Home About us Editorial board Ahead of print Current issue Archives Submit article Instructions Subscribe Search Contacts Login 
  • Users Online: 950
  • Home
  • Print this page
  • Email this page


 
 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 26  |  Issue : 2  |  Page : 237-240

Parry Romberg syndrome: A rare case report


Department of Oral Medicine and Radiology, Panineeya Mahavidyalaya Institute of Dental Sciences and Research Centre, Hyderabad, Telangana, India

Date of Submission14-Aug-2014
Date of Acceptance10-Oct-2014
Date of Web Publication30-Oct-2014

Correspondence Address:
Raj Kumar Badam
Department of Oral Medicine and Radiology, Panineeya Mahavidyalaya Institute of Dental Sciences, Road No.5, Kamala Nagar, Dilshuknagar, Hyderabad - 500 018, Telangana
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.143716

Rights and Permissions
   Abstract 

The Parry Romberg syndrome (PRS) is a rare neurocutaneous disorder characterized by progressive facial hemiatrophy. Parry Romberg syndrome is characterized by a slow progressive atrophy that appears in the early stages of life, primarily affecting the subcutaneous tissue and subjacent fat on one side of the face. Dental findings include delayed tooth eruption, retarded root formation, root resorption, oligodontia, microdontia, dilacerations, pulp stones, reduction in the height and width of the ramus and body of the mandible, and delayed mandibular angular development. We describe the case of a 24-year-old female patient with stabilized, moderate, facial hemiatrophy on the right side of her face with a varied presentation.

Keywords: Atrophy, hemifacial atrophy, parry Romberg syndrome (PRS)


How to cite this article:
Badam RK, Dara BB, Pallerla SR, Boringi M. Parry Romberg syndrome: A rare case report . J Indian Acad Oral Med Radiol 2014;26:237-40

How to cite this URL:
Badam RK, Dara BB, Pallerla SR, Boringi M. Parry Romberg syndrome: A rare case report . J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2019 Oct 21];26:237-40. Available from: http://www.jiaomr.in/text.asp?2014/26/2/237/143716


   Introduction Top


The Parry Romberg syndrome (PRS) was first described by Parry (1825) and Romberg (1846) as trophoneurosis facialis. [1] It is characterized by progressive atrophy of one side of the face, mainly involving the skin, fat, and muscles. The bones and cartilage are rarely affected. The distribution of PRS generally follows the pattern of sensory innervations. Frequently, extensions may include the entire half of the face; ipsilateral body involvement is rarely found. Atrophy occurring bilaterally has been reported in 5 to 10% of the cases. [2] The left side of the face is involved in more than 85% of the cases. This syndrome has a greater prevalence among women and its onset is between five and fifteen years of age. The progression of atrophy often lasts between two and ten years, at which point the process tends to stabilize. The degree of facial deformity is usually more severe if it appears in the first decade of life. In some cases, the disorder is apparent at birth. Neurological symptoms include epilepsy and migraine. [3]

The etiology of PRS is not well-understood, although a reliable theory is that of a genetic alteration in the first stage of embryogenesis of the central nervous system (CNS). Other causes include viral or bacterial infections, trauma, endocrine disturbances, and heredity. [4]

The syndrome is characterized by atrophy of the skin, subcutaneous tissue, muscle, and bone. In advanced forms, the affected areas can become hyperpigmented, with patches of vitiligo. [2] This process can make the skin thin, dry, and atrophied, but with free-moving sebaceous glands. [5]

Oral manifestations include abnormalities of the tongue, lip, masticatory muscles, delayed tooth eruption, tooth loss, deficient root development or root resorption in the teeth, on the affected side. [1],[3] The esthetic impairment is obvious, and therefore, facial reconstruction is often solicited. [1],[6]


   Case Report Top


A 24-year-old female patient reported with a chief complaint of pain in the right lower back region of the jaw since two years. The pain was severe, lancinating, radiating, and intermittent in nature. It got aggravated on talking and chewing food, and lasted for about ten minutes. It was associated with fasciculation on the right side of the face and deviation of the jaw and nose to the right side. The patient also gave a history of slow progressive decrease in the size of the right half of the face since two years, blackish discoloration of the skin below the right corner of the mouth, and decrease in size of the right half of the lower lip. There was no history of trauma or infection. The family and past medical histories were noncontributory. Extraoral examination revealed facial asymmetry and deviation of the mouth to the right side, with atrophy of the right half of the lower lip [Figure 1]. Fasciculations on the right side of the face were noted during clinical examination, which ended with severe pain (with a severity producing tears in the patient's eyes). Intraoral examination revealed mild atrophy and deviation of the tongue toward the right side [Figure 2]. Hard tissue examination showed a full complement of teeth, with no other abnormalities. On the basis of the history and clinical examination, a provisional diagnosis of hemifacial atrophy of the right side of the face was given. A differential diagnosis of Parry Romberg syndrome, Goldenhar syndrome, and partial lipodystrophy were considered. The patient was subjected to investigations. A panoramic radiograph revealed a full complement of teeth, with decreased height of the mandible, with irregular thinning of the cortex on the right body of the mandible [Figure 3]. Biochemical and hematological investigations with an antinuclear antibody (ANA) profile were not contributory. 3D computed tomography (CT) revealed exophthalmoses of the right eye, with decreased fat tissue [Figure 4] and decrease in the height of the right body of the mandible [Figure 5]. Magnetic resonance imaging (MRI) of the brain showed no significant neurological findings, but it revealed a decrease in the volume of the head of the right condyle and inflammation of the muscles attached to the condyle [Figure 6]. The clinical and radiological findings were suggestive of the Parry Romberg syndrome. As the condition was self-limiting, the patient was put on observation and was given analgesics (Aceclofenac, 100 mg) whenever required.
Figure 1: Extraoral photograph of the patient showing facial asymmetry and deviation of the mouth to the right side, with atrophy of the right half of the lower lip

