ICD-11 classes
20 Developmental anomalies
Structural developmental anomalies primarily affecting one...
Structural developmental anomalies of the nervous system
LA06 — Cerebellar structural developmental anomalies
LA06.1 — Hypoplasia or agenesis of cerebellar hemispheres
ICD-11 LA06.1 — Hypoplasia or agenesis of cerebellar hemispheres
Cerebellar hypoplasia corresponds to underdevelopment of cerebellar structures that can involve the vermis and/or the cerebellar hemispheres from partial to total agenesis. It has been described in the context of various clinical entities: chromosomal anomalies, in utero exposure to toxins and infectious agents, metabolic disorders (disorders of glycosylation and CoQ10 deficiencies), and a wide variety of rare genetic neurological diseases. It can be confined to the cerebellum, or affect other CNS structures: the midbrain (molar tooth syndromes), pons and medulla (ponto-cerebellar hypoplasia), cerebral cortex (lissencephaly cerebellar hypoplasia syndromes).
The diagnosis includes nothing.
It excludes 1 item.
- PHACE syndrome (LD2F.1)
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: