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A-typical Fixation in Strabismal Amblyopia as First Manifestation of an Arachnoid-cyst

Krist D., Gusek-Schneider G. C.,
Friedrich-Alexander-Universität, Augenklinik (Erlangen)

Background: Arachnoidal cysts are cerebrospinal fluid collections within the leptomeninges. Usually they represent a congenital malformation and are asymptomatic. However, an enlargement of the cyst may lead to acute neurologic or ocular symptoms.
Patient: A 5-year old girl presented with esotropia on the right eye, manifested in the first year of her life. Visual acuity measured 0.2 in the right eye and 1.0 in the left eye (Cyclo-RM: RA: + 2.0 sph LA: + 0.5 sph). There was a latent jerk nystagmus to the left. Further ophthalmological investigation was unremarkable. Fixation showed a pendular nystagmus with perifoveal excursions on the right eye and central fixation on the left eye. Previous neuro-pediatric investigations including imaging techniques didn't reveal any changes. In spite of intensified occlusion therapy and fixation training there was a further loss of vision to 1/7 reading chart in 1m with unchanged fixation after 1 year. Therefore the neuro-pediatric examination was repeated.
Results: Standard neurological examination didn't show any pathologic changes. Because of an abnormal VEP a MRI was performed. It revealed a large arachnoid cyst in the temporal lobe without any signs of optic compression. Two years later an additional papillary atrophy with corresponding visual field defects was observed. A newly performed MRI scan showed an enlargement of the arachnoidal cyst. Therefore a cysto-peritoneal shunt was performed. Postoperative examination showed improvement of ophthalmological symptoms.
Conclusions: In deep amblyopia associated with strabismus and atypical fixation extra-ocular anomalies have to be excluded using adequate imaging techniques. Because of the potential to rapid progression of cerebral lesions a primarily unremarkable result may have to be confirm later, especially if visual acuity is unresponsive or even decreasing in spite of occlusion therapy and fixation training.

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