Parry-Romberg Syndrome: Severity of the Deformity Does not Correlate with Quality of Life

Raposo-Amaral, Cássio Eduardo; Denadai, Rafael; Camargo, Daniella Nunes; Artioli, Thiago Olivetti; Gelmini, Yara; Buzzo, Celso Luiz; Raposo‐Amaral, Cesar Augusto

doi:10.1007/s00266-013-0142-0

Cited by 28 publications

(14 citation statements)

References 46 publications

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“…Furthermore, the severity of lipoatrophy is correlated with severity of QoL impairment 8. Similar findings have been reported in a study on patients with Parry-Romberg syndrome 11. Our patient experienced feelings of unattractiveness and less desirability, and we believe early intervention could benefit these patients as they are affected during adolescence.…”

Section: Discussionsupporting

confidence: 87%

Barraquer-Simons syndrome: a unique patient's perspective on diagnosis, disease progression and recontouring treatment

2016

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This case report describes a female patient diagnosed with Barraquer-Simons syndrome, a rare form of acquired partial lipodystrophy characterised by symmetrical loss of adipose tissue from face, neck, upper extremities and the trunk with onset in early childhood. Initial symptoms were seen at the age of 8 years. Our patient did not show signs of renal impairment and this may be associated with the syndrome. Treatment of lipoatrophy in these patients is limited to cosmetic restoration, and autologous fat grafting has shown sustained positive effects with no or very little loss of volume at follow-ups. Furthermore, the treatment has resulted in considerable improvements in her quality of life and daily functioning. She has not experienced any adverse effects. Accurate and early diagnosis is important, and clinicians should consider early intervention for these patients. Autologous fat grafting is recommended as a safe procedure.

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Section: Discussionsupporting

confidence: 87%

Barraquer-Simons syndrome: a unique patient's perspective on diagnosis, disease progression and recontouring treatment

2016

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“…10 Progressive hemifacial atrophy usually begins in the first or second decade of life, continues for many years, and ends spontaneously. The main challenges in treatments of PHA include augmentation of soft tissues and restoration of facial symmetry.…”

Section: Discussionmentioning

confidence: 99%

Autologous Fat Grafting With Combined Three-Dimensional and Mirror-Image Analyses for Progressive Hemifacial Atrophy

Yang

et al. 2016

Annals of Plastic Surgery

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“…Studies on the quality of life in children with and without craniofacial anomalies have considered children from five years of age eligible to respond to instruments that measured the quality of life [14][15][16] . Several authors [14][15][16][17] has already used the age threshold of five years for the application of the questionnaire WHOQOL-100 [14][15][16][17] . It has even been described that children from this age on can be self-critical [14][15][16] .…”

Section: Discussionmentioning

confidence: 99%

Traz a deformidade de orelha impacto negativo na qualidadede vida em indivíduos com síndrome de Treacher Collins?

Lodovichi

Oliveira

Denadai

et al. 2018

Ciênc. saúde coletiva

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Resumo A Síndrome de Treacher Collins (STC) é uma síndrome craniofacial de padrão autossômico dominante e expressão clínica variada, em que a orelha pode ou não estar ausente ou malformada. Indivíduos com STC sofrem estigmas podendo repercutir na interação com pares. Instrumentos de qualidade de vida obtidos por meio de questionários de autopercepção são ferramentas de identificação de estigmas e podem permitir o ajuste social desses indivíduos. Objetiva-se avaliar e mensurar a qualidade de vida em indivíduos com STC, aferindo os impactos da deformidade de orelhas na qualidade de vida. Doze voluntários com diagnóstico clínico e genético de STC responderam ao questionário de qualidade de vida da OMS, divididos em grupos com orelhas normais (n = 6) versus orelhas afetadas (n = 6) e seus resultados foram comparados. A escala de Siviero foi usada para estratificar a qualidade de vida em satisfatória, intermediária e insatisfatória. A pontuação geral do grupo com orelhas normais foi de 73,13; a do grupo com orelhas afetadas de 71,81, ambos classificados como níveis intermediários de qualidade de vida e sem diferença significativa entre si. A deformidade de orelha não representa um ônus na qualidade de vida dos indivíduos que já se apresentam com outras deformidades e com escores de qualidade de vida intermediários.

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Parry-Romberg Syndrome: Severity of the Deformity Does not Correlate with Quality of Life

Abstract: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Cited by 28 publications

References 46 publications

Barraquer-Simons syndrome: a unique patient's perspective on diagnosis, disease progression and recontouring treatment

Barraquer-Simons syndrome: a unique patient's perspective on diagnosis, disease progression and recontouring treatment

Autologous Fat Grafting With Combined Three-Dimensional and Mirror-Image Analyses for Progressive Hemifacial Atrophy

Traz a deformidade de orelha impacto negativo na qualidadede vida em indivíduos com síndrome de Treacher Collins?

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