Short rib polydactyly syndrome

Short rib polydactyly syndrome(s) (SRPS) comprise of a rare group of severe osteochondrodysplasias. There are four major recognised types present:

There may also be other very rare types described, such as 9:

While there is some variation in clinical spectrum, they can be collectively characteristed by 2-3:

Genetics

Collectively, most cases carry an autosomal recessive inheritance.

Associations

Multiple anomalies of major organs are common 5. These include

SRPSs are lethal anomalies and management is often supportive.

Share article

Article information

rID: 13709
Section: Syndromes
Synonyms or Alternate Spellings:
  • Short rib polydactyly syndromes
  • Short rib polydactyly syndromes (SRPS)
  • Short rib polydactyly (SRP) syndrome
  • SRP syndrome
  • SRPS

Support Radiopaedia and see fewer ads

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.