Labio-chin hypoesthesia revealing Parry–Romberg syndrome

Manel Gharbi; Imen Chaabani; Mohamed Ben Khelifa; Touhami Ben Alaya

doi:10.4103/jomr.jomr_27_20

CASE REPORT

Year : 2020 | Volume : 8 | Issue : 3 | Page : 66-70

Labio-chin hypoesthesia revealing Parry–Romberg syndrome

Manel Gharbi¹, Imen Chaabani¹, Mohamed Ben Khelifa², Touhami Ben Alaya¹
¹ Department of Radiology, Teaching Dental Clinic of Monastir, Monastir, Tunisia
² Department of Dental Medicine, Fattouma Bourguiba Hospital, Monastir, Tunisia

Date of Submission	24-Dec-2020
Date of Decision	02-Jan-2021
Date of Acceptance	25-Jan-2021
Date of Web Publication	26-Feb-2021

Correspondence Address:
Manel Gharbi
Department of Radiology, University Dental Clinic, Avicenne Street, 5000 Monastir
Tunisia

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jomr.jomr_27_20

Abstract

Parry–Romberg syndrome (PRS) is a rare condition that still having no clear etiology. It is marked by a progressive hemifacial atrophy (PHA) of the muscular, bony, glandular, and fat tissues. Such manifestations are especially present in the lower third of the face. It causes important esthetic problems together with functional and psychological issues due to facial asymmetry. The present case report involves a 33-year-old woman with PHA that started at the age of 12. Despite the evident involvement of the left side tissues and the early age of onset, she had neither sight changes nor dental malformations. PRS is a rare disorder causing PHA that could be misdiagnosed as linear scleroderma. It affects the patient's life quality on various levels. Hence, further studies and a multidisciplinary approach are necessary to determine the exact etiology and to establish precise guidelines for the treatment planning.

Keywords: Asymmetry, atrophy, hemifacial, Parry–Romberg syndrome, treatment

How to cite this article:
Gharbi M, Chaabani I, Khelifa MB, Alaya TB. Labio-chin hypoesthesia revealing Parry–Romberg syndrome. J Oral Maxillofac Radiol 2020;8:66-70

How to cite this URL:
Gharbi M, Chaabani I, Khelifa MB, Alaya TB. Labio-chin hypoesthesia revealing Parry–Romberg syndrome. J Oral Maxillofac Radiol [serial online] 2020 [cited 2023 Mar 29];8:66-70. Available from: https://www.joomr.org/text.asp?2020/8/3/66/310384

Introduction

Parry–Romberg syndrome (PRS) is a rare acquired condition displaying a unilateral progressive hemifacial atrophy (PHA) affecting different tissues. Its cause is still unknown, but several etiologies such as autoimmunity disorders, trauma, and infection have been suggested.^[1]

PRS generally manifests in the first two decades of life. It evolves over a few years before ceasing with no spontaneous recovery.

This article reports the case of an adult woman suffering from PRS without cerebral radiological findings.^[2] This is one of the few case reports of North African patients suffering from PRS.

Case Report

A 33-year-old woman was referred to oral radiology department with left labio-chin hypoesthesia as a chief complaint. She presented neither history of facial trauma, surgery or injections to the affected area, nor a preceding illness. The patient reported that retraction of both chin skin and lips had been evolving since the age of 12, but it was only 2 years ago that a left hemifacial pain first appeared.

The medical questionnaire revealed that she was suffering from pain in V1, V2, and V3 dermatomes, temporoparietal headaches, bruxism, and masseter contractions of 2–3 min each, appearing and disappearing spontaneously and occurring in both rest and movement.

Extraoral examination revealed a gross asymmetry with left hemifacial atrophy and a scar on the patient's chin and lips. The mouth opening path was deviated toward the left side. A hyperpigmentation on the left side of the neck skin was also observed [Figure 1] and [Figure 2].

Figure 1: A front view of the patient's face; note the labial scar-like skin depression (red arrows)

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Figure 2: Mouth opening deviation toward the left (affected) side (red arrow)

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Intraoral examination revealed a poor oral hygiene, a left lingual hemiatrophy, a generalized severe inflammatory gingival swelling, multiple deep periodontal sockets, and an important coronal filling (amalgam) on tooth #36.

