PARRY ROMBERG SYNDROME

2.  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

3. 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%.

4.  No genetic predispositions, no hereditary traits
defined,no ethnic preferences
 Nervous system hyperactivity ?
 Etiological hypothesis resume to
Infection
Peripheral 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

5.  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

6. “coup de sabre

7. 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

8. 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.

9.  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

10. 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

11.  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

14. BEFORE AFTER TREATMENT WITH
MULTIPLE FAT GRAFTS

15.  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