autosomaal dominante hereditaire aandoening	bevinding betreffende orbitarand	congenitale afwijking van orbita	hereditaire aandoening van visueel systeem	hereditaire ontwikkelingsstoornis	polymalformatief syndroom met defect van aangezicht als voornaamst kenmerk
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syndromale oogkasrandhypoplasie (aandoening)
	syndromale oogkasrandhypoplasie
	syndroom van Urrets-Zavalia
	Syndromic orbital border hypoplasia
	Urrets Zavalia syndrome Syndromic hypoplasia of orbital border
	Syndromic orbital border hypoplasia is a rare disorder observed in two families to date with characteristics of agenesis of the orbital margin, varying defects of the lacrimal passages, hypoplasia of the palpebral skin and tarsal plates and atresia of the nasolacrimal duct.

Id	717337001
Status	Primitive

Associated morphology	morfologische afwijking
Finding site	structuur van aangezicht
Occurrence	congenitaal
Pathological process	proces van pathologische ontwikkeling

Associated morphology	hypoplasie
Finding site	structuur van margo orbitalis
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	Q87.0
Term	Congenitale gestoorde-ontwikkelingssyndromen waarbij voornamelijk aangezicht is aangedaan

SNOMED CT to Orphanet simple map

98606

SNOMED CT to ICD-10 extended map

Target	Q87.0
Rule	TRUE
Advice	ALWAYS Q87.0
Correlation	SNOMED CT source code to target map code correlation not specified