Introduction
Background
How do normal eye movements occur?
The 6 muscles that control the movement of the eye are attached to the outside of the wall of the eye. In each eye, there are 2 muscles that move the eye horizontally. The lateral rectus muscle pulls the eye out toward the ear and the medial rectus muscle pulls the eye in toward the nose. Four other muscles move the eye up or down and at an angle. Each eye muscle receives the command for movement from cranial nerves that exit the brain.
What is Duane syndrome?
Duane syndrome (DS) is a rare, congenital disorder of eye movement. In most patients, DS is diagnosed by the age of 10 years.
DS is a miswiring of the eye muscles that causes some eye muscles to contract when they should not and other eye muscles not to contract when they should. People with DS have a limited and sometimes absent ability to move their eye outward toward the ear (ie, abduction), and in most cases, they have a limited ability to move the eye inward toward the nose (ie, adduction).
This probably occurs around the sixth week of pregnancy and is due to poor development of tiny parts of the brainstem that control the eye muscles. In DS, the sixth cranial nerve that controls the lateral rectus muscle (the muscle that rotates the eye out toward the ear) does not develop properly. Why the nerve does not develop is not yet understood. Thus, the problem is not primarily with the eye muscle itself but with the nerve that transmits the electrical impulses to the muscle. There is also irregular innervation of a branch from the third cranial nerve, which controls the medial rectus muscle (the muscle that rotates the eye toward the nose). This is why abnormalities may be found in both left gaze and right gaze.
Often, when the eye moves toward the nose, the eyeball also pulls into the socket (ie, retraction), and the eye opening narrows. In some cases, the eye moves upward or downward. Many patients with DS develop a habit of turning their face to maintain binocular vision and thus compensate for improper turning of the eyes. In about 80% of all cases of DS, only 1 eye is affected, most often the left eye. However, in some cases, both eyes are affected; usually, 1 eye is affected more than the other.
In 70% of patients, DS is the only disorder the individual has. However, other conditions and syndromes have been associated with DS. These include malformation of the skeleton, ears, eyes, kidneys, and nervous system, as well as the following: Okihiro syndrome (an association of DS with forearm malformation and hearing loss), Wildervanck syndrome (fusion of neck vertebrae and hearing loss), Holt-Oram syndrome (abnormalities of the upper limbs and heart), morning-glory syndrome (abnormalities of the optic disc or blind spot), and Goldenhar syndrome (malformation of the jaw, cheek, and ear, usually on 1 side of the face).
What is Duane retraction syndrome?
Sinclair (in 1895), Bahr (in 1896), Stilling (in 1887), Turk (in 1899), and Wolff (in 1900) first described Duane retraction syndrome (DRS).1,2,3,4,5 In 1905, Duane reported 54 cases, summarizing all the clinical findings, reviewing previous work, and offering theories on the pathogenesis and treatment of the disease.6
Brown classified the condition into 3 subtypes, based upon clinical observations.7 Type A corresponded to limited abduction and less limited adduction (as described originally by Duane). Type B corresponded to limited abduction but normal adduction. Type C corresponded to limitation of adduction that is greater than limitation of abduction, giving rise to a divergent deviation and a head posture in which the face is turned away from the side of the affected eye.
In 1974, with the support of electromyography (EMG) and following the suggestions of Lyle and Malbran, Huber classified DRS into the 3 types: Duane 1, Duane 2, and Duane 3.8,9,10 Type 1 is marked limitation of abduction (corresponds to Type B in Brown's classification) explicable by maximum innervation reaching the lateral rectus muscle only when the affected eye is adducted. Type 2 is limitation of adduction (corresponds to Type C in Brown’s classification), which Huber explains as being caused by co-innervation of both medial and lateral rectus muscles on attempted adduction. Type 3 is limitation of both adduction and abduction (corresponds to Type A in Brown’s classification), which Huber explains as being due to co-contraction, accompanied by a loss of innervation to the lateral rectus muscle on attempted abduction.
DS is a congenital ocular motility disorder characterized by limited abduction and/or limited adduction. The palpebral fissure narrows (ie, the globe retracts) on attempted adduction. Upward or downward deviation may occur with attempted adduction because of a leash effect. Often associated with this condition is a tether phenomenon consisting of overelevation, overdepression, or both during adduction as the retracted globe escapes from its horizontal rectus restrictions. It is a condition of aberrant innervation that results in co-contraction of the medial and lateral recti in the affected eye. Therefore, DS can be considered to be congenital miswiring of the medial and the lateral rectus muscles such that globe retraction occurs on adduction.
Pathophysiology
Neuropathologic, neuroradiologic, and neurophysiologic data
Findings from neuropathologic, neuroradiologic, and neurophysiologic studies support the hypothesis that DS results from an absence of cranial nerve VI (abducens nerve). Neuropathologic evidence comes from postmortem examinations of individuals who had DS. These studies have shown an absence of cranial nerve VI and its corresponding alpha motor neurons in the pons, as well as aberrant innervation of the lateral rectus muscle by a branch of cranial nerve III.
MRI studies of an individual with DS also revealed the absence of the abducens nerve. Neurophysiologic evidence of neuronal involvement in DS is derived from EMG studies, which show that the medial and lateral recti muscles are electrically active in individuals with DS. However, when individuals with DS attempt to move their eyes inward (ie, adduct it), both of these muscles contract at the same time, causing the eyeball to retract inward and the eye opening (palpebral fissure) to become narrowed. These findings support aberrant innervation of the lateral rectus muscle.
Autopsy specimens have shown agenesis of the sixth nerve nucleus and innervation of the lateral rectus muscle by the third nerve nucleus. This observation explains the globe retraction on attempted adduction. In neuropathologic terms, the cause of DS is an absent sixth nerve nucleus and innervation of the lateral rectus by a branch of the inferior division of the third nerve.
Condensation of the mesoderm around the eye results in development of the extrinsic eye muscles. When the embryo is 7 mm long, they form 1 mass, which is supplied by only the third nerve. When the embryo is 8-12 mm long, that is, when the fourth nerve and the sixth nerve arrive, this mass divides into separate muscles. Because of an absence of or an aplasia of the abducens nerve, a branch of the oculomotor nerve (as a substitute) conceivably enters the part of the muscle mass that is to become the lateral rectus muscle.
Given the evidence that DS results from an absence of the abducens nerve (cranial nerve VI) and that DS is associated with other anomalies in some cases, DS is thought to reflect a disturbance of normal embryonic development. Either a genetic factor or an environmental factor may be involved when the cranial nerves and ocular muscles are developing at 3-8 weeks of gestation.
The association of DS with other ocular anomalies and congenital facial, skeletal, or neural abnormalities in 30-50% of patients further substantiates the hypothesis of disturbed embryogenesis. A teratogenic event during the second month of gestation seems to cause most ocular and extraocular abnormalities observed in combination with DS. DRS is present from birth, even if it is not recognized during infancy. An abnormal head posture and strabismus are often visible in old photographs taken in early childhood.
Genetic and environmental factorsThe genetic basis of DS is being studied, but genetic loci for DS have not. Similar to congenital fibrosis of the extraocular muscles (CFEOM), DS is classified as strabismus, under the subclassification of incomitant strabismus and extraocular muscle fibrosis syndromes. Although the term muscle fibrosis suggests that syndromes under this heading are primary disorders of muscle, evidence suggests that DS (and other syndromes under this heading, including CFEOM) may be primary disorders of nerve innervation. A review of fibrosis syndromes can be found in Engle's article in 1998.11
Both genetic factors and environmental factors are likely to play a role in the development of DS. Most cases of DS cases are sporadic, with only approximately 2-5% of patients having a familial pattern; families with large involvement are rarely reported. Both dominant forms and recessive forms of DS have been documented. In some families with dominant DS, the disease skips a generation (reduced penetrance) and ranges in severity (variable expressivity). Most familial cases are not associated with other anomalies.
