vitalwiki

Corpus callosum agenesis-intellectual disability-coloboma-micrognathia syndrome

ORPHA:52055· ICD-10 Q87.8

Definition

Corpus callosum agenesis-intellectual disability-coloboma-micrognathia syndrome is a developmental anomalies syndrome characterized by coloboma of the iris and optic nerve, facial dysmorphism (high forehead, microretrognathia, low-set ears), intellectual deficit, agenesis of the corpus callosum (ACC), sensorineural hearing loss, skeletal anomalies and short stature.

Prevalence
<1 / 1 000 000
Inheritance
X-linked recessive
Age of onset
Infancy, Neonatal