Hemiatrofia facial de romberg: relato de caso

Figueiredo Neto, Nicandro De; Martins, Johnny Wesley G.; Farage Filho, Miguel; Henriques, Fernando Guilhon

doi:10.1590/S0004-282X1995000500015

Resumos

Os autores apresentam o caso de uma paciente de 45 anos de idade com história de 15 meses de evolução, de hemiatrofia progressiva da face e língua à esquerda. A investigação laboratorial e de imagens foi normal, incluindo exames de sangue de rotina, líquido cerebro-espinhal, provas de função hepática, renal e reumáticas; além de raio X, tomografia computadorizada e ressonância magnética de crânio. A eletroneuromiografia mostrou alterações neurogênicas periféricas dos nervos trigêmio motor e hipoglosso, predominantemente à esquerda. Esses achados são compatíveis com o diagnóstico de hemiatrofia facial de Romberg, motivando a discussão de diversos aspectos desta rara doença de acordo com o relato e com a literatura.

hemiatrofia facial; síndrome de Parry-Romberg; doença de Romberg

The authors report the case of a 45-years-old female patient, with progressive hemiatrophy of the left face and tongue. The laboratorial and image studies were normal, including routine blood exams, cerebral-spinal fluid, hepatic function tests, renal and rheumatic; besides X ray, computerized tomography and magnetic resonance of skull. The electroneuromyography showed peripheral neurogenic alterations of motor trigeminal and hypoglossal nerves, markedly on the left side. Those findings are compatible with Romberg's facial hemiatrophy diagnosis, motivating discussion of several aspects of this rare disease according to the account and literature.

facial hemiatrophy; Parry-Romberg syndrome; Romberg's syndrome

Hemiatrofia facial de romberg. Relato de caso

Romberg's facial hemiatrophy: case report

Nicandro De Figueiredo Neto^I; Johnny Wesley G. Martins^I; Miguel Farage Filho^II; Fernando Guilhon Henriques^III

^IHospital de Base do Distrito Federal:Médico Residente da Unidade de Neurocirurgia

^IIHospital de Base do Distrito Federal:Chefe da Unidade de Neurocirurgia

^IIIHospital de Base do Distrito Federal:Chefe da Unidade de Neurologia

RESUMO

Os autores apresentam o caso de uma paciente de 45 anos de idade com história de 15 meses de evolução, de hemiatrofia progressiva da face e língua à esquerda. A investigação laboratorial e de imagens foi normal, incluindo exames de sangue de rotina, líquido cerebro-espinhal, provas de função hepática, renal e reumáticas; além de raio X, tomografia computadorizada e ressonância magnética de crânio. A eletroneuromiografia mostrou alterações neurogênicas periféricas dos nervos trigêmio motor e hipoglosso, predominantemente à esquerda. Esses achados são compatíveis com o diagnóstico de hemiatrofia facial de Romberg, motivando a discussão de diversos aspectos desta rara doença de acordo com o relato e com a literatura.

Palavras-chave:hemiatrofia facial, síndrome de Parry-Romberg, doença de Romberg.

SUMMARY

The authors report the case of a 45-years-old female patient, with progressive hemiatrophy of the left face and tongue. The laboratorial and image studies were normal, including routine blood exams, cerebral-spinal fluid, hepatic function tests, renal and rheumatic; besides X ray, computerized tomography and magnetic resonance of skull. The electroneuromyography showed peripheral neurogenic alterations of motor trigeminal and hypoglossal nerves, markedly on the left side. Those findings are compatible with Romberg's facial hemiatrophy diagnosis, motivating discussion of several aspects of this rare disease according to the account and literature.

Key words:facial hemiatrophy, Parry-Romberg syndrome, Romberg's syndrome.

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Full text available only in PDF format.

Agradecimentos - Ao Dr. Tito Lívio Mundim (Clínica Villas Boas), pela realização da ressonância magnética de crânio.

Aceite: 14-agosto-1995.

Dr. Nicandro de Figueiredo Neto - SQS 113, Bloco C, Apto 106 - 70373-030 Brasília DF - Brasil.

