Causes, Types, Comorbidities, and Treatments of Duane Retraction Syndrome (DRS)

 

 

Duane Retraction Syndrome (DRS) is a congenital ocular motility disorder caused by miswiring of cranial nerves, primarily affecting abduction/adduction and causing globe retraction and palpebral fissure narrowing.

🔹 Pathophysiology

• Normally, CN III innervates the medial rectus and CN VI the lateral rectus.
• In DRS, CN VI is absent or underdeveloped, and CN III innervates both recti, causing co-contraction.
• Result: globe pulled backward (retraction) during attempted adduction → palpebral fissure narrowing.

🔹 Clinical Presentation & Classification

• Type 1: Abduction limitation (most common), esotropia in primary gaze
• Type 2: Adduction limitation, exotropia
• Type 3: Both adduction and abduction limitation
• Binocular vision and visual acuity are often preserved.
• Abnormal head posture is common to compensate for movement limitation.

🔹 Associated Findings

• Sensorineural hearing loss (16%), spinal anomalies, CNS dysfunction
• Syndromic associations: Goldenhar, Wildervanck, Klippel-Feil

🔹 Management

• Observation if cosmetic and functional issues are minor
• Surgery indicated for significant strabismus in primary gaze, severe retraction, or cosmetic concern

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📋 DRS Summary Table (English)

CategoryDescription

Pathogenesis	CN VI absent or CN III aberrant innervation → co-contraction of recti
Symptoms	Globe retraction, lid fissure narrowing, limited abduction/adduction
Types	Type 1: abduction limitation Type 2: adduction limitation Type 3: both
Associations	Hearing loss, scoliosis, ocular anomalies, CNS disorders
Treatment Criteria	Primary gaze misalignment, abnormal head posture, severe retraction

 

 

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