Home | About us | Editorial board | Search | Ahead of print | Current issue | Archives | Submit article | Instructions| Reviewers

Login 
  Home Print this page Email this page Small font sizeDefault font sizeIncrease font size Users Online: 1039    
     


 
Table of Contents   
CASE REPORT
Year : 2016  |  Volume : 6  |  Issue : 4  |  Page : 387-390
Parry–Romberg syndrome affecting one half of the body


1 Department of Oral and Maxillofacial Surgery, Kalinga Institute of Dental Sciences, KIIT, Bhubaneswar, Odisha, India
2 Department of Oral and Maxillofacial Pathology, Kalinga Institute of Dental Sciences, KIIT, Bhubaneswar, Odisha, India

Date of Submission14-Apr-2016
Date of Acceptance21-Jun-2016
Date of Web Publication25-Jul-2016

Correspondence Address:
Dr. Abikshyeet Panda
Department of Oral and Maxillofacial Pathology, Campus 5, Kalinga Institute of Dental sciences, KIIT, Bhubaneswar, Odisha
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2231-0762.186792

Rights and Permissions

   Abstract 

Parry-Romberg syndrome, which is also known as progressive hemifacial atrophy, is a poorly understood rare condition. In this condition, the face shows unilateral, slowly progressive atrophy. Disturbance in fat metabolism, viral infection, trauma, heredity, endocrinal disturbances, and autoimmunity are few possible factors in its pathogenesis. Rarely, only this syndrome progresses and involves one half of the body. Our attempt is to present a case of Parry–Romberg syndrome involving one half of the body, which is a rarity in itself.


Keywords: Coup de Sabre, Parry–Romberg syndrome, progressive hemifacial atrophy


How to cite this article:
Pathi J, Mishra P, Kumar H, Panda A. Parry–Romberg syndrome affecting one half of the body. J Int Soc Prevent Communit Dent 2016;6:387-90

How to cite this URL:
Pathi J, Mishra P, Kumar H, Panda A. Parry–Romberg syndrome affecting one half of the body. J Int Soc Prevent Communit Dent [serial online] 2016 [cited 2019 Oct 21];6:387-90. Available from: http://www.jispcd.org/text.asp?2016/6/4/387/186792



   Introduction Top


Parry–Romberg syndrome is a condition characterized by a slowly progressive shrinkage of the soft tissues, and sometimes bones of the face, unilaterally and occasionally bilaterally. Atrophy of the facial tissues including muscles, bones, and skin of one side are usually noticed in this condition.[1] This condition, otherwise known as progressive hemifacial atrophy, as labelled by Eulenberg (1871), is very rare and was first described by Caleb Parry in 1815 followed by Maritz Romberg in 1846.[2],[3],[4] This condition results in several aesthetic, functional, and psychological problems when the symmetry of the face is lost. The incidence and cause of this condition is unknown, however, a cerebral disturbance on fat metabolism is blamed. Trauma, viral infection, endocrine disturbances, autoimmunity, and heredity have also been implicated in the pathogenesis.[5] It manifests in the first and second decade of life, beginning as atrophy of the skin and subcutaneous structure of the face, and progresses at a variable rate affecting the dermatomes of one or more branches of the trigeminal nerve, and then becomes stable. Hypoplasia of the underlying bone may also occur if the condition starts during the first decade.[5],[6] Other associated conditions are ocular changes, neurologic disturbances, such as focal epileptiform seizures, and trigeminal neuralgia.[7] Many times the affected skin shows discolouration due to hypopigmentation or hyperpigmentation before the atrophy sets in. Patchy alopecia may also be observed.[8] Bilateral facial atrophy or unilateral involvements of entire body are rare presentations. There is a high incidence noticed in females then males.[6] Here, we present a case of Parry–Romberg syndrome with unilateral progressive atrophy of one side of the face along with unilateral involvement of the entire body which is rarely encountered.


   Case Report Top


A 21-year-old male patient reported to the outpatient department of Kalinga Institute of Dental Sciences with a complaint of progressive deformity of the right side of the face since last 9 years. On examination, the patient had asymmetry of face on the right side with marked hypoplasia and hyperpigmentation of the skin. Lip was deviated to the right side. Right eyebrow showed loss of hair [Figure 1]. There was a linear scar (coup de sabre) extending from the forehead to mandibular menton dividing the affected side from the unaffected. On intraoral examination, right side soft palate and faucial area showed atrophy [Figure 2]. Right buccal mucosa was stiff to palpate. General examination revealed that the entire right side of the body was atrophied when compared with the left side [Figure 3]. Cone beam computed tomography (CBCT) three-dimensional (3D) reconstruction showed decreased vertical height of the right side mandible compared to the left side [Figure 4]. Clinical and radiographic findings led to a diagnosis of Parry–Romberg syndrome. Surgical intervention and alloplastic fat graft was suggested to the patient but he refused any such intervention due to financial constraints. The patient was recalled after 6 months. There was no further progression of the disease.
Figure 1: Facial profile showing marked hypoplasia of the right side of the face resulting in facial asymmetry

Click here to view
Figure 2: Intraoral image showing atrophy of the right side soft palate and faucial area

Click here to view
Figure 3: Right side of the body shows atrophy in comparison to the left side

Click here to view
Figure 4: Cone beam computed tomography image showing decreased vertical height of the right side mandible

Click here to view



   Discussion Top


Parry–Romberg syndrome is a degenerative condition which is uncommon in nature. A slowly progressive unilateral atrophy involving the facial tissue, including muscles bones and skin, is characteristic of this condition. The etiology and incidence are unknown. The primary cause is proposed to be a cerebral disturbance of fat metabolism. Viral infection, trauma, endocrine imbalance, autoimmunity, and heredity are considered to be pathogenetic.[8],[9] The condition has a high female predilection and presents mostly on the left side of the face.[4],[9],[10] However, in our case the patient was male and had presentation on the right side. The skin can be dry and can show pigmentation. A demarcation line can be present between the normal and abnormal skin, mimicking a big linear scar called coup de sabre.[4],[11],[12] The present case also showed a similar hyperpigmentation and coup de sabre. Enopthalmous is the most common ocular manifestation resultant of fat loss around the orbit. Other common complications are eyelid atrophy, uveitis, retinal vasculitis, occulomotor defects, and glaucoma.[12] Some patients present with smaller ear on the affected side due to atrophy.[11] The present case did not present any ocular manifestations or ear anomalies. Alopecia is also reported on the affected side, as seen in the present case on the right parietal region and right eyebrow.[9] Cases may be associated with neurological complications such as trigeminal neuralgia, movement disorders secondary to brain lesions, hemiplegic migraines, facial palsy, headache, epilepsy, behavioral changes, sympathetic hyperactivity, progressive intellectual deterioration due to cerebral hemiatrophy (Rasmussen encephalitis), and occulomotor nerve palsy.[5],[13],[14] The present case did not show any such complications. Frank elevation of the mouth, nose, facial midline, and dental midline is characteristic. Upper lip atrophy leads to exposure of maxillary anterior teeth. There can be atrophy of the tongue.[5] The present case showed deviation of mouth and nose to the affected side.

Hemifacial microsomia ( first and second branchial arch syndrome) and its variants such as Goldenhar syndrome, post-traumatic atrophy, and partial lipodystrophy (Barraquer-Simon Syndrome) can be considered in the differential diagnosis.[7] Treatment is limited and unsatisfactory. Transfer of autologous fat tissue to the affected side, silicon injection, inorganic implants, and bovine collagen are few modalities that have been explored.[15],[16] However, these methods can never completely restore the appearance of the patient. Our patient was suggested a surgical alloplastic fat graft but he refused the treatment.

