INTRODUCTION
Parry-Romberg syndrome is also called as facial hemiatrophy. It is slowly progressive atrophy of the soft tissues of half of the face and also progressive wasting of subcutaneous fat with atrophy of skin,cartilage,bone and muscle.
ETIOLOGY
- The primary factor being the cerebral disturbances which leads to increased and unregulated activity of the sympathethic nervous system,which inturn leads to localized atrophy.
- the other factors include:
extraction of teeth
local trauma
infection
genetic factors
disruption of stapedial artery
CLINICAL FEATURES
SEX: females are more affected than males with ratio of 3:2
AGE: occurs generally in the first decade
SITE: Mostly occurs on the left than the right side
CLINICAL PRESENTATION-
- COUP DE SABRE
It is a painless cleft near the midline of the face or forehead.
Marks the boundary between normal and atrophic tissue.
- ATROPHY
Bluish hue may appear in the skin overlying atrophic fat.
The affected area extends with atrophy of skin,cartilage,alveolar bone and soft palate on that side of the face.
facial wasting: ipsilateral salivary glands and hemiatrophy of the tongue,unilateral involvement of the ear,larynx,oseophagus,diaphragm,kidney and brain.
- Pigmentation disorders
- Facial naevi
- Contralateral jacksonian epilepsy
- Contralateral trigeminal neuralgia
- occular abnormalities
ORAL MANIFESTATIONS
- Incomplete root formation
- delayed eruption of teeth
- difficulty with mastication
- hemiatrophy of lips and tongue
- eruption of teeth on the affected side is retarded.
TREATMENT
No specific treatment but cosmetic surgeries are recommended.
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