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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.

Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical manifestation of systemic sclerosis and is strongly related to esophageal dysmotility. However, there are multiple other contributing factors in each step in the physiology of swallowing that may contribute to development of severe dysphagia. The oral phase of swallowing may be disrupted by poor mastication due to microstomia and poor dentition, as well as by xerostomia. In the pharyngeal phase of swallowing, pharyngeal muscle weakness due to concurrent myositis or cricopharyngeal muscle tightening due to acid reflux can cause disturbance. The esophageal phase of swallowing is most commonly disturbed by decreased peristalsis and esophageal dysmotility. However, it can also be affected by obstruction from chronic reflux changes, pill-induced esophagitis, or Candida esophagitis. Other contributing factors to dysphagia include difficulties in food preparation and gastroparesis. Understanding the anatomy and physiology of swallowing and evaluating systemic sclerosis patients presenting with dysphagia for disturbances in each step can allow for development of better treatment plans to improve dysphagia and overall quality of life.

The present case report highlights the management of a 6 years old female child who suffered oral and maxillofacial injury due to explosion of a fire cracker inside the mouth which was managed by primary closure after complete debridement and to prevent the post treatment microstomia, a modified microstomia prevention intraoral prosthetic appliance was given and followed up for 15 months.

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.

Making impressions in patients with microstomia is often rather problematic due to their restricted mouth opening. Herein, this report describes a novel digital workflow for making impressions with computer-aided design and computer-aided manufacturing (CAD/CAM) custom sectional trays for a 58-year-old female patient with scleroderma and microstomia. CAD/CAM custom sectional trays were made based on digital dentition models from another case with similar arch scale. After the sectional impressions were obtained, the sectional casts were scanned and digitally aligned to form the final dentition models. The removable partial dentures were designed on the final digital models and printed using a 3D printer. This procedure was executed with a successful prosthetic outcome that included good fit and acceptable esthetics. The patient also reported a high level of satisfaction.

Introduction Scleromyxedema is a rare primary cutaneous mucinosis characterized by numerous firm, waxy, confluent papules. Recently, intravenous immunoglobulin (IVIG) is accepted by many authors as the first-line treatment option for severe cases. We report a 69-year-old male patient who has been suffering from scleromyxedema, with reduced mouth opening. He has been on a high-dose IVIG regime for 5 years. Methods The patient stated that he had difficulty in wearing and removing his dentures because of reduced mouth opening lately. Before considering to add any other immunosuppressants to his regime, we injected 1500 IU of hyaluronidase in total in one session periorally. The patient has been told open his mouth maximum and photographs have been taken before injections and after one month. We used a photo measurement application when evaluating microstomia to increase accuracy. We also took punch biopsies in order to evaluate effect of hyaluronidase histopathologically before and one month after injections. Results One month later, he was able to reattach and remove his dentures without adding any adjuvant immunosuppressants other than hyaluronidase. Mouth opening was increased in measurements and histopathologically, mucin deposition, fibroblastic proliferation, and perivascular lymphocytic infiltration were decreased. Conclusions We think hyaluronidase is a safe, easily accessible, and effective treatment option for microstomia caused by scleromyxedema.

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 .

Microstomia is a clinical condition of reduced mouth opening that can be acquired or congenital in origin. Problems associated with microstomia can be related to function, esthetics, or both. Management of microstomia due to facial burns is complex due to the presence of hypertrophic and contracture scars. Available treatment options can be broadly classified as surgical, nonsurgical, or both. Splints can be used to prevent the contraction of perioral musculature or to recuperate lost mouth opening. Various intraoral or extraoral, tooth-borne or tissue-borne, and static or dynamic appliances are in clinical use, but their designs are case specific. This case report explains the management of microstomia secondary to facial burns by using a dynamic splint in combination with intralesional injections of triamcinolone acetonide and hyaluronidase.

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.

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.

