Parry-Romberg syndrome: A case report and literature review.
Abstract
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.
추출된 의학 개체 (NER)
| 유형 | 영어 표현 | 한국어 / 풀이 | UMLS CUI | 출처 | 등장 |
|---|---|---|---|---|---|
| 합병증 | asymmetry
|
비대칭 | dict | 2 | |
| 시술 | botulinum toxin
|
보툴리눔독소 주사 | dict | 1 | |
| 시술 | orthognathic surgery
|
안면윤곽술 | dict | 1 | |
| 해부 | fat
|
scispacy | 1 | ||
| 해부 | subcutaneous
|
피하조직 | dict | 1 | |
| 해부 | mandible
|
하악골 | dict | 1 | |
| 해부 | maxilla
|
상악골 | dict | 1 | |
| 해부 | eye
|
scispacy | 1 | ||
| 해부 | temporalis
|
scispacy | 1 | ||
| 해부 | medial
|
scispacy | 1 | ||
| 해부 | masseter
|
scispacy | 1 | ||
| 해부 | risorius
|
scispacy | 1 | ||
| 해부 | zygomaticus
|
scispacy | 1 | ||
| 해부 | orbicularis oris muscles
|
scispacy | 1 | ||
| 해부 | subcutaneous tissues
|
scispacy | 1 | ||
| 해부 | muscles
|
scispacy | 1 | ||
| 해부 | bones
|
scispacy | 1 | ||
| 합병증 | facial
|
scispacy | 1 | ||
| 약물 | PRS
→ Parry-Romberg syndrome
|
C0015458
Facial Hemiatrophy
|
scispacy | 1 | |
| 약물 | levatorangulioris
|
scispacy | 1 | ||
| 질환 | Parry-Romberg syndrome
|
C0015458
Facial Hemiatrophy
|
scispacy | 1 | |
| 질환 | PRS
→ Parry-Romberg syndrome
|
C0015458
Facial Hemiatrophy
|
scispacy | 1 | |
| 질환 | neurocutaneous
|
scispacy | 1 | ||
| 질환 | craniofacial disorder
|
scispacy | 1 | ||
| 질환 | hemifacial wasting
|
scispacy | 1 | ||
| 질환 | atrophy
|
C0333641
Atrophic
|
scispacy | 1 | |
| 질환 | wasting
|
C0006625
Cachexia
|
scispacy | 1 | |
| 질환 | hypoplasia of maxilla, mandible, and zygomatic arch
|
scispacy | 1 | ||
| 질환 | enophthalmos
|
C0014306
Enophthalmos
|
scispacy | 1 | |
| 질환 | hemifacial pain
|
scispacy | 1 | ||
| 질환 | ocular abnormalities
|
C5436858
Ocular abnormalities
|
scispacy | 1 | |
| 기타 | children
|
scispacy | 1 | ||
| 기타 | patients
|
scispacy | 1 | ||
| 기타 | female
|
scispacy | 1 | ||
| 기타 | zygomatic arch
|
scispacy | 1 | ||
| 기타 | lateral pterygoid
|
scispacy | 1 | ||
| 기타 | buccinator
|
scispacy | 1 | ||
| 기타 | levator labii superioris
|
scispacy | 1 | ||
| 기타 | vascular factors
|
scispacy | 1 |
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