Langer-Giedion syndrome

Langer-Giedion syndrome, also known as trichorhinophalangeal syndrome type II, is an extremely rare autosomal dominant genetic disorder.

Pathology

The effects are seen mainly on the skeletal system and primarily involves

  • hair: tricho
  • nose: rhino
  • digits of the hands and feet: phalangeal

There can be however multiple of other features such as:

  • multiple exostoses
  • mental retardation
  • lose redundant skin
Genetics

It occurs from a deletion within chromosome 8.

History and etymology

Named after 2,3:

  • Andreas Giedion, Swiss radiologist
  • Leonard O Langer Jr, American (US) radiologist 
Share article

Article information

rID: 10592
Section: Syndromes
Synonyms or Alternate Spellings:
  • Trichorhinophalangeal syndrome type II

Support Radiopaedia and see fewer ads

Cases and figures

  • Trichorhinophalan...
    Case 1
    Drag here to reorder.
  • Updating… Please wait.

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

     Thank you for updating your details.