gms | German Medical Science

The aim of the study was to present a child with exostrabismus of sudden onset, referred the neurologist, who recognised the Dandy-Walker syndrome in this patient.

5-years-old girl, with normal mental development, was examined by ophthalmologist because of exostrabismus of sudden onset associated with nystagmus. Full ophthalmic examination was performed, with evaluation of visual acuity (VA), angle of strabismus, anterior and posterior part of the eye and refraction. Further ophthalmic examination - visual evoked potentials (VEP) and visual field testing with Goldmann perimeter was performed. The child was referred to the neurologist.

Results of the examination: VA: RE 0.9, LE 0.9, with both eyes 1.0, the angle of strabismus -22, full ocular movements, anterior part of the eye - normal, on the fundus: slightly elevated optic disc with blurred margin in upper, nasal and down part, dilated veins. The amplitudes of VEP were diminished and there was a constriction of visual field to 20 degree. Because of changes in nuclear magnetic resonance (NMR) the Dandy-Walker syndrome was recognised. Specialist in neurosurgery found no signs of elevated intracranial pressure and decided to perform control examination after 4 months and careful ophthalmic control.

Because of associated coarctation of the aorta, which was operated previously, partial PHACE-syndrome (- posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, eye abnormalities) was recognised in this patient.