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Reconstruction of the lower lip is complex. The Colmenero flap is an effective albeit rarely described method for the repair of medium- to large-sized defects of the lower lip.A retrospective review was carried out using data gathered from patients who had undergone Colmenero flap reconstruction of the lower lip at our centre between 2015 and 2020. We analysed demographic, histologic and anatomic variables as well as surgical results. This review assessed flap functionality based on proper mouth closure, absence of microstomia and oral competence.Thirteen Colmenero flaps were performed in 9 patients, with the flap being used bilaterally in four cases. All patients had squamous cell carcinoma of the lower lip. The mean length of the reconstructed defect was 4.1 cm (ranging between 3-7.5 cm). None of the flaps exhibited signs of necrosis. Five patients required minor surgical touch up during the second procedure: two for dehiscence, two for oral leakage and one for esthetic improvement. All patients had excellent functional and aesthetic final outcomes.The Colmenero flap is a good resource for medium- and large-sized lower lip reconstructions due to its reliability, limited complications, and good aesthetic and functional results.Ricostruzione del labbro inferiore mediante lembo di Colmenero dopo ampia chirurgia demolitiva: una opzione affidabile.La ricostruzione del labbro inferiore è complessa. Il lembo di Colmenero è un metodo efficace anche se raramente descritto per la riparazione di difetti medio-grandi del labbro inferiore.È stata effettuata una revisione retrospettiva utilizzando i dati raccolti da pazienti sottoposti a ricostruzione del labbro inferiore mediante lembo di Colmenero presso il nostro centro tra il 2015 e il 2020. Abbiamo analizzato le variabili demografiche, istologiche e anatomiche nonché i risultati chirurgici ottenuti. Questa revisione ha valutato la funzionalità del lembo in base alla corretta chiusura della bocca, all’assenza di microstomia e ripristino della competenza orale.Tredici lembi di Colmenero sono stati eseguiti in 9 pazienti, in quattro dei quali bilateramente. Tutti i pazienti erano affetti da carcinoma a cellule squamose del labbro inferiore. La lunghezza media del difetto ricostruito è risultata di 4,1 cm (compresa tra 3-7,5 cm). Nessuno dei lembi ha manifestato segni di necrosi. Cinque pazienti hanno richiesto piccoli ritocchi chirurgici mediante una seconda procedura: due per deiscenza, due per incontinenza orale e uno per miglioramento estetico. Tutti i pazienti hanno avuto ottimi risultati finali funzionali ed estetici.Il lembo Colmenero è una buona risorsa per ricostruzioni del labbro inferiore di medie e grandi dimensioni grazie alla sua affidabilità, alle modeste complicazioni e ai buoni risultati estetici e funzionali.

The perioral region is the most important area for facial expression, after the periocular structures. For the reconstructions of this region, an aesthetic outcome is as important as functional results in terms of the patient’s social life. The purpose of this study was to discuss the functional and aesthetic outcomes achieved with transversely advanced V–Y flaps in reconstructions of the perioral region. The study included 17 patients in whom transverse V–Y advancement flaps were used to repair full-thickness perioral defects resulting from malignant tumor resection. The patients were evaluated in terms of functional and aesthetic outcomes, complications, and need for revision surgery. To evaluate postoperative outcomes, a control group with no history of surgical interventions to the lips or perioral region was selected. Measurements of the lower and upper lips, mouth opening height, and mouth opening width were obtained from the controls and postoperatively from the patients. Lower lip reconstruction was performed in 13 patients (76.4%) and upper lip reconstruction was performed in 4 patients (23.5%). Total lower lip reconstruction was performed in 3 patients. Postoperative measurements were statistically equivalent to those of individuals with no surgical history. No cosmetic deformity formation or microstomia which require surgical treatment were observed in any of our patients. This technique is a simple, useful, functional method with short procedure time and satisfying aesthetic outcomes for patients with total or partial lower and upper lip defects. Level of evidence: Level IV, therapeutic study.

BACKGROUND Moebius syndrome (MBS) is a nonprogressive and rare congenital neuromuscular disorder involving the facial nerve and abductor nerve; it mainly manifests as facial paralysis and eye strabismus paralytic symptoms. Tissues in the oral cavity are also compromised, characterized by microstomia, micrognathia, tongue malformation, cleft lip, high arched palate or cleft palate, bifid uvula, and dental malocclusion. Therefore, dentistry plays a fundamental and crucial role in caring for these individuals. However, there is limited available data on MBS treatment, particularly regarding dental management. CASE SUMMARY This case report presents dental treatment of a 21-year-old man with MBS and discusses crucial interactions among oral complications of MBS. In this case, the patient was clinically characterized by congenital neuromuscular disorder, occlusal disorders, and tooth and gum problems. It is noteworthy that the patient presented early eruption of deciduous teeth 2 mo after birth, which has not been reported in other MBS cases and suggests a potentially new clinical manifestation of this syndrome. It is important to note that MBS cannot be cured, and oral manifestations of this syndrome can be managed by a multidisciplinary health care team that helps the patient maintain oral hygiene and dental health. After a series of oral treatments, no obvious poor oral hygiene, swollen gums, or abnormal imaging results were observed after 2 years of follow-up. CONCLUSION This case addressed the oral clinical manifestations of MBS and difficulties experienced during dental management, and suggested early tooth eruption as a potentially new clinical manifestation of this syndrome. Knowledge of the loop-mediated association among oral complications of this syndrome is essential to perfecting treatments.

Systemic sclerosis is a rare multisystem autoimmune disorder that significantly impacts the orofacial region. Several oral features including microstomia and increased tooth loss contribute to the mouth-related disability. Prosthetic rehabilitation is very challenging in these patients. As the spectrum of dental implants indications has been recently extended to patients with various systemic disorders, the aim of this systematic review was to evaluate the outcome of dental implants in patients with systemic sclerosis. A literature search was conducted in Medline/PubMed database to identify eligible case reports. Ten publications were included in qualitative synthesis. A total of 71 implants have been reported in 10 patients with systemic sclerosis with a mean of 7.1 ± 3.8 implants per patient. Preimplant surgeries have been described for 3 patients. Implant survival rates were higher than 98%, but the mean follow-up time was only 28.3 ± 18.6 months. Complications have been observed in 3 patients with 1 implant failure and peri-implant bone resorption in 2 patients. Although implant survival rates were high, an individualized assessment of risk-benefit balance is mandatory before indicating implant-based rehabilitation in patients suffering from systemic sclerosis and a scrupulous maintenance program has to be implemented. Further studies are strongly required to establish clinical guidelines.

Background Despite many options for upper lip reconstruction, each method's advantages and disadvantages are unclear. Objective To summarize complications and functional and aesthetic outcomes of localized skin flaps for oncological reconstruction of the upper cutaneous lip (PROSPERO CRD42020157244). Methods The search was conducted in Ovid MEDLINE, Ovid EMBASE, and CENTRAL on December 14, 2019. Two reviewers screened 2,958 results for eligibility. Bias assessment was conducted using ROBINS-I criteria. Results Our search identified 12 studies reporting outcomes of V-Y advancement, ergotrid, rotation, Karapandzic, alar crescent, and propeller facial artery perforator flaps. Flap complications (infection, hemorrhage/hematoma, wound dehiscence, and flap necrosis) ranged from 0% to 7.69%. Functional outcomes (salivary continence, microstomia, and paresthesia) were poorest for Karapandzic flaps. Aesthetic outcomes, when reported, stated satisfaction rates greater than 90%. V-Y advancement flaps reported the highest rates of poor scarring (0%-20%) and need for revision surgery (0%-46.7%). Conclusion Our results provide dermatologic surgeons an overview of upper cutaneous lip flap outcomes reported in the literature. In general, we noted high patient satisfaction rates and low complication rates. Additional research into outcomes of other commonly used flaps is needed. Standardization of reported outcomes could allow further comparison across different flaps or across studies of the same flap.

Agnathia/Otocephaly is a rare lethal syndrome characterised by mandibular hypoplasia/aplasia, ventromedial malposition or fusion of ears, microstomia and microglossia/aglossia. We present 26 cases of prenatally diagnosed agnathia/otocephaly, the largest published series so far, with a broad review of available literature and updated concepts on the etiopathogenesis. This is a retrospective case series identified between May 2000 and April 2018 in a tertiary fetal medicine centre in South India where we evaluated the ultrasound features of agnathia/ otocephaly and assessed the associated anomalies and syndromes. Out of 26 cases identified, 42% were isolated and 58% were associated with other anomalies. The most common associations were skeletal (7) followed by central nervous system anomalies (4) and genitourinary anomalies (2). Syndromes identified were Nager acrofacial dysostoses, Treacher Collins, Al Awadi Raas Rothschild and Femoral Facies syndrome. A simple method of observing the mandibular dot in-line with the maxilla in the sagittal view and the absence of the same would help to raise the suspicion of agnathia/severe micrognathia. The difference between these two and syndromic associations may not be brought out in ultrasound. Postnatal clinical assessment or pathologic diagnosis is mandatory as it helps in the counselling process and in the guidance of subsequent pregnancies.

Objectives This study aims to evaluate the effect of a home-based orofacial exercise program on the oral aperture (OA) of systemic sclerosis (SSc) patients. Patients and methods This single-blind prospective randomized controlled study performed between March 2017 and January 2019 included 56 SSc patients (3 males, 53 females; mean age 52.9±10.6 years; range, 31 to 70 years) with an OA of

Burns of the face and neck are devastating injuries with significant psychological and functional morbidity. A detailed understanding of the severity of head and neck burns is key to effective acute management of these injuries and prevention of long-term complications. This article focuses on acute management of burns of the face and skin both in the inpatient setting and managing the sequelae of facial burns including hypertrophic scarring, contractures, and functional issues such as microstomia and ectropion.

Systemic sclerosis is a connective tissue disease. Skin involvement of the mouth and hand may compromise function and quality of life. Autologous fat grafting has been described as a specific treatment of these clinical features. We report the results of our prospective study designed to treat and prevent skin complications in systemic sclerosis. We treated 25 patients with mouth and/or hand involvement (microstomia, xerostomia, skin sclerosis, Raynaud’s phenomenon and long-lasting digital ulcers) with autologous fat grafting, according to the Coleman’s technique, around the mouth and/or at the base of each finger. The surgical procedures were repeated in each patient every 6 months for a total of two or three times. Clinical data were collected before the first surgery and again 6 months after each surgical procedure. Pain, skin thickness, saliva production and disability were assessed with validated tests. Overall we performed 63 autologous fat grafting sessions (either on the mouth, on the hands or on both anatomical areas). Results at 6 moths after the last session included improvement of xerostomia evaluated with a sialogram, reduction of the skin tension around the mouth and, in the hands, reduction of the Raynaud phenomenon as well as skin thickness. Pain was reduced while the perception of disability improved. Digital ulcers healed completely in 8/9 patients. Our results confirm the efficacy and safety of autologous fat grafting for the treatment of skin complications and digital ulcers due to systemic sclerosis. In addition, the patients’ subjective well-being improved. Level of evidence V 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 .

Stevens-Johnson syndrome (SJS) is a rare disease in which extensive toxic epidermolysis occurs after medication. Skin and mucous membranes are involved in about 90% of SJS cases, and webbing of mouth corners (microstomia) may occur when they are affected. Few reports have been issued on microstomia in SJS, and no consensus has been reached regarding treatment methods, timings, or results. We encountered a case of microstomia following SJS after ofloxacin medication in a 22-year-old woman treated by commissuroplasty using a lozenge-shaped excision. We present an appropriate correction method and surgical timing for microstomia following SJS.

The management of laryngotracheal stenosis (LTS) in the pediatric burn patient is complex and requires a multidisciplinary approach. The mainstay of treatment for LTS is laryngotracheal reconstruction (LTR), however, limited reports of burn-specific LTR techniques exist. Here, we provide insight into the initial airway evaluation, surgical decision making, anesthetic challenges, and incision modifications based on our experience in treating patients with this pathology. The initial airway evaluation can be complicated by microstomia, trismus, and neck contractures—the authors recommend treatment of these complications prior to initial airway evaluation to optimize safety. The surgical decision making regarding pursuing single-stage LTR, double-stage LTR, and 1.5-stage LTR can be challenging—the authors recommend 1.5-stage LTR when possible due to the extra safety of rescue tracheostomy and the decreased risk of granuloma, which is especially important in pro-inflammatory burn physiology. Anesthetic challenges include obtaining intravenous access, securing the airway, and intravenous induction—the authors recommend peripherally inserted central catheter when appropriate, utilizing information from the initial airway evaluation to secure the airway, and avoidance of succinylcholine upon induction. Neck and chest incisions are often within the TBSA covered by the burn injury—the authors recommend modifying typical incisions to cover unaffected skin whenever possible in order to limit infection and prevent wound healing complications. Pediatric LTR in the burn patient is challenging, but can be safe when the surgeon is thoughtful in their decision making.

Systemic sclerosis (SSc) is a collagenosis with insufficiently known etiopathogenesis, characterized by microvasculopathy and excessive fibrosis in the context of an autoimmune disorder. The incompletely elucidated pathogenesis and limited therapeutic options, disabling aspects, skin lesions and pain determine important functional and psychological deficiencies which affect the quality of life. It is imperative to observe and correlate individual clinical and paraclinical data to optimize disease management. A group of 22 patients diagnosed with SSc, hospitalized in a university clinic in Bucharest was included in an observational study. The evolution of digital ulcers was evaluated as an indicator of vasculopathy and their status and dynamics were correlated with clinical elements reflecting the fibrotic aspect of the disease. The present study shows that the Raynaud phenomenon is almost always present during the course of the disease, but its presence is not always associated with digital ulcers. The existing data in the literature show that fibrosis is subsequent to vasculopathy, but this study did not reveal causality between these two aspects of pathogenesis. The presence of microstomia and digital contracture was identified in the presence of digital ulcers, but also in their absence. The etiopathogenic mechanisms with multiple unknown involved factors open the opportunity to investigate many aspects of SSc for optimal aiming of therapeutic interventions.

Reconstruction of defects measuring approximately two-thirds of the lower lip width is traditionally reconstructed utilizing loco-regional flap utilizing lip and cheek tissues. This often results in microstomia and unsatisfactory aesthetic outcome. This may hinder the psychosocial aspect of a recovering cancer survivor. Here we describe a single-stage reconstruction in a 79-year-old male patient who received lower lip resection for squamous cell carcinoma. The reconstruction was accomplished using a facial artery musculomucosal flap together with a free radial forearm flap for vermilion and soft tissue defect reconstruction after lip tumor resection with uneventful postoperative course. The patient remains disease-free after 2-year follow-up and does not require revision surgeries for functional or aesthetic reason. This approach may be considered a good option for reconstruction of missing lip and soft tissue simultaneously when the facial vessels are well-preserved during neck dissection. Aesthetically pleasing and functionally satisfactory outcomes may be produced.

