Dr.SARTHAK SURESH KOTHARI, K20241
Case 1: 12 yrs girl, presented with complains of defective vision in both eyes (LE > RE). Her BCVA was 6/12 in RE & 6/36 in LE. On examination, anterior segment was normal except for slowly reacting pupils and rotatory nystagmus. Fundus revealed LE optic nerve head hypoplasia. Systemic examinations was normal. MRI brain scan showed B/L schizencephaly and absent septum pellucidum – confirming diagnosis of De Morsier Syndrome.
Case 2: 4 yrs boy, presented with complains of abnormal movement of eyes. His BCVA was 6/15 in both eyes. On examination, anterior segment was normal except for nystagmus – jerky nystagmus with rotational component, up beating in up gaze, horizontal beating in other gazes. Fundus revealed B/L optic disc pallor. On systemic examination, he had clasp knife spasticity, brisk deep tendon reflexes and lurching gait. CT scan showed U/L open lip schizencephaly.
Conclusion: Not all congenital nystagmus are benign and some may require imaging to find out underlying lesion.


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