Parry romberg syndrome

Neurocutaneous syndrome characterized by progressive shrinkage and degeneration of the tissues beneath the skin usually on only one side of face but occasionally extending to other parts of the body

The condition is often accompanied by significant neurological, ocular and oral signs and symptoms

Progressive facial hemiatrophy

There is an estimate that 1 in every 700.000 births present this syndrome

The earlier the onset of the disease, skeletal compromise is more likely, due to skeletal growth and development.

In 95% of the time, the face is compromised unilaterally

PRS is not a congenital disease with onset typically in the first or second decade of life

The syndrome usually affects more than one branch of the trigeminal nerve dermatomes of the trigeminal nerve, being V1 (ophthalmic division) damaged in 35% of the cases, V2 (maxillary division) in 45% and V3(mandibular division) in the remaining 20%.

Incidence

No genetic predispositions, no hereditary traits defined,no ethnic preferences

Nervous system hyperactivity ? Etiological hypothesis resume to

InfectionPeripheral trigeminal neuritis – a trigeminal neuritis would begin with episodes of pain followed by tissue atrophy.Sympathetic Hypothesis – based on an association among Horner Syndrome, pilomotor reflex alterations, unilateral mydriasis, vasomotor diseases, unilateral migraine and transpiring diseases

Etiology

The syndrome often begins with a circumscribed

patch of SCLERODERMA  in the frontal region of the

scalp which is associated with a loss of hair and the

appearance of a depressed linear scar extending

down through the midface on the affected side.

This scar is referred to as a "coup de sabre"

lesion

Clinical features

“coup de sabre

The affected area extends progressively with the

atrophy of the skin, subcutaneous tissue, the muscles,

bones, cartilages, alveolar bone and soft palate on that

side of the face.

The mouth and nose are typically deviated towards the

affected side of the face

The process may eventually extend to involve

tissues between the nose and upper corner of lip,

the upper jaw ,the angle of mouth, the area around the

eye and brow, the ear, and/or the neck

Onset of the disease is commonly in childhood or puberal phase and continues evolving during adolescence, compromising the esthetic structure and often facial dynamics as well.

Inigo et al proposed a classification for PRS based on skin, subcutaneous tissue and bony atrophy in trigeminal dermatomes:

A) Mild: Atrophy of skin and subcutaneous tissue of only one trigeminal dermatome. No bone involvement

B)  Moderate: Two trigeminal dermatomes involved, no bony structures affected.

C) Severe: All three trigeminal territories affected or bone involvement. In the initial phases of the disease, there may be cutaneous hardness, hypercromia or hypocromia (similar to scleroderma) of skin, hair, Iris and even cicatricial alopecia

Classification

Systemic Associations

Neurological ocular

Seizures -most common

Migraneous crisis Aneurisms Cerebral atrophy Cerebral vascular

anomalies Facial muscular

spasms

Enophtalmia Uveitis Retinal vasculitis Paralysis of III cranial

nerve Glaucoma and eyelid

atrophy  Mydriasis,miosis,

vasomotor or secretory reactions

oculo-pupillary phenomena

Treatment is divided into two philosophies: the first

consists in trying to stop the disease process through

immunosupression which also improve associated

symptoms, while the second regards the repair of

acquired deformities after stabilization of the disease

process. For such, many reconstructive and esthetic

procedures have been tried, such as free grafts,

microsurgery, flaps and alloplastic material

Treatment

BEFORE AFTER TREATMENT WITH MULTIPLE FAT GRAFTS

Parry Romberg Syndrome continues to be an

challenge for research in plastic surgery. It

represents an infirmity with an obscure etiology,

unknown physio-pathology, wide array of clinical

presentations, whose treatment still demands

more than one procedure