Abstract
Background
The aims of this study were to (1) describe the main clinical findings of Parry-Romberg syndrome (PRS), (2) evaluate surgical strategies and outcomes, and (3) investigate the quality of life of patients according to their disease severity.
Methods
This retrospective observational study involved 14 patients treated between 2005 and 2011. The surgical treatment strategies were based on the proposed system for grading severity, and postoperative outcomes were assessed. The patients answered two questionnaires that covered the clinical manifestations of the syndrome and their quality of life in the postoperative period. A comparative analysis between the severity of the deformity and the quality of life was performed using the Kruskal–Wallis and Mann–Whitney tests.
Results
The most prevalent clinical manifestation of PRS was progressive hemifacial atrophy (85.71 %). The surgical strategy was individualized based on the severity of disease of each patient. Surgical strategies included free-fat grafts, dermal fat grafts, and bone grafts associated with a temporoparietal fascia flap. Regardless of approach, all patients had an overall improvement in their facial appearance and were free of complications during the follow-up period. Our data showed no association between the severity of the deformity and the quality of life of these patients (all p > 0.05).
Conclusions
In this series, there was a predominance of clinical features of PRS that had been previously reported. Satisfactory outcomes were obtained using different surgical strategies that varied according to the severity of the deformity. The severity of the deformity did not impose a reduced quality of life on PRS patients.
Level of Evidence IV
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References
Baek R, Heo C, Kim BK (2011) Use of various free flaps in progressive hemifacial atrophy. J Craniofac Surg 22:2268–2271
Bannink N, Maliepaard M, Raat H, Joosten KF, Mathijssen IM (2010) Health-related quality of life in children and adolescents with syndromic craniosynostosis. J Plast Reconstr Aesthet Surg 63:1972–1981
Brent B, Byrd HS (1983) Secondary ear reconstruction with cartilage grafts covered by axial, random, and free flaps of temporoparietal fascia. Plast Reconstr Surg 72:141–152
Cano SJ, Browne JP, Lamping DL, Roberts AH, McGrouther DA, Black NA (2006) The patient outcomes of surgery-head/neck (POS-head/neck): a new patient-based outcome measure. J Plast Reconstr Aesthet Surg 59:65–73
Cano SJ, Klassen A, Pusic AL (2009) The science behind quality-of-life measurement: a primer for plastic surgeons. Plast Reconstr Surg 123:98e–106e
Cheng J, Shen G, Tang Y, Zhang Z, Qiu W, Lu X (2010) Facial reconstruction with vascularised serratus anterior muscle flap in patients with Parry-Romberg syndrome. Br J Oral Maxillofac Surg 48:261–266
Clauser LC, Tieghi R, Consorti G (2010) Parry-Romberg syndrome: volumetric regeneration by structural fat grafting technique. J Craniomaxillofac Surg 38:605–609
De Figueiredo Neto N, Martins JW, Farage Filho M, Henriques FG (1995) Romberg’s facial hemiatrophy: a case report. Arq Neuropsiquiatr 53:795–798
De Sousa A (2008) Psychological issues in oral and maxillofacial reconstructive surgery. Br J Oral Maxillofac Surg 46:661–664
de Vasconcelos Carvalho M, do Nascimento GJ, Andrade E, Andrade M, Sobral AP (2010) Association of aesthetic and orthodontic treatment in Parry-Romberg syndrome. J Craniofac Surg 21:436–439
