ulnar hypoplasia-split foot syndrome
Findings
No curated finding names ulnar hypoplasia-split foot syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Ulnar hypoplasia-split foot syndrome is characterized by the association of severe ulnar hypoplasia, absence of fingers two to five, and split-foot. It has been described in four males belonging to two generations of the same family. X-linked recessive inheritance is suggested, but autosomal dominant transmission cannot be excluded.
Definition from the Mondo Disease Ontology (MONDO:0010750), read 2026-09-29. CC BY 4.0.
Features
4 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.
- Aplasia/Hypoplasia of the radiusHPOHP:0006501
- Very frequent (80% to 99% of cases)
- Hypoplasia of the ulnaHPOHP:0003022
- Very frequent (80% to 99% of cases)
- Split handHPOHP:0001171
- Very frequent (80% to 99% of cases)
- Split footHPOHP:0001839
- Frequent (30% to 79% of cases)
Where it sits
Other names
2 names
Resolves to: ulnar hypoplasia-split foot syndrome
- Also called
- ulnar hypoplasia-lobster-claw deformity of feet syndromeVan den Berghe-Dequecker syndrome