Autosomal recessive hereditary disorder	Developmental hereditary disorder	Hereditary gingival fibromatosis	Multiple malformation syndrome with facial defects as major feature
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Gingival fibromatosis with facial dysmorphism syndrome (disorder)
	Gingival fibromatosis with facial dysmorphism syndrome
	A very rare syndrome with the association of gingival fibromatosis and craniofacial dysmorphism. It has been described in two siblings. Craniofacial dysmorphism consists of relative macrocephaly, bushy eyebrows with synophrys, hypertelorism, downslanting palpebral fissures, flattened nasal bridge and high arched palate. The patients have normal intellect.The condition seems to be hereditary, transmitted as an autosomal recessive trait.
	syndroom van gingivale fibromatose en faciale dysmorfie

Id	719687007
Status	Primitive

Associated morphology	Fibromatosis
Finding site	Gingival structure
Pathological process	Pathological developmental process

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

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q87.0
Rule	TRUE
Advice	ALWAYS Q87.0 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified