Orofacial Complications of the Connective Tissue Disease Systemic Sclerosis.

Scleroderma (systemic sclerosis, SSc) is an autoimmune fibrosing connective tissue disease of unknown etiology. SSc patients show increased levels of autoantibodies, profibrotic cytokines, and extracellular matrix remodeling enzymes that collectively cause activated (myo)fibroblasts, the effector cell type of fibrosis. Despite these impacts, no disease-modifying therapy exists; individual symptoms are treated on a patient-to-patient basis. SSc research has been principally focused on symptoms observed in the lung and skin. However, SSc patients display significant oral complications that arise due to fibrosis of the not only skin, causing microstomia, but also the gastrointestinal tract, causing acid reflux, and the oral cavity itself, causing xerostomia and gingival recession. Due to these complications, SSc patients have impaired quality of life, including periodontitis, tooth loss, reduced tongue mobility, and malnutrition. Indeed, due to their characteristic oral presentation, SSc patients are often initially diagnosed by dentists. Despite their clinical importance, the oral complications of SSc are severely understudied; high-quality publications on this topic are scant. However, SSc patients with periodontal complications possess increased levels of matrix metalloproteinase-9 and chemokines, such as interleukin-6 and chemokine (C-X-C motif) ligand-4. Although many unsuccessful clinical trials, mainly exploring the antifibrotic effects of anti-inflammatory agents, have been conducted in SSc, none have used oral symptoms, which may be more amenable to anti-inflammatory drugs, as clinical end points. This review summarizes the current state of knowledge regarding oral complications in SSc with the goal of inspiring future research in this extremely important and underinvestigated area.

Digitally designed and manufactured sectional custom tray for a patient with microstomia: A case report.

Microstomia presents a challenge for the patient and dental provider. This report describes a partial digital workflow for the fabrication of a mandibular complete denture for a patient with microstomia. Computer-aided design and computer-aided manufacturing technology was utilized to 3D print a sectional custom tray with a unique design. The sectional custom tray was used to make a conventional border molded impression of the edentulous arch to fabricate a flexible complete mandibular denture for a 58-year-old female patient with scleroderma and microstomia. This treatment resulted in a successful prosthetic outcome and high patient satisfaction.

Hyaluronidase Injections for Oral Microstomia in Systemic Sclerosis and Mixed Connective Tissue Disease.

This case series evaluates hyaluronidase for oral microstomia in a cohort of patients with autoimmune sclerosing disease.

Free Fibula Flap for the Treatment of Agnathia in a 10-Year-Old With Severe Agnathia-Otocephaly Complex.

Agnathia-otocephaly complex (AOC), a first branchial arch defect, is characterized by mandibular hypoplasia or aplasia, ear abnormalities, microstomia, and macroglossia and is a rare and often fatal diagnosis. Herein, the technical considerations and details of mandibular reconstruction using virtual surgical planning (VSP) and a vascularized free fibula flap for further mandibular reconstruction in a 10-year-old boy are presented. The patient's preoperative examination was consistent with agnathia (absence of mandibular symphysis, bilateral mandibular bodies, condyles, coronoids, rami, and temporomandibular joint), severe microstomia, and a Tessier # 30 cleft (maintained to allow oral access until later in treatment). Virtual surgical planning was utilized to plan a 3-segment fibula for the reconstruction of the mandibular symphysis and bilateral body segments, and bilateral costochondral grafts were planned for the rami. To the authors' knowledge, this represents the first application of virtual surgical planning for mandibular reconstruction with a vascularized free fibula flap in a pediatric patient with severe agnathia-otocephaly complex.

[Clinical effects of ultra-pulsed fractional carbon dioxide laser in the treatment of mild to moderate microstomia after burns].

