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| + | ====== Duane Syndrome ====== | ||
| + | ===== Description ===== | ||
| + | |||
| + | * Congenital abnormality of the 6th cranial nerve | ||
| + | * 4th-8th week of gestation | ||
| + | * Missing nucleus in most cases (4th week) | ||
| + | * Abnormal innervation from 3rd cranial nerve | ||
| + | |||
| + | ^Type^Abduction^Adduction^Deviation^Frequency^ | ||
| + | |I|Poor|Normal|Ortho- or Esotropia|70%| | ||
| + | |II|Normal|Poor|Ortho- or Exotropia|15%| | ||
| + | |III|Poor|Poor|Ortho-, | ||
| + | |||
| + | ===== Clinical Characteristics ===== | ||
| + | |||
| + | * Most sporadic, 5% autosomal dominant inheritance | ||
| + | * More common in females than males. | ||
| + | * OS affected more often than OD. | ||
| + | * 80% of cases are unilateral, when bilateral it is usually asymmetric. | ||
| + | * Gender and Laterality: | ||
| + | |||
| + | ^Female^Male^OS^OD^OU^ | ||
| + | |58%|42%|59%|23%|18%| | ||
| + | |||
| + | * Gender and Laterality in 835 cases of Duane Syndrome. DeRespinis PA, Wagner RS, Guo S; Duane’s Retraction Syndrome. Surv Ophthalmol 38:257, 1993 | ||
| + | |||
| + | ===== Exam Findings ===== | ||
| + | * Narrowing of the palpebral fissure and retraction of the globe of the involved eye on attempted adduction. | ||
| + | * The amount of limitation depends on the amount of co-contraction of the medial rectus and lateral rectus. | ||
| + | * Abnormal firing of the lateral rectus is found with EMG testing. | ||
| + | * Angle of deviation almost always less than 30D , usually less than 15D . | ||
| + | * Face turn toward the side of limited movement- permits fusion. | ||
| + | * Anisometropic Ambylopia 10-30% | ||
| + | * Ipsilateral congenital hearing loss 5% | ||
| + | |||
| + | ===== Selected Associated Syndromes ===== | ||
| + | |||
| + | * Most cases are isolated | ||
| + | * **Goldenhar syndrome** (hemifacial microsomia, ocular dermoids, ear abnormalities, | ||
| + | * **Wildervanck syndrome** (sensorineural hearing loss, Klippel-Feil anomaly- shortness of neck with fused or missing vertebrae) | ||
| + | |||
| + | ===== Treatment ===== | ||
| + | |||
| + | * Treat Refractive Error | ||
| + | * Consider treating Hyperopia > +2.50 if ET | ||
| + | * Treat Amblyopia | ||
| + | * Goals of Surgery: | ||
| + | * Improve primary position alignment | ||
| + | * Improve head turn | ||
| + | * Decrease up-shoot or down-shoot | ||
| + | * Surgical Treatment Possibilities | ||
| + | * Eso-Duanes | ||
| + | * Medial Rectus (MR) Recession | ||
| + | * Bilateral Medial Rectus Recessions | ||
| + | * Superior Rectus and/or Inferior Rectus transpositions | ||
| + | * Exo-Duanes | ||
| + | * Lateral Rectus (LR) Recession(s) | ||
| + | * Up-shoots/ | ||
| + | * Recession with Y split or posterior fixation of LR | ||
| + | |||
| + | {{tag> | ||