Dr. Maurice Goldenhar, a renowned Swiss ophthalmologist classified the clinical features and named the malformation complex as Goldenhar Syndrome and described it as a congenital defect characterized by constellation of malformations classically involving the face, eyes and ears. Principal deformities of the Goldenhar syndrome are often combined with various malformations, like Cleft lip and/or palate, tongue cleft, unilateral tongue hypoplasia, and parotid gland aplasia, Rib anomalies, Congenital heart disease (ventricular septal defects). A 12-year-old female patient diagnosed with Goldenhar syndrome at secondary care Regional Hospital Bilaspur, Himachal Pradesh. She was diagnosed with a case of Goldenhar syndrome on clinic-radiological investigations. The patient reported to ophthalmology OPD of the hospital with the chief complaint of soft tissue structure in left eye with decreased vision. On examination limbal dermoid was observed. Patient was having pre-auricular tissue tag on both sides, hemifacial micrognathia and microstomia. Correlating the history and physical findings a provisional diagnosis of Goldenhar Syndrome was made with differential diagnosis of Franschetti syndrome or Treacher – Collins syndrome, Nager syndrome or acro-facial dysostosis and Townes-Brocks syndrome. Based on the clinical and radiographic findings a diagnosis of Goldenhar syndrome was made. Severe cases of GS can affect the routine and social life of the patient. Early detection can help avoid complications at a later stage of life. Such patients will have an increased risk for psychosocial difficulties. The study of this condition is still controversial because the symptoms and the physical features may vary greatly in range and severity from case to case.

Introduction Epidermolysis bullosa (EB) is a heterogeneous group of genetic diseases characterized by cutaneous and/or mucosal fragility. Blisters can occur spontaneously or because of minor friction on facial skin or the oral cavity. The repercussions of these dermatoses complicate the management of patients during surgery; for example, wisdom teeth removal might be complicated because of the limited mouth opening and mucosal lesions may be aggravated when the area of the wisdom teeth is being explored. Here, we describe the orofacial surgical management procedure for the extraction of the wisdom teeth of a patient with microstomia complicated by dystrophic epidermolysis bullosa (DEB) with high risk of mucosal lesion aggravation. Technique A 25-year-old woman with recessive DEB sought extraction of four wisdom teeth because of inclusion, recurrent pain, and dental caries. Anaesthesia was supported with conventional sequence induction and fibre-optic device-assisted nasotracheal intubation. A paediatric cuffed endotracheal tube and a fiberscope were lubricated before use in order to reduce friction against the naso-oropharyngeal mucosa. Hydrocellular foam dressing was applied on the face with soft adhesive silicone to avoid cutaneous wounds. No pressure was exerted on the patient's body during surgery. Surgical retractors were covered with hydrocellular foam dressing with silicone, and the labial commissures were protected with petroleum jelly. The dental extractions were performed with caution. Discussion Intraoral blistering in EB and scarring may result in microstomia and obliteration sulci, which can lead to difficulties in navigating the oral cavity. Consensus on a reasonable treatment protocol is lacking because no large cohort study exists. Lack of preparation of the facial skin and surgical instruments can lead to aggravation of lesions, especially when reaching for the third molars. A standardized procedure with general anaesthesia might pose fewer difficulties during the operation and limit facial and oral lesion aggravation. Prophylactic extraction of the third molar in younger patients with EB should be considered to avoid complications. Conclusion Atraumatic anaesthesia procedures and specific surgical care for these patients can be provided by using a protocol that protects the skin and mucous membranes from traumas.

Diffuse cutaneous systemic sclerosis may occur in women of childbearing age. Pregnancies in this population are associated with a markedly increased risk of adverse obstetric and maternal outcomes even before the onset of symptoms related to sclerosis. We report a case involving the management and outcome of pregnancy in a 30-year-old woman with diffuse cutaneous systemic sclerosis. The course of her pregnancy was good and was assisted by a group consultation including obstetricians and rheumatologists. Vaginal delivery was the patient’s preferred choice because she had irregular skin tightness in her lower abdominal skin. She underwent induction of labor and combined spinal-epidural analgesia, and successfully delivered. Importantly, these pregnancies need to be planned, where possible, to allow the opportunity to counsel women and their partners in advance and to decrease any risks. These pregnancies should be considered high risk, and they require close antenatal monitoring and good supervision from an expert multidisciplinary team experienced in high-risk pregnancies. The management of delivery for patients with cutaneous systemic sclerosis is challenging, and vaginal delivery with labor analgesia is an alternative option to cesarean section.

Routine custom trays are difficult to use to make impressions for patients with microstomia. Several types of split custom trays have been introduced to accommodate these situations. A method of fabricating mandibular and maxillary sectional special trays and impression technique for a patient with small oral opening is described. Sectional maxillary and mandibular custom trays can enhance its stabilization during border molding and final impression making. Thus it helps in fabricating a better fitting complete denture for such patients and also makes the impression procedure easy for patient with limited opening as the tray can easily be inserted in two pieces. Keywords: Impression, Microstomia, Sectional custom tray.

BACKGROUND Most patients with scleroderma suffer from microstomia, which can have debilitating consequences on their quality of life. Unfortunately, treatment options remain limited. No specific guidelines exist; hence, microstomia remains a challenge to treat in this patient population. OBJECTIVE This review aims to evaluate the different medical and surgical treatment modalities currently available for microstomia in patients with scleroderma and make recommendations for future research. MATERIALS AND METHODS A search of PubMed, Ovid MEDLINE, and Ovid Embase was conducted to identify articles discussing the treatment of microstomia in scleroderma. Twenty articles discussing surgical therapy and one article discussing medical therapy were reviewed. RESULTS Mostly because of a scarcity of high-level evidence, no individual therapy has documented long-term efficacy. Some treatments demonstrate positive results and warrant further research. CONCLUSION Given the variability of results, specific recommendations for the treatment of microstomia in patients with scleroderma are difficult to establish. A multifaceted approach that includes surgical and medical therapy is likely the best option to improve oral aperture in this patient population. Surgical treatments such as neurotoxins, autologous fat grafting, and ultraviolet A1 phototherapy may hold the most potential for improvement.

Purpose: The aim of this study was to develop an exercise protocol to improve maximal mouth opening (MMO), tongue protrusion (Tprot), tongue strength (Tstren), and lip strength (Lstren), and to assess its effects on subjects with scleroderma. Methods: We performed four replicated single-system studies in a consecutive sample of subjects with scleroderma. An instrumented assessment measured MMO, Tprot, Tstren, and Lstren. Each day, subjects were assessed and performed orofacial exercises conducted by speech therapists. Treatments were first aimed at improving mouth physical characteristics by impairment-oriented exercises and then to improve skills with function-oriented exercises. Results: The mean phase differences between assessment and treatment phases across subjects were from 0.88 to 9.56 mm in MMO, from 2.03 to 12.3 mm in Tprot, from –0.12 to 5.35 N in Tstren, and from –0.84 to 5.19 N in Lstren. After treatment, 3 subjects crossed the 5th percentile discriminating normal from abnormal performances for both Tstren and Tprot, while this occurred in 2 subjects for MMO and Lstren. Conclusions: The results of this study suggest that rehabilitation appears to be useful in reducing tongue and lip impairments and in improving oral functions in subjects with scleroderma.

