• Korean Journal of Cleft Lip And Palate
• /
• v.15 no.1
• /
• pp.1-10
• /
• 2012

Parry-Romberg syndrome(PRS) is a degenerative disease characterized by progressive hemifacial atrophy. A 10-year-old girl who had been treated for linear scleroderma at the dermatologic department visited the orthodontic department. The frontal facial photograph showed mild facial asymmetry. On the left side, mild atrophy of soft tissue, enophthalmos, cheek depression, and dry skin with dark pigmentation were observed. The radiograph showed the hypoplasia of both the maxilla and mandible on the left side. This case report describes the treatment of a patient with PRS for 7 years. To minimize the effect of progressive atrophy on the facial growth, a hybrid appliance was used. The facial photos and radiographic records were periodically taken to analyze the progression of PRS. Although it is impossible to prevent the progression of facial asymmetry, it appears to be possible to limit the atrophic effect. After the stabilization of PRS, the orthodontic treatment by the fixed appliance was performed. Additionally, autologous fat graft was performed three times at 6 month intervals. After the treatment, the patient had a confident smile and facial asymmetry was improved.

• The korean journal of orthodontics
• /
• v.50 no.6
• /
• pp.407-417
• /
• 2020

Objective: To investigate the dental phenotypes and treatment modalities (Tx-Mod) in Korean patients with Parry-Romberg syndrome (PRS) using longitudinal data. Methods: The samples consisted of 10 PRS patients, who were treated and/or followed-up at Seoul National University Dental Hospital between 1998 and 2019. Using a novel PRS severity index based on the numbers of the atrophy-involved area and asymmetry-involved item, we classified them into mild (n = 3), moderate (n = 2), and severe (n = 5). Dental phenotypes, including congenitally missing tooth (Con-Missing-Tooth), microdontia, tooth with short root (Short-Root), tooth with dilacerated root, and delayed eruption/impacted tooth, were investigated along with Tx-Mod. Results: The side of occurrence of all dental phenotypes showed 100% concordance with the side of PRS involvement. The most two common dental phenotypes were Con-Missing-Tooth and Short-Root (n = 29 and n = 17 in six patients). The sums of the average number of Con-Missing-Tooth and Short-Root increased from mild PRS to moderate PRS and severe PRS cases (1.0, 6.0, and 6.2). In terms of Tx-Mod, growth observation due to mild atrophy, fixed orthodontic treatment, and grafting were used for mild PRS cases. Tx-Mod for moderate PRS cases involved growth observation for surgery due to an early age at the initial visit. For severe PRS cases, diverse Tx-Mod combinations including unilateral functional appliance, fixed orthodontic treatment, growth observation, grafting, and orthognathic surgery were used. Conclusions: The novel PRS severity index may be useful to provide primary data for individualized diagnosis and treatment planning for PRS patients.

• Annals of dermatology
• /
• v.30 no.6
• /
• pp.704-707
• /
• 2018

Parry Romberg Syndrome (PRS), also known as idiopathic progressive hemifacial atrophy, is a rare neurocutaneous disorder characterized by loss of skin and subcutaneous fat of face, muscles, and bones causing unilateral atrophy. Most patients require only soft tissue augmentation although syndrome has varying grades of severity. In the majority of reported cases, it has been treated with surgical flap or autologous fat transplantation. However, these treatments need complicated surgical skills which take a lot of time and cost. Herein we report the first case of PRS augmented by hyaluronic acid (HA) filler in a 42-year-old female patient to suggest that HA filler could be a safe, simple, and even rational economic alternative to surgical treatment.

• Clinical and Experimental Pediatrics
• /
• v.58 no.9
• /
• pp.354-357
• /
• 2015

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the left leg. At 4 years of age, she presented with localized scleroderma over the right parietal region of her scalp; her face gradually became asymmetric as her right cheek atrophied. Brain magnetic resonance imaging revealed hemiatrophy of the face and skull base, and T2-weighted images showed increased signal in the right hemipons and hemicerebellar peduncle. Magnetic resonance angiography findings were unremarkable. She was treated with oral prednisolone, and her recurrent gait ataxia diminished within 2 months of the follow-up period. To the best of our knowledge, this is only the second case of PRS presenting with an abnormal involvement of the ipsilateral hemipons.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.37
• /
• pp.29.1-29.7
• /
• 2015

Facial asymmetry is found in patients with or without cosmetic facial alterations. Some patients have facial asymmetry that manifests underlying skeletal problems, while others have only limited soft-tissue facial asymmetry. Orthognathic surgery brings about a dermatic change, as soft tissue covers underlying bones. Limited soft-tissue asymmetry, meanwhile, is difficult to correct. The treatment modalities for the creation or restoration of an esthetically pleasing appearance were autogenous fat grafts, cartilage graft, and silicon injections. A young female patient had right-side facial asymmetry. The clinical assessment involved visual inspection of the face and palpation to differentiate soft tissue and bone. Although the extra-oral examination found facial asymmetry with skin atrophy, the radiographic findings revealed no mandibular atrophy or deviation. She was diagnosed as localized scleroderma with muscle spasm. In conclusion, facial asymmetry patients with skeletal asymmetry can be esthetically satisfied by orthognathic surgery; however, facial atrophy patients with skin or subdermal tissue contraction need treatment by cosmetic dermatological surgery and orthodontic correction.