Bencze syndrome
Findings
No curated finding names Bencze syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
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.
Definition from the Mondo Disease Ontology (MONDO:0007711), read 2026-09-29. CC BY 4.0.
Features
8 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Extension of hair growth on temples to lateral eyebrowHPOHP:0005325
- Very frequent (80% to 99% of cases)
- Facial asymmetryHPOHP:0000324
- Very frequent (80% to 99% of cases)
- AmblyopiaHPOHP:0000646
- Frequent (30% to 79% of cases)
- Open biteHPOHP:0010807
- Frequent (30% to 79% of cases)
- StrabismusHPOHP:0000486
- Frequent (30% to 79% of cases)
- TelecanthusHPOHP:0000506
- Frequent (30% to 79% of cases)
- Upslanted palpebral fissure
Where it sits
Other names
1 name
Resolves to: Bencze syndrome
- Also called
- hemifacial hyperplasia-strabismus syndrome