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1

describe what duane's retraction syndrome is

Limitation of horizontal eye movements with retraction of the globe on adduction

2

list 7 features of duane's

- Limitation of horizontal eye movements

- Abnormal head posture: depending on the underlying deviation they have in pp
Eso deviation face turn to affected side
Exo deviation with limited adduction face turn to unaffected side

- Manifest deviation (may or may not have)

- Retraction of globe on adduction with narrowing of palpebral fissures

- Widening of palpebral fissures on abduction

- Up/down shoots on adduction

- Poor convergence (except in one type of duane's)

3

what is the AHP for a eso deviation in duane's

face turn to affected side

4

what is the AHP for a exo deviation in duane's

face turn to unaffected side

5

list 6 ocular associations of duane's syndrome

- coloboma
- heterochromia irides
- microphthalmos
- cataract
- crocodile tears
- persistent pupillary membrane

6

explain what the central defect aetiology of duane's syndrome is

Defective Innervation
Omission, substitution or duplication of nerve supply of the LR muscle

Co-contraction theory
LR muscle partially innervated by III nerve, with or without VI innervation
MR and LR muscle fire simultaneously leading to limitation of horizontal movement and globe retraction seen on adduction

7

explain what the peripheral defect of duane's syndrome is

Mechanical
Abnormal extraocular muscles or their tendons
Posterior insertion of MR (acts as a retractor)

Inelastic fibrous LR or fibrous bands
Hypoplasia of LR
Prevent LR from relaxing or contracting

8

what is the 2 ways of classifying duane's syndrome

- Brown (1950) clinical picture of OM
Type A, B, C

- Huber’s (1974) based on EMG findings and co-contraction theory where LR receives co-innervation from III and VI nerves
Many patients do not fit neatly into Type I, II and III

9

which classification is done by looking at ocular motility picture that we see in clinic

Brown (1950) clinical picture of OM
Type A, B, C

10

what is an easy way to remember the Huber’s classification of duane's syndrome

Type 1 (has 1 letter D) characterised by abduction deficit

Type 2 (has 2 letter D) characterised by adduction deficit

Type 3 (has 3 letter D) characterised by adduction and abduction deficit

11

which type of duane's syndrome classification is generally used in a hospital based practice

Brown (1950) clinical picture of OM
Type A, B, C

12

briefly describe Type: A, B and C classification of duane's syndrome

- Type A limited abduction > adduction
limited adduction and abduction, but abduction is greater affected than adduction

- Type B limited abduction, but normal adduction
convergence will be fine because adduction is normal

- Type C limited adduction > abduction thus exotropia in pp
limited abduction and adduction, but adduction is greater affected than abduction

13

describe the clinical signs of type A of the Brown (1950) classification of duane's syndrome of:
Globe retraction
limited ABD
AHP
Cover test without AHP
PCT angle
Reduced convergence
BSV

Globe retraction: Yes

limited ABD: abd > adduction

AHP: Face turn affected side, Dist > near

Cover test without AHP: E / ET for near, ET for distance

PCT angle: D>N

Reduced convergence: Defective

BSV: Yes

14

describe the clinical signs of type B of the Brown (1950) classification of duane's syndrome of:
Globe retraction
limited ABD
AHP
Cover test without AHP
PCT angle
Reduced convergence
BSV

Globe retraction: Yes

limited ABD: Abduction only, (adduction normal)

AHP: Face turn affected side, Dist > near

Cover test without AHP: E / ET for near, ET for distance

PCT angle: D>N

Reduced convergence: Normal

BSV: Yes

Convergence is normal, px is still likely to have an ESOT in pp

15

describe the clinical signs of type C of the Brown (1950) classification of duane's syndrome of:
Globe retraction
limited ABD
AHP
Cover test without AHP
PCT angle
Reduced convergence
BSV

Globe retraction: Yes

limited ABD: Add > abduction

AHP: Face turn unaffected side, Near > list

Cover test without AHP: XT for near, XT/ X for distance

PCT angle: N>D

Reduced convergence: Defective / absent

BSV: Yes

Convergence will be reduced as adduction is greater than abduction, px will have EXOT in pp

16

what will be seen in a patient in type A duane's retraction syndrome in the left eye:
when they look to the left
primary position
when they look to the right

when they look to the left: L eye doesn't abduct as well, but R eye is fine i.e. adducts fully

primary position: slight left esotropia

when they look to the right: adduction of L eye is also limited but to a lesser amount than the abduction deficit

