Primary concomitant horizontal strabismus as a clinical biomarker of severity in West Syndrome.
About two-thirds of children with West syndrome had concomitant horizontal strabismus.
Primary concomitant horizontal strabismus as a clinical biomarker of severity in West Syndrome.
Ophthalmic associations of West syndrome, a rare clinical triad comprising infantile spasms, a pathognomonic electroencephalogram pattern of paroxysmal activity called hypsarrhythmia and global developmental delay, usually associated with neuro-radiological anomalies, have been sparsely reported.
This was a cross-sectional, observational, hospital-based study designed to descriptively record ophthalmic findings in children with West Syndrome with the aim to establish the array of ophthalmic associations of this condition.
Patients with primary concomitant horizontal strabismus had a significantly earlier age of onset as well as significantly increased number of drugs required to control the spasms as compared to the children without strabismus.
40/80 (50%) subjects of West Syndrome with primary concomitant horizontal strabismus demonstrated palpebral aperture anomalies, especially epicanthal folds, significantly more than subjects of WS without primary concomitant horizontal strabismus.
Primary concomitant horizontal strabismus along with eyelid and palpebral fissure anomalies are visible phenotypes and thus, very useful ophthalmological clinical biomarkers for early identification of children with increased severity of West Syndrome, which aids the pediatric neurologist toward early management of this life-threatening recalcitrant seizure disorder, while imaging and electro-encephalography evaluation is ongoing.