Rare diseases · Sign or symptom
Split hand
Split-hand
HP:0001171
What it means
A condition in which middle parts of the hand (fingers and metacarpals) are missing giving a cleft appearance. The severity is very variable ranging from slightly hypoplastic middle fingers over absent middle fingers as far as oligo- or monodactyl hands.
Rare diseases that can present with this39
Very common80–99%
16- Acrocardiofacial syndrome
- Acro-renal-mandibular syndrome
- Acrorenal syndrome
- Charlie M syndrome
- Cooks syndrome
- Craniosynostosis, Herrmann-Opitz type
- EEC syndrome
- Femur-fibula-ulna complex
- Holt-Oram syndrome
- Mirror polydactyly-vertebral segmentation-limbs defects syndrome
- Mucopolysaccharidosis type 1
- Ring chromosome 4 syndrome
- Tetramelic monodactyly
- Thanatophoric dysplasia type 1
- Tibial aplasia-ectrodactyly syndrome
- Ulnar hypoplasia-split foot syndrome
Common30–79%
12- Adams-Oliver syndrome
- Alopecia-intellectual disability syndrome
- Autosomal recessive Charcot-Marie-Tooth disease with hoarseness
- FATCO syndrome
- Fibular aplasia-ectrodactyly syndrome
- Hartsfield syndrome
- Hypoglossia-hypodactyly syndrome
- Isolated tibial hemimelia
- Müllerian duct anomalies-limb anomalies syndrome
- Orofaciodigital syndrome type 4
- Thalidomide embryopathy
- Wolf-Hirschhorn syndrome
Sometimes5–29%
10- Autosomal dominant popliteal pterygium syndrome
- Autosomal dominant spastic paraplegia type 17
- Autosomal recessive Robinow syndrome
- Charcot-Marie-Tooth disease type 1E
- Charcot-Marie-Tooth disease type 4D
- HNRNPA1-related adult-onset distal myopathy
- Isolated split hand-split foot malformation
- Pentalogy of Cantrell
and 2 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Ectrodactyly of the hand · Hand ectrodactyly
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.