Nondecussating retinal-fugal fiber syndrome: Case report of a rare cause of congenital nystagmus
Journal Title: Medpulse International Journal of Ophthalmology - Year 2018, Vol 8, Issue 3
Abstract
Congenital nystagmus is a condition presenting itself in neonatal period or early infancy and Persists throughout the life. The overall prevalence of nystagmus is reported to be between 2-3 cases per 10000 individuals. In majority of the cases the nystagmus is pendular or see saw. Though there are numerous causes of congenital nystagmus, congenital absence of optic chiasma is one of the rare causes. It is usually diagnosed on Magnetic resonance Imaging which is frequently done in infants presenting with nystagmus. We present here a case of 3 years old female child who presented to us with history of nystagmus since infancy and was found to be having congenital absence of optic chiasma on MRIimaging.Case Report:3 years old female child was brought to OPD with the complaints of decreased vision in both eyes and abnormal eye movements. No significant family history. There was also a history of delayed developmental milestones in the form of delayed neck holding. On torchlight examination there was presence of pendular nystagmus. Slit lamp examination was in normal limits. Dilated Fundoscopy was normal. An MRI was ordered which showed absence of optic chiasma. In view of imaging findings, a diagnosis of congenital pendular nystagmus due to achiasma was made and bilateral Medial and lateral rectus recession surgery was done. Flash Visual evoked potential showed normal amplitudes with good reproducibility bilaterally whereas pattern Visual evoked potentials showed reduced amplitude with delayed latency for larger checker and extinguished peaks for smaller checker pattern in right eye and extinguished peaks for larger checker pattern in left eye. Patient was discharged with glasses prescription.Conclusion:Though rare, possibility of congenital achiasma remains undiagnosed unless MRI is advised.
Authors and Affiliations
JKavya Rao, Maheshwari .
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