Bencze syndrome

Bencze syndrome, also known as hemifacial hyperplasia with strabismus, is a very rare condition that is present from birth. It mainly affects one side of the face, causing it to grow larger or differently compared to the other side (a feature called facial asymmetry or hemifacial hyperplasia). People with Bencze syndrome may also have crossed eyes (strabismus), mild differences in the shape of the skull, and sometimes other subtle physical features on the affected side of the face. Some individuals may have additional findings such as mild limb differences or other minor anomalies. Because Bencze syndrome is extremely rare, it has been described in only a small number of families in the medical literature. The condition appears to run in families, suggesting a genetic cause, though the exact gene responsible has not yet been identified. The syndrome is generally not life-threatening, and most affected individuals have a normal lifespan. Treatment focuses on managing specific symptoms. For example, strabismus may be treated with glasses, eye patches, or surgery. Facial asymmetry may be monitored over time, and in some cases, reconstructive or cosmetic surgery may be considered. A team of specialists, including geneticists, eye doctors, and craniofacial surgeons, can help guide care and support for affected individuals and their families.

Bring these to your next appointment

• Q1.How severe is the facial asymmetry, and is it likely to change as my child grows?,Does my child need treatment for strabismus, and what are the options?,Should we see a craniofacial surgeon or orthodontist?,Is genetic testing recommended for our family, even though the gene is not yet known?,What is the chance that future children could also have this condition?,Are there any support groups or resources for families affected by facial asymmetry conditions?,What should we watch for as my child gets older?

Common questions about Bencze syndrome