A Case of Dandy-Walker Variant Associated with Infantile Esotropia of Abnormaly Directed Extraocular Muscle

ABSTRACT

The Dandy-Wlaker syndrome is a developmental disoeder characterized by cystic dilatation of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis and is usually associated with hydrocephalus. This syndrome is also associated with intracranial malformations such as agenesis of corpus callosum, and extracranial malformations such as facial anormalies (cleft palate, cleft lip, and ocular anomalies), polycystic kidney, and cardiac anormalies. Reported associated ophthalmic anomalies are microphthalmos, coloboma, and congenital cataract.We experienced a case of infantile esotropia in abnormal insertion and direction of lateral rectus muscle as a variant of the Dandy-Walker syndrome accompanied by cleft palate and cleft lip in a 19-month-old male infant. Subsequent treatment of the 30delta esodeviation and left inferior oblique muscle overaction, including resection of the lateral rectus muscle in both eyes and the myectomy of the inferior oblique muscle in the left eye, yielded satisfactory results, so we report this case, with a review of the pertinent literature, as the first case of Dandy-Walker syndrome associated with esotropia with abnormal course of lateral rectus muscle in Korea.