Defects to the lower lip remain surgically challenging to reconstruct. Restoration of aesthetics and function are crucial, of which the aims include restoring the tissues of the lower lip, maintaining oral competence, speech articulation, and restoring lip height and the vermillion. Further surgical techniques continue to be explored, with local flap surgery being the generally preferred method. However, many reconstructions leave the patient with pronounced deformity and morbidity. In this case report, we describe a new technique for the reconstruction of an 80% lower lip defect with an ipsilateral split zygomaticus major muscle sling, nasolabial flap, and facial artery musculomucosal flap. The reconstruction provided good oral competence, speech articulation and aesthetics, in addition to maintaining the oral commissure and lower lip height/length. Iatrogenic microstomia was avoided and zygomaticus functioning was normal. We are confident this technique will be useful to reconstructive surgeons in select cases of significant lip defects.

Systemic scleroderma is a relatively rare, autoimmune, chronic, and debilitating disease, and its etiology is unknown. The disease is characterized by vasomotor disturbances causing skin and mucosa hardening, hand stiffness, excess production of collagen fibers, and impairment of the internal organs associated with the immune system. Extreme mouth-opening limitation is one of the symptoms in the oral cavity, and compromises dental intervention. One of the fundamental steps when performing a dental procedure is dental arch impression. In the presented clinical case, limited mouth opening hindered the impression accuracy. The objective was to present an alternative impression technique for a patient affected by scleroderma, with limited mouth opening and the need for oral rehabilitation with a removable partial prosthesis in the maxillary arch. An individualized tray was developed with design characteristics that allowed access to the oral cavity and removal, after which it was possible to perform the oral impression, reproducing the areas of interest without causing injury to the oral tissues. The orientation plan was elaborated after obtaining the cast. The teeth were assembled, tested in the mouth, and sent to the laboratory for acrylization. The prosthesis was placed once ready. The alternative impression technique enabled oral rehabilitation of the patient despite the mouth-opening limitation, and provided an accurate impression and a good prosthetic result.

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).

This clinical report describes prosthetic rehabilitation applied to an edentulous patient with microstomia. Intraoral scanning was used for preliminary impressions, edentulous models were printed using a 3D printer, custom 2-piece impression trays for definitive impressions were made, and a 2-piece collapsible maxillary and a conventional mandibular denture were fabricated. Intraoral scanning is a useful alternative to conventional impression techniques and can be used safely in patients with microstomia for preliminary impressions.

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.

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.

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

Objectives To present early teeth extractions as a treatment option in severe dental crowding in patients with generalized recessive dystrophic epidermolysis bullosa (RDEB). Materials and methods Three patients with generalized RDEB were treated with early teeth extractions to prevent severe dental crowding. Results Two patients had bilateral upper first premolars extraction, and the third patient had permanent maxillary canine extraction. Crowding was avoided, and no further orthodontic treatment was necessary. Conclusion Considering the challenges of severe mucosal fragility and microstomia in patients with generalized RDEB, early teeth extractions are a reasonable option as an orthodontic management. This approach reduces the severity of dental crowding as the child gets older and reduces the need for orthodontic appliances. Individual factors such as access to dental care, general health, and oral health have an important impact on the decision‐making process. Orthodontic treatment planning should include a multidisciplinary team.

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.

Systemic sclerosis (SSc) is an autoimmune disease that can cause fibrosis in vital organs, often resulting in damage to the skin, blood vessels, gastrointestinal system, lungs, heart, and/or kidneys. Patients with SSc are also likely to develop microstomia, which can render dental treatment difficult and painful, thereby necessitating advanced anesthetic management. This is a case report of a 61-year-old woman with a history of SSc with microstomia, interstitial pneumonia, and gastroesophageal reflux disease in whom intravenous moderate sedation was performed using a combination of dexmedetomidine and ketamine for dental extractions. Both anesthetic agents are known to have analgesic effects while minimizing respiratory depression. Consequently, the increased discomfort caused by opening the patient's mouth and stretching the buccal mucosa was sufficiently managed, permitting an increase in maximum interincisal opening and completion of treatment without complications. Patients with SSc present with serious comorbidities that can negatively impact anesthetic management, so the implementation of an anesthetic plan that takes such risks into account is required. Furthermore, emergency airway management is likely to be difficult in patients with microstomia. For intravenous moderate sedation, combined use of dexmedetomidine and ketamine, which have analgesic effects while minimizing respiratory depression, may be particularly effective in patients with SSc and microstomia.