A panoramic radiography showed a left mandibular horizontal branch smaller than the right one. However, all the teeth on both sides had a normal morphology. No signs of radicular resorption, dilaceration, or coronal atrophy were noted on the left teeth [Figure 3]. For further examination of the facial asymmetry and exploration of any possible mandibular canal involvement which could explain the hypoesthesia reported by the patient, a computed tomography (CT) scan of the maxillofacial bones was indicated.

Figure 3: Panoramic radiography showing the hypoplasia of the mandibular horizontal branch on the left side compared to the right one

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The coronal CT scans showed a remarkable atrophy affecting both the left hemiface soft tissues (masseter, subcutaneous fat) and the hard tissues (mandibular branch) when compared to the right side. The left maxillary sinus dimensions were smaller than the right one [Figure 4]. The axial CT scans showed, in addition to the asymmetry, a hypodense mandibular zone involving the roots of teeth #34 and #35 and destructing the external cortical bone [Figure 5]. Dentascan reconstructions showed that the mandibular canal was not directly involved in the osteolytic mandibular lesion. The mental foramen was also away from the hypodense zone. Nevertheless, the sclerotic aspect of medullar bone in this area showed the presence of a chronic osmotic process which could have indirectly affected the mandibular alveolar nerve and eventually resulted in the hypoesthesia described in this case [Figure 6]. Three-dimensional (3D) reconstructions were valuable as they showed obvious facial asymmetry and an atrophied aspect, mainly in the chin and the inferior lip [Figure 7].

Figure 4: Computed tomography scan coronal slice (bone window); note the bone hypoplasia (red arrows) and muscle and fat hypotrophy (yellow arrows) on the left side compared to the right side

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Figure 5: Computed tomography scan axial slice (bone window); note the muscle and fat hypotrophy on the left side compared to the right side (asterisk), chin depression can be observed (arrow)

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Figure 6: Dentascan oblique reconstructions; note the osteolytic hypodense image surrounding tooth 36 with no direct involvement of the left mandibular alveolar canal (a) and mental foramen (b)

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Figure 7: Three-dimensional reconstructions: Surface mode showing soft-tissue unilateral atrophy (a) bone mode showing parasymphyseal osseous depression together with left hemimandibular atrophy (b)

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In order to explore the patient's neuralgic pain, a cerebral magnetic resonance imaging (MRI) was indicated. It showed neither a vasculonervous (vessel-nerve) conflict nor morphological anomalies within the central structures. Once clinical and radiological data correlation was performed, the diagnosis of PRS was made. The neurologist prescribed carbamazepine and some myorelaxants which stabilized the patient's condition.

Discussion

PHA, also known as PRS, is an uncommon and poorly understood degenerative condition. It is characterized by a slowly progressive atrophy of the skin that may involve the underlying fat, muscle, and osseocartilaginous structures of half of the face.^[2],[3] It usually affects the left side. Bilateral findings are not frequent (5%–10% of cases).^[4] Only few rare cases of ipsilateral body involvement were reported.^[5],[6] This disease affects 1/700,000 subjects with a female predominance (sex ratio is 3:2).^{[7],[8],[9],[10]}

The onset of this rare disorder usually occurs in the first or second decade of life. Few cases of adult onset have been reported.^[4] The progression of the atrophy often takes place over 2 to 10 years after which the process is stabilized. The earlier the disease appears, the greater is the resultant deformity.^[11]

Several hypotheses, including trauma, heredity, autoimmunity problems, endocrine disorders, viral infections, trigeminal neuritis, and disturbance in fat metabolism of the cerebellum, were suggested to this anomaly.^{[8],[10],[12],[13]}

Clinically, some patients have a sharply demarcated line between normal and abnormal skin, resembling a linear scar, called “coup de sabre,” as presented in this case.

