Parry-Romberg Syndrome

What is Parry-Romberg syndrome?

Parry-Romberg syndrome, also call Romberg syndrome or progressive facial hemiatrophy, is a condition where the tissue of one side of the face gradually wastes away. Over time, the soft tissue (muscle and fat) gradually shrinks, the facial bones may change, and the skin may become thin. The severity of this atrophy can vary widely between patients, ranging from mild to severe. Some children and adults with Parry-Romberg syndrome also have headaches, and seizures can also infrequently happen. The signs of Romberg syndrome overlap with another condition called linear scleroderma, and the line between the two is not always clear. The cause of Parry-Romberg syndrome is not known.

Parry-Romberg syndrome is thought to affect roughly one in 250,000 people, but the true incidence is not known because the condition is often misdiagnosed.

Parry-Romberg syndrome is a condition that starts in childhood and usually affects half of the face. In rare cases, both sides of the face are affected.

What are the symptoms of Parry-Romberg syndrome?

The most common symptom is the thinning or shrinkage (atrophy) of the skin, soft tissues, and in some cases muscle, cartilage, and bone.

Because it is a progressive condition, the symptoms worsen over time before entering a stable phase. The deterioration of the muscle and tissue includes areas around the nose, mouth, tongue, eyes, brow, ears, and neck.

The evident physical changes may include:

  • the appearance that the mouth and nose are shifting or leaning toward one side
  • a sunken-in appearance of both the eye, as well as the cheek, on the affected side of the face
  • changes in skin color (pigmentation), including darkening (hyperpigmentation) or lightening (hypopigmentation)
  • facial hair may turn white and fall out (alopecia)
  • facial bone or muscle loss

Systemic associations may include:

  • neurological symptoms (seizures, migraines, or episodes of severe facial pain known as trigeminal neuralgia)
  • ophthalmologic manifestations
  • orthodontic changes

What causes Parry-Romberg syndrome?

Parry-Romberg syndrome is more common in girls. The cause of this disease is still unknown. Some factors thought to cause this disease include:

  • viral or bacterial infections
  • autoimmune diseases
  • nervous system abnormalities
  • inflammation of the brain or meninges (lining of the skull)
  • physical trauma

Why choose Boston Children’s Hospital for Parry-Romberg care?

Families seeking the very best care for their child come to the Cleft and Craniofacial Center at Boston Children’s for our:

  • Nationally recognized care: U.S. News & World Report ranks Boston Children's Hospital higher in more specialties — including neurosurgery — than any other children's hospital in the country.
  • Surgical expertise: Very advanced surgical techniques and precision are required to treat this disease. Our surgeons are world renowned for treating complex craniofacial anomalies. Learn more about treatment of Parry-Romberg syndrome.
  • Research and innovation: Our clinicians are involved in research that moves us closer to a better understanding of — and treatment for — all deformities of the head and face.
  • Customized treatment: Our team will develop a customized treatment plan that meets your child's physical, emotional, and social needs — one that involves you and your family at every step of the treatment, from diagnosis to craniofacial surgery. Learn more about our approach to craniofacial care.
  • Team approach: Our Cleft and Craniofacial Program includes some of the world’s most experienced neurosurgeons, plastic surgeons, oral and maxillofacial (jaw) specialists, dentistry professionals, psychologists, and social workers — all working together to address the many needs of your child and your entire family. Meet our craniofacial team.

Make an appointment

For an appointment with the Cleft and Craniofacial Center, for more information, or to obtain a second opinion for your child, please call us at 617-355-6309 or email samantha.hall@childrens.harvard.edu.

International patients

For families residing outside of the United States, please call Boston Children's Global Services at +01-617-355-5209. Global Services facilitates the medical review of patient records and appointment scheduling, and also provides assistance with customs and immigration, transportation, and hotel and housing accommodations.