Bencze syndrome

Orpha code: 1241OMIM code: 141350

Definition

Bencze syndrome or hemifacial hyperplasia with strabismus is a malformation syndrome involving the abnormal growth of the facial skeleton as well as its soft tissue structure and organs, and is characterized by mild facial asymmetry with unaffected neurocranium and eyeballs, as well as by esotropia, amblyopia and/or convergent strabismus, and occasionally submucous cleft palate. Transmission is autosomal dominant. There have been no further descriptions in the literature since 1979.

Disease data
Classification

Malformation syndrome

Synonyms
Hemifacial hyperplasia-strabismus syndrome
Przerost połowiczy twarzy - zez
ORPHA code
1241
OMIM code
141350
ICD10 code
Q67.4
ICD11 code
-

No additional description.

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