Bencze syndrome

Also known as: Hemifacial hyperplasia-strabismus syndrome

Definition

Orphanet

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.

Go To Source: Orphanet

Symptoms

Medical Term Other Names Description
Esodeviation Convergent strabismus A manifest or latent ocular deviation in which one or other eye tends to deviate nasally.
Cleft palate Cleft of hard and soft palate, Palatoschisis, Uranostaphyloschisis [more] Cleft palate is a developmental defect of the palate resulting from a failure of fusion of the palatine processes and manifesting as a separation of the roof of the mouth (soft and hard palate).
Esotropia Inward turning cross eyed A form of strabismus with one or both eyes turned inward ('crossed') to a relatively severe degree, usually defined as 10 diopters or more.
Growth abnormality Abnormal growth, Growth issue
Amblyopia Lazy eye, Wandering eye Reduced visual acuity that is uncorrectable by lenses in the absence of detectable anatomic defects in the eye or visual pathways.
Strabismus Squint, Heterotropia, Cross-eyed, Squint eyes [more] Strabismus (also known as squint) is a condition in which the eyes are not properly aligned with each other.
Submucous cleft hard palate Submucosal cleft palate, Submucous clefting, Partial thickness cleft hard palate [more] Hard-palate submucous clefts are characterized by bony defects in the midline of the bony palate that are covered by the mucous membrane of the roof of the mouth. It may be possible to detect a submucous cleft hard palate upon palpation as a notch in the bony palate.
Facial asymmetry Asymmetry of right and left side of face, Unsymmetrical face, Crooked face, Unequal sides of face, Uneven sides of face, Asymmetric facies, Asymmetry of face, Unbalanced face, Uneven face [more] An abnormal difference between the left and right sides of the face.

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