PURPOSE: To analyse and evaluate associated ocular symptoms and signs in @entity1644 and their response to treatment. METHODS: From a database of 275 @entity1 treated in a single institution for hindbrain @entity547 and @entity1644 , 39 @entity1 (14%) had ocular symptoms and signs. Only 31 @entity1 were included in this study; the remainder were excluded due to inadequate follow-up information. All @entity1 had confirmed evidence of @entity1644 with MRI scan or CT myelogram. Treatment included craniovertebral decompression or ventriculo-peritoneal shunting. The mean follow-up was 23 months. RESULTS: In addition to the well-recognised sign of downbeat @entity346 , classically associated with foramen magnum abnormalities, a number of other ophthalmic features were identified. Symptoms included @entity892 , oscillopsia, tunnel vision and difficulty in lateral gaze. Signs included @entity346 (downbeat, horizontal, rotatory, and combinations), strabismus, disc pallor, anisocoria, @entity1736 and @entity2194 . @entity1 were categorised into two groups depending on whether the ocular features were manifest at first presentation (group 1, n = 14) or developed later in the course of the disease (group 2, n = 17). The delay in diagnosis from first presentation was 5 and 6 years respectively. All @entity1 underwent surgery. Craniovertebral decompression was performed in 13 @entity1 in group 1 and in 15 @entity1 in group 2. Ventriculo-peritoneal shunt was inserted in 1 @entity1 in group 1 and in 3 @entity1 in group 2, for the associated @entity1276 . Following surgery, 100% of @entity1 in group 1 and 82% of @entity1 in group 2 had complete or partial resolution of their ocular symptoms and signs. CONCLUSIONS: The presence of unexplained ophthalmic features such as @entity346 or oscillopsia should alert one to the potential diagnosis of @entity1644 . Delay in diagnosis is often associated with poorer outcome. Surgical treatment can offer excellent results for these @entity1 .

Ocular manifestations of XXXX and outcome following craniovertebral decompression.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients', 'patient'] @entity2194 :: ('MESH:D005128', 'Disease') :: ['field defect'] @entity1644 :: ('MESH:D013595', 'Disease') :: ['syringomyelia', 'hindbrain-related syringomyelia'] @entity1276 :: ('MESH:D006849', 'Disease') :: ['hydrocephalus'] @entity547 :: ('MESH:D006547', 'Disease') :: ['hernia'] @entity892 :: ('MESH:D004172', 'Disease') :: ['diplopia'] @entity1736 :: ('MESH:D001763', 'Disease') :: ['ptosis'] @entity346 :: ('MESH:D009759', 'Disease') :: ['nystagmus']
@entity1644 :: (MESH:D013595,Disease) :: ['hindbrain-related syringomyelia']