There is a controversial relationship between Parry–Romberg syndrome and localized scleroderma. The term Parry–Romberg syndrome is advocated to be used for progressive hemifacial atrophy without features of cutaneous scleroderma. However, patients with Parry–Romberg syndrome often report cutaneous changes. Hence, it can be suggested that localized scleroderma and Parry–Romberg syndrome may represent the same disease process.[7]


   Conclusion Top


Parry–Romberg syndrome or progressive hemifacial atrophy is an uncommon condition whose pathophysiology is poorly understood. Involvement of one side of the body is still a rarity, which is seldom ever encountered. Such a rare case with involvement of entire half of the body is reported in the present case. Treatment of such cases becomes a challenge as we do not have treatment options to offer. Poorly understood conditions like this should be highlighted in the literature for enhancing the clinical acumen.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Patel H, Thakkar C, Patel K. Parry-romberg syndrome: A rare entity. J Maxillofac Oral Surg 2010;9:247-50.  Back to cited text no. 1
[PUBMED]    
2.
Parry CH. Collections from the unpublished medical writings of the late Caleb Hillier Parry. London: Underwoods; 1825. p. 478.  Back to cited text no. 2
    
3.
Romberg HM. Klinische Ergebnisse. Berlin: Forrtner; 1846. p. 75-81.  Back to cited text no. 3
    
4.
Tolkachjov SN, Patel NG, Tollefson MM. Progressive hemifacial atrophy: A review. Orphanet J Rare Dis 2015;10:39.  Back to cited text no. 4
    
5.
Pinheiro TP, Silva CC, Silveira CS, Botelho PC, Pinheiro Md, Pinheiro Jde J. Progressive Hemifacial Atrophy--Case report. Med Oral Patol Oral Cir Bucal 2006;11:E112-4.  Back to cited text no. 5
    
6.
Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and Maxillofacial Pathology 3rd ed. Philadelphia: Saunders; 2004.  Back to cited text no. 6
    
7.
Deshingkar SA, Barpande SR, Bhavthankar JD, Humbe JG. Progressive hemifacial atrophy (Parry-Romberg Syndrome) Contemp Clin Dent 2012;3:S78-81.  Back to cited text no. 7
    
8.
Jun JH, Kim HY, Jung HJ, Lee WJ, Lee SJ, Kim DW, et al. Parry-romberg syndrome with en coup de sabre. Ann Dermatol 2011;23:342-7.  Back to cited text no. 8
    
9.
Aydın H, Yologlu Z, Sargın H, Metin MR. Parry-Romberg syndrome: Physical, clinical, and imaging features. Neurosciences 2015;20:368-71.  Back to cited text no. 9
    
10.
Rangare AL, Babu SG, Thomas PS, Shetty SR. Parry-Romberg Syndrome: A Rare Case Report. J Oral Maxillofac Res 2011;2:e5  Back to cited text no. 10
    
11.
Mazzeo N, Fisher JG, Mayer MH, Mathieu GP, Mcade FG. Progressive hemifacial atrophy (Parry Romberg Syndrome). Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;79:30-5.  Back to cited text no. 11
    
12.
Fea AM, Aragno V, Briamonte C, Franzone M, Putignano D, Grignolo FM. Parry Romberg syndrome with a wide range of ocular manifestations: A case report. BMC Ophthalmol 2015;15:119.  Back to cited text no. 12
    
13.
Lee YJ, Chung KY, Kang HC, Kim HD, Lee JS. Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia. Korean J Pediatr 2015;58:354-7.  Back to cited text no. 13
    
14.
Vix J, Mathis S, Lacoste M, Guillevin R, Neau JP. Neurological Manifestations in Parry–Romberg Syndrome: 2 Case Reports. Medicine 2015;94:e1147.  Back to cited text no. 14
    
15.
Roddi R, Riggio E, Gilbert PM, Hovius SE, Vaandrager JM, van der Meulen JC. 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. 15
    
16.
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. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
1 Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?
Irina Khamaganova
Frontiers in Medicine. 2018; 4
[Pubmed] | [DOI]



 

Top
Print this article  Email this article
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,113 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed1226    
    Printed7    
    Emailed0    
    PDF Downloaded125    
    Comments [Add]    
    Cited by others 1    

Recommend this journal