Facial muscles may atrophy with possible facial bone loss. The atrophy may involve the tongue as well. In addition, the skin overlying the affected areas may become discolored (usually hyperpigmented) and dehydrated. Localized areas of cicatricial alopecia are sometimes observed.^[14],[15] This degenerative condition is also occasionally accompanied by neurologic disturbances such as trigeminal neuralgia, facial paresthesia, severe headache, migraine, and epilepsy.^{[16],[17],[18],[19]} CT and MRI scans are the most regular modalities used to explore the neurologic manifestations of PRS. Sometimes, the neurological abnormalities are detected years after the onset of the cutaneous manifestations.^{[20],[21],[22]}

In this case report, the CT scans of our patient did not detect any neurological or intracranial vascular abnormalities.

Ocular involvement is common. The most frequent manifestations are enophthalmos, uveitis, and retinal vasculitis. Even sight loss cases are rarely observed.^{[23],[24],[25]}

Oral manifestations include unilateral atrophy of the oral mucosa, the salivary glands as well as the chin and lips resulting in a unilateral exposure of teeth. Hemiatrophy of the tongue may also be seen. The present case clearly showed these features of facial asymmetry, but the lingual hemiatrophy was not noticed.^[26]

If PRS onset takes place in the early years of the first decade, the atrophy may coincide with the stage of permanent dentition formation, development, and eruption. Therefore, dental problems in PRS patients could be diverse, including congenitally missing, delayed eruption/impacted teeth, microdontia, and dilacerated roots.^[13],[27] Various squelettic involvements such as unilateral posterior crossbite and anterior open bite are often resulting from one-sided jaw hypoplasia.^{[13],[23],[27],[28]}

Temporomandibular joint involvement is usually expressed as pain episodes reported while masticating together with jaw movement hindrance.^[1] These functional manifestations were noted in the case of our patient.

Communication disorders and dysphonia can also be present, especially when the disease onset happens in the early childhood years.^[14]

Radiologically, PRS patients may show hypoplasia of the mandibular horizontal branch and/or ramus on the affected side which was confirmed by our patient's the radiographs. Teeth may present some deficiency in root development, but they are clinically vital.^[13],[23]

As for maxillofacial imaging, both conventional CT and cone-beam CT (CBCT) are very interesting when making linear, angular, and volumetric measurements and estimating the importance of asymmetry, giving the maxillofacial surgeon and the orthodontist a valuable insight to the therapeutic approach.^{[7],[14],[28]}

The role for 3D imaging is paramount to detect and monitor osseocartilaginous changes. Several papers present multidetector computed tomography (MDCT) and CBCT reconstructions as perioperative planning tools. Although highly irradiating, MDCT provides an excellent resolution of bone and soft structures. CBCT, requiring less radiation exposure, has been suggested as lower cost method of disease assessment, but it has considerable limitations when it comes to examine lesions involving the head soft tissues.^{[28],[29],[30]} In our case, the CT scans were tremendously efficient in assessing the degree of atrophy in bone, muscles, and adipose layers.

Many differential diagnoses to PRS have been cited in literature such as other forms of juvenile localized scleroderma, Barraquer–Simons syndrome, and congenital hemiatrophy^[4] but the main one to be mentioned when facing PHA syndrome is linear morphea (scleroderma). The relationship between the two conditions is still controversial as these entities can have overlapping manifestations.^{[29],[31],[32],[33]}

In fact, many authors consider PRS and linear morphea existing in the same disease spectrum with a main difference regarding the involvement of the lower third of the face in PRS, while for linear morphea, it is seen especially on the upper two-thirds of the face along with a remarkable scar-like linear skin depression on the forehead and the frontal scalp.^[31]

There is currently no treatment to cure or stop the progression of PRS. When deeply affecting multiple layers of the head, PRS requires a multidisciplinary intervention of maxillofacial surgeons, dentists, phonoaudiologists, neurologists, and psychologists. The treatment planning usually consists in repositioning the adipose tissue that was lost due to atrophy. This can be achieved through numerous procedures, including autologous fat grafts, cartilage grafts, silicon and hyaluronic acid injections, and silicon prostheses.^{[34],[35],[36],[37],[38]}

Conclusion

PRS pathogenesis have not been elucidated yet, and despite the improvement of diagnosis, there is still some delay due to the gradual onset of symptoms and no specific scheme to follow for the treatment planning. A proper assessment and a multidisciplinary approach are essential for the management of this disfiguring disorder.

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.

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