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Year : 2013  |  Volume : 4  |  Issue : 3  |  Page : 130-133

Parry-Romberg syndrome: A case report and an insight in to the advances in its pathophysiology and treatment

Department of Oral and Maxillofacial Pathology, Royal Dental College (KUHS), Chalissery, Kerala, India

Date of Submission17-May-2013
Date of Acceptance15-Jul-2013
Date of Web Publication12-Dec-2013

Correspondence Address:
Deepthi Karukayil
Department of Oral and Maxillofacial Pathology, Royal Dental College (KUHS), Chalissery, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0976-6944.122960

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Parry-Romberg syndrome is a rare degenerative condition of unknown etiology characterized by slowly progressive atrophy of facial tissues including subcutaneous tissue, muscle, bone and skin. Along with the loss of facial symmetry it leads to several functional and psychological problems. Though the disease does not deteriorate further after few years of progress the deformity caused by this disease will not disappear. Moreover, there is no universally accepted treatment or permanent cure for this condition. Here a 45-year-old female with Parry-Romberg syndrome is presented along with a review of the literature concerning the general characteristics, etiology, pathophysiology, differential diagnosis and treatment.

Keywords: Coup-de-sabre, hemi-facial atrophy, Parry-Romberg, progressive

How to cite this article:
Karukayil D, Sunil A, Mukunda A, Basheer AB. Parry-Romberg syndrome: A case report and an insight in to the advances in its pathophysiology and treatment. Indian J Oral Sci 2013;4:130-3

How to cite this URL:
Karukayil D, Sunil A, Mukunda A, Basheer AB. Parry-Romberg syndrome: A case report and an insight in to the advances in its pathophysiology and treatment. Indian J Oral Sci [serial online] 2013 [cited 2020 Feb 20];4:130-3. Available from: http://www.indjos.com/text.asp?2013/4/3/130/122960

  Introduction Top

 Parry-Romberg syndrome More Details (progressive facial hemiatrophy), a slowly progressive but self limited hemifacial atrophy of the skin and subcutaneous tissue, was first reported by Caleb Parry in 1815 and then described as syndrome by Moritz Romberg in 1846. [1],[2],[3] There is progressive wasting of subcutaneous fat, at times, accompanied by the atrophy of the skin, cartilage, bone and muscles. [4] In most of the instances atrophy is confined to one side of the face and cranium; however it may occasionally spread to the neck and one side of the body. The onset is insidious and usually occurs during the first two decades of life. [5] It is usually accompanied by neurological complications like trigeminal neuralgia, migraine, epilepsy and changes in the eyes and hair. [6],[7],[8]

Though hemifacial atrophy occurs sporadically, some familial distribution has been reported. The disease is common in females (F:M ratio is 3:1). The cause and pathophysiology are unknown. There is no specific treatment. [4],[5] Sometimes progressive hemifacial atrophy is treated with autotransplantation of thoracodorsal flaps, and other facial reconstructive surgery with free fatty tissue. [9]

  Case Report Top

A 45-years-old female was presented with history of slowly progressive atrophy of left half of the face of ten years duration. There was no relevant family history, history of trauma or infections. However, the patient noticed the change soon after her second delivery. On clinical examination there was atrophy of right half of the chin, lower lip and upper lip. A slight hyperpigmentation on the affected side of the chin and coup de sabre on the chin extending to the lower lip were also noticed [Figure 1]. Intraoral examination showed atrophy and fissuring of the affected side of the tongue [Figure 2]. Left half of mandible was hypoplastic. There was no neurological deficit. A diagnosis of Parry-Romberg syndrome was made based on the clinical features.
Figure 1: Atrophy of left side of the chin and coup de sabre

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Figure 2: Atrophy and a deep cleft on the left half of the tongue

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  Discussion Top

Parry-Romberg syndrome is a rare degenerative disease with prevalence rate of 1 in 700,000. It is seen more often in females and has a predilection for left side of the face, both of which is true in this case. The common age of onset of the disease is first or second decade of life and progresses rapidly in the first two to ten years and then stabilizes. [4]

The precise etiology and pathogenesis of this acquired disorder remains unknown. The primary cause may be cerebral disturbance of fat metabolism, vascular insult to trigeminal ganglia or trophic malformation of cervical sympathetic nervous system. [10],[11] Pathogenesis of the disease has been related with trauma, viral infections, endocrine disturbance, heredity and autoimmunity. [10] Some consider it as a variant of localized scleroderma. Blink reflex and immunological studies in these cases have indicated abnormalities in the brain stem as well. [10] According to Wartenberg the primary disturbance involves the higher centers resulting in unregulated control over lower centers and secondary involvement of peripheral nervous system causes local disturbances in fat metabolism and subcutaneous tissue. [12] Schievink et al., suggested the possibility of neural crest defect in this syndrome. [13]

