Facial Atrophy Research Articles

Inflammatory Myopathy with Severe Tongue Atrophy in Pembroke Welsh Corgi Dogs

A disease characterized by tongue and facial muscle atrophy has been recognized sporadically among Pembroke Welsh Corgi (PWC) dogs in Japan. The present study describes the pathologic findings of this canine syndrome. Histopathologic examinations were performed in 2 dogs, including a case of muscular biopsy. Identification and characterization of autoantibodies were attempted by fluorescent antibody test (FAT) and Western blot (WB) by using sera from 7 PWC dogs with typical clinical features, 6 PWC dogs with other clinical signs, and 2 from other breeds with polymyositis. Clinically, the 7 affected PWC dogs exhibited dysphagia with severe tongue atrophy, facial muscular atrophy, and occasional walking difficulty. Histopathologic examinations of the 2 dogs with clinical symptoms revealed moderate to severe inflammatory lesions characterized by lymphohistiocytic infiltration and muscular atrophy in the tongue and/or femoral muscles. The tongue lesions were very severe and accompanied by diffuse fatty infiltration. There were no major lesions in the nervous tissues examined. By FAT, an autoantibody against the cross striation of skeletal muscle was detected in sera from 5 affected PWC dogs. By using WB analysis, the autoantibodies recognized a 42-kDa molecule in striated muscle but not in the nervous tissues. All of the findings indicated that the unique disease of PWC dogs might be generalized inflammatory myopathy, whereas the detailed etiology concerning the dominant involvement of tongue muscles and the role of the autoantibody in the canine disease remain to be clarified.

A Minimally Invasive Technique for Burying Free Flaps and Grafts

The placement of large-volume flaps and grafts into a subcutaneous pocket often requires extensive incisions for accurate placement. We describe a technique that allows for the precise, atraumatic placement of these tissues through minimal incisions. No unusual or expensive surgical instrumentation is required, and the technique is easy to learn. We have found the technique especially useful in the augmentation of severe facial atrophy.

Iodine‐125 orbital irradiation : improvement of cosmetic results for patients needing postenucleation irradiation

Abstract Purpose Patients enucleated for malignant intraocular tumors (as retinoblastoma or uveal melanoma) that have extrascleral extension and/or optic nerve resection margin involvement, are at risk of orbital recurrence. These enucleated orbits were previously treated with external beam radiotherapy which induced late sequelae, in particular facial atrophy. Iodine 125 Orbital brachytherapy (BRT) was first realised in 1983 in South Africa as a technique to irradiate the content of the orbit while minimizing the dose to the surrounding bone and eyelids. We report our experience since 2000 with this technique Methods Six nylon tubes containing 125 I seeds were implanted though the eyelids around the periphery of the orbit. Each contained a metal gutter that screened the outer part of the seeds from the bony orbit. A seventh unscreened tube was placed in the center and a golden plaque with 125 I seeds on its inner surface was secured beneath the eyelid. The reference dose around the target was 45 Gy for retinoblastoma and 60 Gy for uveal melanoma Results Since 2000 13 patients underwent BRT : 9 retinoblastomas (median follow up 36 months, 1 child died of craniospinal metastasis), 3 uveal melanomas (median follow up 55 months, 1 patient developed a metastatic disease), and 1 medulloepithelioma (median follow up 45 months). No patient developed orbital recurrence after BRT. Regarding cosmesis, results were much more acceptable than with other forms of irradiation with a normal size prothesis adaptation Conclusion 125 I orbital brachytherapy is an effective method of irradiating the orbit to prevent recurrent tumor after enucleation with better cosmesis results than with other forms of irradiation, especially in case of children Commercial interest

Simultaneous maxillomandibular distraction osteogenesis in severe progressive hemifacial atrophy with two distractors

