Benign Enlargement of Subarachnoid Space in Infancy: "A Review with Emphasis on Diagnostic Work-Up"

Macrocephaly is one of the most frequent reasons for referral to a pediatric neurologist. Benign enlargement of subarachnoid space (BESS) in infancy is the most common cause of macrocephaly and characterized clinically with large head circumference, normal or mildly motor and language delay and increased cerebrospinal fluid (CSF) in the subarachnoid space with normal ventricles or mild ventriculomegaly. In this review, we describe the etiology, epidemiology, clinical presentation, pathogenesis, neuroimaging, differential diagnosis, treatment and outcome of this entity from current literature with emphasis on diagnostic work-up.