Parry-Romberg syndrome: A case report and literature review.

Radiology case reports 2024 Vol.19(6) p. 2230-2238

Sharma PK, Sekar A, Amir AP, Prabhu ALR

관련 도메인

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)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
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

🔗 함께 등장하는 도메인

이 논문이 속한 카테고리와 같은 논문에서 자주 함께 다뤄지는 카테고리들

관련 논문