A Rare - Facial deformity

1. PARRY ROMBERG SYNDROME
By – Dr.Anchal Agarwal

2. Differential Diagnosis
• Hemifacial Microsomia – congenital disorder ,
• Baraquer-Simons syndrome 
- an acquired partial progressive cephalothoracic lipodystrophy
- that presents with a gradual onset of  symmetrical bilateral subcutaneous fat loss
from 
- face, neck, upper extremities, thorax, and abdomen but sparing the lower
extremities.
- Central nervous system findings of deafness, epilepsy, and intellectual disability have
also been described.
- The bilateral nature of this disease and
- systemic involvement of the kidneys may differentiate these processes
•

3. Localized scleroderma (LS)
• Relationship between scleroderma and PRS is controversial.
• Localized scleroderma maybe the preceding lesion of PHA
• And in patients with localized scleroderma, PHA may develop
in several years.
• Hence regarding the clinical features and and clinical course,
PRS and LS may represent differential spectra of the same
disease process
• LS responds to drug therapy, PRS - Progressive

4. Scleroderma
Mayo Clinic Classification
1 . Plaque morphea,
2. Generalized morphea,
3. Bullous morphea,
4. Linear scleroderma - including subtypes that involve the head
and face,
linear scleroderma ‘en coup de saber’ (LScs) and progressive
facial hemiatrophy (PFH),
• 5. Deep morphea.

5. • Localized Scleroderma,
- It is commonly seen in the paramedian forehead region.
- In “en coup de sabre” - atrophy of underlying muscle or bone is
not seen.

7. • Anti-nuclear anti-body titres are often raised with active linear
scleroderma, but rarely so with Romberg’s disease.

8. Parry Romberg Syndrome
• Progressive hemifacial atrophy
• Uncommon degenerative condition  characterized by  slow and
progressive atrophy  of the facial tissues, including muscles, bones and
skin
• Unilateral , right side more commonly affected
• More in females
• First described by – Caleb Hiller Parry – 1825
• Later described in detail by – Mortiz Heinrich Romberg - 1846

9. • Often accompanied by significant – neurological, ocular and oral
signs and symptoms
• Syndrome usually affects more than one branch of the trigeminal
nerve dermatome
• V1 – damaged in 35% cases
• V2 – 45% cases
• V3 – remaining 20%

10. Etiology
• four theories :
1) infection hypothesis,
2) trigeminal-peripheral neuritis hypothesis,
3) sympathetic hypothesis.
4) Trauma induced

11. • The infectious hypothesis -
• historically linked to an irritation of nerves.
• It has been suggested that a Bell’s palsy or herpes zoster in the
trigeminal distribution may be associated with PRS;
• however, studies have not been able to corroborate this theory
• infectious hypothesis may be remain a tenable etiology until a
definitive understanding of this disorder is truly established.
• The trigeminal-peripheral neuritis hypothesis suggests – neuritis
involving the trigeminal nerve,
• supported by episodes of pain in the involved areas prior to the onset
of tissue involution.

12. • Trauma induced hypothesis –
• many of the studies focusing on PRS have noted trauma in a
significant cohort of their study population.
• The large survey found
- 27% of responders had a childhood head injury; however,
- only 12% of responders had injuries that seemed relevant to the
authors
Stone J. Parry-Romberg syndrome: a global survey of 205 patients using the Internet. Neurology. 2003;61(5):674–6

13. • In summary, a distinct etiology of PHA is still elusive;
• however, a combination of auto- immune, vascular, and
autonomic dysfunction is likely

14. Classification
• Inigo et al – 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 bony involvement
- B) MODERATE –
- two trigeminal dermatomes involved, no bony structures
affected

15. • C) SEVERE –
• all three trigeminal territories affected , or bony involvement
In initial phases of the disease, there maybe
- cutaneous hardness,
- hypercromia or hypocromia (similar to scleroderma) of skin, hair
, iris and even
- cicatricial alopecia

16. TREATMENT

17. Classification and Treatment of Facial Tissue
Atrophy in Parry Romberg Disease
- To determine the most appropriate treatment for each case,
it is convenient first to classify the type of tissue atrophy
exhibited by each patient :
Guerrerosantos J. Classification and treatment of facial tissue atrophy in Parry – Romberg Disease.Anesth
Plastic Surg 31: 424-434 , 2007