Introduction: The aim of this study was to evaluate the associations between malnutrition and the clinical features of the disease and depression in patients with systemic sclerosis (SSc). Method: Patients with SSc who were followed up in our clinic were enrolled in the study. Malnutrition risk was assessed using the Malnutrition Universal Screening Tool (MUST). Skin involvement was assessed using the modified Rodnan skin score (mRSS) and interincisal distance (ID) measurements were used to assess the maximal mouth opening capacity. The Beck Depression Inventory (BDI) was used for measuring the severity of depression. Results: Ninety-eight patients with SSc (84.7% women; mean age 52.67 ± 11.26 years) were included in the study. According to the MUST scores, 61.2%, 15.3%, and 23.5% of patients had low, medium, and high risk for malnutrition, respectively. The mRSS was significantly higher in the group with high malnutrition risk compared with low-risk group (p = 0.014). Malnutrition risk was associated with interstitial lung disease and bowel involvement (p = 0.044 and p = 0.021, respectively). Interincisal distance was lower in the group with high malnutrition risk compared with the low-risk group (p = 0.003). Malnutrition risk was higher in patients who had mild-to-severe depressive symptoms than in those without (p = 0.012). Interincisal distance and bowel involvement were the most relevant factors for malnutrition. Conclusions: The risk of malnutrition is increased in patients with SSc. In our study, microstomia and bowel involvement were the most relevantly associated factors with malnutrition. Key Points: • The risk of malnutrition is increased in patients with systemic sclerosis (SSc). • Microstomia and bowel involvement are found to be the most important factors associated with malnutrition. • Depressive symptoms are seen frequently among patients with SSc, and depression seems to be one of the etiologic factors or the result of malnourishment in SSc. • Assessment of nutritional status and the presence of depression should be a part of routine clinical visits of patients with SSc. © 2019, International League of Associations for Rheumatology (ILAR).

Introduction: Systemic Sclerosis (SSc) is a multi-system connective tissue disorder of unknown aetiology characterised by thickening and stiffening of skin. SSc is clinically classified as limited cutaneous systemic sclerosis (LCSSc) and diffuse cutaneous systemic sclerosis (DCSSc). Objective: To compare the cutaneous and systemic features of LCSSc with those of DCSSc. Materials and Methods: A cross-sectional, descriptive study was conducted over a period of eight years in a tertiary care centre. All the patients of SSc were classified as LCSSc and DCSSc. Cutaneous features, systemic features and laboratory parameters were evaluated and compared between these two groups. Results: 54 cases of SSc were recruited for the study; of which 18 belonged to LCSSc and 36 to DCSSc. Female to male ratio was 5:1 in LCSSc and 11:1 in DCSSc. Mean age were 26.10±10.68 years and 34.72±15.95 years in LCSSc and DCSSc respectively. Statistically significant difference was observed in parameters like mean age, duration of Raynaud’s phenomenon, digital pitted scars, microstomia, diffuse hyperpigmentation, salt and pepper pigmentation, dyspnea on exertion, arthralgia, raised ESR and abnormality in spirometry. Conclusion: The duration of Raynaud’s phenomenon and digital pitted scars were significantly higher in LCSSc than DCSSc whereas microstomia, diffuse hyperpigmentation, salt and pepper pigmentation, dyspnea on exertion, arthragia, raised ESR and restrictive ventilatory defect were significantly higher in DCSSc than LCSSc. Pulmonary, renal and cardiac involvement were relatively less in our study population compared to other studies. Keywords: Limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, Raynaud’s phenomenon, icrostomia, digital pitted scars, salt and pepper pigmentation.

Clinical description Freeman-Burian syndrome (FBS) is a rare congenital myopathic craniofacial syndrome. Considerable variability in severity is seen, but diagnosis requires the following: microstomia, whistling-face appearance (pursed lips), H or V-shaped chin defect, and prominent nasolabial folds. Some patients do not have limb malformations, but essentially all do, typically camptodactyly with ulnar deviation of the hand and talipes equinovarus. Neuro-cognitive function is not impaired. Epidemiology Population prevalence of FBS is unknown. Aetiology Environmental and parental factors are not implicated in pathogenesis. Allelic variations in embryonic myosin heavy chain gene are associated with FBS. White fibrous tissue within histologically normal muscle fibres and complete replacement of muscle by fibrous tissue, which behaves like tendinous tissue, are observed. Management Optimal care seems best achieved through a combination of early craniofacial reconstructive surgery and intensive physiotherapy for most other problems. Much of the therapeutic focus is on the areas of fibrous tissue replacement, which are either operatively released or gradually stretched with physiotherapy to reduce contractures. Operative procedures and techniques that do not account for the unique problems of the muscle and fibrous tissue replacement have poor clinical and functional outcomes. Important implications exist to facilitate patients’ legitimate opportunity to meaningfully overcome functional limitations and become well.

Made available in DSpace on 2021-06-25T10:58:42Z (GMT). No. of bitstreams: 0 Previous issue date: 2021-01-01 Scleroderma is a rare autoimmune disease characterized by excessive collagen production. The oral manifestations of the patient with scleroderma can include microstomia, xerostomia, and changes in the resorption teeth. We report the case of a 7-year-old female patient diagnosed with systemic scleroderma where photobiomodulation therapy was used to treat xerostomia associated with hyposalivation. She attended a pediatric clinic and presented with dry and rigid facial skin, trismus, xerostomia, malocclusion, and difficulty swallowing. Stimulated salivary flow was assessed before, during, and after treatment. Photobiomodulation therapy was conducted at four points at the sublingual glands with 660 nm, 100 mW, and 0.8 J/cm2 to each point; eight points at the parotid glands; and six points at the submandibular glands with 808 nm, 100 mW, and 0.8 J/cm2 for 8 seconds at each point. After this therapy, an increase in salivary flow, remission of the xerostomia, and an improvement in mastication and swallowing were observed. Photobiomodulation therapy was effective in controlling xerostomia in this pediatric patient, resulting in increased salivary flow and an improvement in her quality of life. Universidade Estadual de São Paulo (Unesp) Faculdade de Odontologia de Araraquara Departamento de Morfologia e Clínica Infantil Universidade Estadual de São Paulo (Unesp) Faculdade de Odontologia de Araraquara Departamento de Diagnóstico e Cirurgia Universidade Estadual de São Paulo (Unesp) Faculdade de Odontologia de Araraquara Departamento de Morfologia e Clínica Infantil Universidade Estadual de São Paulo (Unesp) Faculdade de Odontologia de Araraquara Departamento de Diagnóstico e Cirurgia

Restricted mouth opening (RMO) in patients with anatomical anomalies is a major challenge in dental and oral surgery. There are many causes of restricted mouth opening. The origin of the anatomic anomaly may be acquired, such as in burn victims and in patients with disorders of the temporomandibular joint, or developmental, such as in Marfan syndrome.

Systemic sclerosis is a complex autoimmune connective tissue disease of unknown cause that causes sclerosis and inflammation of the skin and subcutaneous tissue. Sclerosis involving the face can lead to microstomia, leading to difficulties with mastication, phonation, and oral hygiene. Although many agents have been used to treat internal organ involvement, they often have compromised efficacy on cutaneous manifestation including facial dermal sclerosis. In this case presentation, we will introduce a surgical technique to correct scleroderma-induced microstomia.

