Hemiatrophy Research Articles

Successful Management of Parry-Romberg Syndrome With Lipografting: A Case Report

Parry-Romberg Syndrome (PRS), or progressive hemifacial atrophy, is a rare disorder marked by the progressive atrophy of skin and soft tissues, sometimes affecting muscles, cartilage, and bones. Typically manifesting in childhood, PRS has a 66% female predilection and sporadic occurrence. Diagnosis is clinical, supplemented by tests to exclude other conditions. Treatment options include bone/cartilage grafts, fat grafts, dermal fillers, and free tissue transplantation. Lipoinjection, using autologous adipose tissue enriched with stem cells, is a promising technique. A 33-year-old female with PRS exacerbated by pregnancy presented with severe right hemifacial atrophy. Initial lipoinjection elsewhere resulted in significant graft reabsorption. At our institution, a second lipoinjection was performed at age 34 after disease remission. Five months postoperatively, 30% graft reabsorption was noted, but significant improvement in facial symmetry was achieved. A second lipoinjection session further enhanced facial volume and skin quality. Four months postoperatively, the patient showed substantial improvement, with 90% facial symmetry and stable disease remission. Autologous lipoinjection is an effective treatment for PRS-related facial atrophy, offering minimal tissue rejection and satisfactory aesthetic outcomes. The presented case highlights the potential of this technique in achieving significant clinical improvement and facial symmetry in PRS patients.

Most common congenital syndromes with facial asymmetry: A narrative review.

Symmetry is present in various aspects of everyday life. A symmetrical face is considered attractive, whereas a lack of facial symmetry is regarded as a source of functional and aesthetic problems. Most of the people exhibit slight asymmetries, but some of them reveal severe asymmetries. Among patients presenting with severe facial asymmetries, there may be those with congenital defects. Congenital defects may manifest at the time of birth or be a result of birth trauma.One of the most prevalent asymmetrical birth defects is cleft lip and/or cleft palate. Other congenital defects include craniofacial syndromes, such as Treacher Collins syndrome (TCS) and Goldenhar syndrome. Among the rare syndromes with facial asymmetries, Klippel-Feil syndrome (KFS), PHACE (posterior fossa brain malformation, hemangiomas, arterial anomalies, cardiac anomalies, and eye abnormalities) syndrome, plagiocephaly, and Parry-Romberg syndrome are worth noticing. The majority of craniofacial asymmetries require surgery to improve the patient's facial appearance. The treatment is multidisciplinary and long, and the most common procedures involve reparative and regenerative surgeries. The aim of this review was to present the most common congenital defects with facial asymmetry.

Nasolabial Transfer Flap with Auricular Cartilage Graft after Fat Grafting for Nasal Ala Reconstruction in Parry-Romberg Syndrome.

Alar atrophy associated with Parry-Romberg syndrome (PRS) can lead to significant nasal contour deformities. The primary objective of this study was to address nasal alar deformities in PRS patients through the application of a nasolabial transfer flap combined with an auricular cartilage graft after sequential fat grafting. All PRS patients with alar deformities who underwent our reconstructive surgery were included in this case series. Sequential fat grafting was performed on the affected nasolabial area, followed by a nasolabial transfer flap combined with an auricular cartilage graft. Surgical outcome and complications were recorded during postoperative follow-up. From September 2021 to November 2023, a total of 14 PRS patients presenting nasal alar defects underwent nasolabial transfer flap combined with an auricular cartilage graft after sequential fat grafting. Complications at 1 month postoperatively included partial flap necrosis (21%), hyperplastic scarring (14%), undercorrection (14%), overcorrection (7%), and pain (7%). The surgery significantly improved the patient's nasal symmetry. FACE-Q questionnaire indicated the overall aesthetic outcome and quality of life were deemed satisfactory for all patients. After sequential fat grafting, the nasolabial fold transfer flap with an auricular cartilage graft is effective to address alar deformities associated with PRS. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266.

Superior retention of aged fat graft by supplementing young adipose-derived stromal cells in a murine model.