Duymaz A, Karabekmez FE, Keskin M, Tosun Z (2009) Parry-Romberg syndrome: facial atrophy and its relationship with other regions of the body. Ann Plast Surg 63:457–461
Duymaz A, Karabekmez FE, Tosun Z, Keskin M, Karamese M, Savaci N (2008) Reconstruction with galeal frontalis flap of depressed forehead region in progressive hemifacial atrophy. J Craniofac Surg 19:1104–1106
Eckstein DA, Wu RL, Akinbiyi T, Silver L, Taub PJ (2011) Measuring quality of life in cleft lip and palate patients: currently available patient-reported outcomes measures. Plast Reconstr Surg 128:518e–526e
Fleck MP, Louzada S, Xavier M, Chachamovich E, Vieira G, Santos L, Pinzon V (1999) Application of the Portuguese version of the instrument for the assessment of quality of life of the World Health Organization (WHOQOL-100). Rev Saude Publica 33:198–205
Grimaldi M, Gentile P, Labardi L, Silvi E, Trimarco A, Cervelli V (2008) Lipostructure technique in Romberg syndrome. J Craniofac Surg 19:1089–1091
Guerrerosantos J, Guerrerosantos F, Orozco J (2007) Classification and treatment of facial tissue atrophy in Parry-Romberg disease. Aesthetic Plast Surg 31:424–434
Hu J, Yin L, Tang X, Gui L, Zhang Z (2011) Combined skeletal and soft tissue reconstruction for severe Parry-Romberg syndrome. J Craniofac Surg 22:937–941
Hunstad JP, Shifrin DA, Kortesis BG (2011) Successful treatment of Parry-Romberg syndrome with autologous fat grafting: 14-year follow-up and review. Ann Plast Surg 67:423–425
Inigo F, Rojo P, Ysunza A (1993) Aesthetic treatment of Romberg’s disease: experience with 35 cases. Br J Plast Surg 46:194–200
Karaaltin MV, Akpinar AC, Baghaki S, Akpinar F (2012) Treatment of en coup de sabre deformity with adipose-derived regenerative cell-enriched fat graft. J Craniofac Surg 23:e103–e105
Karande S, Kulkarni S (2009) Quality of life of parents of children with newly diagnosed specific learning disability. J Postgrad Med 55:97–103
Klassen AF, Stotland MA, Skarsgard ED, Pusic AL (2008) Clinical research in pediatric plastic surgery and systematic review of quality-of-life questionnaires. Clin Plast Surg 35:251–267
Menascu S, Padeh S, Hoffman C, Ben-Zeev B (2009) Parry-Romberg syndrome presenting as status migrainosus. Pediatr Neurol 40:321–323
Moore MH, Wong KS, Proudman TW, David DJ (1993) Progressive hemifacial atrophy (Romberg’s disease): skeletal involvement and treatment. Br J Plast Surg 46:39–44
Moreno AB, Faerstein E, Werneck GL, Lopes CS, Chor D (2006) Psychometric properties of the World Health Organization Abbreviated Instrument for quality of life assessment in the Pro-Saude study. Cad Saude Publica 22:2585–2597
Obermoser G, Pfausler BE, Linder DM, Sepp NT (2003) Scleroderma en coup de sabre with central nervous system and ophthalmologic involvement: treatment of ocular symptoms with interferon gamma. J Am Acad Dermatol 49:543–546
Orozco-Covarrubias L, Guzmán-Meza A, Ridaura-Sanz C, Carrasco Daza D, Sosa-de-Martinez C, Ruiz-Maldonado R (2002) Scleroderma ‘en coup de sabre’ and progressive facial hemiatrophy. Is it possible to differentiate them? J Eur Acad Dermatol Venereol 16:361–366
Palmero ML, Uziel Y, Laxer RM, Forrest CR, Pope E (2010) En coup de sabre scleroderma and Parry-Romberg syndrome in adolescents: surgical options and patient-related outcomes. J Rheumatol 37:2174–2179
Pensler JM, Murphy GF, Mulliken JB (1990) Clinical and ultrastructural studies of Romberg’s hemifacial atrophy. Plast Reconstr Surg 85:669–674
Pu LL, Coleman SR, Cui X, Ferguson RE Jr, Vasconez HC (2008) Autologous fat grafts harvested and refined by the Coleman technique: a comparative study. Plast Reconstr Surg 122:932–937