7q11.23 Microduplication (dup7q11.23) syndrome is a rare autosomal dominant disorder due to a recurring 1.5 to 1.8 Mb duplication of the Williams&ndash, Beuren Syndrome critical region. Dup7q11.23 has been associated with several neuro-behavioral characteristics such as low cognitive and adaptive functioning, expressive language impairment, anxiety problems and autistic features. In the present study, we analyze the clinical features of ten individuals in which array-CGH detected dup7q11.23, spanning from 1.4 to 2.1 Mb. The clinical characteristics associated with dup7q11.23 are discussed with respect to its reciprocal deletion. Consistent with previous studies, we confirm that individuals with dup7q11.23 syndrome do not have a homogeneous clinical profile, although some recurring dysmorphic features were found, including macrocephaly, prominent forehead, elongated palpebral fissures, thin lip vermilion and microstomia. Minor congenital malformations include patent ductus arteriosus, cryptorchidism and pes planus. A common finding is hypotonia and joint laxity, resulting in mild motor delay. Neuropsychological and psychodiagnostic assessment confirm that mild cognitive impairment, expressive language deficits and anxiety are recurring neurobehavioral features. New insights into adaptive, psychopathological and neurodevelopmental profiles are discussed.

A 37-year-old woman with Raynaud’s phenomenon in all fingers and toes for 7 years presented with painful redness of both eyes for 2 weeks. On examination, she had digital ulcers ([figure 1][1]), skin tightening with microstomia (modified Rodnan’s skin score was 15/51), bilateral vision was 6/6

Highlights • Oro-facial fibrosis presents a significant disease burden in systemic sclerosis. • There is currently no treatment available for oro-facial fibrosis. • Autologous fat grafting is a novel therapeutic method for oro-facial fibrosis., Introduction Oro-facial fibrosis is a common and disabling manifestation of systemic sclerosis (SSc), causing a plethora of functional, aesthetic and social compromise, yet is without effective treatment. Autologous lipotransfer is an established minimally invasive surgical procedure that is postulated to exert anti-fibrotic effects by adipose-derived stem cells, and presents a novel method in the treatment of fibrotic conditions. This study aims to assess the safety and efficacy of autologous lipotransfer for facial involvement in SSc. Methods and analysis This is the first randomised controlled study with an open label design to assess autologous lipotransfer for oro-facial involvement in systemic sclerosis. The goals of this study are to assess the feasibility of using a range of quantitative and qualitative outcome measures to effectively measure disease severity and treatment outcome, and to assess patient acceptability for future multi-centre trials. A total of 50 participants will be randomised to a treatment or control group. The treatment group will receive autologous fat transfer to the peri-oral region by a single surgeon. Dermal fibroblasts and adipose-derived stem cells will be isolated from tissue samples. All outcome measures will be taken at baseline, then at 6 weeks, 3 months and 6 months from the time of intervention in the treatment arm, or from baseline in the control arm. Ethics and dissemination The study has ethical approval (REC reference 19/LO/0718). Results will be available to patients, patient user groups, clinicians and the public through presentations at national and international rheumatology conferences and published in peer reviewed journals. Trial registration Registered on ISRCTN registry (ISRCTN17793055).

Background: Peri-oral and oral soft tissue defects can be caused by several conditions, as resection of malignant and benign tumors, congenital malformations, traumatic injury. They can cause alterations in function and appearance of the affected area. Primary closure is the most commonly used technique for small defects, whilst medium and large defects require reconstruction with local, regional or free flaps. Local flaps are a good choice for reconstruction of medium-size defects of the lip, because they provide the same type of tissue, they are situated in proximity to the resected area, and they can be performed also in patients with multiple co-morbidities.Aim: To evaluate the role of lip and perioral reconstruction in plastic surgery unit of General Surgery Department, Zagazig University Hospitals.Methods: This study was carried out in plastic surgery department, Zagazig University Hospitals, from August 2018 to January 2019. It included 18 patients with perioral defects undergoing surgical tumor ablation. These patients were operated upon and referred to our outpatient clinic for the follow-up visits.Results: Out of 18 cases included, Karapandzic flap was used in 6 cases while mucosal advancement, rhomboidal flap were used in 4 cases. Hypothesia in 27.8%. Sialorrhea was in 16.7%. Microstomia was in 33%, partially satisfied in 50% and satisfied 50% after 6 months of the study.Conclusion: Local flaps provide the most satisfactory results and should be considered as the first choice for reconstruction of perioral defects.Keywords: Perioral reconstruction, local flaps, perioral defects.

Mild- to moderate-sized lip defects that affect one- to two-thirds of the total lip length often require that utilization of local soft tissue rearrangement in order to re-establish lip anatomy, function and esthetics. The use of local flaps in lip reconstruction often follows oncologic resection of common skin malignancies including basal cell carcinoma and squamous cell carcinoma. When considering the types of flaps to use for lip defects of one- to two-third total length, a variety of flaps designs have been described, each with its benefits in drawbacks. In this article, we reintroduce the use and value of the bilateral mucomyocutaneous ‘bandoneon’ flaps for lower lip reconstruction in two of our patients. When compared with other flap designs, it can be used as a single-stage procedure with a very good esthetic outcome, minimal to no microstomia and preservation of lip competence and sensory function.

Children suffering from conductive or mixed hearing loss may benefit from a bone-anchored hearing aid system (BAHA Attract implantable prosthesis). After audiological rehabilitation, different aspects of development are improving. The objective of this case report is to propose a comprehensive framework for monitoring cortical auditory function after implantation of a bone-anchored hearing aid system by using electrophysiological and neuropsychological measurements. We present the case of a seven-year-old boy with a congenital hearing loss due to a plurimalformative syndrome, including outer and middle ear malformation. After the diagnosis of hearing loss and the audiological rehabilitation with a BAHA Attract implantable prosthesis, the cortical auditory evoked potentials were recorded. We performed a neuropsychological evaluation using the Wechsler Intelligence Scale for Children – Fourth Edition, which was applied according to a standard procedure. The P1 latency was delayed according to the age (an objective biomarker for quantifying cortical auditory function). The neuropsychological evaluation revealed that the child’s working memory and verbal reasoning abilities were in the borderline range comparing with his nonverbal reasoning abilities and processing abilities, which were in the average and below-average range, respectively. Cortical auditory evoked potentials, along with neuropsychological evaluation, could be an essential tool for monitoring cortical auditory function in children with hearing loss after a bone-anchored hearing aid implantation.

Thermal disease presents a major burden to individual patient morbidity, healthcare cost as well as to over all economy. Burns also also represent a significant per-patient utlilisation of finite healthcare resources. Secondary complications in these patients, such as multiple drug resistant organisms, may have a devastating effect. Laser surgery has recently come of age as an optimal tool in the secondary reconstruction of burn injury, that is able to simultaneously address significant sheet scar tightness, hypertrophic, atrophic, and keloid complications, pruritus, microstomia, ectropion, skin graft honeycombing, and improve range of movement whilst reducing the risk of infection to1%. Yet, cutaneous laser surgery is often underutilised due to the perceived concerns about the sustainability of a new service with relatively high startup cost. We present a dual methodology to explore this concern: an evidence-based background review of the last 5 years of current best evidence, and a 22-year cost-analysis comparison at an established, high volume UK Centre of reconstructive surgery. We report that fiscal viability for laser surgery services for secondary burn reconstruction is supported by: level 2 (one systematic review) level 4 evidence (2 studies) and level 5 evidence (expert reports). Evidence over 22 years from an established super-regional NHS laser centre shows that introduction of this service led to sustained and substantial cost saving, producing excellent surgical results at a fraction of the cost of traditional surgery. Analysis of the potential dollar-effect of these advantages to the general population supports state investment in expertise and capital equipment as a medium to long-term cost saving strategy, which may also aid re-integrating patients into the workforce making a meaningful contribution to the economy.

Background Systemic sclerosis is a systemic connective tissue disease characterized by endothelium damage, fibrosis, and subsequent atrophy of the skin. Perioral fibrosis produces a characteristic microstomia together with microcheilia, both of which cause severe difficulties and affects patients’ daily life, such as eating and oral hygiene. Since there are no effective and specific therapies, we have aimed at evaluating the response to filler injections of hyaluronic acid together with platelet-rich plasma. Methods Ten female patients aged between 18 and 70 were included in this study. Each patient was treated with three filler injections of hyaluronic acid and platelet-rich plasma at an interval of 15 to 20 days. Follow-up check-ups were recorded 1, 3, and 24 months after the end of the treatment. During the therapy and the subsequent follow-up, we evaluated the mouth’s opening, freedom of movement of the lips, and skin elasticity. Results After the treatment, patients had achieved good results already after the first injection and the improvement was maintained in the following months, up to 2 years. In particular, 8 (80%) patients showed a greater mouth’s opening and increased upper lip’s thickness during 1-month follow-up and maintained these results after 2 years (maximum mouth’s opening T0 47.61; T3 49.23; T4 48.60 p p p p p Conclusions Our study demonstrates that filler injections of hyaluronic acid and platelet-rich plasma represent an efficient local therapeutic alternative for patients affected by scleroderma. The treatment has significantly improved patients’ quality of living.

Objectives The aim of this study was to investigate the role of the extended Abbe flap in the reconstruction of complex midfacial defects (upper lip, nasal base and medial cheek). We describe our personal experience with this method and present some interesting aspects from the relevant literature. Materials and methods Between 2003 and 2017 we retrospectively evaluated the records of all patients with defects of the upper lip and nasal base after the resection of malignant tumors or traumata and reconstruction by means of the extended Abbe flap. We looked for information on the origin of the defect, the functional result, the aesthetic outcome and postoperative complications. The functional result of the reconstruction of the oral commissure was assessed with regard to postoperative subjective speech integrity, preservation of competence of the oral sphincter and lip sensation. The aesthetic outcome was evaluated with regard to limitations in facial expression and scar formation. Results The study included 8 patients. Follow–up in the oncological cases was 85 months (range: 20–150 months). No tumor recurrences were detected. All patients achieved normal oral competence and mouth opening. No cases of blunting or microstomia were detected. Conclusion Reconstruction of the upper lip and nasal base with the extended Abbe flap is associated with an outstanding functional outcome that is cosmetically very acceptable.

Purpose Virtual surgical planning (VSP) is an indispensable aid in craniomaxillofacial reconstruction, yet no protocol is established in facial gunshot wounds. We review our experience with computer-aided reconstruction of self-inflicted facial gunshot wounds (SIGSW'S) and propose a protocol for the staged repair. Methods A retrospective case series enrolling patients with SIGSW's managed with the Functional Anatomic Computer Engineered Surgical protocol (FACES) was implemented. Subjects were evaluated at least one month postoperatively. Outcome variables were jaw position, facial projection, oro-nasal communication, lip competence, feeding tube and tracheostomy dependence, descriptive statistics were computed. The FACES protocol implemented during the initial hospitalization is as follows 1) damage control; 2) selective debridement; 3) VSP reconstruction back converted into navigation software 4) navigation assisted midfacial skeletal reconstruction; 5) computer aided oro-mandibular reconstruction with or without microvascular free flaps using custom cutting guides/hardware; 6) navigation assisted, computer aided palatomaxillary reconstruction with or without microvascular free flaps using cutting guides/hardware; 7) navigation assisted reconstruction of the internal orbit; 8) and confirmation of accurate reconstruction using intraoperative CT. Results The sample was composed of 10 patients, mean age of 43 years (range, 28 - 62 years, 70% M), 100% with SIGSW's to the submental/submandibular region. All had satisfactory facial projection (n=10), nine had satisfactory jaw position, were decannulated by one month's follow up and were feeding tube independent (90%). All traumatic oro-antral communications were closed (n=8, 7 surgical, 1 obturator), seven had adequate lip competence (70%). Complications included fibula malunion (n=1), plate exposure (n=2) infection (n=2), intracranial abscess (n=1) and microstomia (n=2). Conclusion Computer-aided surgery is an indispensable tool in the reconstruction of SIGSW's. Successfully implemented, it proved to be a useful adjunct for: the restoration of orbital volume, facial projection and symmetry; the inset of composite tissue, and the facilitation of dental implant supported prosthetic rehabilitation.

Background The repair of extensive lower lip defects is difficult due to the presence of both functional and esthetic requirements. This report describes functional lip repair using the partial latissimus dorsi free flap without nerve coaptation. Methods Reconstruction of the lower lip due to subtotal and total tissue defects was performed using latissimus dorsi free flap on twelve patients, between 2013 and 2017. The etiology was squamous cell carcinoma in six patients, malignant melanoma in one, firearm injury in three and microstomia in two. Following exposure of the lip defect, the partial latissumus dorsi muscle flap was harvested and transfered into the lower lip defect. The microvascular anastomoses was done and no nerve coaptation was performed. The muscle was covered with a skin graft taken from the thigh for mucosal and skin lining. Functional outcomes were assessed using physical examination, electromyography (EMG), electroneuromyography, cold/warm and pain sensation, two point discrimination (TPD), and Semmes Weinstein (SMW) tests. Results Postoperative course was uneventful for all of the flaps. Patients were followed for between fifteen months and four years (mean 28.2 months).Color match between the flap and the face and functional outcome was satisfactory. Reinnervation was detected at EMG in eleven patients undergoing surgery six months postoperatively.After 1 year, the patients demonstrated quite normal results of the test with SMW and TDP results. None of patients perceived both hot and cold sensation. Conclusion The technique is an alternative option in lower lip reconstruction since it provides satisfactory functional and esthetic results despite absence of any neural coaptation.

Reduced oral aperture(Microstomia) and mandibular hypo mobility/trismus are relatively common conditions that can be encountered in patients attending general dental practice, community dental practice and district general or dental teaching hospitals. All dental specialties may see patients with these conditions, and regardless of which environment or specialty, both patient and clinician may experience significant problems. The purpose of this case study is to review the development of problems encountered for patients and clinicians, and to identify options to treat or manage the conditions. Keywords:Mandibular hypomobility, Microstomia, Non Odontogenic, Odontogenic,Trismus.

Moebius/Mӧbius Syndrome (MS) is an extremely rare congenital disorder characterized by uni- or bilateral palsy of the abducens (VI) and the facial (VII) nerves, causing facial paralysis. Dysfunction of cranial nerves III through XII is common, most often the glossopharyngeal (IX) and hypoglossus (XII). Afflicted individuals seeking prosthodontic care, particularly removable prosthetics, present multiple challenges related to inherent facial and tongue muscle weakness and microstomia. Other extraoral anomalies may include congenital multiple arthrogryposis and malformations of the upper and lower limbs. This clinical report describes the rehabilitation of a patient with Moebius Syndrome using a maxillary 2-implant retained overdenture and mandibular partial removable denture prosthesis.

