Congenital bilateral perisylvian syndrome (CBPS): Do concomitant esophageal malformations indicate a poor prognosis

Congenital bilateral perisylvian syndrome (CBPS) is a syndrome of cortical malformation characterized by faciopharyngoglossomasticatory diplegia. We r eport on two cases of CBPS with associated esophageal malformations and a p oor mental and motor development. The association of CBPS and esophageal ma lformations may indicate a subgroup of patients with a very early prenatal injury, characterised by a bad prognosis due to severe cortical disorganiza tion. However, it can not be excluded that the association of CBPS and esop hageal malformation is purely coincidental.