Progressive hemifacial atrophy is a rare disorder characterized by an acquired, idiopathic, self-limited, unilateral facial atrophy involving skin, subcutaneous tissue, fat, muscle, and bone. Symmetry and contour restoration are the main treatment challenges. Among many techniques, microvascular reconstruction has been introduced as the gold standard to correct the atrophic deformity. For some patients with severe manifestations, soft tissue reconstruction alone does not obtain the desired outcome. In this series, we used an effective method to restore the severe progressive hemifacial atrophy by simultaneous maxillomandibular distraction osteogenesis with 2 distractors. The results demonstrate an improvement in both the profile and the occlusion plane of the patients with corresponding satisfactory esthetic and functional outcomes. We conclude that the simultaneous maxillomandibular distraction osteogenesis with 2 distractors is an effective method for hemifacial atrophy and bone frame reconstruction, especially ones involved in the discrepancy of the occlusal plane.

Contact Urticaria from an Oxygen Mask in a Military Pilot

Contact urticaria is a transient, wheal-and-flare response at the site of contact with a substance. We report a case of a military fighter pilot, with a background history of seborrheic dermatitis and steroid-induced facial skin atrophy, who subsequently developed contact urticaria from an oxygen facemask. Skin protection products such as barrier creams were used to try and minimize the provocation of contact urticaria from the oxygen mask whenever he flew, but without success. He was eventually permanently restricted from flying fighter aircraft.

Progressive facial hemiatrophy-With contralateral maxillary mucocele

We report an 8-year-old boy with a six month history of unilateral, progressive facial atrophy, heralded by the development of a morpheaform plaque on the left cheek. The clinical features and investigations were consistent with a diagnosis of progressive hemifacial atrophy with contralateral maxillary mucocele. This case highlights the substantial overlap of progressive facial hemiatrophy with morphea, masqueraded by contralateral maxillary mucocele.

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.

Clinical findings in MuSK‐antibody positive myasthenia gravis: A U.S. experience

We performed a retrospective chart review on 53 muscle-specific kinase antibody (MuSK-Ab)-positive myasthenia gravis (MG) patients at nine university-based centers in the U.S. Of these, 66% were Caucasian, 85% were women, and age of onset was 9-79 years. Twenty-seven patients were nonresponsive to anticholinesterase therapy. Myasthenia Gravis Foundation of America improvement status was achieved in 53% patients on corticosteroids, 51% with plasma exchange, and in 20% on intravenous immunoglobulin (IVIG). Thymectomy was beneficial in 7/18 patients at 3 years. Long-term (> or =3 years) outcome was very favorable in 58% of patients who achieved remission and/or minimal manifestation status. Overall, 73% improved. There was one MG-related death. This survey reinforces several cardinal features of MuSK-Ab-positive MG, including prominent bulbar involvement and anticholinesterase nonresponsiveness. Facial or tongue atrophy was rare. Most patients respond favorably to immunotherapy. The best clinical response was to corticosteroids and plasma exchange, and the poorest response was to IVIG. Long-term outcome is favorable in about 60% of cases.

Parry-Romberg Syndrome

Parry-Romberg syndrome (PRS) is an uncommon disorder and characterized by a slowly, an acquired progressive atrophy involving skin, soft tissue, cartilage, and bony structures. Accompanying atrophies of the other parts of the body are rarely reported. The aim of this study is to report a case that had contralateral lower extremity atrophy with PRS, and to review the related etiologic features, physiopathology, and mechanism. The patient who admitted for his facial atrophy also had atrophy of his contralateral extremity. This extremity was also short in length when compared with other extremity. To obtain detailed information regarding the severity of involvement routine laboratory investigations including antinuclear antibody (ANA), magnetic resonance imagine (MRI) of the craniofacial region and lower extremities, MR angiography of the lower extremities and brain, 3-dimension computed tomography (CT) scan of the craniofacial region were performed. Normal or negative laboratory findings included results of blood count, renal and hepatic function biochemical tests, rheumatoid factor, C-reactive protein, anti-dsDNA antibody except ANA that were positive. His lower extremity and brain MR angiography were normal. In the 3-dimensional cranial CT, there was no abnormality or defect in the bony structures. His brain MRI showed no pathologic changes, and his facial MRI demonstrated noteworthy atrophy to the sternocleidomastoid, masseter, pterygoid muscles, and subcutaneus soft tissues on the right side of the face. Additionally, MR investigation of his lower extremities revealed decreased volume in muscles and bony structures of the effected extremity compared with the other extremity, but pathologic evidence of fatty degeneration associated with muscle atrophy was not demonstrated. The patient had isolated contralateral lower extremity involvement combined with hemifacial atrophy (without affecting any other part of the body). Although more accepted theory is the sympathic nervous system dysfunction, autoimmunity may play a roll in the etiology of our case as ANA abnormality was found in multiple tests.

Facial reconstruction with vascularised serratus anterior muscle flap in patients with Parry–Romberg syndrome

Objective Parry–Romberg syndrome, popularly known as progressive hemifacial atrophy, is a rare and complicated craniofacial deformity characterized by idiopathic and progressive atrophy of patient's face with compromised facial aesthetics and functions. In the present study, we performed a retrospective review on the vascularised serratus anterior muscle flap transfer for the treatment of the hemifacial atrophy in 4 consecutive Chinese patients with Parry–Romberg syndrome. Methods The serratus anterior muscle flaps were harvested and transferred to correct the facial defects together with simultaneous nerve anastomosis between the long thoracic nerve and the ipsilateral cervical branch of facial nerve in the patients with Parry–Romberg syndrome. The postoperative results from patients’ own perception of improvement of facial profile and symmetry were also evaluated. Results In this patient series, both of the vessels and nerves of all flaps were anastomosed successfully in the recipient facial regions without any microvascular failures. Satisfactory facial contour and favorable symmetry were achieved with minimal donor-site morbidity, complications, recurrence and little resorption in surgical reconstruction of hemifacial atrophy during our follow-up period. Conclusions Our results suggested that vascularised serratus anterior muscle flap transfer combined with simultaneous nerve anastomosis is a reliable and effective treatment of choice that offers satisfactory results in the surgical correction of facial atrophy associated with Parry–Romberg syndrome.

Polymethylmethacrylate Facial Implant: A Successful Personal Experience in Brazil for More Than 9 Years

Synthetic permanent fillers for soft-tissue augmentation have acquired an important role in cosmetic improvement of patients with facial aging, facial atrophic scars, or lipoatrophy leading to facial defects. Polymethylmethacrylate (PMMA) is a filler introduced to the market as one option for long-lasting treatment. PMMA microspheres are purified, and the particles are larger than 20 microm. The product used as a filler has proven to be safe, effective, and long lasting. To determine the efficacy and safety of PMMA as a facial filler. Two hundred sixty-six patients (aged 17-72; 154 women) received injections of PMMA to correct facial defects. The number of sessions ranged from one to four, with an interval of 40 to 60 days between applications. Seventy-nine subjects (30%) had facial atrophy related to HIV infection, 159 (60%) had photoaging signs with deep wrinkles and atrophic areas, 25 (9%) had depressive scars from acne process, and three (1%) had residual depressions from lupus profundus. The results were satisfactory and long lasting. Side effects were transient. No late complications were observed. PMMA used for the treatment of atrophy, depressions, and facial wrinkles has been shown to be effective, long lasting, safe, and gratifying to patients and physicians.

Facial atrophy following accidental subcutaneous extrusion of sodium hypochlorite

Facial atrophy of the buccal fat pad following inadvertent subcutaneous extrusion of sodium hypochlorite is very rare. It is a complication which leaves the patient with a defect which is very difficult to treat. We present the case of a 46-year-old lady who presented with almost complete unilateral atrophy of the buccal fat pad. She has undergone multiple surgeries over the past two years. We highlight the precautions that must be taken to prevent such a complication and the difficulties in rehabilitating such a patient.

Sensoric and motor recovery of cranial nerves: a case report

A case of neurological complication after surgical procedure for a Meningeoma of the left cavernous sinus is presented. It concerns a healthy 27-year-old woman who developed left side facial muscle atrophy in combination with dysesthesia and hypalgesia in the left face. The diagnostic and therapeutical approach is described in detail. Adequate and functionally orientated diagnostic procedures are mandatory to establish an individual treatment plan. An interdisciplinary treatment, clearly focused on the recovery of the functions of the stomatognathic system, restores functions and improves local muscular and neuronal properties. In addition, the stricken psychic situation of the affected patient can be considerably improved by this approach. A route of capitulation should not be applied in such situations. An active and pre-visional treatment concept has to be implemented, although the actions taken have to be arranged on an individual base.

Hypertrophie du tissu adipeux après Lipostructure ® chez un sujet séropositif traité par antirétroviraux. Cas clinique et revue de littérature

HIV-related facial atrophy is a common complication due to antiretroviral treatment. The social handicap due to this lipodystrophy increase the demand of surgical correction. The usual treatment is the Lipostructure described by Coleman. The hypertrophy is one of the complications which can occur after a correction by Lipostructure. This complication is different from the overcorrection by the free time before his appearance. The treatment by liposuction is possible but there is a high risk of recurrence. The best treatment, after the literature review, seems to be the direct excision.

Progressive hemi facial atrophy - Parry Romberg syndrome presenting as severe facial pain in a young man: a case report

We present a 30-year-old South Indian man who presented with complaints of left sided headache and facial pain for past 3 months, severe for past 10 days. On physical examination, right side of the face appeared normal. Left side of the face showed signs of hemi atrophy with minimal drooping of left eyelid. All Systems were found to be normal. Routine blood and urine investigations results were within normal limits. X-ray chest revealed no abnormalities and x-ray skull showed both sides equal. Computerized tomogram of the brain showed left minimal sub dural hygroma with no midline shift, and no evidence of cerebral edema or cerebral atrophy. Nerve conduction study showed features suggestive of trigeminal neuralgia. MRI of the skull base was also normal and showed no evidence of trigeminal nerve compression. Interestingly, he had minimal response to analgesics, steroids, and propranolol, but showed immediate response to carbamazepine. Hence this patient indeed had Parry Romberg syndrome: Hemi facial atrophy with trigeminal neuralgia.

Use of Diced Bone Grafts Wrapped in Surgicel in the Augmentation of Maxillofacial Area

The correction of asymmetry in craniomaxillofacial region presents many problems for reconstructive surgeons. For this purpose, varieties of different materials and techniques have been used. We present our experience with diced bone graft wrapped in Surgicel applied to 5 patients with facial atrophy due to various causes between the years 2005 and 2008. The average follow-up period was 18 months (range, 9-25 months). All bone grafts were diced into small pieces with a maximum size of 2 to 3 mm using a costotome. The smaller bone grafts were prepared according to the technique described by Erol (Plast Reconstr Surg. 2000;105:2229-2241) for cartilage grafts. Results of the patients' satisfaction were well. Wrapping bone grafts in Surgicel holds them together. Thus, it becomes easier to adapt the diced bone grafts to the area to be augmented. Molding of diced bone grafts wrapped in Surgicel in accordance with the three-dimensional structure of the face with gentle movements after adaptation is an important advantage, which classical bone grafts do not have. In the later periods, collagen-enhancing effect of Surgicel not only prevents the bone grafts' being noticed, but also softens its consistency. This in turn facilitates adaptation of bone grafts to the facial area.