Genetic linkage studies of a large family with DS established the location of a DS gene on chromosome 2. Although a genetic cause of DS has long been accepted, these studies were the first to show a statistically significant linkage. In addition, cytogenetic results of individuals with DS have shown, in rare cases, abnormalities that suggest the contribution of more than 1 gene. Deletions on chromosomes 4 and 8 and an extra marker chromosome thought to be derived from chromosome 22 have been documented in individuals with DS.
Familial occurrence with dominant inheritance patterns has been reported. DS has also been described in monozygotic twins. However, most cases are sporadic rather than familial. Pairs of identical twins with mirror-image DS have been described. Its most common variant (type 1, 85%) and most commonly manifests in the left eye (60%) and in girls (60%) as severely limited or absent abduction. In 90% of cases, the patient has no family history of DS. Ten percent of patients will have an affected family member, and these tend to be cases where both eyes are involved. There is currently no test that can determine whether a patient has a hereditary form.
See also the Causes section.
Frequency
United States
The frequency of DS in the general population of individuals with eye movement disorders (strabismus) is approximately 1-5%. The most common clinical presentation is type 1 DS (70-80%) followed by type 2 (7%) and type 3 (15%). Involvement of both eyes is less common than involvement of 1 eye only. Approximately 80% of cases are unilateral. Of the unilateral cases, the left eye is most often affected (60-72%).
See also the International section.
International
DS accounts for 1% of all cases of strabismus. Unilateral retraction syndrome is the rule; the left eye is distinctly involved in 60% of cases, the right eye is involved in 20% of cases, and both eyes are involved in 20% of cases.
Mortality/Morbidity
About 40% of patients develop esotropia and tight medial rectus muscles; therefore, they adopt a head turn toward the eye to maintain single binocular vision, or they maintain a straight head but accept esotropia, abnormal retinal correspondence (ARC), and suppression, if available.
In about 30% of patients, compensatory head positions are necessary to achieve binocular single vision. Before surgery is contemplated, coexisting and clinically significant refractive errors, anisometropia, and amblyopia must be treated. Kirkham found that amblyopia was present in 21% of his patients with DRS, and nearly 50% had anisometropia.12 In 2 large series, the incidence of amblyopia in DRS patients was 10% and 14%, respectively. However, Maruo and coworkers found a 3.6% prevalence of amblyopia among 220 patients with DRS.13 Tredici and von Noorden also reported a 3% incidence of amblyopia in 72 patients and commented that this incidence is similar to that in the general population.14,15
Race
No racial preference is reported.
Sex
The female-to-male ratio is 3:2, but this female predominance is not explained.
Age
This syndrome is a congenital anomaly. Most cases are diagnosed by the age of 10 years.
Clinical
History
Patients can present with a history of the following:
- Strabismus
- Head tilt
- Loss of binocular vision
- Reduced abduction
- Picture of paralytic squint
Physical
DS can be isolated, or it can be associated with other congenital anomalies. DS is an isolated finding in approximately 70% of patients, but it may be associated with other malformations. Major anomalies associated with DS can be grouped into 4 categories: skeletal, auricular, ocular, or neural. DS can also be associated with other well-defined syndromes, including Okihiro syndrome, Wildervanck syndrome, Holt-Oram syndrome, morning-glory syndrome, and Goldenhar syndrome.
DS can be associated with both ocular anomalies and systemic anomalies. Ocular anomalies commonly associated with DRS include dysplasia of the iris stroma, pupillary anomalies, cataracts, heterochromia, Marcus Gunn jaw-winking, coloboma, crocodile tears, and microphthalmos. Systemic anomalies include Goldenhar syndrome, Klippel-Feil anomalad, and congenital labyrinthine deafness.
Most cases are sporadic. The congenital anomaly consists of absence of the abducens nerve nucleus in the brainstem and simultaneous innervation of the affected lateral rectus muscle by the inferior branch of the oculomotor nerve. The left eye is involved in 60% of cases. DS is often bilateral but asymmetrical.
Clinical types of Duane syndrome
DS is often clinically divided into 3 types (see the Table below). Different clinical types may be present within the same family; this occurrence suggests that the same genetic defect may produce a range of clinical presentations. The clinical spectrum of different types of DS result from the variability of the 3 innervational portions of the lateral rectus muscle, namely, the normal abducens innervated portion, the abnormal oculomotor innervated portion, and the noninnervated fibrotic portion.
In DS type 1, the ability to move the affected eye outward toward the ear (abduction) is limited, but the ability to move the affected eye inward toward the nose (adduction) is normal or nearly so. The palpebral fissure narrows, and the eyeball retracts into the orbit when the patient looks inward toward the nose (adduction). When he or she looks outward toward the ear (abduction), the reverse occurs.
In DS type 2, adduction of the affected eye is limited, whereas abduction of the eye is normal or only slightly limited. The palpebral fissure narrows, and the eyeball retracts into the globe when the affected eye attempts to adduct.
In DS type 3, adduction and abduction of the affected eye is limited. The palpebral fissure narrows, and the eyeball retracts when the affected eye attempts to adduct.
These 3 types can be further classified into 3 subgroups designated A, B, and C to describe the eyes when looking straight (in primary gaze). In subgroup A, the affected eye is turned inward toward the nose (esotropia). In subgroup B, the affected eye is turned outward toward the ear (exotropia). In subgroup C, the eyes are in a straight primary position.
Types of DSOpen table in new window
Table
| Category | Description |
| Type | |
| 1 (70%-80%) | Inability to abduct Normal or minimal defect in adduction Esotropia with head straight A or V pattern Usually updrift or a downdrift of affected eye on adduction or attempted abduction Globe retraction and palpebral-fissure narrowing on adduction Usual face turn to affected side Normal stereo possible |
| 2 (about 7%) | Inability to adduct Normal or minimal defect in abduction Exotropia of the affected eye Marked upshoot Globe retraction and palpebral-fissure narrowing on adduction Stereo normal or suppressed Face turn to normal side |
| 3 (about 15%) | Inability ability to abduct and adduct Globe retraction and palpebral-fissure narrowing on attempted adduction Possible upshoot and downshoot on adduction Straight or nearly straight head position Usually, normal stereo |
| 4 | Not usual DS or DS-type appearance Large-angle exotropia Face turn to uninvolved side Limited adduction Simultaneous abduction when looking toward uninvolved side Usually suppresses 1 eye |
| Subtype | |
| A | Affected eye turned inward toward the nose (esotropia) |
| B | Affected eye turned outward toward the ear (exotropia) |
| C | Eyes in a straight primary position |
| Category | Description |
| Type | |
| 1 (70%-80%) | Inability to abduct Normal or minimal defect in adduction Esotropia with head straight A or V pattern Usually updrift or a downdrift of affected eye on adduction or attempted abduction Globe retraction and palpebral-fissure narrowing on adduction Usual face turn to affected side Normal stereo possible |
| 2 (about 7%) | Inability to adduct Normal or minimal defect in abduction Exotropia of the affected eye Marked upshoot Globe retraction and palpebral-fissure narrowing on adduction Stereo normal or suppressed Face turn to normal side |
| 3 (about 15%) | Inability ability to abduct and adduct Globe retraction and palpebral-fissure narrowing on attempted adduction Possible upshoot and downshoot on adduction Straight or nearly straight head position Usually, normal stereo |
| 4 | Not usual DS or DS-type appearance Large-angle exotropia Face turn to uninvolved side Limited adduction Simultaneous abduction when looking toward uninvolved side Usually suppresses 1 eye |
| Subtype | |
| A | Affected eye turned inward toward the nose (esotropia) |
| B | Affected eye turned outward toward the ear (exotropia) |
| C | Eyes in a straight primary position |
- Clinical manifestations
- The aberrant innervation produces several manifestations. These manifestations include a face turn with strabismus in primary position, an upshoot or downshoot during adduction, a vertical deviation in primary position, retraction during adduction, and enophthalmos.
- On occasion, fusion is impossible in a patient with DS, usually because bilateral involvement or a vertical deviation precludes fusion with any head posture.
- DS is characterized in its typical and most frequently observed form by abduction deficiency, globe retraction and palpebral fissure narrowing on attempted adduction, widening of the palpebral fissure on attempted abduction, and normal or only slightly defective adduction.
- Sensory adaptation
- Most patients with sensory adaptation demonstrate a peculiar sensory adaptation with excellent binocular functions in directions of gaze where visual axes are aligned and usually suppression without diplopia in the field of the paretic muscles.
- Some suggest that the second image is ignored, rather than suppressed, in view of the difficulties encountered in plotting the suppression scotoma.
- Signs of DS include the following:
- Head turn to maintain fusion (about 66% of cases, 76% of unilateral cases)
- Strabismus (about 77% of cases)
- Globe retraction
- Palpebral fissure narrowing
- Anisometropia and/or amblyopia (though not more prevalent than in the general population)
- Heterochromia
- Iris dysplasia
- Ptosis
- Nystagmus
- Choroidal coloboma
- Hypoplasia of the optic nerve
Clinical evaluation
A thorough family history and an ocular examination are conducted, with special attention to other ocular or systemic malformations. Measurements of visual acuity, ocular misalignment, ocular range of motion, head turn, globe retraction, size of the palpebral fissure size, and upshoots and downshoots are indicated. In addition, an examination of the cervical and thoracic spine, palate, vertebrae, hands, and hearing is recommended to rule out disorders associated with DS.
Differential diagnosis and other problems to be consideredOne type of inherited DS that is associated with other birth defects is DS with radial-ray anomalies. The association of DS with radial-ray anomalies is referred as Duane/radial dysplasia syndrome, DR syndrome (acronym for Duane anomaly, deafness, radial dysplasia, renal dysplasia), and Okihiro syndrome.
The familial association of DS with radial-ray anomalies appears to be autosomal dominant with incomplete penetrance and variable expressivity. DS can be unilateral or bilateral. Radial dysplasia can range from hypoplasia of the thenar eminence (base of the thumb) with or without thumb abduction and apposition weakness, hypoplasia or absence of the thumb, and hypoplasia or absence of the radial and ulnar bones to an absent forearm. Hearing loss, dysmorphic facies and cardiac, renal, and vertebral anomalies are variably expressed in some families. The chromosomal location of the gene for this syndrome is unknown, but families are being enrolled to identify the gene or genes involved in this condition.
Other diagnostic considerations include medial-wall fracture with incarceration of orbit contents might be a consideration; retraction of globe with attempted abduction is seen on examination. Retraction of convergent nonfixating eye with associated loss of conjugate lateral gaze and occurrence of the near reflex on attempted lateral gaze might also be considered. Because DS mimics sixth nerve paresis, the differential diagnoses can be similar to those described for sixth nerve paresis.
In 1977, Duane and coauthors described entirely unrelated pseudo-DS in which abduction is restricted and narrowing of the palpebral fissure and retraction of the globe occur after fracture of the medial orbital wall with entrapment of the medial rectus muscle.16
Causes
DS is thought to be a miswiring of the medial and the lateral rectus muscles, or the muscles that move the eyes. Also, patients with DS lack the abducens nerve, or the sixth cranial nerve, which is involved in eye movement. However, the etiology or origin of these malfunctions remains a mystery. Many researchers believe that DS results from a disturbance due to either genetic factors or environmental factors during embryonic development. Because the cranial nerves and ocular muscles are developing between the third and eighth weeks of pregnancy, this is most likely when the disturbance happens. It appears that several factors may be involved in DS, and a single mechanism is unlikely to be responsible for this condition.
Although DS has been well described clinically, the etiology remains unclear. Various theories have been put forward on the basis of data collected from surgical, autopsy, and EMG studies. A high prevalence of ocular and systemic disorders associated with DS suggests that a common teratogenic stimulus at 8 weeks of gestation may cause this problem. The most noted and believed theory is based on an absence of cranial nerve VI, an absence of the abducens nucleus in the pons, and a marked abnormality of the lateral rectus muscle.
Most likely, both genetic factors and environmental factors play a role in the development DS. In patients with evidence of a genetic cause, both dominant forms and recessive forms of DS have been found. The chromosomal location of the proposed gene is currently unknown. Research shows that more than 1 gene may be involved. Evidence suggests that the gene involved in the development of DS is located on chromosome 2. Also, deletions of chromosomal material from chromosomes 4 and 8, as well as an extra marker chromosome thought to be derived from chromosome 22, have been linked to DS. See also Genetic and environmental factors in the Pathophysiology section.
- Structural anomalies
- In the early literature, most authors believed that structural anomalies were the cause of the retraction phenomenon. People found posterior insertion of the medial rectus muscle, which acts as a retractor bulbi.
- von Noorden found a broad, flat, tendinous band containing a few visible fibers inserted into the sclera at a point 6 mm behind the medial rectus insertion.15
- Turk believed that fixation of the globe by a nonelastic lateral rectus muscle was the cause of retraction on adduction.4
- The abnormal vertical eye movement that frequently occurs with adduction has been blamed on oblique overaction. Narrowing of the palpebral fissure usually is explained as passive adjustment of the lid to retraction of the globe, rather than active innervation of the orbicularis muscles.
- Innervational anomalies
- Evidence accumulated from EMG studies has indicated an innervational mechanism rather than anatomic abnormalities as the cause of this syndrome.
- The currently favored theory is that DS is a neurogenic disorder involving either a supranuclear lesion or a cranial nerve anomaly in which branches of the oculomotor nerve innervate the lateral rectus.
- Hoyt and Nachtigaller have proposed that ocular retraction during adduction, as well as the EMG findings of synergistic innervation of medial and lateral rectus muscle, can be explained on the basis of substitute innervation of the paretic lateral rectus muscle by an extra branch of the oculomotor nerve.17
- Primary brainstem anomaly
- On autopsy, authors at the Wilmer institute found the absence of abducens nuclei and nerves from the brainstem. Matteucci has described absence of abducens nerve and hypoplasia of its nucleus.18
- Given the clinical evidence, anomalies of the vestibulo-ocular reflex, auditory evoked responses, optokinetic nystagmus, and frequent association of gustatolacrimal reflex (crocodile tears) with DS suggests that a primary brainstem abnormality is a likely cause in at least some cases.
- Iatrogenic factors
- DS may occur on an iatrogenic basis unrelated to congenital, mechanical, or innervational anomalies. von Noorden reports a patient who had normal ocular motility and developed retraction syndrome after massive scarring following removal of a dermolipoma from the temporal aspect of the bulbar conjunctiva of the left eye.15
- At this time, several etiology factors may be involved, and it is doubtful that a single mechanism is responsible for this disturbance.
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Further Reading
Keywords
DS, Duane's syndrome, Duane retraction syndrome, DRS, congenital retraction syndrome, DRS, Stilling-Turk-Duane retraction syndrome, Duane radial dysplasia syndrome, Duane-Stilling-Turk syndrome, Stilling's syndrome, Stilling syndrome, Turk's syndrome, Turk syndrome, Turk-Stilling syndrome, Duane 1, Duane 2, Duane 3
