Lens subluxation combined with parry-romberg syndrome: case report.

BACKGROUND: Parry-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 PRESENTATION: A 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. CONCLUSIONS: Here 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.

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

BACKGROUND: Progressive hemifacial atrophy (PHA) is a rare disease characterized by progressive atrophy of skin, soft tissue, muscles, and underlying bone structures. For severe PHA patients with obvious bone deformities, skeletal framework reconstruction is needed in addition to soft-tissue augmentation. The authors propose a new combinatorial surgical method using rib cartilage graft and free adipofascial flap for restoring facial symmetry. To improve the surgical accuracy, preoperative three-dimensional planning and printing was used. METHODS: Twelve patients with severe facial atrophy were included in the authors' study. Three-dimensional facial image analyses were performed preoperatively to quantify the facial asymmetry. Rib cartilages were harvested and sculptured to the appropriate shape created by three-dimensional planning and fixed to the atrophic bone. The circumflex scapular artery-based adipofascial flap was transplanted to repair soft-tissue deficiency. A residual small monitor flap was left with the adipofascial flap. A revision surgery was performed to perfect the repair if the contour was suboptimal 6 months postoperatively. RESULTS: The adipofascial flaps survived in all 12 patients. All patients achieved good healing without complications. At 1 more year after surgery, the rib cartilage was still in position and rarely absorbed. The morphologic and volumetric difference between the affected side and the unaffected side was improved significantly postoperatively. All patients were satisfied with the results, and no more additional operations were required. CONCLUSION: The combinatorial surgery of rib cartilage graft and free adipofascial flap in the setting of three-dimensional planning and printing can be a good choice in restoring facial symmetry in severe cases of PHA. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Application of free serratus anterior muscle-fascial composite tissue flap and facial lipofilling in repairing progressive hemifacial atrophy.

INTRODUCTION: Progressive hemifacial atrophy (PHA) is a nonnegligible disease, and its treatment currently lacks consensus. We aim to conduct an analysis of PHA patients to summarize the postoperative effect. Moreover, we introduced the free serratus anterior muscle-fascial composite tissue flap as a safe and novelty surgical procedure for moderate-severe PHA. METHODS: This clinical study included four patients who received a free serratus anterior muscle-fascial composite tissue flap and 19 patients who received Coleman fat transplantation. Preoperative (preoperative photograph and imageological examination) and postoperative (postoperative photograph, complications, therapeutic effect, and satisfaction) assessments were performed for all PHA patients. Body Image Concern Inventory (BICI), Self-rating Anxiety Scale (SAS), Self-rating Depression Scale (SDS) were performed preoperatively and postoperatively. RESULTS: All the cases were cured with a good appearance with two kinds of operations. Free serratus anterior muscle-fascial composite tissue flap could correct face defects in one surgery and achieve good long time and short-time postoperative satisfaction in moderate-severe PHA. Fat transplantation could also enhance appearance in numerous operations for mild-moderate PHA. The volume of free-fat grafts decreased obviously after implantation in many cases. So, many patients (42.11%) accepted a series of operations to achieve satisfied postoperative effect. BICI, SAS, SDS score decreased a year later in all patients. CONCLUSION: Free serratus anterior muscle-fascial composite tissue flap transplantation is an effective and safe treatment for moderate to severe PHA.

A case of progressive crossed hemiatrophy mistaken as panniculitis.

Progressive crossed hemiatrophy is an extremely rare clinical type of facial hemiatrophy that presents primarily as unilateral facial atrophy and contralateral trunk or limb involvement. The undistinguishable clinical manifestation and pathological changes complicate diagnosis, especially at the onset of the disease when presenting with less clinical evidence. Here, we report a case of a 9-year-old boy started with left scalp induration, following with subcutaneous tissues atrophy on the right trunk. He was mistaken as panniculitis based on the pathologic findings and treated with topical tacrolimus without any improvement. Immune-related tests were implemented to exclude connective tissues. Imaging examinations such as magnetic resonance was conducted to evaluate the range and degree of the involvement of the skin, soft tissue, and cranial changes. Although no effective treatment to hold back the progress has been reported so far, surgeries might work to restore the appearance to some extent or improve central nerves symptoms if they exist.

Soft-Tissue Reconstruction in Progressive Hemifacial Atrophy: Current Evidence and Future Directions.

BACKGROUND: Progressive hemifacial atrophy is a rare disorder characterized by gradual unilateral soft-tissue atrophy in the face, which may also include clinically significant degeneration of underlying muscle and bone. In recent years, there has been a growing body of evidence regarding different soft-tissue reconstructive strategies in progressive hemifacial atrophy and the impact of intervention timing on disease progression. This article provides a comprehensive synthesis of the latest evidence to guide optimal management. METHODS: A comprehensive multidatabase search was performed through April of 2020 using relevant search terms to identify clinical studies. Outcomes, complications, and disease- and patient-related indications pertaining to different soft-tissue reconstructive strategies in progressive hemifacial atrophy were collected and critically appraised. RESULTS: Thirty-five articles reporting on a total of 824 progressive hemifacial atrophy patients were evaluated; 503 patients (61 percent) were managed by microvascular free flaps, 302 patients (37 percent) were managed by autologous fat grafts, and 19 patients (2 percent) were managed by pedicled flaps. A detailed synthesis of outcomes is presented in this article, as is a comparative evaluation of different microvascular free flap options. CONCLUSIONS: Soft-tissue reconstruction in progressive hemifacial atrophy remains an evolving field. Operative decision-making is often multifaceted, and guided by specific volumetric, aesthetic, and functional deficiencies. Serial fat grafting is the primary modality used for patients with mild soft-tissue atrophy, whereas microvascular free flaps widely remain the treatment of choice for reconstruction of large-volume defects. There exists a growing role of graft supplementation to improve fat graft survival, whereas recent evidence demonstrates that early intervention may help curb disease progression.

Multi-Staged Surgeries for Coexisting Facial Asymmetry and Strabismus in Parry-Romberg Syndrome.

ABSTRACT: Parry-Romberg syndrome (PRS) is a rare disorder resulting in disfiguring facial asymmetry. Ocular manifestations can result in complex strabismus. There were limited reports on the treatment of PRS with coexisting strabismus. We present a multistaged surgical approach to manage the facial asymmetry and strabismus.

Combined Surgical-Orthodontic Treatment of Patients With Severe Parry-Romberg Syndrome.

ABSTRACT: Parry-Romberg syndrome (PRS) refers to a relatively rare dysfunction disease that is characterized by chronic progressive maxillofacial atrophy, especially one side of facial skin, subcutaneous tissue, muscle, and bone. According to the atrophy degree of skin, subcutaneous tissue, and skeleton in the area innervated by the trigeminal nerve, PRS can be classified into mild, moderate, and severe. In general, cases with different severity have specific treatment regimens. For mild and moderate cases, soft tissue augmentation techniques are the optimal strategy for aesthetic reconstruction. In this study, the authors report a 19-year-old female with severe PRS. Considering the severity of the case, a combined surgical and orthodontic treatment was performed, which was involved in alveolar bone augmentation, preoperative and postoperative orthodontic treatment in combination with orthognathic surgery, medpor filling of zygomatic and maxillary complex, free fat grafting, as well as angulus oris and lip trimming. Comprehensive treatment is recommended for severe cases with extensive atrophy of soft tissue and craniofacial bone, obvious deviation of the chin and occlusal plane.

Fundus changes of Parry-Romberg syndrome: A report of three cases and literature review.

PURPOSE: To report three cases of Parry-Romberg syndrome (PRS) with progressive hemifacial atrophy and similar fundus changes. METHODS: Retrospective case series of three patients who presented to the ophthalmology department with blurred vision in one eye. All of them underwent three-dimensional reconstruction of head computed tomography (CT), related physical examinations, and also monthly ocular examinations of both eyes, including the slit-lamp microscope, fundus autofluorescence, optical coherence tomography (OCT), and fundus fluorescein angiography (FFA). We also reviewed the current literature about the fundus change in PRS from inception to March 2020 via PubMed. RESULTS: Coats-like fundus and exudative retinal detachment were found in all three eyes of three patients during the follow-up. No systematic changes were found except for hemifacial atrophy. The final diagnoses of these three patients were PRS. CONCLUSION: Only a subset of PRS patients have concomitant ocular changes, but it still should be noticed that the diagnosis of PRS should be considered if adults presented with Coats-like retinopathy and hemifacial atrophy.

High-Density Fat Grafting Assisted Stromal Vascular Fraction Gel in Facial Deformities.

ABSTRACT: Autologous fat grafting is commonly used for soft tissue augmentation and reconstruction, this technique is limited by a high rate of graft absorption. The stromal vascular fraction gel (SVF-gel) grafting for facial volume augmentation can exert a positive effect on skin rejuvenation, but its major limitation is the low rate of conversion of Coleman fat. The purpose of our study was to investigate a novel surgery using performing high-density fat in combination with SVF-gel in the treatment of hemifacial atrophy, or Romberg diseases. From October 2017 to October 2019, 13 patients with hemifacial atrophy underwent high-density fat transfer with SVF-gel injection. The outcome was determined by the difference in presurgery and postsurgery FACE-Q modules (FACE-Q conceptual framework: 1, Satisfaction with Facial Appearance; 2, Health-Related Quality of Life; 3, Negative Sequelae; 4, Satisfaction with Process of Care), which were designed as patient-reported outcome instrument to evaluate the unique outcomes of patients undergoing facial cosmetic procedures.The excellent cosmetic results were observed during follow-up periods, with no adverse events was seen in the treatment group. All patients showed improvements in facial augmentation and contour. In patients with facial volume loss, high-density fat transfer with SVF-gel facial injection resulted in significantly higher improvement scores and better patient satisfaction. The patient-reported FACE-Q modules presurgery and postsurgery results showed statistically significant improvement (P < 0.05). This high-density fat in combination with SVF-gel is an effective method of correcting the facial volume loss that leave no complications during follow-up, having a satisfactory volumization effect. This could largely facilitate the clinical utilization of fat.

Comparative Study of Three-Dimensional Volume Measurement for Facial Fat Grafting.

ABSTRACT: The three-dimensional (3D) volume measurement after facial fat grafting is of great significance to plastic surgeons. It has been ascertained that reliable results rely on the accurate measurement of 3D softwares. Some 3D softwares in literatures have proposed various ways to optimize each step of the procedure, including the pre- and post-operative image acquisition, alignment, calculation, and analysis. Good image alignment between pre- and post-scan is essential to quantify the volumetric change. Once the pre- and post-operative image alignment has slightly bias or deviation, the subsequent volume measurement would also be affected. To our knowledge, 2 types of 3D software have been widely applied in clinic, primarily based on the image-automatically alignment and image-manually alignment. This study aimed to compare the accuracy, repeatability, and reproducibility of the Geomagic Qualify 12.0 software and the 3-Matic 7.0 software in a relatively ideal model of virtual facial fat grafting. A simulated facial fat grafting was first performed for 10 preoperative patients diagnosed with progressive hemifacial atrophy, and the known volumetric change was named as the true value (T value). Then, the facial volumetric change of every case was remeasured 10 times with above 2 kinds of software separately. The mean volumetric change was calculated as Q value and M value. The paired t test, intraclass correlation coefficient, and Bland-Altman analysis showed that the Geomagic Qualify 12.0 software demonstrated a statistically higher accuracy, repeatability, and reproducibility in comparison with the 3-Matic 7.0 software.

Aesthetic Restoration of Severe Hemifacial Atrophy With Free Adipofacial Antero Lateral Thigh Flap Followed by Autologous Fat Grafting; Optimizing Result and Long-Term Outcome.

BACKGROUND: Hemifacial atrophy (Romberg disease) is characterized by progressive soft tissue and bone atrophy on 1 side of the face. The process of atrophy usually starts in the late first or early second decade of life. Romberg Patients usually require soft tissue augmentation for the correction of their defect. There are many reconstructive tools available to correct such facial asymmetry. In this study, we evaluate the outcome of the free adipofacial flap followed by autologous fat grafting in the treatment of Romberg disease, regarding aesthetics and longevity of the treatment. PATIENTS AND METHODS: A retrospective review of 12 patients with moderate to severe hemifacial atrophy from April 2016 till March 2019. All patients received soft tissue augmentation with free adipofascial anterolateral thigh flaps, followed by autologous fat grafting 6 to 12 months later for correction of residual deformity. The average follow-up period was 18 months (range, 6-30). RESULTS: There were 9 females and 3 males in this study. All flaps were survived, and the effect is long-lasting during follow-up. Patients were satisfied with the result especially after refinement of the result of free tissue transfer. The donor sites were closed directly and with no apparent morbidities nor dysfunctions. CONCLUSIONS: Microsurgical free tissue transfer is considered a gold standard tool in the management of moderate and severe form of hemifacial atrophy. Among different flaps available, free adipofacial anterolateral thigh flap (ALT) works as a workhorse flap in the correction of a severe form of the disease. Autologous fat grafting is a versatile and reliable option to correct residual deformities. We believed that such a combination is a superb approach to optimize the outcome of the severe form of the progressive hemifacial atrophy.

Parry­Romberg syndrome / Parry-Romberg-szindróma

Parry-Romberg syndrome is a rare, slowly progressive, autolimitated disease, characterized by unilateral facial atrophy. It is usually manifested in childhood and young adulthood. Because of the degree of atrophy and the variety of symptoms, the diagnosis, prognosis, and treatment of Parry-Romberg syndrome are a major challenge. The purpose of this case presentation is to highlight the difficulty of establishing the diagnosis, furthermore, to present the steps of examining the patient and to draw attention to the importance of proper timing of the surgery. Moreover, it wants to emphasize the importance of looking for diseases that often occur with the mentioned syndrome. Orv Hetil. 2020; 161(28): 1181-1185.

Challenging Traditional Thinking: Early Free Tissue Transfer for Active Hemifacial Atrophy in Children.

