Parry-Romberg syndrome

Parry-Romberg syndrome (PRS), also known as progressive facial hemiatrophy (PFH), is a rare progressive craniofacial disorder (phakomatosis).

It is classically characterised by a slow progressive degeneration (atrophy) of the soft tissues of half of the face (hemifacial atrophy). This involves the skin, underlying soft tissues, cartilaginous structures and bone. 

There may be slightly increased prevalence in females. It is thought to be of sporadic onset.

It typically presents in childhood. The overlying craniofacial skin in the affected region may be hyperpigmented.

Associations

The condition is often accompanied by:

Other possible associations include:

CT/MRI brain
  • may show atrophy on one side of the face +/- scalp
  • high (T2/PD/FLAIR) signal of white matter within the ipsilateral and less commonly contralateral brain parenchyma has also been reported on MRI 8  
  • ipsilateral and less often contralateral leptomeningeal enhancement
  • parenchymal atrophy
  • cerebral microhemorrhages
  • parenchymal calcification
  • intracranial aneurysms

Caleb Hillier Parry was a British physician (1755-1822), and Moritz Heinrich Romberg was a German neurologist (1795-1873).


Share Article

Article Information

rID: 12633
Section: Syndromes
Synonyms or Alternate Spellings:
  • Progressive facial hemiatrophy
  • Parry Romberg syndrome
  • Romberg syndrome
  • Romberg disease
  • Progressive facial hemiatrophy (PFH)
  • Progressive hemifacial atrophy
  • Parry Romberg syndrome (PRS)

Support Radiopaedia and see fewer ads

  • Drag
    Case 1: on right side
    Drag here to reorder.
  • 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.