Ramakrishnan KM, Andal A, Sankar J, Jayaraman V (2007) Facially disfigured children–dedicated approach and rehabilitation. Dev Neurorehabil 10:99–104
Raposo-do-Amaral CE, Raposo-do-Amaral CA, Guidi M, Buzzo C (2010) The role of temporoparietal fascia flap in craniofacial skeleton and secondary ear reconstruction. Rev Bras Cir Craniomaxilofac 13:1–6
Raposo do Amaral CE, Cetrulo CL Jr, Pereira CL, Guidi Mde C, Raposo do Amaral CM (2006) Augmentation gluteoplasty with dermal-fat autografting from the lower abdomen. Aesthet Surg J 26:290–296
Saccomanno F, Bernardi C, Vittorini P (1997) The expanded polytetrafluoroethylene (ePTFE) in the surgical treatment of Parry-Romberg syndrome: case report. Aesthetic Plast Surg 21:342–345
Schmitz S, Weis C, Morley S, DeMey A, Dabernig J (2008) Treatment of facial lipodystrophy syndromes. Lipofilling versus free flap surgery. Eur J Plast Surg 31:305–310
Sommer A, Gambichler T, Bacharach-Buhles M, von Rothenburg T, Altmeyer P, Kreuter A (2006) Clinical and serological characteristics of progressive facial hemiatrophy: a case series of 12 patients. J Am Acad Dermatol 54:227–233
Stone J (2003) Parry-Romberg syndrome: a global survey of 205 patients using the Internet. Neurology 61:674–676
Teng L, Jin X, Wu G, Zhang Z, Ji Y, Xu J, Lu J, Zhang B, Zhou G (2010) Correction of hemifacial atrophy using free anterolateral thigh adipofascial flap. J Plast Reconstr Aesthet Surg 63:1110–1116
Tessier P (1982) Autogenous bone grafts taken from the calvarium for facial and cranial applications. Clin Plast Surg 9:531–538
Vaienti L, Soresina M, Menozzi A (2005) Parascapular free flap and fat grafts: combined surgical methods in morphological restoration of hemifacial progressive atrophy. Plast Reconstr Surg 116:699–711
Vegter F, Hage JJ (2001) Lack of correlation between objective and subjective evaluation of residual stigmata in cleft patients. Ann Plast Surg 46:625–629
Versnel SL, Duivenvoorden HJ, Passchier J, Mathijssen IM (2010) Satisfaction with facial appearance and its determinants in adults with severe congenital facial disfigurement: a case-referent study. J Plast Reconstr Aesthet Surg 63:1642–1649
Versnel SL, Plomp RG, Passchier J, Duivenvoorden HJ, Mathijssen IM (2012) Long-term psychological functioning of adults with severe congenital facial disfigurement. Plast Reconstr Surg 129:110–117
Warschausky S, Kay JB, Buchman S, Halberg A, Berger M (2002) Health-related quality of life in children with craniofacial anomalies. Plast Reconstr Surg 110:409–414
Wojcicki P, Zachara M (2011) Surgical treatment of patients with Parry-Romberg syndrome. Ann Plast Surg 66:267–272
Wong VW, Rustad KC, Longaker MT, Gurtner GC (2010) Tissue engineering in plastic surgery: a review. Plast Reconstr Surg 126:858–868
Xie Y, Li Q, Zheng D, Lei H, Pu LL (2007) Correction of hemifacial atrophy with autologous fat transplantation. Ann Plast Surg 59:645–653
Ye XD, Li CY, Wang C, Yu YS (2010) Superficial temporal fascial flap plus lipofilling for facial contour reconstruction in bilateral progressive facial hemiatrophy. Aesthetic Plast Surg 34:534–537
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Raposo-Amaral, C.E., Denadai, R., Camargo, D.N. et al. Parry-Romberg Syndrome: Severity of the Deformity Does not Correlate with Quality of Life. Aesth Plast Surg 37, 792–801 (2013). https://doi.org/10.1007/s00266-013-0142-0
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DOI: https://doi.org/10.1007/s00266-013-0142-0