Epilepsy in Parry-Romberg syndrome and linear scleroderma en coup de sabre: Case series and systematic review including 140 patients.

Parry-Romberg syndrome (PRS) and linear sclerosis en coup de sabre (LScs) are rare, related, autoimmune conditions of focal atrophy and sclerosis of head and face which are associated with the development of focal epilepsy. The scarcity of PRS and LScs cases has made an evidence-based approach to optimal treatment of seizures difficult. Here we present a large systematic review of the literature evaluating 137 cases of PRS or LScs, as well as three new cases with epilepsy that span the spectrum of severity, treatments, and outcomes in these syndromes. Analysis showed that intracranial abnormalities and epileptic foci localized ipsilateral to the external (skin, eye, mouth) manifestations by imaging or EEG in 92% and 80% of cases, respectively. Epilepsy developed before external abnormalities in 19% of cases and after external disease onset in 66% of cases, with decreasing risk the further from the start of external symptoms. We found that over half of individuals affected may achieve seizure freedom with anti-seizure medications (ASMs) alone or in combination with immunomodulatory therapy (IMT), while a smaller number of individuals benefitted from epilepsy surgery. Although analysis of case reports has the risk of bias or omission, this is currently the best source of clinical information on epilepsy in PRS/LScs-spectrum disease. The paucity of higher quality information requires improved case identification and tracking. Toward this effort, all data have been deposited in a Synapse.org database for case collection with the potential for international collaboration.

Mechanical Supplementation With the Stromal Vascular Fraction Yields Improved Volume Retention in Facial Lipotransfer: A 1-Year Comparative Study.

Background: Unpredictable volume maintenance in the long term is a major limitation of autologous fat grafting. Objectives: The authors compared results of autologous lipotransfer to the face with or without enrichment of fat with the stromal vascular fraction (SVF). Methods: Thirty patients with asymmetric depletion of facial volume were included in a prospective study. Patients were randomly assigned to undergo a single session of autologous fat transfer with washed adipose tissue (control group) or with washed adipose tissue combined with the pellet of centrifuged lipoaspirate, which contained the SVF (enriched group). Patients were evaluated clinically and from photographs. A subset of 5 patients in each group underwent computed tomography (CT) preoperatively and 12-months postoperatively for quantitative assessment of graft retention. Washed and fractionated lipoaspirates were evaluated histochemically and with flow cytometry to determine relative abundances of viable cells. Results: No major complications occurred. CT findings 12 months postoperatively indicated that patients who received SVF-enriched fat had significantly better volume retention (9.6% volume loss vs 24% in the control group; P = 0.013). Independent surgeons more frequently rated long-term aesthetic outcomes as "excellent" for patients in the enriched group (82.5% vs 47.6% for control group). Laboratory results indicated that each pellet contained approximately 16,000 intact adipose-derived stem cells. Conclusions: Lipotransfer with SVF-enriched adipose tissue is safe and associated with improved volume retention, compared with transplantation of unenriched fat. The SVF can be dissociated from lipoaspirate by centrifugation to yield a large quantity of viable regenerative cells, without enzymatic digestion. Level of Evidence: 2.

Simulation of lipofilling reconstructive surgery using coupled Eulerian fluid and deformable solid models.

We present a method to simulate the outcome of reconstructive facial surgery based on fat-filling. Facial anatomy is complex: the fat is constrained between layers of tissues which behave as walls along the face; in addition, connective tissues that are present between these different layers also influence the fat-filling procedure. To simulate the end result, we propose a method which couples a 2.5D Eulerian fluid model for the fat and a finite element model for the soft tissues. The two models are coupled using the computation of the mechanical compliance matrix. Two contributions are presented in this paper: a solver for fluids which couples properties of solid tissues and fluid pressure, and an application of this solver to fat-filling surgery procedure simulation.

Case of progressive facial hemiatrophy with cervical sympathetic hyperactivity as underlying aetiology.

OBJECTIVE: We report a case of progressive facial hemiatrophy with cervical sympathetic hyperactivity as a possible underlying aetiology, based on clinical findings, three-dimensional computed tomography and thermographic imaging. METHODS: We present a case report in which we describe the investigation and clinical course of progressive facial hemiatrophy, and we also review the world literature on this condition. RESULTS: To our knowledge, this is the first report in the world literature of progressive facial hemiatrophy with cervical sympathetic hyperactivity indicated as a possible underlying aetiology, based on clinical findings, three-dimensional computed tomography and thermographic imaging. CONCLUSION: This syndrome may lead to atrophy of the subcutaneous adipose tissue with hyperfunction of the vegetative system. Although this is a rare syndrome, otolaryngologists should be aware of its symptoms, aetiology and treatment.

A review of Parry-Romberg syndrome.

Parry-Romberg syndrome, also known as progressive hemifacial atrophy, is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, muscles, and sometimes extending to the osteocartilaginous structures. It has been associated with various systemic manifestations, particularly neurologic, ophthalmologic and maxillofacial. In this article, we review Parry-Romberg syndrome with its associated findings (neurologic, ophthalmologic, cardiac, rheumatologic, endocrinologic, infectious, orthodontic and maxillofacial, and autoimmune), underlying cause, differential diagnoses (en coup de sabre, scleroderma, and Rasmussen encephalitis), and therapeutic options.

Orthopedic and orthodontic treatment of Parry-Romberg syndrome.

