Lobar holoprosencephaly

Definition

A form of holoprosencephaly characterized by separation of the right and left cerebral hemispheres and lateral ventricules with some continuity only across the frontal neocortex, especially rostrally and ventrally. Craniofacial features are variable may include ocular hypotelorism, midline cleft lip (complete or partial) and/or flat nose amongst other features.

Disease data

Classification

Clinical subtype

ORPHA code

93924

OMIM code

609637

ICD10 code

Q04.2

ICD11 code

*Soruce