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 Neurocutaneous syndrome characterized by
progressive shrinkage and degeneration of the
tissues beneath the skin usually ...
There is an estimate that 1 in every 700.000 births present
this syndrome
The earlier the onset of the disease, skeletal c...
 No genetic predispositions, no hereditary traits
defined,no ethnic preferences
 Nervous system hyperactivity ?
 Etiolo...
 The syndrome often begins with a circumscribed
patch of SCLERODERMA in the frontal region of the
scalp which is associat...
“coup de sabre
The affected area extends progressively with the atrophy
of the skin, subcutaneous tissue, the muscles, bones,
cartilages,...
Onset of the disease is commonly in
childhood or puberal phase and
continues evolving during
adolescence, compromising the...
 Inigo et al proposed a classification for PRS based on
skin, subcutaneous tissue and bony atrophy in
trigeminal dermatom...
Neurological ocular
 Seizures -most
common
 Migraneous crisis
 Aneurisms
 Cerebral atrophy
 Cerebral vascular
anomali...
 Treatment is divided into two philosophies: the first
consists in trying to stop the disease process through
immunosupre...
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 ...
Parry romberg syndrome
Parry romberg syndrome
Parry romberg syndrome
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Parry romberg syndrome

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PARRY ROMBERG SYNDROME

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Parry romberg syndrome

  1. 1.  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
  2. 2. 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%.
  3. 3.  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
  4. 4.  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
  5. 5. “coup de sabre
  6. 6. 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
  7. 7. 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.
  8. 8.  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
  9. 9. 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
  10. 10.  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
  11. 11. BEFORE AFTER TREATMENT WITH MULTIPLE FAT GRAFTS
  12. 12.  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

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