Parry Romberg Syndrome

Parry-Romberg syndrome is a rare, acquired disorder characterized by slowly progressive shrinkage (atrophy) of the skin and soft tissues of half of the face (hemifacial atrophy).

Other names of Parry Romberg Syndrome #

• Hemifacial atrophy (HFA)

• Progressive facial hemiatrophy

• Progressive hemifacial atrophy

• PRS

• Romberg syndrome

Causes of Parry Romberg Syndrome #

• The cause of Parry-Romberg syndrome is unknown and appears to occur randomly for unknown reasons (sporadically).

• Different theories have been proposed to explain the development of the disorder including abnormal development or inflammation of the sympathetic nervous system; viral infections; inflammation of the brain and membranes (meninges) covering the brain (meningoencephalitis); trauma; abnormalities of blood vessel formation (angiogenesis); or autoimmunity.

• Some individuals with Parry-Romberg syndrome have a history of trauma to face or head.

Symptoms of Parry Romberg Syndrome #

• The symptoms, progression and severity of Parry-Romberg syndrome are highly variable from one person to another and range from mild to severe.

• It is important to note that affected individuals will not have all the symptoms discussed below.

• It seems likely that individuals with milder symptoms are much more common than severely affected individuals.

• Affected individuals should talk to their physician and medical team about their specific case and associated symptoms.

• The characteristic symptom of Parry-Romberg syndrome is thinning or shrinkage (atrophy) of various tissues of the face including fat, skin, connective tissues, muscle, and, in some people, bone.

• The degree of atrophy can vary widely, ranging from mild, barely perceptible changes to significant asymmetry in which one side of the face appears sunken in.

• The initial facial changes associated with Parry-Romberg syndrome usually occur near the middle portion of the face such as the cheek area above the upper jaw bone (maxilla) or between the nose and the upper corner of the lip.

• Some individuals with Parry-Romberg syndrome may develop abnormalities affecting the mouth and teeth.

• Some individuals develop atrophy of half (hemiatrophy) of the upper lip and/or one side of the tongue.

Diagnosis of Parry Romberg Syndrome #

• A diagnosis of Parry-Romberg syndrome is made based upon identification of characteristic symptoms, a detailed patient history, a thorough clinical evaluation and a variety of specialized tests.

• The specific tests that are used depend on which symptoms are present and which symptoms occur first.

• For example, magnetic resonance imaging (MRI) may be used in individuals with neurological symptoms.

• An MRI uses a magnetic field and radio waves to produce cross-sectional images of organs and bodily tissues.

• MRI allows better characterization of the soft tissue and bony changes when present.

Treatment of Parry Romberg Syndrome #

• The treatment of Parry-Romberg syndrome is directed toward the specific symptoms that are apparent in each.

• Treatment may require the coordinated efforts of a team of specialists.

• Pediatricians or internists, surgeons (especially plastic surgeons), dentists, ophthalmologists, dermatologists, neurologists, and/or other health care professionals may need to systematically and comprehensively plan an affected individual’s treatment.

• For example, migraine, epilepsy or uveitis can be treated as they would in any other situation.

• A variety of surgical techniques have been used to improve cosmetic appearance in affected individuals.

• The success rates of these surgical options are highly variable. Surgical treatment is usually not advised until the atrophic changes have ceased and the extent of resulting facial deformity is known.

• Surgical techniques used to treat individuals with Parry-Romberg syndrome include fat or silicone injections, flap/pedicle grafts, or bone implants.