Dandy-Walker syndrome	Developmental hereditary disorder	Disorder of basal ganglia	Hereditary disorder of nervous system	Intellectual disability	Multiple malformation syndrome with facial defects as major feature	X-linked recessive hereditary disease
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X-linked Dandy-Walker malformation with intellectual disability, basal ganglia disease and seizure syndrome (disorder)
	Pettigrew syndrome
	X-linked Dandy-Walker malformation with intellectual disability, basal ganglia disease and seizure syndrome
	A central nervous system malformation with characteristics of severe intellectual deficit, early hypotonia with progression to spasticity and contractures, choreoathetosis, seizures, dysmorphic face (long face with prominent forehead) and brain imaging abnormalities.
	X-gebonden syndroom van Dandy-Walker-malformatie, verstandelijke beperking, basalegangliaziekte en epileptische aanvallen
	X-gebonden syndroom van Dandy-Walker-malformatie, mentale retardatie, basalegangliaziekte en convulsies X-gebonden syndroom van Dandy-Walker-malformatie, verstandelijke handicap, basalegangliaziekte en insulten

Id	719139003
Status	Primitive

Associated morphology	Cystic dilatation
Finding site	Fourth ventricle structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Morphologically abnormal structure
Finding site	Face structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Hypoplasia
Finding site	Cerebellar vermis structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Finding site	Basal ganglion structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Congenital malrotation
Finding site	Cerebellar vermis structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Cystic dilatation
Finding site	Infratentorial brain structure
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q03.1
Rule	TRUE
Advice	ALWAYS Q03.1 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified