afwijkend weefsel van huid en/of huidgeassocieerd slijmvlies	benigne neoplasma van cerebrum	congenitale neuropathie	frontonasale dysplasiesequentie	leukencefalopathie	lipoom van hersenen	mediane cheiloschisis	poliep
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Pai-syndroom (aandoening)
	Pai-syndroom
	syndroom van mediane cheiloschisis, pericallosaal lipoom en cutane poliep syndroom van Pai syndroom van mediane hazenlip, lipoom van corpus callosum en huidpoliep
	Pai syndrome
	Median cleft of upper lip, corpus callosum lipoma, cutaneous polyp syndrome
	A rare frontonasal dysplasia characterized by median cleft of the upper lip (MCL), midline polyps of the facial skin, nasal mucosa, and pericallosal lipomas. Hypertelorism with ocular anomalies are also observed, generally with normal neuropsychological development.

Id	722201004
Status	Primitive

Associated morphology	lipomateuze morfologie
Finding site	structuur van corpus callosum
Occurrence	congenitaal

Associated morphology	poliep
Finding site	structuur van huid en/of huidgeassocieerd slijmvlies
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

Associated morphology	dysplasie
Finding site	botstructuur van cranium
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

Associated morphology	fusiedefect door stoornis in ontwikkeling
Finding site	structuur van bovenlip
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

DHD Diagnosis thesaurus reference set

SNOMED CT to Orphanet simple map

1993

SNOMED CT to ICD-10 extended map

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