BACKGROUND: Hemifacial atrophy (Parry-Romberg syndrome) is an enigmatic craniofacial disorder characterized by progressive facial atrophy. Early age at onset is associated with significant skeletal involvement and jaw abnormalities. Standard reconstructive dogma dictates that the disease should "burn out," with at least 2 years of no disease progression, before pursuing reconstructive intervention. The purpose of this article is to present the senior author's (J.W.S.) experience treating preadolescent children with free soft-tissue transfer for reconstruction of progressing hemifacial atrophy, and to review surgical techniques in this patient population. METHODS: Pediatric free tissue transfer cases performed by the senior author were reviewed. Free tissue transfer using a circumflex scapular adipofasciocutaneous flap was performed to treat deformities arising from hemifacial atrophy. RESULTS: Thirty-six patients aged 3 to 6 years underwent free tissue transfer in the presence of progressing, active disease. Follow-up ranged from 7 months to 25 years. There were no cases of flap loss. In all patients, the disease process seemed to be altered, with no symptoms recurring to date. CONCLUSIONS: The authors believe that early microsurgical correction of hemifacial atrophy in children as young as 3 years is reliable and effective. The authors theorize that the interposition of vascularized, healthy tissue may alter or even halt the progression of disease, and may also prevent the skeletal hypoplasia classically seen in older patients. Further study incorporating autologous fat grafting as a control procedure may provide additional insight into this challenging disease process. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Parry-Romberg Syndrome With Hemimasticatory Spasm: A Rare Combination.

Parry-Romberg syndrome is a rare craniofacial disorder characterized by progressive hemifacial atrophy with systematic manifestations. The combination with hemimasticatory spasm is rare, with only 9 patients reported before. In this study, a study of a young male patient with Parry-Romberg syndrome and hemimasticatory spasm on his left side was presented. Radiologic examinations showed severe atrophy limited to subcutaneous tissue and electromyography demonstrated as hemimasticatory spasm. Injection of botulinum toxin type A was applied for the treatment of hemimasticatory spasm and after 2 separated injections, the patient was significantly relieved from symptom. The theory of focal demyelination of the trigeminal nerve peripheral brunches is the possible link between hemifacial atrophy and hemimasticatory spasm, although the pathogenesis of both diseases requires further study and current therapeutic methods are still limited to symptomatic treatments. Injection of botulinum toxin type A is an effective way to treat hemimasticatory spasm and autologous fat transplant is a promising solution to correct facial asymmetry.

Comprehensive Three-Dimensional Technology Strategies for Autologous Free Fat Graft in Parry-Romberg Syndrome.

Autologous free fat graft is a widely accepted method of facial contour restoration in Parry-Romberg syndrome (PRS); however, complex and unpredictable facial anatomy can render this surgery extremely challenging. The integration of 3-dimensional (3D) technology strategies as a method to enhance surgical aesthetic outcomes has been demonstrated.A retrospective chart review was performed for PRS with autologous, free fat graft between 2016 and 2017. Based on 3D volumetric facial analysis and presurgical simulation, a 3D printed fat graft guide was produced. Surgical outcomes were analyzed by volumetric measurements of the upper, middle, and lower face segments.A total of 9 patients were included in the study. Their upper and middle facial volumes before and after the procedures showed statistically significant volume augmentation (upper face, P = 0.004; middle face, P = 0.002) 6 months postoperatively (T1). Facial asymmetry was also statistically significantly corrected (middle face P = 0.012; lower face P = 0.025) at 1 year after the procedure (T2).The advantageous application of 3D comprehensive technology for aesthetic improvements in patients with PRS with autologous, free fat graft treatment has been demonstrated. Precise preoperative planning based on simulations and postoperative 3D volumetric analyses can help adequately predict fat graft strategies.

Autologous fat transfer for facial recontouring in Parry-Romberg syndrome.

BACKGROUND: Parry-Romberg syndrome (PRS) is a rare condition involving self-limited progressive facial hemiatrophy. AIMS: To analyze esthetic results of fat graft used for correcting soft-tissues atrophy in PRS patients, in the aspect of volume restoration and skin rejuvenation, to present our experience in redefining facial contours in PRS and modification of fat graft accusation technique (based on Coleman's technique). PATIENTS/METHODS: A prospective evaluation study was designed to analyze esthetic results (contour and skin condition), complications, and subjective satisfaction of eight patients with PRS treated with fat graft. RESULTS: All patients underwent two (n = 6) or three (n = 2) procedures in 3 months intervals (the mean 3.7 months). No major postoperative complications were observed. The authors evaluated their esthetic results concerning face contour and symmetry as good (in four patients) and very good (in four patients). Subjective assessment showed high patients' satisfaction with the final result of face contouring (the mean VAS [0-5]-4.75). CONCLUSIONS: Our experience shows that fat grafting in patients with PRS is safe and gives satisfactory results in the aspect of volume restoration and skin quality improvement. Modified technique of graft preparation (112 g for 3 minutes) enables to obtain less dense fat graft, which is more appropriate in case of PRS patients with thin atrophic skin.