Autosomal recessive hereditary disorder	Congenital anomaly of cornea	Corneal thinning	Ehlers-Danlos syndrome	Hereditary disorder of the visual system
\|	\|	\|	\|	\|

Brittle cornea syndrome (disorder)
	Brittle cornea syndrome
	Ehlers-Danlos syndrome type 6B
	Brittle cornea syndrome (BCS) is a form of Ehlers-Danlos syndrome with characteristics of severe ocular manifestations due to extreme corneal thinning and fragility with rupture in the absence of significant trauma. BCS generally progresses to blindness.
	'brittle cornea'-syndroom
	BCS

Id	719096006
Status	Primitive

Associated morphology	Morphologically abnormal structure
Finding site	Connective tissue structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Morphologically abnormal structure
Finding site	Corneal structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Dysplasia
Finding site	Bone structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Dysplasia
Finding site	Skin structure
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q79.6
Rule	TRUE
Advice	ALWAYS Q79.6
Correlation	SNOMED CT source code to target map code correlation not specified