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Biomedical & Pharmacology Journal Vol.

7(1), 207-211 (2014)

Parry Romberg Syndrome-A Review of Treatment Options


BANU SARGUNAR1, VIJAY EBENEZER2, R. BALAKRISHNAN3 and SWARNA PRIYA4

Department of Oral and Maxillofacial Surgery, Sree Balaji Dental College and Hospital,
Bharath University, Chennai, 600100, India.
*Corresponding author E-mail: drbanuabe@gmail.com

http://dx.doi.org/10.13005/bpj/473

(Received: January 10, 2014; Accepted: February 02, 2014)

ABSTRACT

Parry-Romberg Syndrome, also known as Progressive Hemifacial Atrophy, is an uncommon


degenerative condition, characterized by a slow and progressive atrophy affecting one side of the face.
A cerebral disturbance of fat metabolism, atrophic malformation of Cervical Sympathetic Nervous
System has been proposed as the primary cause. Other possible factors that are involved in the
pathogenesis include trauma,viral infections, heredity, endocrine disturbances and auto-immunity.
The objective of this work is, to accomplish a literature review concerning general characteristics,
etiology, physiopathology, differential diagnosis and treatment of progressive hemifacial atrophy. A
review of literature with variety of treatment options have been discussed in an attempt to treat
the atrophic deformties from mild to severe cases. These have included free fat grafts, dermis fat
grafts, fascia,muscle grafts, cartilage and bone augmentation ,Orthognathic surgery,microvascular
reconstruction and stem cell regeneration of bone and soft tissues.

Key words: Hemifacial atrophy, Parry-Romberg syndrome, physiopathology, treatment options.

INTRODUCTION neuralgia, facial paresthesia, severe headache and


contra lateral epilepsy. The presence of antinuclear
Hemifacial atropy is a uncommon antibodies in the serum suggested that the Parry-
degenerative slowly progressing unilateral atrophy Romberg syndrome may be a form of localized
of the facial tissues including the skin, subcutaneous scleroderma.
fat, muscle, cartilage and bone. This was first
described by Parry and Romberg in the year 1846. Clinical features
Onset of Disease occurs at first decade of life and The shape and symmetry of head is
progresses slowly over years frequenly for 2 to 10 abnormal with a cleft on the midline. The head is flat
years and reaches a stable burn out phase. 10 It is in the anterior region along the midline. Flattening
uncommon and unilateral with a higher incidence is seen in the cranial vault at midline extending
rate in females18. The extension of the atrophy is till hair line. Depression or furrow is present at
frequently limited to one side of the face16, and anterior fontanelle region and at the mid line of
cranium, it may occasionally spread to the neck and fore head close to hair line. Shape of the forehead
one side of the body and it is accompanied usually by is asymmetrical with a depression on midline of
ocular involvement, the most frequent manifestation forehead close to glabella. Enopthalmus of the right
is enophthalmy, deviation of mouth and nose to the eye due to atrophy of orbital fat occurs. Supra orbital
affected side, and unilateral exposure of teeth (when margin appears normal with a prominent infraorbital
the lips are involved). Occasionally, there may be margin. Nasal bones appear normal with depression
some neurological complications, such as trigeminal seen on the affected side of nose close to the tip.
208 SARGUNAR et al., Biomed. & Pharmacol. J., Vol. 7(1), 207-211 (2014)

The nose is deviated to the right side. Ears appear Transaxial FLAIR MR Images demonstrate
normal. Zygoma, maxilla and mandible appear atrophy of skin and subcutaneous tissues overlying
asymmetrical on the affected side. Atrophy of soft the left frontal calvarium as well as ipsilateral cerebral
tissue is seen in the infraorbital, zygomatic and atrophy and diffuse white matter hyperintensities
mandibular region. On palpation, the supraorbital, involving the left frontal, parietal, and occipital
infraorbital, zygoma, maxilla and mandible appear lobes, external capsule, and corpus callosum
almost symmetrical on both the sides. There is loss splenium. Axial T2*-weighted, gradient-echo MR
of soft tissue bulk on upper and lower eye lid. The images demonstrate ipsilateral microhemorrhages
eye looks sunken on the affected side due to loss involving the left isthmus of the cingulate gyrus,
of orbital fat14. There is also loss of hair in the lower parietal and occipital white matter, thalamus, and
eye lid. Due to complete loss of soft tissue bulk in corpus callosum splenium. Additionally, a cystic
the cheek and the mandibular region on the affected lesion lined by hemosiderin is demonstrated in
side, there is stretching of skin on the entire aspect the left superior frontal lobe, consistent with old,
of the right side, causing depression of ala of nose, encapsulated hematoma21.The finding of unilateral
retraction of upper and lower lip, prominent exposure cerebral microhemorrhages ipsilateral to facial
of infraorbital rim and zygoma, angle of mandible. hemiatrophy suggests that some cases of Parry-
Skin is pigmented more on the right zygomatic Romberg syndrome may be secondary to a small-
region, ramus region and the corner of the mouth. vessel neurovasculopathy.
There is scarring in the infraorbital region extending
till hair line in front of the ear. On clenching, the According to Wells and luce classification
masseter and temporalis muscles appear prominent. of defect, Parry Romberg syndrome is considered
Muscle bulk is comparatively less on the affected as type 2 defect
side. There is excessive exposure of teeth on the Type 1 Cutaneous defect, subcutaneous and
affected side during smiling. Upper and lower lip underlying bony frame work intact eg.
is thin. There is a deep cleft seen on the affected nevi, scar
side of the chin. Maxilla and mandible appears Type 2 Deep soft tissue defect, involving
to be prognathic. The most important features of muscles, require greater bulk to restore
this pathology are enophthalmy, the deviation of facial contour Eg. Romberg syndrome,
mouth and nose to the affected side, and unilateral lipodystrophy, hemifacial microsomia
exposition of teeth when lips are affected13,16. Type 3 Full thickness defect of cheek due to
resection of malignant neoplasm
The condyle is slightly larger on the affected Type 4 Deformities of bony structures or frame
side compared to the other. The roots of the posterior work of maxilla and zygoma leading to
teeth on the affected side appear slightly shorter. esthetic and functional morbidity of eye
and dentition
There is mild deviation of the nasal septum Type 4a Partial loss of maxilla with loss of palate
to the affected side. The mandible and condyle and alveolar ridge
appears larger on the affected side. There is pain Type 4 b Extensive loss of maxillar y bone
and tenderness over messetric region on wide including nasomaxillary, zygomaticom-
opening. axillary region and floor of orbit