Reconstruction of the oral commissure is necessary after trauma, pathological resection, or electrical, chemical, or thermal burns. Using dental appliances is strongly recommended to prevent microstomia in victims with oral commissure injury caused by burns. These appliances can be modified from dynamic to static for more patient comfort during the healing period and used in trauma patients to prevent rounding of the labial commissure. Preventing relapse of microstomia and forming acute angles at the corner of the mouth are 2 optimal goals when performing this surgery.

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.

This clinical report describes a digital workflow using extraoral digital photographs and volumetric datasets from cone beam computed tomography (CBCT) imaging to create a 3-dimensional (3D), virtual patient with photorealistic appearance. In a patient with microstomia, hinge axis approximation, diagnostic casts simulating postextraction alveolar ridge profile, and facial simulation of prosthetic treatment outcome were completed in a 3D, virtual environment. The approach facilitated the diagnosis, communication, and patient acceptance of the treatment of maxillary and mandibular computer-aided design and computer-aided manufacturing (CAD-CAM) of immediate dentures at increased occlusal vertical dimension.

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.

Purpose: This review was intended to discuss the various possible modifications suggested in the literature for prosthetic steps and surgical corrective procedures in nonresponding or complicated cases during rehabilitation of patients with restricted mouth opening. Material and Methods: Medline, PubMed, and Google were searched electronically for articles using keywords: microstomia and treatment options for restricted mouth opening. The various articles on prosthodontic rehabilitation in microstomia were segregated. From these, various modifications in the prosthetic steps were reviewed. Results: Oral hygiene maintenance is difficult for patient either due to limited access or due to associated lack of manual dexterity, so dental decay and periodontal problems are more extensive in such patients; hence, tooth loss is a common finding. All prosthetic procedures require wide mouth opening to carry out various steps, starting from tray placement during impression making to the final prosthesis insertion, especially removable prosthesis. Various prosthetic modifications given by authors are included in this review for each step in prosthodontic management. A total of eight stock tray designs, 12 custom tray designs, and 17 removable prosthesis designs are discussed along with fixed (either tooth-supported or implant-supported) and maxillofacial prosthesis. However, some patients require surgical intervention also for the correction of microstomia either for function or for esthetic purpose before prosthetic rehabilitation and are also enumerated here. Conclusion: Among all prosthetic restorative options, removable prosthesis is most difficult for dentist to fabricate as conventional methods are either very difficult or impossible to apply. To get a more accurate final prosthesis, we need to modify these steps according to the existing case. Several modifications available are discussed here which can help while managing these patients.

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.

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.

Microstomia, an abnormally small oral orifice, is a complication of perioral facial burns. In this case, contraction of the circumoral tissues and hypotonia of the musculature is responsible for this microstomia, which can produce aesthetic and functional impairment with eating, swallowing, communication (speech and facial expressions), compromised dental care and maintenance due to limited oral access, social interactions, and psychological well-being. Conservative management involves providing physical resistance to scar contracture, with opposing horizontal and vertical circumoral forces by means of appliances that aim to stretch the commissures and fibrotic muscles. Numerous appliances, either intraoral or extraoral, have been described to prevent or treat microstomia by delivering a static or dynamic stretch horizontally or vertically, with most designed to stretch the mouth horizontally. Finding a comfortable effective way to stretch the mouth vertically has proved to be a challenge. This article describes the fabrication of a dynamic commissural appliance, constructed using acrylic resin and expansion screws, which provide simultaneous horizontal and vertical circumoral forces. This appliance is constructed easily and inexpensively without the need for taking impressions, can be adjusted so that it is almost painlessly inserted, and is progressively activated. It is convenient for use because the patient controls the pressure that is applied by the appliance. Its use in a case is described where the appliance has improved mouth opening and consequently functional outcomes.

Reconstruction of a composite defect of the lower lip after oncological resection is challenging, and it is essential to consider both functional and aesthetic components when repairing lips. We report a technique that can be used to repair anything ranging from 30% to the whole of the lower lip with a bilateral commissurotomy and advancement of skin, muscle, and mucosal flaps. This technique helps to achieve good oral function, excellent lip function, and a pleasant aesthetic appearance. It also prevents microstomia and allows patients to maintain normal sensory innervation.

In most cases, prosthetic rehabilitation of patients suffering from microstomia is inconvenient for the patient and challenging for both the dentist and dental technician. In such cases, conventional impression-taking requires an individualized tray design and modified impression techniques, amplifying the risk for impression and cast deformation and thereby compromising the final outcome. An intraoral scanner (IOS) might overcome these limitations due to its reduced size compared to an impression tray. In the present case history report, a microstomia patient was successfully restored with a telescopic prosthesis by digitizing both retaining teeth and the palate with an IOS.

Prosthetic techniques commonly employed for the rehabilitation of edentulous patients might not be adequate in the treatment of patients with microstomia.The purpose of this paper is to systematically review all the prosthetic techniques that have been used in the oral rehabilitation of patients with microstomia.Data sources, including PubMed, Google Scholar, SCOPUS and Web of Science, were searched for case reports and case series published through September 2017. Three investigators reviewed and verified the extracted data. Only case reports and case series on prosthetic rehabilitation in microstomia patients published in the English language were considered eligible.A total of 212 records were identified from the database search. Forty duplicate records were removed. The remaining 172 articles were assessed for eligibility, and 139 articles were removed because they did not satisfy the inclusion criteria. A total of 34 cases (including 32 case reports and 1 case series) were finally included in the qualitative analysis. The review revealed the use of a modified impression technique with flexible and sectional trays to record impressions in patients with microstomia. Modified forms of oral prostheses ranging from sectional, flexible, collapsible and hinged dentures to implant-supported prosthesis were fabricated to overcome the limited mouth opening. The success of the prosthetic technique primarily depended on the extent of the microstomia and the nature of the cause of the microstomia.Even though the patient acceptance of the prosthetic techniques summarized in the systematic review were high, long-term success rates for each option could not be assessed because of the short follow-up time in most of the included case reports and series.

Correction of microstomia is challenging with a high rate of recurrence. We report the successful treatment of microstomia using acellular dermal matrix (ADM) as an adjunct for intraoral lining with >1 year of follow-up.A 9-year-old international patient with severe immunodeficiency presented with severe microstomia because of recurrent oral infections. She had undergone 3 previous failed attempts to re-establish an adequate oral opening and was dependent on enteral nutrition via gastrostomy tube. She underwent release of the oral commissure scar contracture and orbicularis oris and the resultant mucosal defect was lined with ADM. A postoperative splint was used for 8 weeks. One-year follow-up demonstrated maintenance of the oral aperture with complete mucosalization of the ADM; the patient was able to resume oral diet and regular dental hygiene.Mucosal reconstruction with ADM is a viable alternative to local flaps and in this case exhibited minimal soft tissue contraction.

Current evidence suggests awaiting for scars to fully mature before engaging surgical reconstruction unless acute indications to prevent secondary damage, such as microstomia and eyelid ectropion are apparent. To evaluate the efficacy of ablative fractional CO2 laser intervention early in the acute treatment of panfacial burn injury. A 39-year-old Asian male with 60% TBSA flame burns including panfacial involvement was developing microstomia and upper and lower eyelid ectropion early proceeding epithelialization. At 6-weeks postinjury, ablative fractional CO2 laser treatment was commenced while still in the intensive care unit, and subsequently delivered at regular intervals. Nonsurgical scar contracture management was provided concurrently as per site specific standard protocols. Measurements and photographic data relative to deficits in eye and mouth competence were obtained at rest, as well as maximal opening at baseline and routinely until scar stabilization was reached. The outcomes were subsequently compared with facial burn patient historical data within our facility. No significant difference was identified in the functional ROM for mouth and eye regions; treatment duration was, however, shorter and aesthetic outcomes were considered superior to their surgical reconstruction counterparts in the historical cohort. This case report reveals that early ablative fractional CO2 resurfacing treatment, coalesced with nonsurgical scar management is an efficacious interventional approach to abate contractures to the face, accelerates and enhances scar maturation processes and may alleviate the need for surgical scar reconstructions. Moreover, optimal aesthetic outcomes may be achieved compared with traditional reconstructive methods.

