Abstract
Progressive facial hemiatrophy (PFH), also known as Parry-Romberg or Romberg syndrome, is a sporadic, but not so rare disease (OMIM # 141300) characterised by progressive and self-limited shrinking and deformation of one side of the face, which involves different tissues, scar-like cutaneous changes, subcutaneous connective and fatty tissue’ atrophy, circumscribed osteoporosis, bone deformation accompanied usually by contralateral Jacksonian epilepsy, trigeminal neuralgia (and/or peripheral nerve dysfunction), and changes in the eyes and hair. Evidence of mendelian basis is lacking. Larner and Bennison (1993) reported discordance in a pair of monozygotic twins and Anderson et al. (2005) reported two first cousins with PFH, whose fathers were dizygotic twins and whose mothers were sisters.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Anderson PJ, Molony D, Haan E, David DJ (2005) Familial Parry-Romberg disease. Int J Pediatr Otorhonolaryngol 69: 705–708.
Asher SW, Berg BO (1982) Progressive facial hemiatrophy: report of three cases, including one observed over 43 years, and computed tomography findings. Arch Neurol 39: 44–46.
de Crecchio G, Forte R, Strianese D, Rinaldi M, D’Aponte A (2008) Clinical evolution of neuroretinitis in Parry-Romberg syndrome. J Pediatr Ophthalmol Strabismus 45: 125–126.
Dupont S, Catala M, Hasdboun D, Semah F, Baulac M (1997) Progressive facial hemiatrophy and epilepsy: a common underlying dysgenetic mechanism. Neurology 48: 1013–1018.
Fry JA, Alvarellos A, Fink CW, Blaw ME, Roach ES (1992) Intracranial findings in progressive facial hemiatrophy. J Rheumatol 19: 956–958.
Guerrerosantos J, Guerrerosantos F, Orozco J (2007) Classification and treatment of facial tissue atrophy in Parry-Romberg disease. Aesthetic Plast Surg 31: 424–434.
Kanchwala SK, Bucky LP (2007) Invited discussion: correction of hemifacial atrophy with autologous fat transplantation. Ann Plast Surg 59: 654.
Khan AO (2007) Restrictive strabismus in Parry-Romberg syndrome. J Pediatr Ophthalmol Strabismus 44: 51–52.
Larner AJ, Bennison DP (1993) Some observations on the aetiology of progressive hemifacial atrophy (“Parry-Romberg syndrome”). J Neurol Neurosurg Psychiatr 56: 1035–1039.
Moon WJ, Kim HJ, Roh HG, Oh J, Han SH (2008) Diffusion tensor imaging and fiber tractography in Parry-Romberg syndrome. Am J Neuroradiol 29: 714–715.
Parry CH (1825) Collections from the unpublished medical writings of the late Caleb Hillier Parry. London: Underwoods, pp. 478–480.
Pensler JM, Murphy GF, Mulliken JB (1990) Clinical and ultrastructural studies of Romberg’ hemifacial atrophy. Plast Reconstr Surg 85: 669–674.
Rees TD (1976) Facial atrophy. Clin Plast Surg 3: 637–646.
Rogers BO (1963) Progressive facial hemiatrophy (Romberg’s disease): a review of 772 cases. In: Transactions of third international congress of plastic surgery (International Congress Series No. 66) Washington: Excerpta Medica Foundation, pp. 681–689.
Romberg MH (1846) Klinishe Ergebnisse. Berlin: A Förstner, pp. 75–81.
Terstegge K, Kunath B, Felberg S, Speciali JG, Henkes H, Hosten N (1994) MR of brain involvement in progressive facial hemiatrophy (Romberg disease): reconsideration of a syndrome. Am J Neuroradiol 15: 145–150.
Theodossiadis PG, Grigoropoulos VG, Emfietzoglou I, Papaspirou A, Nikolaidis P, Vergados I, Theodossiadis GP (2008) Parry-Romberg syndrome studied by optical coherence tomography. Ophthalmic Surg Lasers Imaging 39: 78–80.
Tollefson MM, Witman PM (2007) En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol 56: 257–263.
Wolf SM, Verity MA (1974) Neurological complications of progressive facial hemiatrophy. J Neurol Neurosurg Psychiatry 37: 997–1004.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2008 Springer-Verlag/Wien
About this chapter
Cite this chapter
Pascual-Castroviejo, I., Restivo, D.A., Milone, P. (2008). Progressive facial hemiatrophy (parry-romberg syndrome). In: Ruggieri, M., Pascual-Castroviejo, I., Di Rocco, C. (eds) Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-211-69500-5_58
Download citation
DOI: https://doi.org/10.1007/978-3-211-69500-5_58
Publisher Name: Springer, Vienna
Print ISBN: 978-3-211-21396-4
Online ISBN: 978-3-211-69500-5
eBook Packages: MedicineMedicine (R0)