vitalwiki

Vici syndrome

ORPHA:1493· ICD-10 Q87.8

Definition

Vici syndrome is a very rare and severe congenital multisystem disorder characterized by the principal features of agenesis of the corpus callosum, cataracts, oculocutaneous hypopigmentation, cardiomyopathy and combined immunodeficiency.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Antenatal, Neonatal