Click here to view
Figure 2: Intraoral photograph revealing mild atrophy and deviation of the tongue toward the right side

Click here to view
Figure 3: Panoramic radiograph revealing a full complement of teeth with decreased height of the mandible, with irregular thinning of the cortex on the right body of the mandible

Click here to view
Figure 4: 3D CT revealing exophthalmoses of the right eye, with decreased fat tissue on the right side when compared to the left side

Click here to view
Figure 5: 3D CT showing a decrease in the height of the right body of the mandible

Click here to view
Figure 6: MRI revealing a decrease in the volume of the head of the right condyle and inflammation of the muscles attached to the condyle

Click here to view



   Discussion Top


The Parry Romberg syndrome is a rare, acquired, neurocutaneous syndrome of unknown etiology. The syndrome has a higher prevalence in females, and typically appears between five and fifteen years of age with involvement of the left side of the face, more commonly. Our patient presented to us in the third decade, with the right side of her face affected. The principle features include atrophy of the soft tissues and sometimes the bone, on one half of the face or forehead, without facial weakness. Sometimes the atrophy extends to the limbs, usually on the same side, and there may be various ophthalmological and neurological complications. [7] Our patient did not have atrophy of the limbs or any other neurological complications. Progressive atrophy is generally localized to a small area of the skin, with atrophy of the underlying muscles, bones, and cartilage, otorhinolaryngological disorders associated with hearing loss and parotid gland atrophy. [8] Our patient had atrophy of the right body of the mandible, with no residual muscular atrophy.

Inego et al., proposed the classification of PRS as mild, moderate, and severe, depending on the involvement of the skin, subcutaneous tissue, nerve, and bone involvement. [9] Oral manifestations include atrophy of the upper lip and tongue of the affected side. The soft and hard palate may also be deficient in all directions on the affected side, leading to facial concavity. The intraoral soft tissue and muscles of mastication can be affected by the disease process, but they usually function normally without impairment of movement, speech or deglutition. [2] A decrease in the depth and width of the retromolar region may occur. [10] The disturbances in jaw growth can result in unilateral malocclusion on the involved side and deviation of the facial and dental midlines. The dental findings include delayed ipsilateral tooth eruption, missing teeth, atrophic root development, retarded root formation, root resorption, oligodontia, microdontia, dilacerations, unilateral crossbite, and pulp stones on the affected side. [1] No such findings were manifested in our patient. Apart from the above, our patient presented with fasciculations on the affected side, which were sparsely reported in literature. [9]


   Conclusion Top


Although the condition is rare and self-limiting, it often mandates a thorough clinical evaluation for proper treatment planning, since the clinical presentation of the syndrome is diverse and it may be confused with other conditions like linear scleroderma, Goldenhar syndrome, hemifacial microsomia or partial lipodystrophy.

 
   References Top

1.
Sujatha SR, Devaraju D, Kunal A. Parry Romberg syndrome: Report of two cases with rare dental and skeletal findings. J Dent 2012;2:212-5.  Back to cited text no. 1
    
2.
O'Flynn S, Kinirons M. Parry-Romberg syndrome: A report of the dental findings in a child followed up for 9 years. Int J Paediatr Dent 2006;16:297-301.  Back to cited text no. 2
    
3.
Duymaz A, Karabekmez FE, Tosun Z, Keskin M, Karamese M, Savaci N. Reconstruction with galeal frontalis flap of depressed forehead region in progressive hemifacial atrophy. J Craniofac Surg 2008;19:1104-6.  Back to cited text no. 3
    
4.
Terenzi V, Leonardi A, Covelli E, Buonaccorsi S, Indrizzi E, Fenicia V, et al. Parry-Romberg syndrome. Plast Reconstr Surg 2005;116:97-102e.  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.
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. 6
    
7.
Lazaridou E, Giannopoulou C, Apalla Z, Fotiadou C, Trigoni A, Ioannides D. Parry-Romberg syndrome. J Dermatol Case Rep 2010;4:30-2.  Back to cited text no. 7
    
8.
Mazzeo N, Fisher JG, Mayer MH, Mathieu GP. Progressive hemifacial atrophy (Parry-Romberg syndrome). Case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;79:30-5.  Back to cited text no. 8
    
9.
Grimaldi M, Gentile P, Labardi L, Silvi E, Trimarco A, Cervelli V. Lipostructure technique in Romberg syndrome. J Craniofac Surg 2008;19:1089-91.  Back to cited text no. 9
    
10.
Sande A, Risbud M, Kshar A, Paranjpe AO. Progressive hemifacial atrophy. Dent Res J 2013;10:108-11.  Back to cited text no. 10
[PUBMED]  Medknow Journal  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

   Abstract Introduction Case Report Discussion Conclusion Article Figures
  In this article
 References

 Article Access Statistics
    Viewed957    
    Printed22    
    Emailed0    
    PDF Downloaded176    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]