• Users Online: 596
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Partners Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 2  |  Page : 121-124

Progressive facial deformity from childhood to adulthood in a patient of Parry-Romberg syndrome


Department of Orthodontics, Dr. Harvansh Singh Judge Institute of Dental Sciences and Hospital, Panjab University, Chandigarh, India

Date of Submission03-Feb-2020
Date of Acceptance02-Jun-2020
Date of Web Publication31-Jul-2020

Correspondence Address:
Dr. Deepak Kumar Gupta
#301, Third Floor, Department of Orthodontics, Dr. Harvansh Singh Judge Institute of Dental Sciences and Hospital, Panjab University, Sector 25, Chandigarh - 160 014
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jclpca.jclpca_3_20

Rights and Permissions
  Abstract 


Parry-Romberg syndrome (PRS) is an uncommon degenerative craniofacial condition characterized by a slow, progressive, and unilateral atrophy of facial tissues, including muscles, cartilage, nerves, bones, and skin. This article reports a severe case of PRS in a 22-year-old adult female, exhibiting facial asymmetry, hypoplasia of the right side of the face, areas of skin hyper pigmentation, and oral changes involving the mandible and teeth in whom orthodontic intervention is being done.

Keywords: Electromyography, facial hemiatrophy, hyperpigmentation, lipoinjections, orthodontic treatment, Parry-Romberg syndrome


How to cite this article:
Yadav N, Gupta DK, Utreja A, Garg AK. Progressive facial deformity from childhood to adulthood in a patient of Parry-Romberg syndrome. J Cleft Lip Palate Craniofac Anomal 2020;7:121-4

How to cite this URL:
Yadav N, Gupta DK, Utreja A, Garg AK. Progressive facial deformity from childhood to adulthood in a patient of Parry-Romberg syndrome. J Cleft Lip Palate Craniofac Anomal [serial online] 2020 [cited 2020 Aug 8];7:121-4. Available from: http://www.jclpca.org/text.asp?2020/7/2/121/291142




  Introduction Top


 Parry-Romberg syndrome More Details (PRS) or progressive hemifacial atrophy is a rare degenerative craniofacial condition, characterized by slow, progressive, and unilateral atrophy of facial tissues, including muscles, cartilage, nerves, bones, and the skin. It was first described by Caleb Hillier Parry (1825)[1] and later by Moritz Heinrich Romberg (1846) as a syndrome.

The onset of this syndrome occurs in the first two decades of life; being more severe if present in first decade, affecting females more than males and is usually restricted to one side of the face which sometimes involve the arms, trunk, and legs.[2]

The etiological factors can be viral infections, trauma, genetic factors, cerebral disturbance of fat metabolism, endocrine disturbances, and peripheral trigeminal neuritis. It may be associated with various deformities such as en coup de sabre, linear scleroderma, port wine stain, endocrine, cardiac, ophthalmologic and orthodontic abnormalities.[3]

Classification

Various classification methods have been proposed to classify PRS.

According to Guerrerosantos et al.,[4] this case can be classified as type 4, Type C according to Kawamoto,[5] and severe according to both Raposo-do-Amaral et al.[6] and Iñigo et al.[7]


  Case Report Top


A 22-year-old female patient reported with a chief complaint of irregularly placed upper front teeth and spacing in lower front teeth.

On extraoral examination, the patient has a large area of hyper pigmentation on the right side of the face and neck, grossly asymmetrical face, cheek depression, and a noticeable difference in eye level with the right eye at a lower level with a convex facial profile [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d.
Figure 1: Extra oral photographs of the patient showing facial asymmetry and hyperpigmentation on the affected side of the face. (a) Profile view. (b) Frontal view. (c) Frontal smiling view. (d) Three quarter view

Click here to view


The clinical history revealed that the progressive atrophy of the right half of the face initiated at the age of 5 years and continued until 17 years [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e. She had undergone multiple lipoinjection surgeries in which liposuction was done and about 100 ml of lipoaspirate was obtained from the medial aspect of both the thighs and periumbilical region; about 28 ml (as per the surgeon's record) of lipoaspirate was injected into right lower face around the border of mandible and around lateral orbital margin 5 years back for 3 years at an interval of 6 months each by a plastic surgeon. The patient reported with no associated central nervous system and ophthalmic problems.
Figure 2: Serial facial photographs of the patient showing the progression of deformity since childhood. (a) At the age of 8 years. (b) At the age of 12 years. (c) At the age of 15 years. (d) At the age of 17 years. (e) At the age of 22 years

Click here to view


Intraorally, mild maxillary anterior crowding and retroclination, spacing in mandibular anteriors, cross-bite from 13 to 15, 100% overbite and hypoplasia of 21 in the distoincisal area [Figure 3]a, [Figure 3]b, [Figure 3]c, asymmetric arches with lingually tilted 46, 47, and occlusal canting [Figure 3]d, [Figure 3]e and [Figure 4] was seen.
Figure 3: Intraoral photographs of the patient (a) Right lateral view in occlusion. (b) Frontal view in occlusion. (c) Left lateral view in occlusion. (d) Maxillary occlusal view. (e) Mandibular occlusal view showing lingually tilted premolars and molars and increased depth of the vestibule

Click here to view
Figure 4: Extraoral photograph of the patient showing canting of the occlusal plane

Click here to view


Radiographically, the panoramic radiograph and posteroanterior cephalogram [Figure 5]a and [Figure 5]b revealed asymmetry with decreased ramal height (35 mm) and mandibular body length on the right side (65 mm) compared to 46 mm and 82 mm, respectively, on the left side supported by the difference in Condylion–Antegonion of 11 mm, hypoplastic right condyle and deficient pulp cavity and roots from 44 to 47 along with shift of mandibular midline toward right side by 5 mm. The affected right side gonial angle could not be established, whereas on the left side, it was 136°.
Figure 5: (a) Orthopantomogram showing the affected right side of ramus and body of the mandible. (b) Posteroanterior cephalogram. (c) Lateral cephalogram

Click here to view


Lateral cephalogram [Figure 5]c findings revealed that the patient has skeletal Class II jaw bases due to retrusive mandible with a normodivergent growth pattern. Upper incisors are severely retroclined, and lower incisors are mildly proclined.

The surface electromyography (EMG) of masseter and temporalis muscle is done on both sides of the face. It is found that motor unit action potential (MUAP) on the right side are 592 and 1907 μV and on left side 1085 and 1809 μV, respectively.


  Discussion Top


PRS may manifest several clinical characteristics, but the occurrence and severity of the symptoms vary individually. The severity of the atrophy can range from barely noticeable asymmetry to severe disfigurment. The osseous defects are related to the age of onset and can vary greatly. Late onset usually causes more skeletal changes in the lower third of the face which is observed in this case.

The esthetic treatment of atrophied face is recommended only when the complete evolution of the disease has ended as was done in the present case after the pubertal growth spurt and continued up till 17 years of age.

Nair et al.[8] stated that around 50% of affected individuals present with deviation of the nose, mouth, and dental midline toward the affected side as was seen in the present case. The stunting of roots of premolars and molars of affected side of face and other teeth has also been reported by El-Kehdy et al.

Latha et al.[9] stated that although there are no standard treatment strategies that have been accepted for PRS, symptomatic relief and halting the disease progression are the primary objectives to be provided as soon as it is diagnosed. The various treatment modalities to address the facial deformity include pulse dye lasers, dermal fat grafts, autologous fat grafts, muscle flap grafts, free silicone injections, and bone augmentations. In this case, lipoinjection surgeries were done repeatedly as was done by Castro-Govea et al. also.[10]

An interesting finding which may not have been reported in the literature so far is an increase in the depth of the lingual vestibule on the affected side of the face [Figure 3]e, which may be attributed to the overall decreased activity of the muscles. As the EMG on the affected side showed a decrease in MUAP of masseter muscle compared to the contralateral side and temporalis muscle showed a comparable value on both the sides, so, it is concluded that any nerve involvement in this case can be ruled out.

The chief complaint of the patient, i.e., malocclusion, is being addressed by comprehensive orthodontic treatment with fixed mechanotherapy [Figure 6]a, [Figure 6]b, [Figure 6]c, [Figure 6]d, [Figure 6]e and [Figure 7].
Figure 6: Mid treatment photographs. (a) Right lateral view in occlusion. (b) Frontal view in occlusion. (c) Left lateral view in occlusion. (d) Maxillary occlusal view. (e) Mandibular occlusal view

Click here to view
Figure 7: Predebonding orthopantomogram

Click here to view


Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Al-Aizari NA, Azzeghaiby SN, Al-Shamiri HM, Darwish S, Tarakji B. Oral manifestations of Parry-Romberg syndrome: A review of literature. Avicenna J Med 2015;5:25-8.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
El-Kehdy J, Abbas O, Rubeiz N. A review of Parry-Romberg syndrome. J Am Acad Dermatol 2012;67:769-84.  Back to cited text no. 2
    
3.
Parry-Romberg syndrome: An orthodontic perspective. Int J Recent Surg Med Sci 2017;3:48-52.  Back to cited text no. 3
    
4.
Guerrerosantos J, Guerrerosantos F, Orozco J. Classification and treatment of facial tissue atrophy in Parry-Romberg disease. Aesthetic Plast Surg 2007;31:424-34.  Back to cited text no. 4
    
5.
Li A, Buck A, Lee J, Meals C, Dipple K, Sedano H, et al. The Kawamoto classification scheme for Parry Romberg syndrome. Plast Reconstr Surg 2013;132:12.  Back to cited text no. 5
    
6.
Raposo-do-Amaral C, Denadai R, Buzzo C, Raposo-do-Amaral C. Therapeutic appro therapeutic approach to the Parry-Romberg syndrome based on a severity grading system. Rev Bras Cir Plást 2001;29:57-65.  Back to cited text no. 6
    
7.
Iñigo F, Rojo P, Ysunza A. Aesthetic treatment of Romberg's disease: Experience with 35 cases. Br J Plast Surg 1993;46:194-200.  Back to cited text no. 7
    
8.
Nair M, Ajila V, Hegde S, Babu GS, Ghosh R. Clinical and radiographic features of parry-Romberg syndrome. J Istanb Univ Fac Dent 2017;51:45-9.  Back to cited text no. 8
    
9.
Latha S, Jain S, Sur J, Khan F, Jain S, Ratna S. Parry Romberg Syndrome: A case report and review. International Journal of Applied Dental Sciences 2016;2:75-8.  Back to cited text no. 9
    
10.
Castro-Govea Y, De La Garza-Pineda O, Lara-Arias J, Chacón-Martínez H, Mecott-Rivera G, Salazar-Lozano A, et al. Cell-assisted lipotransfer for the treatment of Parry-Romberg syndrome. Arch Plast Surg 2012;39:659-62.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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)

 
  In this article
Abstract
Introduction
Case Report
Discussion
References
Article Figures

 Article Access Statistics
    Viewed83    
    Printed0    
    Emailed0    
    PDF Downloaded13    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]