Freeman-Sheldon syndrome

Orpha code: 2053OMIM code: 618436

Definition

A rare congenital, distal arthogryposis syndrome characterized by microstomia, whistling-face appearance, Chin with V- or H- shaped creased, and prominent nasolabial folds; most patients present club foot and congenital joint contractures of the hands and feet. It is the most severe form of distal arthrogryposis.

Disease data
Classification

Malformation syndrome

Synonyms
Craniocarpotarsal dysplasia
Dysplazja czaszkowo-nadgarstkowo-skokowa
Dystalna artrogrypoza typu 2A
Dystrofia czaszkowo-nadgarstkowo-skokowa
Zespół gwiżdżącej twarzy
Craniocarpotarsal dystrophy
Distal arthrogryposis type 2A
Freeman-Burian syndrome
Whistling face syndrome
ORPHA code
2053
OMIM code
618436
ICD10 code
Q87.0
ICD11 code
LD26.4Y

No additional description.

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