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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.

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.

Restricted mouth opening (microstomia) can be defined as a reduction in the circumference of the oral cavity, although the intraoral structures may be of normal size. It is observed that the size of the oral opening decreases, the difficulty increases in required dental treatment. Prosthetic rehabilitation of microstomia patients due to oral sub-mucous fibrosis (OSMF) presents difficulties at all stages, right from preliminary impressions to insertion of prostheses, as the maximal oral opening is smaller than the size of a removal intra-oral prosthesis. To rehabilitate this type of patient successfully, the methods and designs incorporated in the fabrication of prosthesis have to be modified to achieve favourable mastication, retention and aesthetics. This article describes modified primary impression procedure without using tray and other techniques used to fabricate maxillary and mandibular custom sectional trays, sectional denture bases and removable sectional complete dentures for completely edentulous patient with limited oral opening caused by OSMF. Fabricating the sectional denture allows the patient to insert and remove the denture with ease.

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.

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 .

Background Microstomia is defined as a condition with a small sized-mouth that results in functional impairment such as difficulty with food intake, pronunciation, and poor oral hygiene and cosmetic problems. Several treatment methods for microstomia have been proposed. None of them are universally applicable. This study aims at analyzing the cases treated at our institution critically reviewing the pertinent literature. Methods The medical records of all microstomia patients treated in our hospital from November 2015 to April 2018 were reviewed retrospectively. Of these, all patients who received surgical treatment for microstomia were included in the study and analyzed for etiology, chief complaint, surgical method, and outcomes. The functional outcomes of mouth opening and intercommissure distance before and after the surgery were evaluated. The cosmetic results were assessed according to the patients' satisfaction. Results Five patients with microstomia were corrected. Two cases were due to scar contracture after chemical burn, two cases derived from repeated excision of skin cancer, and one patient suffered sequela of Stevens-Johnson syndrome. The following surgical methods were applied: one full-thickness skin graft on the buccal mucosa, three buccal mucosal advancement flaps after triangular excision of the mouth corner, and one local buccal mucosal flap. Mouth opening was increased by 6.0 mm, and the intercommissure distance improved by 7.2 mm on average. Follow-up was 9.6 months (range, 5-14 months). Cosmetic assessment was as follows: two patients found the results excellent, three judged it as good. Conclusion Microstomia has several causes. In order to achieve optimal functional recovery and aesthetic improvement it is important to precisely evaluate the etiologic factors and the severity of the impairment and to carefully choose the appropriate surgical method.

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.

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.

Agnathia-otocephaly complex (AOC) is a rare malformation complex of the first pharyngeal arch that is characterized by agnathia/dysgnathia, microstomia, aglossia/hypoglossia and variable displacement of the ears. Only 11 post-infancy patients with severe AOC have been described in the literature, and the incidence of this malformation complex is estimated to be 1 per 70,000 births. In this brief clinical study, the authors describe the case of an 18-year-old female diagnosed with AOC who underwent a 3-step mandibular distraction protocol with an external distraction device. The surgical protocol the authors used was unique in that we first placed a tissue expander in the submental area to enlarge the skin envelope in an effort to mitigate skeletal relapse from soft tissue forces. Furthermore, the way in which the authors slowed the activation of the distraction device to allow for soft tissue healing behind the pins was a novel component of the patient's treatment. The 3-step mandibular distraction protocol the authors present in this study increased the length of the mandible by 20 mm, and nearly doubled the size of the patient's mandible from an initial volume of 3.62 cm to a post-operative volume of 6.89 cm. Future surgeries will aim to improve the function of our patient's expanded mandible. Most important of all, the surgical treatment authors are presenting led to a significant improvement in our patient's physical appearance and 3d quality of life.

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.

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.

Routine complications faced by the dentist include atrophic ridge, microstomia, flabby tissue, xerostomia, bony exostosis, labially inclined premaxilla, esthetic demand, bruxism, systemic disorders, patient’s demand for duplicating dentures, etc. Management of these difficulties can be done by proper incorporating of suitable materials and advanced techniques. This article describes the unconventional approaches to various modalities so as to provide ultimate satisfaction for the patient. Keywords: Unconventional dentures, Liquid supported dentures, Xerostomia.

A patient with microstomia is a challenge for the prosthodontist at every stage of rehabilitation. A contemporary technique using sectional stock tray loaded with putty material and a rigid sectional custom tray incorporated with press button assembly for extra oral stabilisation has been described. The above two techniques were used to impart ease of tray insertion and comfort to the patient during impression making procedures. Also, a prefabricated easily available hinge was incorporated into the denture bases to facilitate easy intra-oral placement of the prosthesis.

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.

Patients with burns to the head and neck may be difficult to intubate or ventilate via facemask. Furthermore, post-burn scarring and microstomia may reduce the success of rescue supraglottic airway placement. While awake tracheal intubation using a flexible intubation scope is considered the optimal technique for these patients, it may not always be feasible in the pediatric population. We report a case of successful management of a difficult airway in a child with extensive post-burn head and neck deformity using a noninvasive positive pressure system to aid with inhalational induction and deep sedation during intubation using a flexible scope.