Parry-Romberg syndrome is generally unilateral but 5-10% cases are described as being bilateral. Initial changes usually involve the area of the face covered by the temporal or buccinator muscles and then it progressively spreads from the initial location, resulting in atrophy of the skin and its adnexa, as well as underlying subcutaneous structures such as connective tissue, (fat, fascia, cartilage, bones) and/or muscles of one side of the face. The mouth and nose are typically deviated towards the affected side of the face along with the deviation of facial and dental midlines. [14]

Some common symptoms are sunken cheek, unilateral atrophy of the lip and tongue, hyperpigmented or hypopigmented with patches of unpigmented skin overlying affected areas (in 20% of cases), a depressed linear scar, referred to as "coup de sabre", extending down through the midface demarcating the normal and abnormal skin (61% of cases) and face pain which is usually felt around the cheek and nose. As regards in this patient almost all these findings, like unilateral atrophy of lower lip and tongue, hyperpigmented skin overlying the chin and very prominent coup de sabre were present. [8]

Ophthalmic changes like enophthalmos, pupillary disturbance. Heterochromia, uveitis, pigmentary disturbance of ocular fundi and restrictive strabismus have also been reported in patients with this syndrome. [15] The cartilage of the nose, ear and larynx can be affected and there may be associated otorhinolaryngological disorders in the form of unilateral hearing loss, frontal sinus deformity and parotid gland atrophy in Parry-Romberg disease. It may also be associated with hemiatrophy of brain, breast and half of the body along with pigmentary skin abnormalities such as vitiligo, facial nevus and morphea along with sweating and lacrimation abnormalities. The involvement of the arm, trunk or leg though unusual has been reported usually on the same side as the face. Some people may have just small patches of atrophy on their body. The hair located on the affected side of the head may turn white, and the eyelashes and eyebrow hair may fall out. People with Parry-Romberg syndrome may rarely experience nausea and vomiting. Degenerative brain lesions, intracranial calcifications, sensory impairment, excessive sweating, tear duct dysfunction may be seen. [16]

Oral manifestations like unilateral atrophy of muscle of the tongue and upper lip, delayed eruption of permanent teeth causing dental crowding, unilateral posterior cross bite and wasting away of erupted teeth have been reported. Even though there are involvement of intraoral soft tissue and muscles of mastication, the normal functions like speech and deglutition are not disturbed. In this patient in addition to atrophy of lower lip and right tongue, deep fissuring of tongue and hypoplastic mandible may be present. No radiological deficiency is seen although in some cases teeth on the affected side appear smaller with short roots compared to the normal side. [16] Parry Romberg syndrome is associated with neurological complication including the trigeminal neuralgia (52%), epilepsy (11%), facial pain (46%) and headache. [8],[15]

The differential diagnosis of Parry-Romberg syndrome is linear scleroderma. Half of the face is involved in Parry-Romberg syndrome, while in linear scleroderma only the forehead that too the skin show the changes. There is no involvement of tongue in linear scleroderma. However, both conditions occur in the same way and there are children who have areas of atrophic facial skin that do not clearly fall in to either category. [17]

Histologically, along with the atrophy of epidermis, dermis and subcutaneous tissue, variable infiltrate of lymphocytes and monocytes in dermis are discerned. Degenerative alterations on vascular endothelia are observed in electron microscopy.

  Treatment Top

Pharmacologic therapies for Parry-Romberg syndrome involve methotrexate with or without monthly pulses of systemic glucocorticoids, cyclophosphamide, and azathioprine. Since no randomized controlled trials have been conducted to evaluate such treatments, the benefits have not been clearly established. [18] A variety of microsurgical and reconstructive surgical interventions can help to correct facial atrophy in patients with Parry-Romberg syndrome such as transfer of an island parascapular fasciocutaneous flap or a free flap from the groin, rectus abdominis muscle (Transverse Rectus Abdominis Myocutaneous or "TRAM" flap) or latissimus dorsi muscle to the face. [19],[20],[21],[22],[23] Severe deformities may require additional procedures, such as pedicled temporal fascia flaps, cartilage grafts, bone grafts, orthognathic surgery, and bone distraction. [24],[25] Minimally invasive procedures like autologous lipofilling and a combination of lipofilling, poly-L-lactic acid and intensed pulse therapy have also been tried. [26],[27] The timing of surgical intervention is controversial; some surgeons prefer to wait until the disease has run its course while others recommend early intervention.

  Conclusion Top

There is no universally accepted and satisfying treatment modality till date scientists are working on autologous stem cell transplantation which may result in providing relief to the Parry-Romberg syndrome patients.

  References Top

1.Gulati S, Jain V, Garg G. Parry Romberg syndrome. Indian J Pediatr 2006;73:448-9.  Back to cited text no. 1
2.Parry CH. Collection from the unpublished medical writings of the Late Calet Hillier Parry. London, England: Under-woods; 1825. p. 478.  Back to cited text no. 2
3.Romberg MH. Trophoneurosen Kliniske Ergenbrisse. Berlin, Germany: Forster; 1846. p. 75-81.  Back to cited text no. 3
4.Stone J. Parry Romberg syndrome. Pract Neurol 2006;6:185-8.  Back to cited text no. 4
5.Hickman JW, Sheils WS. Progressive facial hemiatrophy. Report of a case with marked homolateral involvement. Arch Intern Med 1964;113:716-20.  Back to cited text no. 5
6.Sagild JC, Alving J. Hemiplegic migraine and progressive hemifacial atrophy. Ann Neurol 1985;17:620.  Back to cited text no. 6
7.Chbicheb M, Gelot A, Rivier F, Roubertie A, Humbertclaude V, Coubes P, et al. Parry-Romberg's syndrome and epilepsy. Rev Neurol (Paris) 2005;161:92-7.  Back to cited text no. 7
8.Stone J. Parry-Romberg syndrome: Global survey of 205 patients using the Internet. Neurology 2003;61:674-6.  Back to cited text no. 8
9.Davis WB. Reconstruction of hemiatrophy of face. Case report. Plast Reconstr Surg 1968;42:489-91.  Back to cited text no. 9
10.Lonchampt P, Emile J, Pélier-Cady MC, Cadou B, Barthelaix A. Central sympathetic disregulation and immunologic abnormalities in a case of progressive facial hemiatrophy (Parry-Romberg disease). Clin Auton Res 1995;5:199-204.  Back to cited text no. 10
11.Taylor HM, Robinson R, Cox T. Progressive facial hemiatrophy MRI appearances. Dev Med Child Neurol 1997;39:484-6.  Back to cited text no. 11
12.Wartenberg R. Progressive facial hemiatrophy. Arch Neurol Psychiatry 1945;54:75-96.  Back to cited text no. 12
13.Schievink WL, Mellinger JF, Atkinson JL. Progressive intracranial aneurysmal disease in a child with progressive hemifacial atrophy (Parry-Romberg disease) case report. Neurosurgery 1996;28:1237-41.  Back to cited text no. 13
14.Cory RC, Clayman DA, Faillace WJ, McKee SW, Gama CH. Clinical and radiologic findings in progressive facial hemiatrophy (Parry-Romberg syndrome). AJNR Am J Neuroradiol 1997;18:751-7.  Back to cited text no. 14
15.Kumar AA, Kumar RA, Shantha GP, Aloogopinathan G. Progressive hemi facial atrophy-Parry Romberg syndrome presenting as severe facial pain in a young man: Case report. Cases J 2009;2:6776.  Back to cited text no. 15
16.Terstegge K, Kunath B, Felber S, Speciali JG, Henkes H, Hosten N. MR of brain involvement in progressive facial hemiatrophy (Romberg disease): Reconsideration of a syndrome. AJNR Am J Neuroradiol 1994;15:145-50.  Back to cited text no. 16
17.Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients. J Am Acad Dermatol 2007;56:257-63.  Back to cited text no. 17
18.Stone J. Neurological rarity: Parry-Romberg syndrome. Pract Neurol 2006;6:185-8.  Back to cited text no. 18
19.Iñigo F, Jimenez-Murat Y, Arroyo O, Fernandez M, Ysunza A. Restoration of facial contour in Romberg's disease and hemifacial microsomia: Experience with 18 cases. Microsurgery 2000;20:167-72.  Back to cited text no. 19
20.Jurkiewicz J, Nahai F. The use of free revascularized grafts in the amelioration of hemifacial atrophy. Plast Reconstr Surg 1985;76:44-55.  Back to cited text no. 20
21.Moore MH, Wong KS, Proudman TW, David DJ. Progressive hemifacial atrophy (Romberg's disease): Skeletal involvement and treatment. Br J Plast Surg 1993;46:39-44.  Back to cited text no. 21
22.Foster TD. The effects of hemifacial atrophy of dental growth. Br Dent J 1979;146:148-50.  Back to cited text no. 22
23.Roddi R, Riggio E, Gilbert PM. Clinical evaluation of techniques used in the surgical treatment of progressive hemifacial atrophy. J Craniomaxillofac Surg 1994;22:23-32.  Back to cited text no. 23
24.Muchnick RS, Aston SJ, Rees TD. Ocular manifestations and treatment of hemifacial atrophy. Am J Ophthalmol 1979;88:889-97.  Back to cited text no. 24
25.de la Fuente A, Jimenez A. Latissimus dorsi free flap for restoration of facial contour defects. Ann Plast Surg 1989;22:1-8.  Back to cited text no. 25
26.Donofrio LM. Panfacial volume restoration with fat. Dermatol Surg 2005;31:1496-505.  Back to cited text no. 26
27.Onesti MG, Monarca C, Rizzo MI, Mazzocchi M, Scuderi N. Minimally invasive combined treatment for Parry Romberg syndrome. Aesthetic Plast Surg 2009;33:452-6.  Back to cited text no. 27


  [Figure 1], [Figure 2]


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