome Research Institute (http://www.genome.gov/11508984)

Dr. Norliza Lecture Notes on Nonstrabismic anomaly.

Lecture notes Bengkel penglihatan Binokular dan Terapi Visual Tahap 1/2013.

E-His systems, Hospital Sultanah Bahiyah, Alor Setar.

ACKNOWLEDGEMENT

Special thanks to all clinician optometrist in Sultanah Bahiyah Hospital.

Special thanks to Ms Nur Shafiah ( Supervisor’s optometrist) for this case.