Was to assess age-related changes in mouth opening and tongue mobility in children with various forms of epidermolysis bullosa (EB). Materials and methods. The study comprised 50 EB children (mean age 8.8±3.9 years). Oral slit width (the distance between mouth commissures at rest), mouth opening amplitude (the distance between incisive edges of the lower and upper incisors) were measured by orthodontic caliper. Tongue mobility was assessed using Bristol Tongue Assessment Tools (BTAT). The results were compared with 55 healthy age-matched controls (mean age 9.3±3.7 years).Mean moth opening in EB group was 22.6±11.1 mm which is twice lower than in controls (44.3±7.2 mm). The amplitude was progressively increasing in EB patients in the mixed dentition period but then dropped drastically in the permanent dentition because of oral fibrosis. Tongue mobility was lower in EB group when compared to controls even in cases with anatomically appropriate frenulum fixation sites. In permanent dentition maximal tongue raising was 8 times lower than in controls. Microstomia and ankyloglossia were very specific for dystrophic EB patients while in EB simplex, junctional EB and Kindler syndrome the values were similar to healthy controls.Microstomia, limited mouth opening and tongue mobility are typical oral features of dystrophic EB patients. Age-related dynamics shows progressive growth of the values in mixed dentition with significant lowering after so preventive measures for oral fibrosis are more feasible before permanent dentition phase.Оценить открывание рта и подвижность языка у детей с различными формами буллезного эпидермолиза (БЭ) во временном, сменном и постоянном прикусах.В исследование были включены 50 детей с БЭ (средний возраст 8,8±3,9 года). При помощи ортодонтического штангенциркуля измеряли ширину ротовой щели (расстояние между углами рта в покое при сомкнутых губах), амплитуду открывания рта (расстояние между режущими краями резцов при максимально открытом рте), а также оценивали подвижность языка по Бристольской системе оценки (Bristol Tongue Assessment Tools — BTAT). Результаты измерений сравнили с аналогичными данными, полученными в группе контроля, в которую вошли 55 здоровых детей (средний возраст 9,3±3,7 года).Средняя амплитуда открывания рта у детей с БЭ составила в 22,6±11,1 мм, что в 2 раза меньше (44,3±7,2 мм), чем в группе контроля, причем у детей с БЭ амплитуда нарастала в сменном прикусе по сравнению со временным, а затем уменьшалась в постоянном прикусе, что связано с патологическим рубцеванием слизистой оболочки. Даже при анатомически правильном прикреплении уздечки языка у детей с БЭ отмечалось ограничение его подъема, что можно считать проявлением фиброза, приводящего к уменьшению эластичности тканей. Максимально возможный уровень подъема языка у детей с БЭ с постоянным прикусом был в 8 раз меньше в сравнении с тем же возрастом в группе контроля. Развитие микростомии и анкилоглоссии напрямую зависело от формы заболевания — у детей с дистрофической формой БЭ степень ограничения открывания рта, рубцевания ротовой щели и анкилоглоссия наиболее выражены. У детей с простой и пограничной формами БЭ, синдромом Киндлер все показатели близки к результатам, полученным у здоровых детей.Для детей с дистрофической формой БЭ характерны выраженное ограничение открывания рта, микростомия и ограничение подвижности языка. Учитывая, что максимальные значения измерений у детей с БЭ получены в сменном прикусе, а далее наблюдается снижение всех показателей, профилактические мероприятия против патологического рубцевания необходимо проводить до формирования постоянного прикуса.

