The Clinical and Imaging Profile of Skew Deviation: A Study of 157 Cases

Skew deviation, a vertical misalignment of the eyes caused by a lesion in the vestibulo-ocular pathway, is a common manifestation of brainstem dysfunction, yet comprehensive information about its clinical profile is lacking. The aim of this study was to document presenting symptoms, causes, ocular alignment features, accompanying neurologic signs, pertinent brain imaging abnormalities, and measures used to relieve diplopia.

We searched the electronic medical records text from 2000 to 2018 for "skew" or "skew deviation" at a tertiary care academic center, including only patients diagnosed under supervision of faculty neuro-ophthalmologists. After excluding patients with features suggesting an imitator of skew deviation, we collected data useful in answering the selected clinical issues.

In a cohort of 157 patients, vertical misalignment ranged between 1 and 30 prism diopters (PD) (median 5 PD) and was comitant in 100 (64%) patients. Diplopia was reported by 87% and blurred vision by 11% of patients. Blurred vision was more common when vertical misalignment was less than 3 PD. At least one accompanying neurologic sign was present in 133 (85%) patients, most often nystagmus, followed by gaze paresis, ataxia, saccadic pursuit, and internuclear ophthalmoplegia. Stroke affecting the thalamus, brainstem, or cerebellum accounted for 82 (52%) of cases. Stroke was usually ischemic, mostly unprovoked, but also occurring after intracranial or extracranial surgical procedures. Brainstem tumor and operative injury caused most of the remaining cases. A subgroup of 17 (11%) patients had skew deviation as the only new clinical sign and had no pertinent brain imaging abnormalities. Resolution of skew deviation, documented in 58 (42%) of 137 patients who had at least one follow-up visit, usually occurred within 3 months, but sometimes not until after 12 months. Of 110 patients who still had diplopia on follow-up examinations, the diplopia was successfully relieved with prism spectacles in 68 (62%). The cause of the skew deviation, amount of vertical misalignment, and degree of incomitance did not predict the success in relieving diplopia. Accompanying neurologic signs, including ataxia, endured in 44% of patients and were often more debilitating than the diplopia of skew deviation.

The amplitude of misalignment in skew deviation varies widely but is generally 5 PD or less. When misalignment is 3 PD or less, patients report blurred vision rather than diplopia. Skew deviation is usually accompanied by other neurologic signs reflecting brainstem dysfunction. Yet there is a small subgroup in which vertical misalignment is an isolated sign, and there are no supporting brain imaging abnormalities. The main cause of skew deviation is ischemic stroke, which affects not only the brainstem but also the thalamus. Diplopia from skew deviation frequently persists, in which case prism spectacles may be successful in palliating it. Accompanying neurologic signs, especially ataxia, may outlast skew deviation and be more debilitating.