Objective: To investigate the clinical effects of ultra-pulsed fractional carbon dioxide laser (UFCL) in the treatment of mild to moderate microstomia after burns. Methods: A retrospective observational study was conducted on 19 patients with mild to moderate microstomia after burns who were admitted to Inner Mongolia Baogang Hospital from January 2018 to January 2022, including 15 males and 4 females aged (35±14) years. Patients had an average course of 71 d of microstomia, with 8 cases of moderate microstomia and 11 cases of mild microstomia. All the patients received UFCL treatment every 2-3 months until the microstomia was corrected or the treatment bottleneck was reached. The times of UFCL treatment for patients and the time interval from the last treatment to the last follow-up were recorded. Before the first treatment and at the last follow-up, the opening degree of mouth (finger measurement method), oral gap width, and the distance between the upper and lower incisors during mouth opening were recorded. Before the first treatment and at the last treatment, the new Vancouver scar scale (VSS) was used to evaluate the scar. At the last follow-up, the degree of satisfaction was evaluated by the Likert 5 scale by the patients themselves, and the satisfaction ratio was calculated; the adverse reactions such as pigmentation, blisters, infection, and persistent erythema in the treatment area were counted. Data were statistically analyzed with Mann-Whitney rank sum test or paired sample t test. Results: Patients received UFCL treatment of 3 (2, 6) times. The interval from the last treatment to the last follow-up was 26 months at most and 4 months at least. At the last follow-up, the opening degree of mouth of patients was significantly improved than that before treatment (Z=4.68, P<0.01). At the last follow-up, the oral gap width of patients was (35±6) mm, and the distance between upper and lower incisors during mouth opening was (3.2±0.4) cm, which was significantly improved compared with those before treatment (with t values of 10.73 and 18.97, respectively, P<0.01). The VSS score after the last treatment was 4.1±1.6, which was significantly better than that before treatment (t=22.96, P<0.01). At the last follow-up, the satisfaction ratio of patients with treatment was 18/19, and no pigmentation, blisters, infection, persistent erythema, and other adverse reactions of all patients in the treatment area occurred, however, one of the patients reported that the disease recurred about half a year after treatment. Conclusions: UFCL is an effective method for treating mild to moderate microstomia after burns, with which patients are highly satisfied, and it is worth of further study and promotion.

Firecracker Maxillofacial Injury in a 6-Year-Old Child- A Case Report.

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.

Clinical and histopathological improvement of scleromyxedema-induced microstomia after hyaluronidase injection.

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.

Manifestações bucais em portadores de epidermólise bolhosa residentes em um município baiano / Oral manifestations of epidermolysis bullosa in a municipality of the state of Bahia

Introdução: a Epidermólise Bolhosa hereditária (EB) é uma doença rara, caracterizada por formações de bolhas na pele e nas mucosas que sofrem mínimos traumas ou aparecem espontaneamente. As principais divisões de EB são Epidermólise Bolhosa Simples, Epidermólise Bolhosa Juncional, Epidermólise Bolhosa Distrófica e a Síndrome de Kindler. As manifestações bucais geralmente encontradas nos pacientes com EB são as bolhas no epitélio oral, microstomia, anquiloglossia, doença periondontal, hipoplasia do esmalte, cárie dentária, atrofia da maxila e prognatismo mandibular, variando para cada tipo da doença. Objetivo: relatar as manifestações bucais dos portadores de Epidermólise Bolhosa residentes no município de Barra da Estiva. Metodologia: trata-se de um estudo transversal, quantitativo e descritivo que foi desenvolvido no município de Barra da Estiva ­ BA com a população de portadores de Epidermólise Bolhosa, através da realização de anamnese e exame clínico. Estatística descritiva foi utilizada para tabular os dados coletados. Resultados: foram analisados 5 portadores de Epidermólise Bolhosa, a maioria do sexo masculino (60%), com média de idade de 5,6 anos. As manifestações bucais encontradas foram bolhas orais (100%), anquiloglossia (100%), língua despapilada (100%), hipoplasia de esmalte (80%), cárie (40%) e abertura de boca limitada em média de 28,6 mm. Conclusão: os portadores de Epidermólise Bolhosa apresentaram manifestações orais características da doença, tendo como consequências maiores dificuldades para realizar higienização bucal e tratamento odontológico, sendo necessário conhecimento prévio dos cirurgiões-dentistas para o atendimento desses pacientes.

Introduction: inherited Epidermolysis Bullosa (EB) is a rare disease, characterized by blistering of the skin and mucous membranes that suffer minimal trauma or appear spontaneously. The main divisions of EB are Simple Epidermolysis Bullosa, Junctional Epidermolysis Bullosa, Dystrophic Epidermolysis Bullosa and Kindler Syndrome. The oral manifestations usually found in patients with EB are blisters in the oral epithelium, microstomy, ankyloglossia, periodontal disease, enamel hypoplasia, dental caries, atrophy of the jaw and mandibular prognathism, varying for each type of disease. Objective: to report the oral manifestations of Epidermolysis Bullosa patients living in the municipality of Barra da Estiva. Methods: cross-sectional study, quantitative and descriptive that was developed in the municipality of Barra da Estiva-BA with the population of people with Epidermolysis Bullosa, through anamnesis and clinical examination. Descriptive statistics was used to tabulate the data collected. Results: 5 children with Epidermolysis Bullosa were analyzed, most male (60%), with an average age of 5,6 years. Oral manifestations found were oral blisters (100%), anquiloglossia (100%), loss of papillae of the tongue (100%), enamel hypoplasia (80%), tooth decay (40%) and mouth opening limited to the average of 28.6 mm. Conclusion: epidermolysis Bullosa children presented oral manifestations common to the disease, leading to difficulties in performing oral hygiene and dental treatment, requiring prior knowledge of dental surgeons for the care of these patients.