Background:A sound experience has been accumulated up to date with the use of rituximab (RTХ) for treatment of systemic sclerosis (SSc). Some studies reported improvement of skin fibrosis following treatment with RTХ, but long-term follow-ups are really few.Objectives:to evaluate the effect of RTХ on the manifestations of skin fibrosis in patients (pts) with SSc in the long-term follow-up.Methods:This prospective study included 71 pts aged 46 years (17-66) on average, 59 (83%) pts were females, mean disease duration was 5,6±4,4 years, and mean follow-up - 42 months (12-72) (mo). Diffuse SSc was established in 42 (59%) pts. All pts received glucocorticoids in low doses. 45% of pts were receiving immunosuppressants at study entry. The following parameters were evaluated: Rodnan skin score (mRSS), interdigital space(IDS) (the distance between the tips of 1 and 5 fingers at maximum extension), oral aperture (OAp) and activity index (EScSG-AI) over the periods: 12-18 mo, 24-30 mo, 36-42 mo, 48-54 mo and 60-72 mo after initiation of RTX therapy. The results are presented as: mean values, delta (Δ), median, upper and lower quartiles.Results:RTX therapy resulted in significant decrease of disease activity index, which statistically significantly correlated with decrease of mRSS - the main indicator of the severity of skin fibrosis (r=0,39;p=0,001). Changes in parameters by follow-up periods are presented in the Table 1.Table 1.Changes in clinical and instrumental parameters at RTX treatment (delta; median; lower quartile; upper quartile).Parameters12-18 mo (n=71)24-30 mo (n=55)36-42 mo (n=36)48-54 mo (n=24)60-72 mo (n=17)ΔmRSS3,32 [3.3; 0; 8]5,4 [3; 0; 10]5,1 [3,5; 0; 9]5,3 [3; 0; 10]7,3 [5; 1; 14]p=0,001p=0,001p=0,001p=0,001p=0,001ΔOAp, cm0,24 [0,1; 0; 0,5]0,26 [0,1; 0; 0,6]0,39 [0,2; 0; 0,8]0,31 [0.3; 0; 0,7]0,36 [0,2; 0; 0,8]p=0,0009p=0,0006p=0,004p=0,006p=0,009ΔActivity index (EScSG-AI)1,49 [1,5; 0; 2,5]1,64 [1,5; 0; 2,5]1,11 [1; 0; 2]2 [2; 1; 3]2,17 [2; 1,5; 2]p=0,001p=0,001p=0,0001p=0,0001p=0,0001Cumulative dose of RTX, g1,43±0,62,97±0,83,45±1,33,96±1,15,15±1,7Decreasing of mRSS statistically significantly correlated with increasing cumulative dose of RTX (r=0,29;p=0,01). Decreasing disease activity index correlated with increasing cumulative dose of RTX (r=-0,37; p=0,01). IDS improvement was documented at all assessment time periods, although statistically insignificant.Conclusion:The results of this study confirm reported positive effect of RTX on the reduction of skin fibrosis in SSc. Long-term follow-up demonstrated steadily decreasing skin fibrosis and improvement of microstomia with increasing oral aperture in parallel with a decrease of the disease activity index and increasing cumulative dose of RTX.Disclosure of Interests:None declared

Although it is a rare developmental malformation, van der Woude syndrome is the most common form of syndromic orofacial clefting, accounting for approximately 2% of all cleft cases. The lower lip pits with or without a cleft lip or palate is characteristic of the syndrome. Findings, such as hypodontia, limb deformities, popliteal webs, ankylogossia, ankyloblepheron, and genitourinary and cardiovascular abnormalities, are rarely associated with the syndrome. This paper reports a rare case of van der Woude syndrome in a 10-year-old male patient with a single median lower lip pit and a repaired bilateral cleft lip and cleft palate that were associated with microstomia, hypodontia, and clubbing of the left foot with syndactyly of the second to fifth lesser toes of the same foot.

Background: Systemic sclerosis (SSc) is an autoimmune disease with multisystem involvement, dominated by a general fibrosis. The early stage of the disease is associated with progressive damage to microcirculation, particularly in the respiratory tract, the gastrointestinal tract and skin. The face assumes a typical appearance characterized by microstomia (reduction of mouth opening) and microcheilia (thinning of the lips). These conditions cause a considerable reduction in performance status of patients. We treated them by fat grafting, rich in adipose stem cells, and we evaluated through time clinical, functional and aesthetic evaluation of oral pathology associated with SSc. Materials and Method: From September 2014 to May 2016, we enrolled and treated seven patients in the plastic, reconstructive and aesthetic surgery clinic. Through time, we evaluated the following parameters: evaluation of mouth opening (maximum opening in superior–inferior and lateral directions) and lip thicknesses, both measured by doctors of the aforementioned operating unit; variation in the quality of life as perceived by patients according to the MHISS scale (Mouth Handicap Systemic Sclerosis); variation in severity of labial fibrosis assessed by microscopic analysis of pre- and post-fat transfer samples in the pathology clinic; safety of the protocol, according to the management of side effects resulting from the procedure; aesthetic evaluation, made by external observers and non-experts in the field, on pre- and post-operative photographs. Results and Conclusions: We reported satisfying results, both functionally and aesthetically, for all parameters except one, for which the sample size might have proven critical. These data should be a starting point for further experimental research and clinical trials. Level of Evidence IV: 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. Background: Systemic sclerosis (SSc) is an autoimmune disease with multisystem involvement, dominated by a general fibrosis. The early stage of the disease is associated with progressive damage to microcirculation, particularly in the respiratory tract, the gastrointestinal tract and skin. The face assumes a typical appearance characterized by microstomia (reduction of mouth opening) and microcheilia (thinning of the lips). These conditions cause a considerable reduction in performance status of patients. We treated them by fat grafting, rich in adipose stem cells, and we evaluated through time clinical, functional and aesthetic evaluation of oral pathology associated with SSc. Materials and Method: From September 2014 to May 2016, we enrolled and treated seven patients in the plastic, reconstructive and aesthetic surgery clinic. Through time, we evaluated the following parameters: evaluation of mouth opening (maximum opening in superior–inferior and lateral directions) and lip thicknesses, both measured by doctors of the aforementioned operating unit; variation in the quality of life as perceived by patients according to the MHISS scale (Mouth Handicap Systemic Sclerosis); variation in severity of labial fibrosis assessed by microscopic analysis of pre- and post-fat transfer samples in the pathology clinic; safety of the protocol, according to the management of side effects resulting from the procedure; aesthetic evaluation, made by external observers and non-experts in the field, on pre- and post-operative photographs. Results and Conclusions: We reported satisfying results, both functionally and aesthetically, for all parameters except one, for which the sample size might have proven critical. These data should be a starting point for further experimental research and clinical trials. Level of Evidence IV: 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.

Objective: To compare a physical therapy program to usual care on disability of systemic sclerosis (SSc) patients. Methods: A 12-month follow-up, parallel-group randomized controlled trial involving a modified Zelen design was conducted in 4 tertiary-care hospitals. Patients were enrolled if they had a disability rating≥0.5 on the Health Assessment Questionnaire Disability Index (HAQ-DI) or complaints of decreased mouth opening or limited range of motion of at least one joint. The experimental intervention (n=112, 110 analyzed) was a 1-month personalized supervised physical therapy program provided by trained care providers followed by home sessions. The comparator (n=108, 108 analyzed) was usual care that could include ambulatory physical therapy. The primary outcome was the HAQ-DI score. Results: There was no statistically significant difference in disability at 12 months (HAQ-DI between-group difference, -0.01; 95% CI -0.15 to 0.13; p=0.86). Disability was reduced at 1 month for patients in the physical therapy group (HAQ-DI between-group difference -0.14; 95% confidence interval [CI] -0.24 to -0.03; p=.01), at 6 months the HAQDI scores between-group difference was -0.12; 95% CI, -0.23 to 0.01; p = 0.054. There was a statistically significant difference for hand mobility and function, and pain, at 1 month. Microstomia was lower in the physical therapy group at 1, 6 and 12 months (between-group difference at 12 months, 1.62; 95% CI 0.32 to 2.93; p=0.01). No differences in adverse effects were observed. Conclusion: A personalized physical therapy program did not reduce disability at 12 months but had short-term benefits for patients with SSc. This article is protected by copyright. All rights reserved.

