Progressive facial hemiatrophy associated with an astrocytoma in the cerebellopontine region

Abstract

Progressive hemifacial atrophy was originally described by Parry in 1825 and Romberg in 1846. This condition was later referred to as Parry-Romberg or just Romberg syndrome. It is characterized by acquired, idiopathic, unilateral atrophy of the face, variably involving skin, fat, muscle, and, less frequently, bone. Usually, the disease is manifested during the first or second decade. 1 Paradise JE Raney RB Whitaker LA Progressive facial hemiatrophy: Report of a case associated with Ewing's sarcoma. Am J Dis Child. 1980; 134: 1065 Crossref PubMed Scopus (3) Google Scholar , 2 Cohen Jr, MM Perspectives on craniofacial asymmetry. III. Common and/or well-known causes of asymmetry. Int J Oral Maxillofac Surg. 1995; 24: 127 Abstract Full Text PDF PubMed Scopus (33) Google Scholar Prompt recognition of progressive facial hemiatrophy is important, not only because effective cosmetic management is available, but also because failure to appreciate its various complications (eg, unusual manifestations of epilepsy) may result in delayed or inappropriate therapy. 1 Paradise JE Raney RB Whitaker LA Progressive facial hemiatrophy: Report of a case associated with Ewing's sarcoma. Am J Dis Child. 1980; 134: 1065 Crossref PubMed Scopus (3) Google Scholar , 3 Wolf SM Verity MA Neurological complications of progressive facial hemiatrophy. J Neurol Neurosurg Psychiatry. 1974; 37: 997 Crossref Scopus (52) Google Scholar , 4 Rees TD Facial atrophy. Clin Plast Surg. 1976; 3: 637 PubMed Google Scholar , 5 Asher SW Berg BO Progressive hemifacial atrophy. Arch Neurol. 1982; 39: 44 Crossref PubMed Scopus (56) Google Scholar , 6 Taylor HM Robinson R Cox T Progressive facial hemiatrophy: MRI appearances. Dev Med Child Neurol. 1997; 39: 484 Crossref PubMed Scopus (34) Google Scholar Although the etiology and pathogenesis are unknown, neurodystrophic and sclerodermal origin is strongly suspected. 7 Roddi R Riggio E Gilbert PM et al. Progressive hemifacial atrophy in a patient with lupus erythematosus. Plast Reconstr Surg. 1994; 93: 1067 Crossref PubMed Scopus (26) Google Scholar Trauma, infections, sympathetic dysfunction, and intracerebral vascular malformations are also proposed causes. 6 Taylor HM Robinson R Cox T Progressive facial hemiatrophy: MRI appearances. Dev Med Child Neurol. 1997; 39: 484 Crossref PubMed Scopus (34) Google Scholar Auger and Piepgras 8 Auger RG Piepgras DG Hemifacial spasm associated with epidermoid tumors of the cerebellopontine angle. Neurology. 1989; 39: 577 Crossref PubMed Google Scholar reported that neoplasms (eg, epidermoid tumors) located at the cerebellopontine angle could be an underlying cause of progressive hemifacial atrophy. We present a case with acquired facial asymmetry associated with an astrocytoma in the cerebellopontine region.