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 report on two cases of CBPS with associated esophageal malformations and a poor mental and motor development. The association of CBPS and esophageal malformations may indicate a subgroup of patients with a very early prenatal injury, characterised by a bad prognosis due to severe cortical disorganization. However, it can not be excluded that the association of CBPS and esophageal malformation is purely coincidental.

Key words

Polymicrogyria - Congenital bilateral perisylvian syndrome - Dysphagia - MRI - Foix-Chavany-Marie syndrome - Esophageal malformation - Worster-Drought syndrome

References

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