Congenital extraocular muscular defects

Published on Mar 1, 1992in Eye2.455
· DOI :10.1038/eye.1992.36
John P. Lee20
Estimated H-index: 20
(Moorfields Eye Hospital)
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Abstract
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• Duane's retraction syndrome (DRS) is a congenital eye movement disorder characterized by abduction deficiency, adduction limitation, globe retraction, and palpebral fissure narrowing on attempted adduction. In a case of unilateral DRS, the right side of the brainstem, cavernous sinus, and orbit were completely normal. The left abducens nucleus contained no cell bodies from motor neurons, but in its rostral portion, it contained several small cell bodies believed to be compatible with internucl...
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Abstract Congenital absence of the superior oblique tendon occurred in 18% of patients with a diagnosis of congenital superior oblique palsy who were treated with an attempt at a tuck of the superior oblique tendon. The presence of horizontal strabismus and amblyopia in a patient with diagnosis of congenital superior oblique palsy should raise the possibility of absence of this tendon. Absence of the superior oblique tendon can be treated successfully with weakening of the antagonist inferior ob...
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• Duane's retraction syndrome (DRS) is a congenital eye movement disorder characterized by abduction deficiency, adduction limitation, globe retraction, and palpebral fissure narrowing on attempted adduction. Although data from surgical and autopsy studies suggest a neurogenic cause for this disorder, only one welldocumented case of DRS has been studied pathologically, and that report was incomplete. We describe the intracranial and orbital pathology of a clinically documented case of bilateral ...
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: Two cases with bilateral Duane's Syndrome are presented. Both showed immediate large angle exotropia following bilateral medial rectus recession for esotropia. In both cases, the forced duction test was then found to be positive in adduction and negative after disinsertion of the lateral rectus. It is suggested that both the medial rectus muscles and lateral rectus muscles are tight in bilateral Duane's Syndrome and that seemingly simultaneous recession of the medial and lateral rectus muscles...
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Background Branching and/or replication of the abducens nerve is not an uncommon occurrence. Although numerous variations have been documented, the rarest forms are duplicated or triplicated nerves, where multiple nerve roots originate from the brainstem, travel intracranially, and attach to the lateral rectus as separate entities. Methods We conducted a systematic literature search on the topic of supernumerary abducens nerve, using PubMed and Google Scholar. Results After screening, 16 studies...
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We report a peculiar case of congenitally restricted sursumduction caused by an anomalous band-like structure within the right orbit which was completely distinct from the extraocular muscles. Imaging
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Duane's retraction syndrome (DRS) is a well-described entity. However, the possibility of this condition having progressive features has not been previously documented. We studied 21 consecutive patients with this disorder and found that the incidence of severe retraction on adduction, the occurrence of enophthalmos in the primary position and the presence of upshoots and downshoots were significantly higher in adults with type I DRS than in children. Awareness that the clinical features may inc...
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A characteristic pattern of dissociated eye movements was observed in a large proportion of our patients with a variety of craniosynostosis syndromes. These anomalies simulate overaction of the inferior oblique and underaction of the superior oblique muscles which, however, cannot fully explain the abnormalities. In a number of cases, excyclorotation of the muscle cone was observed, with the upper pole of the eye tilted away from the midline. It is postulated that such excyclorotation of the eye...
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