1. Abele DC, Bedingfield RB, Chandler FW, Given KS. Progressive facial hemiatropy (Parry-Romberg syndrome) and borreliosis. J Am Acad Dermatoll988, 19:820-825.
2. Archambault L., Fromm NK. Progressive facial hemiatrophy:report of three cases. Arch Neurol Psychiat. 1932, 27; 529-534.
3. Avelar J. Uma nova técnica na correção de deformidades frontais: "coup de sabre'". Anais do simpósio brasileiro do contorno facial. São Paulo, 1983, 341-344.
4. Claudy AL, Segault D, Rousset H, Moulin G. Facial hemiatrophy, homolateral cervical linear scleroderma and thyroid disease. Ann Dermatol Venereol 1992; 119:543-545.
5. Fayad S, Steffensen B. Root resorptions in a patient with hemifacial atrophy. J Endod 1994; 20:299-303.
6. Fry JA, Alvarellos A, Finx CW, Blaw ME, Roach ES. Intracranial findings in progressive facial hemiatrophy. J Rheumatol 1992, 19:956-958.
7. Hoang-Xuan T, Foster CS, Jakobiec FA, Tauber J, Sains de la Maza M, Krebs W. Romberg's progressive hemifacial atrophy: an association with scleral melting. Cornea. 1991, 10:361-366.
8. Inigo F, Rojo P, Ysunza A. Aesthetic treatment of Romberg's disease: experience with 35 cases. Br J Plast Surg. 1993, 46:194-200.
9. La Hey E, Baarsma GS. Fuchs' heterochromic cyclitis and retinal vascular abnormalities in progressive hemifacial atrophy. Eye 1993, 7 (Pt 3):426-428.
10. Lamer AJ, Bennison DP. Some observations on the aetiology of progressive hemifacial atrophy. J Neurol Neurosurg Psychiatry 1993, 56:1035-1036.
11. Lakhani PK, David T J. Progressive hemifacial atrophy with scleroderma and ipsilateral limb wasting (Parry-Romberg syndrome). J R Soc Med 1984, 77:138-139.
12. Lehman JAT. The Parry Romberg syndrome of progressive facial hemiatrophy and linear scleroderma "en coup de sabre": mistaken diagnosis or overlapping conditions ? J Rheumatol 1992, 19:6.
13. Menges-Wenzel EM, Kurlemann G, Pawlowitzki JH. Progressive facial hemiatrophy (Parry-Romberg syndrome). Monatsschr Kinderheilkd 1993, 141:922-924.
14. Morais-Besteiro J.; Aki FE, Mendes JA, Ferreira MC. Microsurgical transplants for facial depressions. Rev Hosp Clin Fac Med São Paulo 1992, 47:271-275.
15. Mordick TG 2d, Larossa D, Whitaker L. Soft-tissue reconstruction of the face:a comparison of dermal-fat grafting and vascularized tissue transfer. Ann Plast Surg 1992; 29:390-396.
16. Moss ML, Crikelair GF. Progressive facial hemiatrophy following cervical sympathectomy in the rat. Arch Oral Biol 1959-60, 1:254-258.
17. Muhling J, Zoller J, Reuther J. The use of "de-epithelized" scapular flap for soft tissue augmentation: a case report. J Craniomaxillofac Surg. 1994, 22:200-204.
18. Nakazawa A, Matsuo I, Ohkido M. Progressive facial hemiatrophy with localized scleroderma. Tokai J Exp Clin Med 1992, 17:85-87.
19. Naumbaev AN, Sharipov ASH, Iakhontov B. V. Zh Nevropatol Psikhiatr Im S S Korsakova 1991, 91:88-89.
20. Nussgens Z, Roggenkamper P. Congenital facial hemiatrophy: report of two pediatric cases. Klin Monatsbl Augenheilkd 1992, 201:119-121.
21. Parry CH. Collections from unpublished medical writings of the late Calleb Hillier Parry. London: Underwoods, 1825:478.
22. Pensler JM, Murphy GF, Mulliken JB. Clinical and ultrastructural studies of Romberg's hemifacial atrophy. Plast Reconstr Surg 1990, 85:669-674.
23. Rezende LAL, Dal Pai V, Alves A. Experimental study of progressive facial hemiatrophy: effects of cervical sympathectomy in animals. Rev Neurol (Paris). 1991, 147:609-611.
24. Roddi R, Riggio E, Gilbert PM, Hovius SE, Vaandrager JM, van der Meulen JC. Clinical evaluation of techniques used in the surgical treatment of progressive hemifacial atrophy. J Craniomaxillofac Surg. 1994, 22:23-32.
25. Roddi R, Riggio E, Gilbert PM, Vaandrager JM, van der Meulen JC. Progressive hemifacial atrophy in a patient with lupus erythematosus. Plast Reconstr Surg. 1994, 93:1067-1072.
26. Romberg HM. Trophoneurosen kliniche Ergebnisse. Berlin: A Forstner, 1846:75-81.
27. Sakuraoka K, Tajima S, Nishi TK. Progressive facial hemiatrophy. Dermatology 1992, 185:196-201.
28. Sener RN, Jinkins JR. MR. of craniocerebral hemiatrophy. Clin Imaging. 1992, 16:93-97.
29. Terstegge K, Henkes H, Kunath B, Felber S, Uhrmeister P, Kern A. Cerebral manifestations of progressive facial hemiatrophy, nuclear spin tomographic findings and literature review. Radiologe. 1993; 33:585-595.
30. Terstegge K, Kunath B, Felber S, Speciali JG, Beckert M, Henkes H, Hosten N. Ressonância magnética do envolvimento cerebral na hemiatrofia facial progressiva (doença de Romberg): reconsideração de uma síndrome. Arq Neuropsiquiatrl995; 53:98-113.
31. Terstegge K, Kunath B, Felber S, Speciali JG, Henkes H, Hosten N. M. R. of brain involvement in progressive facial hemiatrophy: reconsideration of a syndrome. Am J Neuroradiol 1994, 15:145-150.
32. Tsuda N, Yamamotu K, Fukusako T, Morimatsu M. A case of unilateral lingual atrophy suplied by trigeminal nerve in relation to progressive facial hemiatrophy. Rinsho Shinkeigaku 1991, 31:107-109.
33. Wartenberg R. Progressive facial hemiatrophy. Arch Neurol Psychiatr 1945, 54:75-96.
34. Wolf SM, Verity MA. Neurological complications of progressive facial hemiatrophy. J Neurol Neurosurg Psychiatry 1947, 37:997-1004.

