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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.

Sodium hydroxide or caustic soda is a corrosive agent that can cause extensive damage to the oral mucosa, lips, and tongue when ingested either accidentally or intentionally. These injuries include microstomia, shallow vestibule, ankyloglossia, speech impairment, loss of teeth and impairment in facial expression. In the present article, we report a unique case of tongue adhesion to the mouth floor and its surgical management in a 66-year-old female patient, who had a history of caustic soda ingestion.

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

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

Agnathia-otocephaly complex (AOC) is a rare and complex craniofacial malformation characterized by mandibular hypoplasia or agnathia, auricular fusion (synotia), and microstomia with oroglossal hypoplasia or aglossia. It can occur alone or in combination with forebrain anomalies and cardiac malformations and has an extremely poor prognosis. Here, we report a case of AOC diagnosed by systemic fetal screening at a gestational age of 25(+4) weeks. Ultrasound revealed that the S-curve formed by the normal lower jaw and lower lip had disappeared, the lower jaw and mandible were invisible, the mouth was extremely small, and the oral fissure was “pinhole-shaped”. There was a cone-shaped perioral bulge. Both ears were located in the front side of the neck, and the right foot was inverted. Excessive amniotic fluid was observed. The absence of a mandible was confirmed on X-ray examination after induced abortion. Specimen observation showed that the ear positions were extremely low, and both earlobes were connected in the front side of the neck. It was particularly challenging to identify the development of the mandible and locate auricles during prenatal ultrasound diagnosis, and the prenatal diagnosis of AOC was confirmed by combining two-dimensional and three-dimensional ultrasound in our current case.

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.

Here we present a clinical case of microstomia with the focus on the oral rehabilitation of the patient. Microstomia, an abnormal contracture of the mouth, occurred in this case as a result of alveolar resection due to cancer, and scarring impeded mouth opening. The patient needed a partial removable laminar denture for the upper jaw. Prosthetics became possible upon normalization of oral mucosa. We describe the imprint preparation features in conditions of lacking right alveolar appendix and crossover fold after surgery. The formation of the wax board in combination with molding allowed demarcation of denture border. The laminar denture successfully replaced jaw and teeth defects, restored proper mastication, improved mouth moistening and speech, as well as facial aesthetics and quality of life.

Near-total lip defects present a unique challenge preserving both oral competence and aesthetics. While lip reconstruction is commonly accomplished with local flaps to preserve function, in large defects, this technique alone can result in microstomia, speech inarticulation, and facial distortion. Free tissue transfer has become an attractive alternative, yet this approach presents its own set of functional challenges. For complex lip defects, the goal of reconstruction is preserving function and cosmetic appearance while avoiding debilitating microstomia. In this pictorial essay, we describe the anatomy, harvest, inset, and reconstructive approaches for using a combined Karapandzic and radial forearm free flap with dual palmaris longus tendons as an effective option for lip reconstruction in large ablative defects involving the oral commissure. This novel technique uses local, regional, and free tissue to provide an excellent functional and cosmetic reconstructive option for this and similar large lip defects.

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.

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.

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

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

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

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

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

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.

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.

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.

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

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

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

Limited oral opening is an acquired or congenital abnormal condition that compromises patient esthetics, nutrition, and quality of life. In addition, it may hinder conventional prosthetic procedures of edentulous patients, make it challenging, and present difficulties at all its stages. This clinical report presents different clinical treatment options suitable to be chosen by the prosthodontic carer in the case of reduced oral aperture.

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.

Statement of problem Prosthetic techniques commonly employed for the rehabilitation of edentulous patients might not be adequate in the treatment of patients with microstomia. Purpose 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. Materials and methods 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. Results 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. Conclusion 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.

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.