Bilateral Duane's retraction syndrome. A clinical-pathologic case report

Abstract

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 well-documented 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 DRS. Both abducens nuclei and nerves were absent from the brainstem, and the lateral rectus muscles were partially innervated by branches from the oculomotor nerves. This report presents evidence that a cranial nerve anomaly may cause DRS. The reason for this neurogenic abnormality is briefly discussed.