Facial hemiatrophy

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

Etiopathogenesis

• Idiopathic that is for unknown reasons• Familial• localised scleroderma• malfunction of sympathetic nervous system• trigeminal neuralgia• loss of adipose tissue

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

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

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

Diagnosis

• White line or furrow on one side and patient is having coup de sabre

• Reduction of size of bone on affected side

Management

• Surgical reconstruction• orthodontic treatment