Datas de Publicação

Publicação nesta coleção
08 Dez 2010
Data do Fascículo
Dez 1995

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Abele DC, Bedingfield RB, Chandler FW, Given KS. Progressive facial hemiatropy (Parry-Romberg syndrome) and borreliosis. J Am Acad Dermatoll988, 19:820-825.

[2] 2. Archambault L., Fromm NK. Progressive facial hemiatrophy:report of three cases. Arch Neurol Psychiat. 1932, 27; 529-534.

[3] 3. Avelar J. Uma nova técnica na correção de deformidades frontais: "coup de sabre'". Anais do simpósio brasileiro do contorno facial. São Paulo, 1983, 341-344.

[4] 4. Claudy AL, Segault D, Rousset H, Moulin G. Facial hemiatrophy, homolateral cervical linear scleroderma and thyroid disease. Ann Dermatol Venereol 1992; 119:543-545.

[5] 5. Fayad S, Steffensen B. Root resorptions in a patient with hemifacial atrophy. J Endod 1994; 20:299-303.

[6] 6. Fry JA, Alvarellos A, Finx CW, Blaw ME, Roach ES. Intracranial findings in progressive facial hemiatrophy. J Rheumatol 1992, 19:956-958.

[7] 7. Hoang-Xuan T, Foster CS, Jakobiec FA, Tauber J, Sains de la Maza M, Krebs W. Romberg's progressive hemifacial atrophy: an association with scleral melting. Cornea. 1991, 10:361-366.

[8] 8. Inigo F, Rojo P, Ysunza A. Aesthetic treatment of Romberg's disease: experience with 35 cases. Br J Plast Surg. 1993, 46:194-200.

[9] 9. La Hey E, Baarsma GS. Fuchs' heterochromic cyclitis and retinal vascular abnormalities in progressive hemifacial atrophy. Eye 1993, 7 (Pt 3):426-428.

[10] 10. Lamer AJ, Bennison DP. Some observations on the aetiology of progressive hemifacial atrophy. J Neurol Neurosurg Psychiatry 1993, 56:1035-1036.

[11] 11. Lakhani PK, David T J. Progressive hemifacial atrophy with scleroderma and ipsilateral limb wasting (Parry-Romberg syndrome). J R Soc Med 1984, 77:138-139.

[12] 12. Lehman JAT. The Parry Romberg syndrome of progressive facial hemiatrophy and linear scleroderma "en coup de sabre": mistaken diagnosis or overlapping conditions ? J Rheumatol 1992, 19:6.