Parry-Romberg syndrome (PRS) is a degenerative disease characterized by progressive hemifacial atrophy of soft and hard tissues. A 10-year-old girl who had been treated for linear scleroderma at the dermatologic department since the age of 9 years visited the orthodontic department. The frontal facial photograph showed mild facial asymmetry. On the left side, mild atrophy of soft tissue, mild enophthalmos, cheek depression, and dry skin with dark pigmentation were observed. The radiograph showed hypoplasia of both the maxilla and mandible on the left side. Intraorally, she was in the mixed dentition with the dental crowding. This case report describes the treatment of a patient with PRS for 7 years. To minimize the effect of progressive atrophy on facial growth, a functional appliance was used. The facial photographs and radiographic records were periodically taken to analyze the progression of PRS. Although it is impossible to prevent the progress of facial asymmetry, it appears to be possible to limit the atrophic effect on the mandible by stimulating the mandibular growth. After stabilization of PRS, orthodontic treatment by fixed appliance was performed. In addition, autologous fat graft was performed 3 times at 6-month intervals. After the treatment, the patient had a confident smile, and facial asymmetry was improved.

[Facial asymmetry: the benefits of cone beam computerized tomography]. / Asymmetrie van het aangezicht: de meer- waarde van cone beam-computertomografie.

Treatment planning in facial asymmetry is complex and requires precise diagnosis and planning. In the case of a fourteen year old boy with Parry Romberg syndrome, cone beam computerized tomography scanning was used to analyse the asymmetry. Linear, angular and volumetric measurements helped to make clear the extent of the asymmetry, giving the orthodontist and surgeon as well as the patient insight into the possibilities and expectations of treatment. Also the mirror image of the unaffected side superimposed on the affected side was very helpful in this respect. The latter may offer new possibilities for fabrication of individualized alloplastic onlays to further correct asymmetry in the future.

Association of aesthetic and orthodontic treatment in Parry-Romberg syndrome.

Parry-Romberg syndrome (PRS) is an uncommon degenerative condition characterized by a slow, progressive, and, generally, unilateral atrophy of facial tissues, including muscles, bones and skin. Ophthalmological and neurological manifestations have frequently been observed and few oral changes have been reported. This article reports a case of PRS in a 22-year-old woman, exhibiting facial asymmetry, hypoplasia of the right side of the face, areas of skin hyperpigmentation, and oral alterations, involving the mandible and teeth. These clinical and radiological findings led to the diagnosis of PRS. In an attempt to improve the patient's facial aesthetic and the dental functions, oral pentoxifylline, orthodontic rehabilitation, and subcutaneous injections of polymethylmethacrylate microspheres were used as part of the treatment for the facial atrophy. Together, these approaches accounted for a minimal invasive treatment with long term satisfactory results.

Volumetric correction using poly-L-lactic acid in facial asymmetry: Parry Romberg syndrome and scleroderma.

BACKGROUND: Linear scleroderma and Parry-Romberg Syndrome (PRS) are chronic diseases that can affect the face, with patients therefore requiring facial volumetric correction. OBJECTIVE: We have used poly-L-lactic acid (PLLA) to treat facial asymmetry in patients with linear scleroderma and PRS, because it can restore volumetric defects and has been proven to be safe. MATERIALS AND METHODS: Since February 2004, we have treated six patients: four with linear scleroderma and two with PRS. The patients were treated every 4 weeks for three to five sessions. Photographic documentation was taken at the beginning and at the completion of every treatment and 12 and 18 months later. RESULTS: In patients with PRS and linear scleroderma of the face, we noticed good restoration of facial volume and symmetry and improvement in skin quality. The results were found to be permanent at 18 months in patients with PRS and 12 months in patients with linear scleroderma. CONCLUSIONS: PLLA has been shown to be a useful material for the correction of small volumetric defects caused by linear scleroderma and PRS. The authors have indicated no significant interest with commercial supporters.

Minimally invasive combined treatment for Parry-Romberg syndrome.

BACKGROUND: Facial hemiatrophy, a typical manifestation of Parry-Romberg syndrome, produces massive face asymmetry, causing marked aesthetic damage and severe psychological discomfort, with repercussions to the psychophysical status of the patient. This article presents a successful combined treatment that results in resolution of symptoms and patient satisfaction. METHODS: The authors proposed a clinic-therapeutic management comprising a customized program of bio-lipo-intense pulsed light (IPL) remodeling. RESULTS: In 2007, at a 1-year follow-up visit, the patient presented a stable result. She is satisfied with the augmentation treatment, the new volume and contours of the face, and the resolution of the sclerosis and ochrodermia. CONCLUSION: The authors have succeeded with a minimally invasive reconstructive technique for Romberg disease using a customized therapeutic program of poly-L-lactic acid, lipofilling, and IPL therapy. They report a case of good healing without recurrences and complications, with a good cosmetic result, and with satisfaction of the patient.

The history of injectable silicone fluids for soft-tissue augmentation.

BACKGROUND: The debate over the legitimacy of silicone as a safe tool for soft-tissue augmentation has spanned well over half a century. Proponents concede that injections of questionable purity and/or of massive quantities have produced unfavorable outcomes. They assert that in experienced hands with "injectable-grade" silicone, there are very few problems. Despite these claims, the literature is replete with disastrous outcomes following silicone fluid injection, often many years after the initial treatment. METHODS: An extensive review of the English-language literature was conducted using MEDLINE. RESULTS: A comprehensive review of injectable silicones was completed, revealing the origins, misuses, early clinical trials, and support for and against the injection of silicone fluids for the augmentation of soft tissues. CONCLUSIONS: A better understanding of the history of injectable silicone fluids for soft-tissue augmentation can give insight into the pitfalls and complications surrounding its use. There has been an evolution in the technique and type of products used for soft-tissue augmentation. In its current use, silicone oil for permanent soft-tissue augmentation could be a very powerful tool. There is some literature that supports the use of a small amount of purified, high-viscosity silicone oil; however, there has not been a single longitudinal study to date with appropriate follow-up data. The unanswered question remains: Are the risks worth the potential benefits of silicone oil as a permanent filler?