128 slice CT of facial bones shows Aetiology


reduction in the size of right side of skull bones Theories proposed include neurogenic, vascular,
and thickness of soft tissues. There is thinning of exogenous insult and autoimmune mediated
pericranial and facial soft tissues. The orbit and the processes19. A popular hypothesis is the vasomotor
maxillary sinus on the affected side appear smaller trophoneuritis theory, involving the sympathetic
in size. Brain shows no focal lesion. CT and MR nervous system which results in the atrophy of
findings included unilateral focal infarctions in the facial tissues. 2 Immunological evidence shows
corpus callosum, diffuse deep and subcortical white involvement of noradrenergic system 15 in the
matter signal changes, mild cortical thickening.20 brain stem causing hyperactivity of the brain
SARGUNAR et al., Biomed. & Pharmacol. J., Vol. 7(1), 207-211 (2014) 209

stem sympathetic centers, possibly caused by an s &REEFATGRAFTS )NJECTIONOFASPIRATEDFAT


autoimmune process 16,17may be the primary cause s !LLOPLASTICGRAFTMATERIALS
for cutaneous and subcutaneous atrophy in Parry-
Romberg Syndrome .Wartenburg considered the Hemifacial atrophy treated with autologous
primary factor to be a cerebral disturbance leading fat transplantation: 6,7. Extraction of adipose tissue
to increased and unregulated activity of sympathetic through a cannula or a needle and the fat was
nervous system, which in turn produced the localized harvested from the buttocks using a 10cc syringe
atrophy through its trophic functions conducted by to which a 16 G lumbar puncture needle was
way of sensory trunks of trigemminal nerve. Other attached. To and fro’ movement of the LP needle
workers suggested extraction of teeth, local trauma, in the subcutaneus plane (tunneling approach),
infection and genetic factors could also be a cause. creates a culture of adipocytes which is believed
In a paper published in 1973, Poswillo attributed the to lead to more rapid revascularization and less
development of facial deformities to the disruption reabsorption. The prepared fat was injected into the
of stapedial artery. The stapedial artery functions target area in the subcutaneous plane with a 16 G
as a stopgap vascular channel during days 33-45 needle in a retrograde manner depositing ribbons of
of embryologic development (ref) fat in the desired area. Over correction was made to
compensate for subsequent resorption. The injected
Differential diagnosis fat, which was pliable, was sculpted into the designed
Localized Scleroderma, Clinically, linear contour by manual pressure.
scleroderma may present in childhood and it involves
intense loss of subcutaneous fat with ensuing In spite of the satisfactory results achieved
thinning and pigmentation of the skin. It is commonly with lipofilling it may be considered an interesting
seen in the paramedian forehead region. In “en coup solution for soft tissue augmentation of the face
de sabre” atrophy of underlying muscle or bone is especially for moderate adipose defects, due
not seen. There is prolonged nerve conduction in to its repeatability, no donor site morbidity, no
areas affected by scleroderma which do not exist complications at the recipient site such as lesions
in Romberg’s16, 21. Anti-nuclear anti-body titres are resulting from dissection, bleeding, necrosis, etc. This
often raised with active linear scleroderma, but technique can be performed in a day-hospital with
rarely so with Romberg’s disease. The presence short surgery time, at low cost and without a highly
of antinuclear antibodies in the serum suggestive skilled team. For severe grades of adipose atrophy,
of Parry-Romberg syndrome which may be a form because of the low blood supply to these tissues
of localized scleroderma. The other differential which interferes with take of any type of autograft,
diagnosis include, lupus erythematosis, trigeminal we think that free flaps actually represent one of the
neuritis and chronic neurovasculitis. best solutions for soft tissue augmentation11.
Advantages
Treatment options s #OSTEFFECTIVE
Medical management s 3IMPLETECHNIQUE
s #ORTICOSTEROIDSTOPICALANDINTRALESIONAL s .OREJECTIONANDALLERGY
s 2ETINOIDS s 'OOD RESULTS AT THREE YEARS APPEAR
s !NTIOXIDANTS encouraging
s )MMUNOSUPPRESSANTSMETHOTREXATE 
Disadvantage
DISCUSSION Over-correction of the region with gradual
atrophy may require further treatment8.
Surgical treatment options
s &ASCIAGRAFTS Free vascularized tissue transfer should be
s -USCLEGRAFTS considered for young patients, because the free flap
s 0EDICLEmAPS is the best among the procedures for Romberg’s
s -ICROVASCULAR FREE FLAPS ALLOW FOR THE disease for maintaining volume.
permanent correction of large deformities
210 SARGUNAR et al., Biomed. & Pharmacol. J., Vol. 7(1), 207-211 (2014)

