PARRY-ROMBERG SYNDROME

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.

One thought on “PARRY-ROMBERG SYNDROME

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s