The prevention of scar contracture after surgery is an important aspect of lateral facial cleft repair. Maintaining adequate mouth opening is essential for speech, expression, chewing, oral hygiene and psychosocial well-being. Although there have been extensive reviews of non-surgical microstomia management in patients with oral electrical burns, there have been no reports on the use of oral commissure splints to manage the surgical scar in patients with lateral facial clefts. The case study presented here will demonstrate the clinical use of cheek retractors to provide physical resistance to scar contracture. We recommend early rehabilitation with cheek retractors to minimize scar contracture and the negative sequelae of scarred tissues on the dentition after lateral facial cleft repair.

Introduction Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis and microvascular damage. Facial signs are frequent and include perioral skin thickening, facial atrophy and microstomia. Facial handicap is overlooked by physicians but when patients are questioned, they frequently declare discomfort and distress due to their facial disability and appearance. As ideal autologous filler, fat graft has previously been considered in the treatment of facial handicap in SSc. To enhance fat graft survival, we combined fat graft and autologous platelet-rich plasma. Cases Description Two patients suffering from SSc with facial disability, facial skin thickening and aesthetic discomfort were treated with combined subcutaneous microinjection of autologous fat and platelet-rich plasma. Conclusions Regular follow-up until 12 months showed that facial disability decreased, skin texture appeared softer, and mouth opening increased. Patients declared to be very satisfied from a functional and aesthetic point of view until their last follow-up at 12 months. Fat grafting is beneficial in the improvement of facial symptoms of patients with SSc, and the addition of platelet-rich plasma is a feasible, simple and safe method. Further studies are required to better assess the advantage on fat graft retention, trophicity and the lasting effect offered by this combined therapy.

The Abbe-Estlander flap surgery is a cross-lip procedure that is valuable in repairing a defect on the lower lip using a full-thickness flap, consisting of the skin, muscle and mucosa, from the upper lip. As usefulness and practicality of the flap in reconstruction of lower lip surgical defects in Asian ethnicity have not been documented, the authors present a case of successful lower lip reconstruction with a staged, Abbe-Estlander lip switching flap with commissuroplasty as an illustrative example. A 71-year-old male has presented with an ulcerating lip nodule in the middle one third of the lower lip, measuring about 1.5×2 cm across its long and short axes. Wide excision of the tumor was followed by delineation of the triangular Abbe-Estlander flap from the upper lip, in which the medial hinge point of the base was chosen as the pedicle. Then, the flap elevation was carried out from the lateral commissure and then was transferred into the lower lip defect. Three weeks later, commissuroplasty was performed to correct the rounding at the new commissure. The patient is currently performing his daily activities with no apparent compromise in orbicularis oris strength or oral continence. Given the size of the primary defect and the flap-to-defect ratio of size, the degree of microstomia was acceptable. Even with other myriad of reconstructive options at surgeons' disposal, the Abbe-Estlander lip-switching flap is a reliable, and less morbid method of lower lip reconstruction for Asian surgical candidates. The authors illustrate an exemplary case in which a relatively large lower lip defect was successfully repaired using an upper lip flap of a significantly smaller size in an Asian subject of advanced age, without any remarkable long term sequelae which have traditionally been associated with the trans-oral lip switching flap technique.

Arthrogryposis multiplex congenita (AMC) is a rare disorder characterized by non-progressive, multiple contractures. In addition to affected extremities, patients may also present microstomia, decreased temporomandibular joint mobility. Although the etiology of AMC is unclear, any factor that decreases fetal movement is responsible for AMC. Thus, accurate diagnosis and classification are crucial to the appropriate treatment of AMC. The development of ultrasound technology has enabled prenatal diagnosis. Very early treatment is favorable, and multidisciplinary treatment is necessary to improve the function of AMC patients. Most patients require surgery to release contracture and reconstruct joints. However, perioperative care is challenging, and difficult airway is the first concern of anesthesiologists. Postoperative pulmonary complications are common and regional anesthesia is recommended for postoperative analgesia. This review on AMC is intended for anesthesiologists. Thus, we discuss the treatment and perioperative management of patients undergoing surgery, as well as the diagnosis and classification of AMC.

Introduction: The effects of intense pulsed light (IPL) on collagen structures are well known in the treatment of photodamaged skin. Objective: The objective of this study was to investigate the effect of IPL on sclerotic skin by treating patients with microstomia due to systemic sclerosis. Methods and materials: 13 patients all with microstomia and systemic sclerosis were treated with IPL, PR (530-750 nm filter) and/or VL (555-950 nm filter) applicator. They were treated in the perioral area 8 times with 3–4 weeks of interval and follow-up for 6 months. The outcomes were the inter-incisal distance and the inter-ridge distance. Results: A significant increase in mouth opening of 4.1 mm (95% confidence interval, 1726–6638, p < 0.005) was found in the inter-ridge distance when comparing the distance before treatment with the distance at six-month follow-up. No significant difference was found in the inter-incisal distance. The patients experienced improved mobility and better control of lip movements after the treatments. Conclusion: IPL can improve the inter-ridge distance between the lips in patients with microstomia due to systemic sclerosis but does not affect the inter-incisal distance, which is also dependent on the mobility of the mandibular joints. This treatment can be considered an adjunctive therapy in patients with microstomia due to systemic sclerosis.

Background: Sensitive restoration is the primary aim of oral reconstructive surgery. Discriminative sensibility is an important index of innervation density of a tissue. Instruments normally used to assess this type of skin sensibility are bulky and difficult to introduce in the oral cavity, even in healthy patients with a normal mouth opening. This study was intended to evaluate the recovery of static and dynamic two-point discrimination sensitivity of the reconstructed areas of the oral cavity. Methods: Surgical staples, calibrated in predetermined width (from 1 to 30 mm) and introduced in the oral cavity with a Mayo needle holder, were used to evaluate two-point discrimination recovery in 57 patients who underwent reconstructive surgery with buccinator myomucosal flaps. Tests were conducted both on the reconstructive flap and on the non-operated contralateral side. The latter also included the non-operated cheek. Results: All of the considered flaps showed a recovery of tactile sensitivity. The overall average discriminative threshold value assessed on this sample was 9.11 ± 2.46 mm for the static and 6.56 ± 2.46 mm for the dynamic. Conclusions: The use of surgical staples allows easy assessment of tactile sensitivity in all oral cavity areas, even in operated patients who often present lockjaw or microstomia. In our series, buccinator myomucosal flaps demonstrate a much greater recovery of the sensation compared to results found in the literature on fasciocutaneous free flaps, even those reinnervated. Level of Evidence: Level III, prognostic study.

Introduction Systemic sclerosis (SS) is a systemic autoimmune disease characterized by microvascular and macrovascular lesions and fibrotic changes and frequently affects the mouth. Objective To evaluate the oral patterns of systemic sclerosis. Methods A descriptive case series with consecutives patients from November 2015 to October 2016. Systemic features and orofacial parameters were evaluated according to disease type. Results The sample consisted of 50 individuals with SS and 43 controls. A high frequency of orofacial alterations was observed, with orofacial telangiectasia (42/50, 84%), microstomia (35/50, 70%), and oral candidosis (31/50, 62%) being the most frequent findings. Gingival bleeding index was higher in controls (21.57 ± 15.66) than in SS (7.05 ± 7.25) (P Conclusions The pattern of periodontal manifestation is unique and characterized by few gingival bleeding and severe periodontitis.