Congenital ocular motor apraxia (COMA) is an uncommon disorder of ocular motility characterized by inability to initiate voluntary horizontal saccades with associated head thrusts during horizontal fixation shifts. In addition, when ocular movements typical of COMA are observed, detailed systemic and neurologic examination should be performed to rule out any associated congenital neurologic or metabolic disorder. We report two cases of COMA, a 14-year-old male with diminution of vision, esotropia, and his face turned to the right without any systemic disease, and a 9-year-old male with esotropia, no face turn, acute lymphocytic leukemia, and isolated complete agenesis of the corpus callosum. Both cases had abnormal saccadic initiation for horizontal gaze with head thrusts to take fixation. Both cases underwent Medial rectus (MR) recession, which improved face turn and strabismus, respectively.
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