Ophthalmological manifestations of Parry-Romberg syndrome

Surv Ophthalmol. 2016 Nov-Dec;61(6):693-701. doi: 10.1016/j.survophthal.2016.03.009. Epub 2016 Apr 1.


Parry-Romberg syndrome is a rare disease characterized by slowly progressive atrophy affecting facial subcutaneous tissues, including the underlying muscles and osteocartilaginous structures. Various periocular, ocular, and neuro-ophthalmological manifestations have been described in Parry-Romberg syndrome. The most common periocular disorders include enophthalmos, eyelid, and orbit alterations. The most frequent ocular disorders include corneal and retinal changes, and the most common neuro-ophthalmological disorders involve optic nerve, ocular motor and pupillary dysfunction. Besides the characteristic facial abnormalities, systemic manifestations may occur, including neurologic, dermatologic, cardiac, endocrine, infectious, orthodontic, and maxillofacial disorders. So far, mainly brief case reports describe these ophthalmological findings. Therefore, we summarize the ocular, periocular, and neuro-ophthalmological findings in detail, describe diagnostic modalities, and outline therapeutic options.

Keywords: Parry-Romberg syndrome; hemiatrophia faciei; linear scleroderma en coup de sabre; morphea; progressive hemifacial atrophy; scleroderma.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Diagnostic Techniques, Ophthalmological*
  • Disease Progression
  • Eye Diseases* / diagnosis
  • Eye Diseases* / etiology
  • Eye Diseases* / physiopathology
  • Facial Hemiatrophy / complications*
  • Humans