[13] 13. Menges-Wenzel EM, Kurlemann G, Pawlowitzki JH. Progressive facial hemiatrophy (Parry-Romberg syndrome). Monatsschr Kinderheilkd 1993, 141:922-924.

[14] 14. Morais-Besteiro J.; Aki FE, Mendes JA, Ferreira MC. Microsurgical transplants for facial depressions. Rev Hosp Clin Fac Med São Paulo 1992, 47:271-275.

[15] 15. Mordick TG 2d, Larossa D, Whitaker L. Soft-tissue reconstruction of the face:a comparison of dermal-fat grafting and vascularized tissue transfer. Ann Plast Surg 1992; 29:390-396.

[16] 16. Moss ML, Crikelair GF. Progressive facial hemiatrophy following cervical sympathectomy in the rat. Arch Oral Biol 1959-60, 1:254-258.

[17] 17. Muhling J, Zoller J, Reuther J. The use of "de-epithelized" scapular flap for soft tissue augmentation: a case report. J Craniomaxillofac Surg. 1994, 22:200-204.

[18] 18. Nakazawa A, Matsuo I, Ohkido M. Progressive facial hemiatrophy with localized scleroderma. Tokai J Exp Clin Med 1992, 17:85-87.

[19] 19. Naumbaev AN, Sharipov ASH, Iakhontov B. V. Zh Nevropatol Psikhiatr Im S S Korsakova 1991, 91:88-89.

[20] 20. Nussgens Z, Roggenkamper P. Congenital facial hemiatrophy: report of two pediatric cases. Klin Monatsbl Augenheilkd 1992, 201:119-121.

[21] 21. Parry CH. Collections from unpublished medical writings of the late Calleb Hillier Parry. London: Underwoods, 1825:478.

[22] 22. Pensler JM, Murphy GF, Mulliken JB. Clinical and ultrastructural studies of Romberg's hemifacial atrophy. Plast Reconstr Surg 1990, 85:669-674.

[23] 23. Rezende LAL, Dal Pai V, Alves A. Experimental study of progressive facial hemiatrophy: effects of cervical sympathectomy in animals. Rev Neurol (Paris). 1991, 147:609-611.

[24] 24. Roddi R, Riggio E, Gilbert PM, Hovius SE, Vaandrager JM, van der Meulen JC. Clinical evaluation of techniques used in the surgical treatment of progressive hemifacial atrophy. J Craniomaxillofac Surg. 1994, 22:23-32.

[25] 25. Roddi R, Riggio E, Gilbert PM, Vaandrager JM, van der Meulen JC. Progressive hemifacial atrophy in a patient with lupus erythematosus. Plast Reconstr Surg. 1994, 93:1067-1072.

[26] 26. Romberg HM. Trophoneurosen kliniche Ergebnisse. Berlin: A Forstner, 1846:75-81.

[27] 27. Sakuraoka K, Tajima S, Nishi TK. Progressive facial hemiatrophy. Dermatology 1992, 185:196-201.

[28] 28. Sener RN, Jinkins JR. MR. of craniocerebral hemiatrophy. Clin Imaging. 1992, 16:93-97.

[29] 29. Terstegge K, Henkes H, Kunath B, Felber S, Uhrmeister P, Kern A. Cerebral manifestations of progressive facial hemiatrophy, nuclear spin tomographic findings and literature review. Radiologe. 1993; 33:585-595.

[30] 30. Terstegge K, Kunath B, Felber S, Speciali JG, Beckert M, Henkes H, Hosten N. Ressonância magnética do envolvimento cerebral na hemiatrofia facial progressiva (doença de Romberg): reconsideração de uma síndrome. Arq Neuropsiquiatrl995; 53:98-113.

[31] 31. Terstegge K, Kunath B, Felber S, Speciali JG, Henkes H, Hosten N. M. R. of brain involvement in progressive facial hemiatrophy: reconsideration of a syndrome. Am J Neuroradiol 1994, 15:145-150.

[32] 32. Tsuda N, Yamamotu K, Fukusako T, Morimatsu M. A case of unilateral lingual atrophy suplied by trigeminal nerve in relation to progressive facial hemiatrophy. Rinsho Shinkeigaku 1991, 31:107-109.

[33] 33. Wartenberg R. Progressive facial hemiatrophy. Arch Neurol Psychiatr 1945, 54:75-96.

[34] 34. Wolf SM, Verity MA. Neurological complications of progressive facial hemiatrophy. J Neurol Neurosurg Psychiatry 1947, 37:997-1004.