Parry Romberg Syndrome-A Review of Treatment Options

Sargunar, Banu; Ebenezer, Vijay; Balakrishnan, R.; Priya, S.Veni

doi:10.13005/bpj/473

Cited by 5 publications

(15 citation statements)

References 10 publications

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“…A cerebral disturbance of fat metabolism and atrophic malformation of cervical sympathetic nervous system have been proposed as the primary cause. Other possible factors that are involved in the pathogenesis include trauma, infections ( Borrelia burgdorferi and viral), heredity, endocrine disturbances, and autoimmunity [21, 22].…”

Section: Discussionmentioning

confidence: 99%

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Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan – a rare entity from East Africa: a case report

Abdelnour¹,

Abdelnour²,

Kerollos³

et al. 2019

J Med Case Reports

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Background Parry–Romberg syndrome, also known as progressive hemifacial atrophy, is a rare degenerative disorder with numerous distinctive clinical presentations. It is usually slowly progressive, occurring more in females, and affects primarily one side of the face; it causes unilateral atrophy and loss of skin, subcutaneous tissue, muscles, and bones, and can even extend to oral structures. Other involvements that can occur are ocular and neurological; however, it is frequently associated with linear scleroderma, known as en coup de sabre . The etiology of the disorder is unknown, although some consider it a variant of morphea (localized scleroderma) and others proposed a malfunction of the sympathetic system as a cause. Imaging studies can support diagnosis and reveal the extent of the disease. Moreover, with the wide systemic involvement in such a condition, a multidisciplinary approach is crucial. Case presentation A 35-year-old Dinka woman presented with left hemifacial atrophy associated with left-sided body hemihypoesthesia and glaucoma with overlapping linear scleroderma “ en coup de sabre” for 5 years. Conclusions Parry–Romberg syndrome is a very rare entity causing progressive hemifacial atrophy that could also be associated with linear scleroderma. It has devastating outcomes due to its various systemic involvements; therefore, a multidisciplinary approach is required together with further studies to be performed in order to identify the key etiology and construct a clear guideline for management.

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Section: Discussionmentioning

confidence: 99%

“…Fascia grafts, muscle grafts, pedicle flaps, microvascular free flaps, and free fat grafts – injection of aspirated fat and alloplastic graft materials. Some surgical modalities classify the depression type caused by the disease and afterward select the surgical technique to be used [22–24].…”

Section: Discussionmentioning

confidence: 99%

Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan – a rare entity from East Africa: a case report

Abdelnour¹,

Abdelnour²,

Kerollos³

et al. 2019

J Med Case Reports

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“…17,18 Free tissue transfer is also associated with high cost, prolonged surgical times, and the need for staff with microsurgical expertise. 18,19 Treatment of LScs defects with alloplastic fillers such as silicone, inflammation triggered by multiple stimuli. 16,20 Fat grafting combines the advantages of free tissue transfer and alloplastic materials in that it provides an autologous substrate that is easy to harvest without the need for microsurgical expertise.…”

Section: Discussionmentioning

confidence: 99%

Treatment of linear scleroderma “En coup de Sabre” with single‐stage autologous fat grafting: A case report and review of the literature

Ayoub

Saba

2020

J of Cosmetic Dermatology

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Linear scleroderma "en coups de sabre" (LScs) is a variant of localized scleroderma, which is confined to the hemiface but may occasionally be bilateral. 1 First described by Addison in 1854, disease incidence peaks during the fifth decade of life. However, in children, it is often diagnosed between the ages of 2-14 years, and it affects females more than males (3:1). Clinical manifestations include scar-like lesions in the forehead and the scalp, and atrophy of the underlying skin and subcutaneous tissues including muscle, tendon, and bone. 1-5 In the craniofacial region, intracranial neural structures may be involved, with some patients exhibiting seizures. 5 The soft-tissue changes often progress slowly over a period of 2-5 years, after which the disease stabilizes. 6-8 The result is an acute facial asymmetry that can be distressing to the affected young adults. 9,10 Medical treatments in terms of immunosuppressants and anti-inflammatory drugs do not adequately address the soft-tissue atrophy. Thus, surgical correction has continued to be a mainstay of treatment. 2,9,11 It is important to distinguish LScs from Parry-Romberg syndrome, where the latter often initiates in the cheek and nose, is unilateral, and does not exhibit skin changes. 5 In this report, we present a rare case of LScs that was successfully managed by single-stage autologous fat grafting.

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“…ASCs were also used for orthopedic applications, treatment of inflammatory diseases (such as the fistula induced by Crohn’s disease), immunosuppression in GVHD (Graft-versus-host disease), and multiple sclerosis [ 155 , 156 , 157 , 158 , 159 , 160 ]. The transfer of autologous free fat has found several applications especially in plastic surgery [ 161 , 162 , 163 , 164 , 165 , 166 , 167 ]. This includes: reconstruction and re-modelling of breast (either in post-oncologic or in de-novo resections) [ 162 ]; facial and hand rejuvenation [ 161 ]; facial re-modelling, following the appearance of the typical HIV (human immunodeficiency virus) lipodystrophy [ 163 ]; correction of asymmetry in the syndrome of Poland and Parry–Romberg syndrome [ 164 ].…”

Section: Adipose Stem Cells and Regenerative Medicinementioning

confidence: 99%

“…The transfer of autologous free fat has found several applications especially in plastic surgery [ 161 , 162 , 163 , 164 , 165 , 166 , 167 ]. This includes: reconstruction and re-modelling of breast (either in post-oncologic or in de-novo resections) [ 162 ]; facial and hand rejuvenation [ 161 ]; facial re-modelling, following the appearance of the typical HIV (human immunodeficiency virus) lipodystrophy [ 163 ]; correction of asymmetry in the syndrome of Poland and Parry–Romberg syndrome [ 164 ]. It is believed that the presence of ASCs promotes the increase of angiogenesis with the consequent increase in capillary density.…”

Section: Adipose Stem Cells and Regenerative Medicinementioning

confidence: 99%

Adipose Stem Cell Translational Applications: From Bench-to-Bedside

Argentati

Morena

Bazzucchi

et al. 2018

IJMS

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During the last five years, there has been a significantly increasing interest in adult adipose stem cells (ASCs) as a suitable tool for translational medicine applications. The abundant and renewable source of ASCs and the relatively simple procedure for cell isolation are only some of the reasons for this success. Here, we document the advances in the biology and in the innovative biotechnological applications of ASCs. We discuss how the multipotential property boosts ASCs toward mesenchymal and non-mesenchymal differentiation cell lineages and how their character is maintained even if they are combined with gene delivery systems and/or biomaterials, both in vitro and in vivo.

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Parry Romberg Syndrome-A Review of Treatment Options

Cited by 5 publications

References 10 publications

Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan – a rare entity from East Africa: a case report

Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan – a rare entity from East Africa: a case report

Treatment of linear scleroderma “En coup de Sabre” with single‐stage autologous fat grafting: A case report and review of the literature

Adipose Stem Cell Translational Applications: From Bench-to-Bedside

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