18. Type 1 Tissue Depression
• very mild, with a thinness of the
soft facial tissues.
• Occurs - patients with the acute
phase of Parry-Romberg disease
• Between ages of 10 and 20 years.
• deformity is almost imperceptible
to strangers,
• but noticeable to the patient, as
well to family and friends
• This certainly causes emotional
pain for the affected patient.
Guerrerosantos J. Classification and treatment of facial tissue atrophy in Parry – Romberg Disease.Anesth
Plastic Surg 31: 424-434 , 2007

19. Type 2 Tissue
Depression
• characterized by thinness
of the soft tissues,
• with no effect on bone or
cartilage.
• The depression and loss
of volume is more
noticeable than type 1
deformity, and
• recognized by anyone
looking at the patient
Case 2. Preoperative condition of female patient with type 2 tissue
depres- sions in the temporal (A) and cheek (B) regions. (C,D) Patient 2
years after two infiltrations. (E,F) Patient 6 years after treatment with
four sessions of fat infil- trations.

20. Type 3 Tissue Depression
• soft tissues are thinner than with type 2,
• and the bony and cartilaginous tissues are also thinner.
• It is a very evident deformity (Fig. 9A and B).
• Facial depression types 3 and 4 are experienced by patients who experience
Parry Romberg disease by  age of 10 years.

21. Guerrerosantos J. Classification and treatment of facial tissue atrophy in Parry – Romberg Disease.Anesth
Plastic Surg 31: 424-434 , 2007

22. Type 4 Tissue Depression
• the most severe facial tissue depression.
• the soft tissues are so atrophied that in many cases,
• the skin is practically next to the bones.
• The cartilages and bones are thinner than with type 3.
• Besides the aesthetic deformities,
• the patients also have severe functional problems, especially
with the lips and the nose.
Guerrerosantos J. Classification and treatment of facial tissue atrophy in Parry – Romberg Disease.Anesth
Plastic Surg 31: 424-434 , 2007

25. Treatment of Facial Tissue Atrophy Depressions
Patients with
- Depression Types 1 and 2  treated with lipoinjection sessions
(Table 1) as isolated procedures.
Guerrerosantos J. Classification and treatment of facial tissue atrophy in Parry – Romberg Disease.Anesth
Plastic Surg 31: 424-434 , 2007

31. Type 3 depressions  receive a combined treatment that
- include galeal flaps,
- dermis-fat grafts,
- lipoinjection sessions, and
- cartilage and bone grafts.
- free transplantation of flaps with microsurgery.
Type 4 depressions, 
- treatment is similar to that for type 3,
- except that the thickness of the flaps and grafts is thicker.

32. GALEAL FLAP

33. FREE DERMIS FAT GRAFT

39. Treatment
• Many surgeons will defer treatment until the disease “burns out,” or
reaches a stable plateau phase.
• For milder asymmetry and atrophy of the skin and subcutaneous
tissue, 
- injection of collagen and hyaluronic acid derivatives or
- fat injection may provide some short-term benefit.
Grabb & Smith’s Plastic Surgery – 6th Edition, Chapter 29

40. • for small areas of asymmetry, -
• dermal grafts,
• fat grafts, or
• dermal-fascial-fat grafts are considered.
• These can be tailored to smaller defects and provide an acceptable
improvement.
• variability in graft survival, - overcorrection is necessary.
• Overall, the experience in the literature with nonvascularized
transfer of fat tissue, particularly with larger transfers, has been
inconsistent.
Grabb & Smith’s Plastic Surgery – 6th Edition, Chapter 29

41. • Microvascular free tissue transfer is the gold standard in
reconstruction of patients with Romberg disease.
Grabb & Smith’s Plastic Surgery – 6th Edition, Chapter 29

42. • In children with the early onset of the disorder, there is often
distortion of the orbit and the zygomaticomaxillary complex, leading
to vertical orbital dystopia.
• Depending on the severity, of bony involvement  this can be
corrected either through
- corrective osteotomies and
- vertical repositioning of the orbit, or through bone grafting of the
orbital floor.
• Involvement of the V2 and V3 distributions of the trigeminal nerve
can lead to severe maxillary and mandibular asymmetries,
• with distortion of both the facial midline and occlusal plane.
• Bimaxillary surgery is necessary to correct of the occlusal plane.
Grabb & Smith’s Plastic Surgery – 6th Edition, Chapter 29

43. Treatment Plan
• Type III
• Soft tissue correction  dermal fat graft
• Bony correction  mandibular osteotomies
• Depending on the loss  fat graft

44. THANK YOU…