Techniques in Facial Fat Grafting

According to the author, autologous fat transplantation is an ideal technique to correct facial atrophy, providing a stable and potentially long lasting alternative to "off the shelf" fillers. Structural augmentation with fat involves multilayer blunt fat infiltration designed around concepts of volume expansion and resultant tissue shifting. The author has found that retention varies in different parts of the face and fat seems to "take" best in areas in which fat atrophy is the problem, such as in the cheek, periorbital area, and mandible. When fat is being used to replace bony remodeling or mucosal atrophy (both occur in the perioral area), retention can be unpredictable.

ADULTS WITH EOSINOPHILIC MYOSITIS AND CALPAIN-3 MUTATIONS

A recent report found that six patients who presented with eosinophilic myositis in childhood actually had a calpainopathy (LGMD 2A).1 I have encountered two patients previously diagnosed with adult-onset eosinophilic polymyositis who had mutations in the calpain-3 gene ( CAPN3 ). ### Case reports. #### Case 1. A 22-year-old woman presented with a 1-year history of progressive arm and leg weakness and elevated serum creatine kinase (CK) levels as high as 5,528 U/L. There was no family history of myopathy. She was initially evaluated elsewhere and a quadriceps muscle biopsy was interpreted as an inflammatory myopathy with prominent eosinophilic infiltrates. There was no peripheral eosinophilia. She was treated with prednisone, azathioprine, methotrexate, IV immunoglobulin, and rituximab without improvement. She was referred to me for management of her myositis. Manual muscle testing revealed the following Medical Research Council (MRC) grades: neck flexion 4−/5, neck extension 4/5, deltoids, biceps, and triceps 4/5, wrist extension 4+/5, wrist flexors and fingers 5/5, hip flexion/extension/abduction 2/5, knee flexion 3/5, knee extension 4+/5, ankle dorsiflexion and plantar flexion 4+/5. There was no facial weakness, muscle atrophy, or hypertrophy. Serum CK was 845 U/L. Review of her outside muscle biopsy slides demonstrated many necrotic and …

Facial Muscular Atrophy in a Myasthenia Gravis Patient

A 56-year-old man with anti-acetylcholine receptor antibody-mediated myasthenia gravis had bilateral facial muscular atrophy and had noticed blepharoptosis 15 years earlier. From 45 to 51 years of age, 5-10 mg prednisolone and 180 mg pyridostigmine daily relieved his symptoms. Subsequently, these treatments no longer improved the facial weakness, though blepharoptosis was absent. At 56 years of age, the edrophonium test and repetitive supramaximal stimulation testing of the orbicularis oris were negative. Frontalis muscle needle electromyography showed low amplitude polyphasic units and an incomplete interference pattern. Facial muscle atrophy, caused by disuse atrophy from neuromuscular junction depletion, contributed to this patient's facial weakness.

Hyaluronic acid treatment of facial fat atrophy in HIV‐positive patients

Facial lipoatrophy can be devastating for HIV-infected patients, with negative effects on self-esteem. In this study, we treated facial fat atrophy in the nasogenian area with hyaluronic acid (Restylane SubQ; Q-Med AB, Uppsala, Sweden). Twenty patients were included in the study. Treatment effects were evaluated at baseline, and at weeks 6, 24 and 52 using ultrasound, the Global Aesthetic Improvement Scale, the Visual Analogue Scale and the Rosenberg Self-Esteem Scale. Mean (+/-standard deviation) total cutaneous thickness increased from 6+/-1 mm at baseline to 15+/-3 mm at week 6 (P<0.001), and declined to 10+/-2 mm at week 52 (P<0.001 vs baseline). The response rate (total cutaneous thickness >10 mm) was 100% at week 6, 85% at week 24 and 60% at week 52. At week 6, all of the patients classified their facial appearance as very much improved or moderately improved. They also reported increased satisfaction with their facial appearance and had higher self-esteem scores. At week 52, 15 of 19 patients still classified their facial appearance as very much improved or moderately improved, although the mean total cutaneous thickness had gradually declined. Our results indicate that Restylane SubQ is a useful and well-tolerated dermal filler for treating HIV-positive patients with facial lipoatrophy.