Absence of the vermis	Congenital absence of part of brain	Congenital cerebellar hypoplasia	Congenital porencephaly	Multiple system malformation syndrome
\|	\|	\|	\|	\|

Porencephaly, cerebellar hypoplasia, internal malformations syndrome (disorder)
	Porencephaly, cerebellar hypoplasia, internal malformations syndrome
	Bonnemann Meinecke syndrome
	A rare central nervous system malformation syndrome with characteristics of bilateral porencephaly, absence of the septum pellucidum and cerebellar hypoplasia with absent vermis. Additionally, dysmorphic facial features (hypertelorism, epicanthic folds, high arched palate, prominent metopic suture), macrocephaly, corneal clouding, situs inversus, tetralogy of Fallot, atrial septal defects and/or seizures have been observed.
	syndroom van porencefalie, cerebellaire hypoplasie en inwendige malformaties
	syndroom van Bönnemann-Meinecke Bönnemann-Meinecke-syndroom

Id	763821001
Status	Primitive

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

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

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

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q87.8
Rule	TRUE
Advice	ALWAYS Q87.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified