|
SYMPTOMS
|
Diplopia, Typically normal vision
|
|
SIGNS
|
Typically unilateral
|
|
Non-progressive strabismus in primary gaze with compensating head position to align the eyes, May see upshoots or downshoots with adduction (Leash phenomenon), Globe retraction with narrowing of the palpebral fissure when adducting, Possible amblyopia, Hyperopic refractive error that is typically greater than +1.50, Poor binocular vision, Possible morning glory optic nerve
|
|
Type I (most common presentation): Esotropia in primary gaze with a head turn toward the affected side. Limited or absent abduction with normal or slightly limited adduction
|
|
Type II: Exotropia in primary gaze with a head turn towards the side that is not affected. Limited or absent adduction with normal or slightly limited abduction
|
|
Type III: Either an esotropia or exotropia with a head turn toward the affected side. Limited or absent abduction and adduction
|
|
WORK-UP
|
Pupils, EOMs, Cover test, Cycloplegic refraction, Full eye exam with dilation, Strabismus evaluation, Forced duction testing
|
|
TREATMENT
|
Prescribe glasses with best correction and possible prism
|
|
Recommend amblyopia/strabismus therapy if needed
|
|
Refer to a pediatric ophthalmologist for a surgery consult especially if one of the following is present: A head turn that is ≥ 15 degrees, A significant deviation in primary gaze, Reduction of the palpebral fissure ≥ 50% when adducting
|
|
Refer to a pediatrician if suspecting a systemic association
|
|
FOLLOW-UP
|
If prescribing correction with or without prism, the patient should be seen back in 1 month, 3-6 months, and then yearly
|
|
If getting ocular surgery, the patient should be seen back once stable for a follow-up and refraction
|
|
ADDITIONAL LAB | TESTS
|
About 30% of patients with duanes retraction syndrome can be associated with other congenital anomalies and should be ruled out: Okihiro syndrome, Wildervanck syndrome, Moebius syndrome, Holt-Oram syndrome, Goldenhar syndrome
|
|
ETIOLOGY
|
Congenital cranial dysinnervation disorder: Abnormal development of the pontine abducens nucleus or nerve which causes poor innervation of the lateral rectus muscle (this will lead to fibrotic changes in the lateral rectus muscle). There is also aberrant innervation of the lateral rectus by the oculomotor nerve. May also be associated with polymorphisms such one involving CHN1
|
|
DIFFERENTIAL DX
|
Brown syndrome, Cranial nerve 6 palsy, Congenital esotropia
|
|
NOTES
|
The majority of cases involving duanes retraction syndrome are isolated
|
|
Patients with a polymorphism of CHN1 may also have involvement of Cranial nerve 2, 3, and 4
|
