Three-dimensional imaging of progressive facial hemiatrophy (Parry-Romberg syndrome) with unusual conjunctival findings

Balan, Preethi; Gogineni, Subhas Babu; Shetty, Shishir Ram; Dsouza, Deepa

doi:10.5624/isd.2011.41.4.183

Cited by 16 publications

(22 citation statements)

References 11 publications

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“…Parry-Romberg syndrome (PRS) is a rare disorder characterized by atrophy of cutaneous, subcutaneous fatty tissue, muscle tissue and rarely bone structures. It is more common in women, it usually involves one side of the face, the ipsilateral involvement of the body is rare and 20 % of cases are bilateral [ 9 ]. PRS usually begins in the first decade of life [ 1 – 3 , 12 ], although some cases with a late onset have been described [ 12 ], and is slowly progressive over two to twenty years and then stabilizes [ 1 , 2 , 13 – 16 ].…”

Section: Discussionmentioning

confidence: 99%

Parry Romberg syndrome with a wide range of ocular manifestations: a case report

et al. 2015

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BackgroundParry-Romberg syndrome (PRS) is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, muscles, and sometimes extending to the osteocartilaginous structures. Ocular involvement is relatively rare.Case presentationWe present a case of a 23-year-old female caucasian patient with Parry Romberg syndrome and extensive ocular involvement: enophthalmos, uveitis, iris atrophy. Ultrasound biomicroscopy (UBM) demonstrated hypotrophy of the ciliary body. The ciliary body atrophy has been previously reported just once and can be an explanation for the hypotony, frequently present in these patients.ConclusionsParry Romberg syndrome is a rare multidisciplinary disease. Our case presents a full spectrum of ocular manifestations. The pathogenesis of hypotonia is discussed.

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Section: Discussionmentioning

confidence: 99%

Parry Romberg syndrome with a wide range of ocular manifestations: a case report

et al. 2015

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“…In those with dental involvement, infection is frequent. Pain secondary to masticatory muscle spasm, temporomandibular joint pain, and locking of the jaw has also been reported [ 52 , 62 - 69 ].…”

Section: Clinical Descriptionmentioning

confidence: 99%

“…Skeletal hypoplasia of involved areas of the skull is common, although the clinical significance of this is unknown [ 52 , 70 ]. Atrophy of the extremities, both ipsilaterally and contralaterally, as well as the trunk, has been described in rare cases [ 12 , 18 , 71 - 73 ].…”

Section: Clinical Descriptionmentioning

confidence: 99%

Progressive hemifacial atrophy: a review

Tolkachjov

Patel

Tollefson

2015

Orphanet J Rare Dis

116

187

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BackgroundProgressive Hemifacial Atrophy (PHA) is an acquired, typically unilateral, facial distortion with unknown etiology. The true incidence of this disorder has not been reported, but it is often regarded as a subtype of localized scleroderma. Historically, a debate existed whether PHA is a form of linear scleroderma, called morphea en coup de sabre (ECDS), or whether these conditions are inherently different processes or appear on a spectrum (; Adv Exp Med Biol 455:101–4, 1999; J Eur Acad Dermatol Venereol 19:403–4, 2005). Currently, it is generally accepted that both diseases exist on a spectrum of localized scleroderma and often coexist.The pathogenesis of PHA has not been delineated, but trauma, autoimmunity, infection, and autonomic dysregulation have all been suggested. The majority of patients have initial manifestations in the first two decades of life; however, late presentations in 6th and 7th decades are also described [J Am Acad Dermatol 56:257–63, 2007; J Postgrad Med 51:135–6, 2005; Neurology 61:674–6, 2003]. The typical course of PHA is slow progression over 2-20 years and eventually reaching quiescence.Systemic associations of PHA are protean, but neurological manifestations of seizures and headaches are common [J Am Acad Dermatol 56:257–63, 2007; Neurology 48:1013–8, 1997; Semin Arthritis Rheum 43:335–47, 2013]. As in many rare diseases, standard guidelines for imaging, treatment, and follow-up are not defined.MethodsThis review is based on a literature search using PubMed including original articles, reviews, cases and clinical guidelines. The search terms were “idiopathic hemifacial atrophy”, “Parry-Romberg syndrome”, “Romberg’s syndrome”, “progressive hemifacial atrophy”, “progressive facial hemiatrophy”, “juvenile localized scleroderma”, “linear scleroderma”, and “morphea en coup de sabre”. The goal of this review is to summarize clinical findings, theories of pathogenesis, diagnosis, clinical course, and proposed treatments of progressive hemifacial atrophy using a detailed review of literature.Inclusion- and exclusion criteriaReview articles were used to identify primary papers of interest while retrospective cohort studies, case series, case reports, and treatment analyses in the English language literature or available translations of international literature were included.

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“…Parry Romberg syndrome is a degenerative condition characterized by a slow progressive unilateral atrophy of facial tissues including subcutaneous fat, muscles, bone and skin. [1][2][3] This syndrome seems to have higher incidence on Women. [4] It affects individuals who are morphologically normal at birth, Onset is usually in the first or second decade of life.…”

Section: Introductionmentioning

confidence: 99%

Parry Romberg Syndrome: A Rare Entity With Possible Association With Periodontitis

Elghoulbzouri¹,

Erraji²,

Ennibi³

2018

AIMDR

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Background: Parry Romberg syndrome (PRS) or progressive hemifacial atrophy is a rare entity uncommon, degenerative, poorly understood with unknown etiology, but possible factors that are involved in the pathogenesis in this disorder, like trophic malfunction of sympathetic system, disturbance of fat metabolism, trauma, viral infections, heredity, endocrine disturbances and auto-immunity. PRS is seen most generally unilateral, in facial tissues including muscles, bones and skin. This alteration is characterized by the "coup de sabre"; this is clear line of demarcation between the normal and abnormal structures, results in psychological disturbance and communication defects like speech defects, and also dental malformations. It needs multidisciplinary approach including physician, dentist and psychiatric. The objective of this work is to report a rare case of PRS, concerning a young female patient, with persistence of moderate periodontal destruction with severe gingival inflammation. Extensive investigations and a multidisciplinary approach are necessary for successful management of the case.

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Three-dimensional imaging of progressive facial hemiatrophy (Parry-Romberg syndrome) with unusual conjunctival findings

Cited by 16 publications

References 11 publications

Parry Romberg syndrome with a wide range of ocular manifestations: a case report

Parry Romberg syndrome with a wide range of ocular manifestations: a case report

Progressive hemifacial atrophy: a review

Parry Romberg Syndrome: A Rare Entity With Possible Association With Periodontitis

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