From the Guidelines
Surgery is the best alternative to correct the large-angle esotropia and frequent hypotropia and to normalize ocular rotations in patients with Duane syndrome. The management approach for Duane syndrome involves a comprehensive evaluation to determine the severity of the disease and to plan the surgery 1.
Key Considerations
- Measuring the angle between the superior rectus muscle and the lateral rectus muscle (dislocation angle) is helpful in determining the severity of the disease and for planning the surgery 1.
- A forced duction test to determine if limitation to ocular rotation is the result of a medial rectus muscle restriction or, more rarely, an orbital mechanical restriction resulting from the increased axial length and adjacency of the globe to the lateral orbital wall should be performed at the onset of surgery 1.
- If the forced duction test confirms restriction to abducting rotation, the medial rectus muscle should be recessed 1.
- Medial rectus recession is typically accompanied by one of several muscle union procedures that join the superior rectus to the lateral rectus to reposition the muscle paths 1.
Surgical Approaches
- The classical approach is the Yokoyama procedure, a vessel-sparing suture union of the superior rectus and lateral rectus 15mm posterior to their insertions, typically accompanied by a medial rectus recession 1.
- Many variants have been described, including union by a silicone sleeve and the use of a three-suture SR-LR union to successfully reduce more extreme degrees of esotropia 1.
Special Considerations
- In some cases of very long axial myopia and large esotropia, prolapse of the globe beyond the confines of the superior and lateral rectus may not be present, and such cases can be surgically managed the same as for sagging syndrome or basic divergence insufficiency 1.
- Referral to a retina specialist for preoperative evaluation should be considered to ensure that progressive myopic degeneration is not associated with active retinal pathology requiring treatment prior to strabismus surgery 1.
From the Research
Management Approach for Duane Syndrome
The management approach for Duane syndrome is challenging and must be individualized based on the amount of ocular deviation, abnormal head position, associated globe retraction, and overshoots 2, 3.
- Surgical Management: Surgical approach is the primary management strategy for Duane syndrome, aiming to achieve alignment in the primary gaze, reduction in globe retraction, upshoots, and downshoots, and correction of any abnormal head posture 2, 3, 4.
- Differential Recessions: Differential recessions of the lateral and medial rectus muscles are done to correct esotropia or exotropia in the primary gaze 2.
- Y-Split or Periosteal Fixation: Y-split or periosteal fixation of the lateral rectus muscles is done to address globe retraction and shoots, depending on the severity 2.
- Classification-Based Management: Management is also based on the classification of Duane syndrome, with type I being the most common form and type III being the least common 3, 4.
- Individualized Approach: The surgical approach must be individualized based on the specific characteristics of each patient, including the amount of ocular deviation, abnormal head position, and associated anomalies 3, 4.
Key Considerations
- Variable Presentation: Duane syndrome presents with variable horizontal duction deficits, globe retraction, and narrowing of the palpebral aperture, making management challenging 2, 3, 5.
- Aberrant Innervation: The disease is characterized by aberrant innervation of the lateral rectus muscle by the oculomotor nerve, leading to the clinical manifestations of Duane syndrome 5, 6.
- Genetic Factors: Duane syndrome has a genetic component, with several chromosomal loci suggested to contribute to the disease 6.