Autosomal dominant hereditary disorder	Disorder characterized by edema	Inherited optic neuropathy	Splenomegaly
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Optic nerve edema, splenomegaly syndrome (disorder)
	Optic nerve edema, splenomegaly syndrome
	A rare presumably genetic disorder characterized by idiopathic massive splenomegaly with pancytopenia and childhood-onset chronic optic nerve edema with slowly progressive vision loss. Additional reported features include anhidrosis, urticaria and headaches.
	syndroom van oedeem van nervus opticus en splenomegalie
	syndroom van oogzenuwoedeem en splenomegalie

Id	771471002
Status	Primitive

Associated morphology	Edema
Finding site	Optic nerve structure
Occurrence	Childhood

Associated morphology	Enlargement
Finding site	Entire spleen

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	H47.1
Rule	TRUE
Advice	ALWAYS H47.1 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified

Target	R16.1
Rule	TRUE
Advice	ALWAYS R16.1
Correlation	SNOMED CT source code to target map code correlation not specified