Parry-Romberg syndrome

Last revised by Daniel J Bell on 16 May 2021

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

It is classically characterized by a slowly 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.

The condition is often accompanied by:

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

  • may show atrophy on one side of the face +/- scalp
  • high (T2/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
  • ipsilateral parenchymal atrophy
  • ipsilateral cerebral microhemorrhages
  • ipsilateral parenchymal calcification
  • intracranial aneurysms

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

ADVERTISEMENT: Supporters see fewer/no ads

Updating… Please wait.

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

 Thank you for updating your details.