Autosomal recessive hereditary disorder	Developmental hereditary disorder	Ectodermal dysplasia	Hereditary disorder of the integument	Popliteal pterygium syndrome
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Autosomal recessive popliteal pterygium syndrome (disorder)
	Autosomal recessive popliteal pterygium syndrome
	Bartsocas Papas syndrome Lethal popliteal pterygium syndrome
	A rare inherited popliteal pterygium syndrome with characteristics of severe popliteal webbing, microcephaly, a typical face with short palpebral fissures, ankyloblepharon, hypoplastic nose, filiform bands between the jaws and facial clefts, genital abnormalities and additional ectodermal anomalies (absent hair, eyebrows, lashes, nails). It is often fatal in the neonatal period but survival into childhood has been reported.
	autosomaal recessief popliteaal pterygiumsyndroom
	syndroom van Bartsocas-Papas letaal popliteaal pterygiumsyndroom

Id	722376008
Status	Primitive

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

Associated morphology	Congenital webbing
Finding site	Popliteal region structure
Occurrence	Congenital
Pathological process	Pathological developmental process

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

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q87.2
Rule	TRUE
Advice	ALWAYS Q87.2 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified