Ocular manifestations in Arnold Chiari syndrome

Purpose: Arnold‐Chiari disease is a rare congenital malformation of the cerebellum. Symptoms are dominated by occipital headache, torticollis, and sometimes swallowing disorders. Regarding ophthalmological manifestations, convergence abnormalities, oculomotor paralysis and diplopia are the main clinical signs. We report two cases of ocular manifestations in Arnold Chiari type 1 syndrome. Methods: Two case reports and a literature review. Results: Case 1: A 40‐year‐old patient with no medical history presented an acute loss of vision in both eyes. On examination, the patient had a visual acuity limited to 5/63. Biomicroscopic examination of the anterior segment was without abnormalities. Fundus examination revealed a bilateral papillary edema. Visual field test showed a borderline diffuse loss in both eyes. Case 2: A 17‐year‐old patient with no medical history presented intermittent headaches and a progressive loss of vision in both eyes. On examination, the patient had a visual acuity limited to 5/80. Biomicroscopic examination of the anterior segment was without abnormalities. Fundus examination revealed atrophy not excavated in both eyes. Visual field test showed nasal and temporal scotomas in both eyes. Temporal, superior and inferior RNFL defects were detected in both eyes. A brain MRI revealed a Chiari type 1 malformation without associated hydrocephalus in both patients. Conclusions: Arnold Chiari type 1 malformation is an uncommon pathology. The discovery of ophthalmologic signs requires a neurosurgical intervention as soon as possible to stop progression towards the loss of visual function by optic atrophy.