|Year : 2020 | Volume
| Issue : 2 | Page : 121-124
Progressive facial deformity from childhood to adulthood in a patient of Parry-Romberg syndrome
Neeraj Yadav, Deepak Kumar Gupta, Ashok Utreja, Arun Kumar Garg
Department of Orthodontics, Dr. Harvansh Singh Judge Institute of Dental Sciences and Hospital, Panjab University, Chandigarh, India
|Date of Submission||03-Feb-2020|
|Date of Acceptance||02-Jun-2020|
|Date of Web Publication||31-Jul-2020|
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
Source of Support: None, Conflict of Interest: None
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 2021 May 15];7:121-4. Available from: https://www.jclpca.org/text.asp?2020/7/2/121/291142
| Introduction|| |
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) 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.
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.
Various classification methods have been proposed to classify PRS.
According to Guerrerosantos et al., this case can be classified as type 4, Type C according to Kawamoto, and severe according to both Raposo-do-Amaral et al. and Iñigo et al.
| Case Report|| |
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|
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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|
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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|
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|Figure 4: Extraoral photograph of the patient showing canting of the occlusal plane|
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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|
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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|| |
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. 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. 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.
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|
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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
Conflicts of interest
There are no conflicts of interest.
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