Reconstruction of hemifacial atrophy with a free


flap of omentum:9 The ideal host bed for allografts is one
s 0ROVIDEADEQUATETISSUEINTHREEDIMENSIONAL that is covered by well-vascularized thick skin and
plane for reconstruction, subcutaneous tissue that is not subjected to the
s !DEQUATEBLOODSUPPLY stresses of trauma or motion.
s ,ACKOFSUFlCIENTATROPHYOFTHESOFTTISSUE s (IGHDENSITY0OROUSPOLYMER-EDPOR
mass after several years follow-up. s 0OROUSPOLYMERS
s 0OLYTETRAmUROETHYLENE PROPLAST
Disadv s %XPANDEDlBRILLATED04&% GORETEX
Intraabdominal harvest,Difficulty in fixation, s 3OLIDPOLYMERS
and excessive bulk s 3ILICONE
s 0OLYMETHYLMETHACRYLATE
Free Jejunal flaps s ,IQUIDSILICONE
Segment of jejunum can be removed s -ESHEDPOLYMERSnMERSILENE -ARLEX
without impairing absorption and digestion s 0OLYn, LACTICACID
s 0O L Y , L A C T I C A C I D  0 , , ! W H I C H I S
Advantages biocompatible and biodegradable
Pliable and elastic allows the surgeon to mold s Sculptra is used primarily to augment the soft
and adapt to reconstruct the defect tissues of the face, replacing fullness in areas
Galea temporoparietal fascia flap,temporalis of fat loss (lipoatrophy)
muscle flap13 s  )NJECTIONS OF A HYALURONIC ACID lLLER24 into
Reconstruction of orbital ,forehead and cheek the right side of the upper lip improved the
region cosmetic appearance and further treatment
Aesthetic treatment of progressive Hemi facial with autologous fat transfer is planned.
atrophy s Stem cells The controlled study, conducted
use of a pedicled platysma muscle flap. by Dr Kyeung-Suk Ko and Dr Jong-Woo Choi
and led by Dr Jeong-chan Ra of RNL Stem
The platysma muscle flap was used for Cell Technology Institute, painlessly removed
reconstruction in four cases of severe to moderate a few ounces of fat from one group Parry-
disease.7The platysma flap was transected at the Romberg Syndrome patients, harvesting
clavicular level, turned at the mandibular margin, and stem cells from these patients’ fat.s Adding
spread subcutaneously on the affected side from the stem cells to standard-of-care therapies,
nasolabial fold up to the orbital margin and laterally described a revolutionary finding, that “adult”
to the anterior part of the ear.The flap masked the mesenchymal stem cells saw unprecedented
atrophy relatively well, and no complications were improvement in the effectiveness of therapies.
seen during or after the procedure. stem cell transplantation is considered as
Candidate for free flap: standard of care of treatment23.
s 0ATIENTSWITHSEVEREABNORMALITIES
s 9OUNGPATIENTS Prognosis16
s 4IMING FOR RECONSTRUCTION 7AIT FOR   In some cases, the atrophy stops before the
months after atrophy stops entire face is affected. In mild cases, the disorder usually
causes no disability other than the cosmetic effects.
Allografts
Implantation of autogenous tissue for major Recovery period for overall prognosis of
contouring is associated with morbidity from the 0ARRYn2OMBERGSYNDROMEISUNPREDICTABLE)TISAN
donor site, Prolongation of the surgery, Possibility of auto-limitable condition and there is no cure. The
absorption if the tissue is not vascularised,Insufficient treatment is usually based on reposition of adipose
amounts of autogenous material, Difficulties tissue22
in contouring of grafts, necessitates the use of
alloplastic material for facial augmentation.
SARGUNAR et al., Biomed. & Pharmacol. J., Vol. 7(1), 207-211 (2014) 211

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