Parry-Romberg syndrome (PRS) is a rare neurocutaneous and craniofacial disorder characterized by progressive hemifacial wasting and atrophy that predominantly affects children and young adults, with an estimated prevalence of 1 in 700,000 individuals. Despite its rarity, PRS poses significant challenges for patients, their families, and healthcare providers due to its unpredictable course and potential functional and aesthetic impairments. The main aim is to provide a comprehensive overview of PRS, encompassing its clinical features, pathogenesis, and management techniques. We present a case of PRS in a 9-year-old female with pronounced facial asymmetry, with marked wasting and atrophy involving the entire right side of the face. CT scan revealed right sided hypoplasia of maxilla, mandible, and zygomatic arch with enophthalmos of right eye. MRI showed right temporalis, medial and lateral pterygoid, masseter, risorius, buccinator, zygomaticus major and minor, levator labii superioris, levatorangulioris and orbicularis oris muscles atrophy. The clinical presentation of PRS typically involves progressive facial atrophy, predominantly affecting the subcutaneous tissues, muscles and bones. Patients may experience various symptoms as the condition advances, including facial asymmetry, hemifacial pain, dental and ocular abnormalities and neurological manifestations. The exact etiology of PRS remains unknown, although autoimmune, genetic and vascular factors are likely contributors. Treatment of PRS needs a multidisciplinary approach involving dermatologists, plastic surgeons, neurologists, ophthalmologists, and dental specialists. Treatment options aim to alleviate symptoms, improve function and address cosmetic concerns. Surgical interventions such as autologous fat grafting, facial reconstructive procedures and orthognathic surgery have restored facial symmetry and function. Additionally, nonsurgical modalities, including botulinum toxin injections, prosthetic devices and dental interventions, may offer symptomatic relief and enhance overall quality of life.
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http://dx.doi.org/10.1016/j.radcr.2024.02.053 | DOI Listing |
BMJ Mil Health
January 2025
Emergency Medicine, Madigan Army Medical Center, Tacoma, Washington, USA.
Cleft Palate Craniofac J
November 2024
Department of Maxillofacial Surgery, Plastic Surgery Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Objective: To characterize mandibular morphology in patients with Parry-Romberg syndrome (PRS).
Design: Retrospective study.
Setting: A craniofacial center.
Dent Med Probl
December 2024
Department of Oral Pathology, Wroclaw Medical University, Poland.
Symmetry is present in various aspects of everyday life. A symmetrical face is considered attractive, whereas a lack of facial symmetry is regarded as a source of functional and aesthetic problems. Most of the people exhibit slight asymmetries, but some of them reveal severe asymmetries.
View Article and Find Full Text PDFAesthetic Plast Surg
October 2024
Department of Plastic and Cosmetic Surgery, Nanfang Hospital, Southern Medical University, 1838 Guangzhou North Road, Guangzhou, 510515, Guangdong, People's Republic of China.
Background: Alar atrophy associated with Parry-Romberg syndrome (PRS) can lead to significant nasal contour deformities. The primary objective of this study was to address nasal alar deformities in PRS patients through the application of a nasolabial transfer flap combined with an auricular cartilage graft after sequential fat grafting.
Methods: All PRS patients with alar deformities who underwent our reconstructive surgery were included in this case series.
Plast Reconstr Surg
October 2024
Department of Plastic and Reconstructive Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
Background: In 2021, a meta-analysis showed fat graft retention varied from 26 to 83%. In a retrospective study including patients with Parry-Romberg syndrome, the younger age group had higher satisfaction scores (3.8 vs.
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