@entity1645 is defined as a herniation of the cerebellar tonsils into the foramen magnum of more than 5 mm. Symptoms are most commonly dominated by @entity10 , torticollis and sometimes swallowing disorders. Ophthalmologically abnormal convergences, oculomotor palsy and @entity892 are the main clinical signs. We report the case of a 9 year old child, who presented with @entity227 since 6 months. Ophthalmologic examination showed visual acuity of 4/10 in both eyes, @entity1319 and @entity717 . The examination of the anterior segment of the eye showed @entity28180 with no evidence of @entity18382 , iridodonesis associated with @entity194 of the dilator muscle and microcoria with lazy photomotor reflex. @entity2790 was 14 mmHg. Ocular fundus examination, despite difficulties in performing it, objectified @entity1829 (stage II). General physical examination showed torticollis, @entity1635 and a @entity69296 . MRI showed @entity1645 associated with @entity1276 and @entity1644 . Neurosurgical intervention based on internal CSF drainage with occipitocervical osteo-dural decompression was proposed. The evolution was favorable with regression of clinical signs. Ophthalmologically, there was a regression of @entity1829 but visual acuity remained stationary. The occurrence of @entity1829 associated with @entity1645 is rare, it has been only reported in 2% of symptomatic patients. Its pathophysiology is still poorly understood. The originality of our study consists in the association of @entity76 with @entity735 including @entity28180 and microcoria which make ophthalmologic examination more difficult to perform.

[Papillary oedema revealing XXXX : about a case].

@entity227 :: ('MESH:D014786', 'Disease') :: ['visual loss evolving'] @entity1644 :: ('MESH:D013595', 'Disease') :: ['syringomyelia'] @entity1276 :: ('MESH:D006849', 'Disease') :: ['hydrocephalus'] @entity1645 :: ('MESH:D001139', 'Disease') :: ['Chiari malformation type 1', 'Arnold Chiari malformation type 1', 'Chiari malformation type I'] @entity28180 :: ('MESH:C562829', 'Disease') :: ['megalocornea'] @entity1635 :: ('MESH:D012600', 'Disease') :: ['scoliosis'] @entity69296 :: ('MESH:C536498', 'Disease') :: ['tetra-pyramidal syndrome'] @entity2790 :: ('MESH:D009798', 'Disease') :: ['Normal intraocular pressure'] @entity1829 :: ('MESH:D010211', 'Disease') :: ['papilledema', 'bilateral papilledema'] @entity194 :: ('MESH:D001284', 'Disease') :: ['atrophy'] @entity76 :: ('MESH:D002526', 'Disease') :: ['cerebellar malformations'] @entity735 :: ('MESH:D005124', 'Disease') :: ['ocular malformations'] @entity892 :: ('MESH:D004172', 'Disease') :: ['diplopia'] @entity717 :: ('MESH:D009069', 'Disease') :: ['rotational nystagmus'] @entity10 :: ('MESH:D006261', 'Disease') :: ['occipital headache'] @entity18382 :: ('MESH:C564214', 'Disease') :: ['goniodysgenesis'] @entity1319 :: ('MESH:D015835', 'Disease') :: ['retained ocular motility']
@entity1645 :: (MESH:D001139,Disease) :: ['Arnold Chiari malformation type 1']
