Facial hemiatrophy, also known as Parry Romberg syndrome, is characterized by slowly progressive wasting of the soft tissues on half of the face. Key features include a white line furrow on one side of the face near the midline, thinning of tissues under the skin including fat, cartilage, and bone, and a sharp line of demarcation between normal and affected skin resembling a scar. Radiographic imaging reveals reduction in the size of bones on the affected side of the face compared to the unaffected side. There is no known cause, and treatment involves surgical reconstruction and orthodontic management.
2. General information
• Also called as Parry Romberg syndrome,
Romberg hemifacial atrophy, progressive facial
hemiatrophy
• There is slowly progressive wasting of the soft
tissues of half of the face
• wasting is associated with skin, cartilage,
connective tissue, muscles and bone
3. Etiopathogenesis
• Idiopathic that is for unknown reasons
• Familial
• localised scleroderma
• malfunction of sympathetic nervous system
• trigeminal neuralgia
• loss of adipose tissue
4. Clinical features
• Progressive tissue wasting is limited to one half of the
face, usually the left side
• White line furrow or Mark on one side of face or
eyebrow near midline
• coup de sabre-may show sharp line of demarcation
resembling linear scar between normal and abnormal
skin
• Shrinkage and atrophy of tissues beneath the skin in
the layer of fat under the skin and in the underlying
cartilage, muscles and bone
• patient may get skeletal like appearance
• there is dark pigmentation
5. Oral manifestations
• Atrophy of half of the upper lip and tongue
• deviation of jaws while opening the mouth
• delayed eruption or wasting of roots of certain
teeth on the affected site
• malocclusion is also seen
• there is posterior open bite
6. Radiographic features
• reduction in size of bone on affected site
• there is also reduction in size of condyle,
coronoid process or overall dimension of body
and Ramus of mandible
• affected site of the face is smaller in all
dimensions than the opposite side
7. Diagnosis
• White line or furrow on one side and patient is
having coup de sabre
• Reduction of size of bone on affected side
