Autosomal recessive hereditary disorder	Congenital dysplasia	Developmental hereditary disorder	Developmental malformation of branchial arch	Intellectual disability	Multiple system malformation syndrome
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Branchial dysplasia, intellectual disability, inguinal hernia syndrome (disorder)
	Branchial dysplasia, intellectual disability, inguinal hernia syndrome
	Lambert syndrome
	A very rare syndrome described in four siblings of one French family and with characteristics of branchial dysplasia (malar hypoplasia, macrostomia, preauricular tags and meatal atresia), club feet, inguinal hernia and cholestasis due to paucity of interlobular bile ducts and intellectual deficit.
	syndroom van branchiale dysplasie, verstandelijke beperking en hernia inguinalis
	syndroom van Lambert syndroom van branchiale dysplasie, mentale retardatie en hernia inguinalis syndroom van branchiale dysplasie, verstandelijke handicap en hernia inguinalis

Id	732961003
Status	Primitive

Associated morphology	Dysplasia
Finding site	Branchial arch structure
Occurrence	Congenital
Pathological process	Pathological developmental process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

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