In 2021, a meta-analysis showed fat graft retention varied from 26 to 83%. In a retrospective study including patients with Parry-Romberg syndrome, the younger age group had higher satisfaction scores (3.8 vs. 3.0) after fat grafting. Cell-assisted lipotransfer (CAL) could be an alternative to overcome the instability of volume loss; however, no study investigated its effect on old recipients. In an in vitro study, adipose-derived stromal cells (ASCs) from aged and young (52- and 8-weeks old) DsRed B6 mice were characterized by proliferation rates as percentages of Ki-67-positive cells. 68-week-old wild type B6 mice received 150 µL of green fluorescent protein fat (from 69-week-old B6 mice) mixed with saline, 3 x 105 aged or young DsRed ASCs (N, A, and Y groups, respectively) on the scalp (n = 6/group). After 8 weeks, graft volumes were evaluated using micro-computed tomography. Vessel densities were tracked by percentages of CD31 using immunofluorescence staining. Young ASCs showed higher proliferation than the aged (47.1% and 26.2%, respectively, p < 0.05). The Y group showed the highest graft retention (median: N = 41.0%, A = 52.2%, Y = 65.2%, p < 0.05) and percentage of blood vessels (median: N = 27.7%, A = 43.5%, Y = 54.7%, p < 0.05) among the three groups. CAL is effective with old recipients; higher effect was observed by supplementation with younger ASCs due to higher angiogenesis stimulation. However, this is autologous grafting model, further validation of safety, toxicity, and allogenic grafting is required. The results suggest that it is clinically possible to conduct CAL for old adults using autologous ASCs. However, further validation of using allogenic ASCs from young donors to improve graft retention in old adults is required.

A rare case of Parry-Romberg syndrome with restrictive strabismus

Parry-Romberg syndrome is a rare disease characterized by slowly progressive atrophy affecting facial subcutaneous tissues, muscles, osteocartilaginous structures, and possible cerebral involvement. Though bilateral cases have been described, diagnosis is often possible because of the characteristic hemifacial atrophy and resulting facial asymmetry at later stages of the disease. We report a 28-year-old woman with diplopia for the past 3 months with history of gradual loss of subcutaneous fat in the right side of her body. She had severe restriction of adduction of right eye. Multidetector computed tomography scan of the orbit revealed a reduced bulk of the right medial rectus muscle.

Restauración volumétrica autóloga en pacientes con hemiatrofia adiposa facial

IntroductionFacial lipoatrophy lacks standard clinical criteria for its diagnosis and treatment; however, patients with hemifacial atrophy increase, affecting the mental health of patients who suffer from it. Lipografting has been rescued as an effective and safe treatment option. ObjectiveTo show the results of autologous volumetric restoration in patients with facial adipose hemiatrophy. MethodMulticenter, descriptive, longitudinal and prospective study carried out on 29 patients treated at the Hermanos Ameijeiras Hospital, Cuba and the EIMEC clinic, Spain in the period from July 2018 to July 2023. Both sexes were included, between 19 and 75 years old, with facial asymmetry due to unilateral decrease in subcutaneous cellular tissue. ResultsThe average age was 47.9 years. Women predominated with 79.3%. The most common etiologies were idiopathic, autoimmune and orbital trauma, each accounting for 17.2%. According to the degree of severity, grade II was the highest, with 65.5%, and grade III represented 34.5% of the total. Regarding the amount of grafted adipose tissue, 62.1% of the cases were 31 to 60ml, and 27.6% more than 60ml. According to the number of graft applications, 72.4% required one and 27.6% two applications. 93.1% had no complications and 6.9% reported 50% reabsorption of the graft. Patient satisfaction reached 100% and the results were classified as good in 86.21%; when there was improvement, without complications as well as patient satisfaction. ConclusionAutologous lipotransfer assisted with adipose tissue stem cells is a safe treatment option that offers good results in correcting asymmetry caused by hemifacial lipoatrophy.

Unveiling Parry-Romberg Syndrome With Native Demyelinating Etiology as the Underlying Cause

Parry-Romberg Syndrome (PRS) is an atypical condition characterized by hemiatrophy of the face. Despite its rarity, the precise pathophysiological processes underlying its etiology remain elusive. While previous reports have hinted at the potential involvement of autoimmune factors, our case report aims to explore a novel perspective. Specifically, we pose the question of whether multiple sclerosis (MS) could be a plausible causative factor for PRS. Additionally, we investigate the potential association between Radiologically Isolated Syndrome (RIS) and the development of Parry-Romberg Syndrome.

Surgical Management of Parry-Romberg Syndrome: An in-Depth Review of Techniques and Outcomes

Surgical Management of Parry-Romberg Syndrome: An in-Depth Review of Techniques and Outcomes

Managing Soft Tissue Defects in Parry-Romberg Syndrome: An Individualized Approach.

Parry-Romberg syndrome (PRS) is a rare condition characterized by a progressive shrinkage and degeneration of the tissues beneath the skin, usually on one side of the face. Managing this disease presents numerous challenges due to its heterogeneity and unpredictable outcomes. The existing literature is primarily composed of case reports and series, leading to a lack of comprehensive guidelines on surgical intervention for the various manifestations of PRS. We propose an approach to address these challenges and optimize surgical outcomes. We conducted a retrospective review of all patients who underwent surgical correction for PRS between 2012 and 2022. Surgical interventions were determined based on the location and severity of the facial defect. The revision procedures were tailored to each patient until they were satisfied with the results. Eleven patients underwent surgical correction, with an average of 3.2 procedures per patient. Fat grafting or dermal fat grafting was sufficiently effective for mild deficits in all areas and for upper-third deficits regardless of severity. For moderate to severe defects in the mid and lower face, a combination of buried free flaps and fat grafting yielded satisfactory results. Upon final revision, all patients rated their results as satisfactory or excellent. We propose an approach to surgical management that takes into account the specific deficits of each patient. Our approach has proven to yield aesthetically pleasing and reliable results, aligning with findings in the existing literature. This method could provide a foundation for standardized guidelines and improve the prognosis for individuals with PRS.

A patient's experience of progressive hemifacial atrophy.

Progressive hemifacial atrophy (PHA) is a rare idiopathic disorder that can have a significant impact on quality of life. Herein, a patient describes his experience of the condition.

Parry-Romberg syndrome following COVID-19 vaccination.

Parry–Romberg syndrome is an uncommon disorder characterized by hemifacial atrophy of the skin. We present an unusual case of COVID-19 vaccine-induced Parry–Romberg syndrome. To our knowledge, no previous cases have been reported.

Microvascular Decompression Plus Partial Resection of Trigeminal Nerve Motor Root for Hemimasticatory Spasm Combined With Hemifacial Atrophy: A Good Choice or Not?

Hemimasticatory spasm (HMS) combined with hemifacial atrophy is a rare clinical entity with an unclear etiology. The authors report a 58-year-old female suffering from HMS and hemifacial atrophy, which performed microvascular decompression plus partial resection of the trigeminal nerve motor root, which is the first report in worldwide. The vascular compression of the trigeminal nerve motor root was found in surgery, the authors completed the aforementioned surgical method. The symptoms of HMS disappeared after surgery but recurred after 8 months.

Lens subluxation combined with parry-romberg syndrome: case report

BackgroundParry-Romberg syndrome (PRS) is a rare progressive degenerative disorder of unknown etiology. Here we report a rare case of PRS combined with lens subluxation in Eye and ENT hospital of Fudan University, Shanghai. To our knowledge, it is the first reported case of PRS combined with lens subluxation that has been managed surgically with phacoemulsification and CTR placement and IOL implantation in Shanghai.Case presentationA 60-year-old woman was referred for “right visual blur for 2 years” and had persistent right facial paralysis of unknown etiology since the age 12. She had right facial muscle atrophy and paralysis. Eye examination also showed the right eyelid pseudoptosis, enophthalmos, age-related cataract combined with lens subluxation existed in the right eye. The patient was diagnosed as age-related cataract and lens subluxation in the right eye and progressive hemifacial atrophy (Parry-Romberg syndrome). We conducted a combined phacoemulsification, IOL and CTR implantation and pupilloplasty surgery for the patient under general anesthesia and the postoperative UCVA was 20/30 and remained for 1 year’s follow up.ConclusionsHere we reported a rare case of PHA combined with lens subluxation in China. After appropriate eye surgery, the patient achieved satisfying vision result in the right eye.

A retrospective study of adult patients with Parry-Romberg syndrome.

Parry–Romberg syndrome (PRS), also known as progressive hemifacial atrophy, is characterized by atrophy of the skin and subcutaneous tissue, including muscle, bone and cartilage. PRS is a clinical diagnosis, although supportive imaging and histology may be obtained. We present a case series of 10 adult patients with a diagnosis of PRS seen at Massachusetts General Hospital and Brigham and Women’s Hospital from 2000 to 2021. Nine of 10 patients were women, with a mean (SD) age at onset of 27.3 (12.1) years and a mean (SD) of 12.4 (7.8) years between disease onset and diagnosis. Of the 10 patients, 4 had biopsies with supportive histopathology findings and 7 had positive imaging findings. Patients typically received both medical and surgical treatments.

Treatment of Hemimasticatory Spasm and Facial Myokymia Associated with Hemifacial Atrophy and Localized Morphea with Botulinum Neurotoxin and Immunotherapy

Treatment of Hemimasticatory Spasm and Facial Myokymia Associated with Hemifacial Atrophy and Localized Morphea with Botulinum Neurotoxin and Immunotherapy

Late-onset Parry-Romberg Syndrome with Atypical Neurological Manifestations: A Case Report (P11-11.015)

Late-onset Parry-Romberg Syndrome with Atypical Neurological Manifestations: A Case Report (P11-11.015)

Hemimasticatory spasm: a series of 17 cases and a comprehensive review of the literature.

Hemimasticatory spasm (HMS) is a rare movement disorder characterized by paroxysmal spasms or twitches of the unilateral jaw-closing muscles. This study aimed to comprehensively evaluate the clinical features of patients with HMS. Data from 17 patients newly diagnosed with HMS (12 females and 5 males; mean age at onset: 46.7 years) who visited our department were retrospectively analyzed, and a literature search based on electronic medical databases from their inception until November 30, 2023, was conducted. A manual search was conducted for articles cited in the related literature. A total of 117 cases (72 females and 45 males; mean age at onset: 37.1 years) from 57 studies were analyzed. The muscles involved were the masseter (97.4%), temporalis (47.9%), and medial pterygoid (6%). Morphea or scleroderma was observed in 23.9% of the patients, and facial hemiatrophy in 27.4%. In 17.9% of the cases, Parry-Romberg syndrome was either complicated or suspected. Typical electromyographic findings included the absence of a silent period during spasms (23.9%) and irregular brief bursts of multiple motor unit potentials. Oral medicines, such as clonazepam or carbamazepine, alleviated the symptoms for some patients but were often unsatisfactory. Botulinum toxin therapy was effective in most cases. Recently, microvascular decompression surgery is increasingly being used, resulting in complete relief in some cases. In conclusion, highly effective modalities are currently available, and it is necessary to raise awareness of HMS to ensure that it can be diagnosed and treated accurately by both medical and dental professionals.

Parry-Romberg Syndrome and an Associated Complex Vascular Lesion Managed With Hybrid Strategies: A Case Report

BACKGROUND AND IMPORTANCE: Parry-Romberg syndrome (PRS) is a condition of unknown etiology, most commonly characterized by progressive facial hemi-atrophy due to breakdown of the underlying skin and soft tissues. PRS has been associated with neurologic disease such as intracranial aneurysms and vascular malformations. However, its exact role in the incidence of neurovascular disease remains unknown. CLINICAL PRESENTATION: We present a case of a complex fusiform aneurysm involving the A1 segment of the left anterior cerebral artery in a 21-year-old man with PRS. The patient initially presented with a left giant fusiform/dissecting internal carotid artery aneurysm initially managed with overlapping flow diverters. His post-flow diverter treatment course was confounded by progressive growth of the aneurysm with worsening mass effect on the optic chiasm leading to complete visual loss in the left eye and a right hemifield cut. To manage the aneurysm recurrence, retreatment with surgical bypass-supported carotid occlusion was performed. Thereafter, visual symptoms improved, but complete normalization of vision was not achieved. The patient remained clinically stable for several years until follow-up imaging demonstrated a novel adjacent recurrence involving the ipsilateral A1 segment, previously noted to be angiographically normal. The patient underwent superselective angiography followed by endovascular occlusion of the new fusiform lesion. DISCUSSION: The case presented here illustrates the hybrid roles of endovascular and surgical treatment modalities in treating complex vascular lesions. The recurrence of the lesion following initial treatment strategies introduces questions about how the nature and development of the lesion affected the success of treatment at each stage. Considerations for the efficacy of each stage of treatment in this case include the patients underlying connective tissue disorder, the pediatric etiology of the lesion, and the timing of each treatment option given these factors. CONCLUSION: In addition to understanding the biological behavior of flow diversion for complex intracranial aneurysms, the role of PRS in the incidence of neurovascular disease should also be considered when applicable. More research into the mechanisms of PRS in neurovascular disease is needed.

Immunological Profiles in Parry-Romberg Syndrome: A Case-Control Study.

Background: Parry-Romberg syndrome (PRS) is a rare craniofacial disorder. The aim of this study is to provide information on the immunological profile of this pathology. Since PRS can be included in a wider spectrum of sclerodermic diseases, we propose a case-control study comparing a patient affected by PRS with one with a diagnosis of scleroderma, herein used as control (CTR). Methods: B lymphocyte, T lymphocyte, and monocyte phenotypes and functions were assessed by flow cytometry in influenza (Flu)- or anti cluster differentiation (CD)3/CD28-stimulated peripheral blood mononuclear cells (PBMCs). Cytokine concentration was evaluated as well in PBMC supernatants, plasma, and saliva by Luminex assay. Results: T and B lymphocytes were similarly activated in unstimulated PRS and CTR cells but differed following antigen stimulation. T helper (Th)17 lymphocytes were expanded in PRS compared to CTR; this increase correlated with higher interleukin (IL)-17 concentration. Conclusions: Our case-control study is the first to compare the immunological profiles of PRS and scleroderma patients. The higher percentage of Th17 cells in PRS suggests the use of anti-IL17 receptor monoclonal antibody in this rare disease; however, further studies with larger numbers of patients are needed to confirm our findings.

Discussion: Treatment of Progressive Hemifacial Atrophy by Cartilage Graft and Free Adipofascial Flap Combined with Three-Dimensional Planning.

Discussion: Treatment of Progressive Hemifacial Atrophy by Cartilage Graft and Free Adipofascial Flap Combined with Three-Dimensional Planning.