Bencze syndrome

Bencze syndrome, also known as hemifacial hyperplasia with strabismus, is a malformation syndrome. It involves abnormal growth of the facial bones, soft tissues, and organs. The syndrome is characterized by mild facial asymmetry while the braincase and eyeballs remain unaffected. People with this condition may also experience issues with eye alignment such as esotropia, amblyopia, or convergent strabismus. In some cases, a submucous cleft palate is seen. The syndrome is noted to be transmitted in an autosomal dominant pattern. Since the initial description in 1979, there has been very limited additional information in the medical literature.

Common signs in people with Bencze syndrome include mild facial asymmetry, esotropia (inward turning of the eye), amblyopia (reduced vision), and convergent strabismus. Occasionally, a submucous cleft palate may also be present.

Bencze syndrome is caused by a malformation that affects the growth of the facial skeleton and soft tissues. The condition is inherited in an autosomal dominant manner. This means that a change in one copy of a gene may be enough to cause the disorder.

Information about treatment is currently limited for this condition.

Information about prognosis is currently limited for this condition.

There has been very little new research on Bencze syndrome since its initial description in 1979. Information about ongoing research or future directions is currently limited for this condition.