17

what will be seen in a patient in type B duane's retraction syndrome in the right eye:
when they look to the left
primary position
when they look to the right

when they look to the left: adduction of R eye is normal

primary position: slight right esotropia

when they look to the right: limited abduction of right eye

18

what will be seen in a patient in type C duane's retraction syndrome in the right eye:
when they look to the left
primary position
when they look to the right

when they look to the left: level of limitation of adduction is greater compared to abduction of that eye

primary position: right exotropia

when they look to the right: R eye slight limited abduction

19

list 9 things that should be in the orthoptic report of someone with a suspect duane's syndrome

- Case history
Abnormal eye movements
Eye looks sunken / ptosis
One eye slow to follow
AHP (likely reason for presentation to eye clinic)

- Birth history
Any pregnancy problems or medication

- General health

- Family history
As theres some genetic link, so must enquire

- Abnormal head posture
Centralise narrow field of BSV

- Cover test – with and without AHP
- OM - carefully document
- Changes of lid and globe position
- Confirmation of BSV with AHP

20

when a px with duane's syndrome has a EXOT or ESOT in pp, what will they not experience and what will they experience instead

will not experience diplopia, will suppress instead

21

explain why you will carry out a cover test with the AHP and without the AHP in duane's syndrome

- with the AHP: to confirm that they do have a latent deviation that they can control to

- without AHP: to determine if esotropia or exotropia and this will help classify the type of diagnosis that they have

22

list the 7 things you will do in your management for someone with duane's syndrome

- Correct any refractive error

- Fundus and media check
for no other pathology causing the reduced vision

- Amblyopia treatment if necessary
as px can maintain AHP and avoid amblyopia

- Differential diagnosis
- Moebius syndrome (VI and VII CN)
- Sixth nerve palsy

- Generally conservative treatment as:
- Symptom free
- Slight abnormal head posture
- Well compensated deviation
- Comfortable binocular single vision (BSV)

- Manage patient/parent expectations:
- Cannot restore normal eye movements
- Patient will still be able to drive

- Indications for further treatment
- Prisms for compensation
- Moderate AHP
- Poor cosmesis (large esotropia)

23

name the 2 differential diagnosis of duane's retraction syndrome

- Moebius syndrome (VI and VII CN)
- Sixth nerve palsy

24

how can you tell that a patient has duane's retraction syndrome and not Moebius syndrome instead

because patients with Moebius syndrome will have 6th and 7th CN involvement too
and type B duane's syndrome, where their abduction is only affected and adduction is normal would look as if these patients have got a 6th nerve palsy, but with Moebius syndrome they will have 7th nerve involvement too

25

how can you tell that a patient has duane's retraction syndrome and not a sixth nerve palsy instead

because there will be no lid/globe retraction
and the esotropia will be greater in distance fixation compared to near fixation with 6th nerve palsy

26

give 4 reasons why treatment for duane's syndrome is generally conservative

- Symptom free
- Slight abnormal head posture
- Well compensated deviation (in presence of AHP)
- Comfortable binocular single vision (BSV)

27

what 2 things will you explain to the patient/parent about their expectations

- Cannot restore normal eye movements
so if they do require any treatment, the limitations of the eye movements they have will remain exactly the same post operatively. so any surgical treatment is only for the underlying ESOT or EXOT that they have in pp, and not to improve their horizontal eye movements

- Patient will still be able to drive
as px can see BSV in pp

28

list the 3 things that will give indications for further treatment for duane's syndrome
why for each one and what can be done

- Prisms for compensation
if px decompensated later in life or becomes more symptomatic and complains of diplopia
can use a fresnel prism on their glasses

- Moderate AHP
for a large squint in pp and is difficult to maintain BSV wth the AHP, then do surgery to reduce the primary deviation they have, so they can control that to a small AHP, especially if theres poor cosmesis of the underlying deviation

- Poor cosmesis (large esotropia)

29

what are the 3 aims for treating/managing someone with duane's syndrome

- Improve field of BSV
- Reduce AHP
- Improve cosmesis if no BSV

30

what is the surgical management for the esotropia in someone with duane's syndrome and what this depends on
and which 2 types this applies to

Recess (weaken) one or both MR depending on angle or vertical tendon transfer

Type A and B