Orofaciodigital syndrome type 11

Orpha code: 141000OMIM code: 612913

Definition

Orofaciodigital syndrome type 11 is an extremely rare, sporadic form of Orofaciodigital syndrome (OFDS; see this term) with only a few reported cases, and characterized by facial (blepharophimosis, bulbous nasal tip, broad nasal bridge, downslanting palpebral fissures and low set ears) and skeletal (post-axial polydactyly and fusion of vertebrae) malformations along with severe intellectual disability, deafness and congenital heart defects.

Disease data
Classification

Malformation syndrome

Synonyms
OFD11
OFD11
Zespół ustno-twarzowo-palcowy, typ Gabrielli
Oral-facial-digital syndrome type 11
Oral-facial-digital syndrome, Gabrielli type
Orofaciodigital syndrome, Gabrielli type
ORPHA code
141000
OMIM code
612913
ICD10 code
Q87.0
ICD11 code
LD25.00

No additional description.

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