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The authors present a case report of Duane retraction syndrome (DRS) associated with Rubinstein-Taybi syndrome (RTS). RTS is a multisystem condition characterized by psychomotor developmental delay, broad thumbs and great toes, short... more
The authors present a case report of Duane retraction syndrome (DRS) associated with Rubinstein-Taybi syndrome (RTS). RTS is a multisystem condition characterized by psychomotor developmental delay, broad thumbs and great toes, short stature, and characteristic facies with a beaked nose. DRS is an ocular disorder characterized by retraction of the globe with narrowing of the palpebral fissure in attempted adduction, with abduction and/or adduction deficiency, and, frequently, upshoot or downshoot of the affected eye on adduction. The case report described represents the first reported case of DRS associated with RTS, an association not previously recognized. This association raises the possibility that the abnormal CNS development in RTS may occasionally involve the ocular motor nerves.
To evaluate the results of extraocular surgery in patients with large exotropias from... more
To evaluate the results of extraocular surgery in patients with large exotropias from "wall-eyed" bilateral internuclear ophthalmoplegia (WEBINO). Eight patients with WEBINO evaluated between November 1994 and October 2006 underwent extraocular muscle surgery. Three patients required a second procedure. An 85% reduction in exodeviation occurred postoperatively. Fusion responses were demonstrable in 87.5% of patients. Other ocular signs of internuclear ophthalmoplegia (INO) such as adduction limitation, abducting nystagmus, and ocular dysmetria also improved. These results were obtained following two-staged unilateral recession-resection procedures in seven patients; the remaining patient underwent bilateral lateral rectus recessions. Binocular outcomes are achievable in patients with WEBINO following extraocular muscle surgery, and associated ocular signs may be reduced or eliminated.
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