congenitale hydrocefalie	macrencefalie	malformatiesyndroom met betrokkenheid van meerdere systemen	polymicrogyrie
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syndroom van megalencefalie, polymicrogyrie, postaxiale polydactylie en hydrocefalie (aandoening)
	syndroom van megalencefalie, polymicrogyrie, postaxiale polydactylie en hydrocefalie
	syndroom van macrencefalie, polymicrogyrie, postaxiale polydactylie en hydrocephalus MPPH-syndroom
	Megalencephaly, polymicrogyria, postaxial polydactyly, hydrocephalus syndrome
	MPPH syndrome MPPH (megalencephaly, polymicrogyria, polydactyly, hydrocephalus) syndrome
	This syndrome has characteristics of megalencephaly, polymicrogyria and hydrocephalus with variable polydactyly. It has been described in six unrelated patients. Intellectual deficit or slow development is also present. The mode of inheritance of this syndrome is unknown since all cases were sporadic.

Id	722036008
Status	Primitive

Associated morphology	congenitale kleinheid
Finding site	structuur van gyrus van hersenen
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

Associated morphology	dilatatie
Finding site	structuur van liquorcirculatie in hersenen
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

Associated morphology	vergroting
Finding site	structuur van cerebrum
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

DHD Diagnosis thesaurus reference set

RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set

Target	Q04.5
Term	Megalencefalie

Target	Q04.3
Term	Overige onderontwikkeling van hersenen

Target	Q03.9
Term	Congenitale hydrocefalus, niet gespecificeerd

SNOMED CT to Orphanet simple map

83473

SNOMED CT to ICD-10 extended map

Target	Q04.5
Rule	TRUE
Advice	ALWAYS Q04.5
Correlation	SNOMED CT source code to target map code correlation not specified

Target	Q04.3
Rule	TRUE
Advice	ALWAYS Q04.3
Correlation	SNOMED CT source code to target map code correlation not specified

Target	Q03.9
Rule	TRUE
Advice	ALWAYS Q03.9 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified