STRABISMUS SYNDROMES
This group of strabismus are unique and have certain specials features. There are:
1.Duane’s retraction syndrome
2.Brown’s syndrome
3.Mobius’ syndrome
4.Marcus Gunn’s syndrome
6.Double elevator palsy
Duane’s retraction syndrome
The etiologic factors of this syndrome are: Structural anomalies (abnormal posterior insertion
of the medial rectus, non-elastic lateral rectus, fibrosis and contracture of the muscle) and
abnormal innervations (hypoplasia of sixth nerve nucleus, paradoxical innervations lateral,
superior rectus or inferior oblique).
Duane’s syndrome is classified into three types:
1.Type I:Characterized by limitation or absence of abduction with widening of the palpebral
fissure , normal adduction with narrowing of the palpebral fissure and retraction. The patients
usually have esotropia .Fig 1,2.
Fig1.Duane’s syndrome type I in the left eye-adduction with narrowing of the palpebral
fissure and retraction.(by courtesy prof.K.Krzystkowa, Cracow, Poland)
Fig2.Duane’s syndrome type I in the left eye-absence abduction with widening of the
palpebral fissure. (by courtesy prof. K.Krzystkowa, Cracow, Poland)
2.Type II: Limitation or absence of adduction with narrowing of the palpebral fissure and
retraction. Normal or slightly limited abduction. This may be associated exotropia.Fig.3,4
Fig.3Duane’s syndrome type II in the right eye. Note the exotropia in the primary position.
(by courtesy prof.K.Krzystkowa, Cracow, Poland)
Fig.4.The same patient with Duane’s type II syndrome. Note absence of adduction with
narrowing of the palpebral. (by courtesy prof.K.Krzystkowa, Cracow, Poland)
3.Type III: Limitation or absence of both abduction and adduction with narrowing of the
palpebral fissure and retraction of the globe on attempted adduction.Fig.5,6
Fig.5.Duane’s syndrome type III-absence of adduction in the left eye.
(by courtesy prof.K.Krzystkowa, Cracow, Poland)
Fig..6The same patient –limitation of abduction in the left eye. .(by courtesy
prof.K.Krzystkowa, Cracow, Poland)
Clinical findings:





see above: type I,II,III
ussually vision is full
stereopsis is good
diplopia occur when the patient attempts to abduct the affected eye
the face turn to have normal binocular vision
Investigation:



ocular movements exam
Hirschberg test
electromyographic studies
Differential diagnosis:






congenital esotropia
accommodative esotropia
divergens insufficiency
deprivation (sensory) esotropia
congenital exotropia
deprivation (sensory) exotropia


non-comitant strabismus
Moebius’ syndrom
Treatment:

surgery is only indicated when funcional and cosmetic aspects can be evaluated
Prognosis:


face turn or strabismus after the surgery can be smaller
sometimes results of the surgery can be dissapointed
Brown’s syndrome
Brown’s syndrome or superior oblique tendon syndrome is caused by short tight superior
oblique tendon and characterized by deficit in elevation during adduction (Fig.7).This
syndrome could be congenital or acquired.
Fig.7.Brown’s syndrome. Note the restriction of the elevation on adduction of the right eye.
.(by courtesy prof.K.Krzystkowa, Cracow, Poland)
Clinical findings:
 active elevation is limited
 also passive elevation on forced duction test is limited
 presence of head posture
Investigation:
 ocular movements exam
 Hirschberg test
 forced duction test
Differential diagnosis:
 inferior oblique palsy
 superior oblique overaction
 double elevator palsy
Treatment:
 surgery is only indicated to attempt
to restor binocular function in primary position
Prognosis:



face turn after the surgery can be smaller
sometimes results of the surgery can be dissapointed
elevating the involved eye in adduction after surgery of the superior oblique is often poor
Moebius Syndrome
Moebius syndrom is a rare congenital disturbance with bilateral abducens and facial nerve
paralysis .(Fig8,,9)
Clinical findings:






unilateral or bilateral inability to abduct the eyes
convergence and vertical movement are intact
vision and papillary constriction are generally normal
complete or incomplete facial palsy
partial atrophy of the tongue
sceletal and muscle defects are common (hypoplasia of the pectoral muscles,
syndactyly,clubfeet,congenital limb amputations)
Fig.8 Moebius syndrom.(by courtesy prof.K.Krzystkowa, Cracow, Poland)
Fig.9 The syndactyly in patents with Moebius syndrom . .(by courtesy prof.K.Krzystkowa, Cracow,
Poland)
Investigation:






Hirschberg test
esotropia about 50 pd
forced duction test are positive to both adduction and abduction
horizontal muscles are often thickened and fibrotic
electromyographic studies have shown waxing and waning of electrical activity
NMR and CT examination
Differential diagnosis:







congenital esotropia
nystagmus blockage syndrome
infantile accommodative esotropia
congenital fibrosis syndrome (fixed strabismus)
congenital myasthenia
congenital six nerve palsy
Duane’s syndrome
Treatment:

surgery for the esotropia consisting of recession of the medial rectus muscles
Prognosis:


usually patients have a little horizontal movements after surgery
reduction of squint is visible
Marcus Gunn’s syndrome
Marcus Gunn jaw-winking is a synkinesis between the nerve supply to the muscles of
masticacion and the levator palpebrae superiosis.See Fig.10,11,12.
Fig.10 Marcus-Gunn syndrome in a7-years old girl. .(by courtesy prof.K.Krzystkowa,
Cracow, Poland)
Fig.11.The girl opens the mouth and the eye lid elevates. (by courtesy prof.K.Krzystkowa,
Cracow, Poland)
Fig.12 The same patient with Marcus-Gunn syndrome-tried to jaw from side to side we see
the elevation of the eye lid. (by courtesy prof.K.Krzystkowa, Cracow, Poland)
Clinical findings:

when the patient opens or close the mouth or jaw from side to side we see the elevation of the
affected lid above its ptotic level.
Investigation:

observation the eyes asocciated with mouth’s movements
Differential diagnosis:

congenital ptosis
*congenital blepharoptosis




telecanthus
epiblepharon
euryblepharon
paresis of III or V cranial nerves
Treatment:

if significant lid retraction with ptosis is present -directed towards disinserting the levator
muscle and suspending the lid
Prognosis:

treatment result are only partially satisfactory
Double elevator palsy
Congenital or acquired double elevator palsy is the paralysis of both the elevators :superior
rectus and levator palpebrae. (Fig.13,14)
Fig.13.Double elevator palsy. Note the ptosis of the right eye.
Fig.14The same patient with restricted the elevation of the right eye.
Clinical findings:





inability or reduced ability to elevate the eye
paretic eye have the hypotropia with ptosis when fixates non-paretic eye
when fixates paretic eye no hypotropia in the primary position and no ptosis
the lid may become ptotic
patient with chin up position to maintain binocularity


diplopia may usually occured in upward gaze
sometimes the pupillary anomalies can occured
Investigation:






Hirschberg test
ocular movements exam
observation the eyes during alternate fixation
forced duction test
observation the head posture
pupillary exam on the light
Differential diagnosis:





congenital ptosis
congenital myasthenia
congenital fibrosis of inferior rectus
blow-out syndrom
Brown’s syndrome
Treatment:




surgery is not indicated if a patient is ortoptic in primary position
if the forced duction test is negative on the paretic eye, the Knapp’s surgery is indicated
if the paralyzed eye fixates then weakening procedure of the elevators of the non-paretic eye is
done
lid surgery also be required if the ptosis not disappear
Prognosis:


only a modest increase in elevation is usually observed after surgery
often surgery give satisfactory results and head posture disappears
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