A 74-year-old man was referred to a tertiary academic otolaryngology clinic for evaluation of a left-sided neck mass with unknown primary. Nuclear imaging modalities revealed a primary cancer located at the left tongue base. Further investigation revealed the tumour to be a p16 positive squamous cell cancer with metastatic spread to cervical lymph nodes of multiple levels. The patient was found on initial investigation to have microstomia and a retrognathic mandible, which are typically considered unsuitable for robotic surgery due to difficulties obtaining adequate exposure.The patient underwent bilateral neck dissection, followed by transoral robotic-assisted left base of tongue resection. A midline intraoral mandibulotomy was performed to improve robotic access. Following tumour resection, the mandible was repaired using open reduction with internal plate fixation. Postoperative occlusion was maintained, and the patient recovered well from mandibulotomy with none of the morbidity or cosmetic defects associated with a traditional lip-split approach.

Otocephaly complex is a rare and usually lethal syndrome characterized by a set of malformations consisting of microstomia, mandibular hypoplasia/agnathia, and ventromedial malposition of the ears. Those cases that have been diagnosed prenatally have used an ex utero intrapartum treatment procedure to establish a definitive airway. However, prenatal diagnosis continues to be challenging, primarily because of poor diagnostic sensitivity associated with ultrasonography. We present a case of a newborn with an unanticipated otocephaly complex requiring emergent airway management. In this report, we discuss the medical and ethical issues related to the care of a newborn with this frequently fatal condition.

The ability to provide high quality complete dentures remains an important skill for general dental practitioners. The prevalence of edentulism is increasingly concentrated within an older patient cohort and general dental practitioners may face challenges associated with providing care for these patients. This two-part series explores various aspects of complete denture provision and is designed to act as a refresher on the management of edentulous patients. This second part focuses on the copy denture technique as well as discussing strategies for assessing and managing gag reflexes, prominent palatal and lingual tori and microstomia.

The Richieri-Costa-Pereira syndrome (RCPS) is an autosomal-recessive acrofacial dysostosis caused by mutations in EIF4A3, characterized by mandibular cleft comprising other craniofacial anomalies and limb defects such as cleft palate/Robin Sequence, microstomia, absence of mandibular central incisors, minor ear anomalies, clubfeet and first and 5 ray defects. The findings from this study are useful for better understanding the morphological consequences of disorders of EIF4A3, and having a better picture of the anatomic characteristics of the syndrome for a better therapeutic planning. Twenty-four angular and linear variables were measured to assess anteroposterior and vertical (superior-inferior) position of the cranial base, maxilla, mandible, and facial profile. The cephalometric radiographic analysis was performed on 9 individuals with RCPS, obtained at a mean age of 10.3 years, and compared with randomly selected age-matched 9 controls, without clefts and with well-balanced faces, with mean age of 10.6 years (both groups range 8.1 to 13.7 years). t test was used for analysis of means and Levene test for equality of variances. The syndrome group presented severe mandibular hypoplasia and retrognathism (P = 0.009, P = 0.001), greater facial convexity (N'PnPog and N'SnPog, P

BACKGROUND Oropharyngeal features are frequent and often understated in the treatment clinical guidelines of systemic sclerosis in spite of important consequences on comfort, aesthetics, nutrition and daily life. The aim of this systematic review was to assess a correlation between the oropharyngeal manifestations of systemic sclerosis and patients' health-related quality of life. METHODS A systematic search was conducted using four databases [PubMed® , Cochrane Database® , Dentistry & Oral Sciences Source® and SCOPUS® ] up to January 2018, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses. Grey literature and hand search were also included. Study selection, risk bias assessment (Newcastle-Ottawa scale) and data extraction were performed by two independent reviewers. The review protocol was registered on PROSPERO database with the code CRD42018085994. RESULTS From 375 screened studies, 6 cross-sectional studies were included in the systematic review. The total number of patients included per study ranged from 84 to 178. These studies reported a statistically significant association between oropharyngeal manifestations of systemic sclerosis (mainly assessed by maximal mouth opening and the Mouth Handicap in Systemic Sclerosis Scale) and an impaired quality of life (measured by different scales). Studies were unequal concerning risk of bias mostly because of low level of evidence, different recruiting sources of samples and different scales to assess the quality of life. CONCLUSION This systematic review demonstrates a correlation between oropharyngeal manifestations of systemic sclerosis and impaired quality of life, despite the low level of evidence of included studies. Large-scaled studies are needed to provide stronger evidence of this association.