Eficacia de la intervención logopédica en la microstomía en pacientes quemados de cabeza y/o cuello: revisión bibliográfica de las publicaciones “Burns” y “Revista Brasileira de Queimaduras”. Trabajo de fin de grado / Efficacy of speech therapy intervention in microstomy in patients with head and/or neck burns: bibliographic review of the publications “Burns” and “Revista Brasileira de Queimaduras”. Final degree project

El rostro es una región de gran importancia para el ser humano, pues expresa lo más íntimo del individuo. Al quemarse la cara aparecen graves secuelas morfofuncionales, como las contracturas faciales y la microstomía. Estas pueden manifestarse en secuelas que incluyen déficits en la propiocepción facial y labial, deficiente acceso oral e higiénico y alteración de las funciones del sistema estomatognático.(AU)

Review of the Current Medical and Surgical Treatment Options for Microstomia in Patients With Scleroderma.

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.

Modified impression technique for extreme mouth-opening limitation caused by systemic scleroderma.

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.

Digital Workflow for a Scleroderma Patient with Microstomia: A Clinical Report.

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.

Endodontic management of a patient with dystrophic epidermolysis bullosa: A case report.

Epidermolysis bullosa is a congenital genetic disease that causes blistering and erosion of the skin and mucosa. The main known forms include simple, junction, dystrophic and mixed subtypes. This case report presents the endodontic management and 1-year follow-up of a 27-year-old female patient with epidermolysis bullosa who was referred to the Faculty of Dentistry, Kirikkale University, Turkey. An extraoral examination showed that the patient had multiple scars and blisters. The intraoral examination revealed ankyloglossia, microstomia, shallow buccal and vestibular sulci, enamel hypoplasia, gingival inflammation, mouth ulcers, symptomatic and asymptomatic deep caries, a tooth with an apical lesion and a tooth with pulpitis. The dental treatment was divided into four stages: (i) oral hygiene motivation and elimination of gingival bleeding, (ii) restorative and/or endodontic procedures, (iii) extractions and prosthetic treatments and (iv) recall appointments. A 1-year follow-up radiographic examination of the periapical status of the root canal treatments was clear.

Posterior Based Triangular Mucosal Advancement Flap for Surgical Correction of Scleroderma-Induced Microstomia.

ABSTRACT: 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.

Intraoral midline mandibulotomy to improve access for transoral robotic surgery (TORS) base of tongue resection in a retrognathic and microstomic patient.

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.

A case of rare isolated agnathia and literature review.

Agnathia is a rare congenital malformation with unknown etiology characterized by absence of the mandible, microstomia, and tongue aplasia, often found to have other anomalies including holoprosencephaly. The purpose of this paper was to describe the symptoms and imaging of a case of isolated agnathia and to conduct a comprehensive literature review of reported patients with isolated agnathia. Case reports of isolated agnathia are very rare, with most infants as stillborn. We report a child's management of isolated agnathia with microstomia and tongue aplasia. A literature review was performed with focus on diagnosis, airway, and feeding management of isolated agnathia. Polyhydramnios was a common pregnancy complication reported in 25 out of the 39 patients in the case study. Five infants were stillborn, while 23 died within the neonatal period. Of the deceased infants within the neonatal period, 19 died within minutes to hours while four died within days to weeks. There are nine patients with agnathia that survived past infancy. The results of this study suggest that isolated agnathia is a rare malformation which requires a multi-disciplinary approach for airway and feeding management.

Rehabilitation for a scleroderma patient with severe microstomia using digital and conventional methods.

BACKGROUND: Microstomia is a principal clinical symptom in patients with scleroderma. It is characterized by an abnormally small mouth opening, which makes traditional prosthetic rehabilitation difficult. METHODS: First, the primary impression and jaw relation record were acquired using an intraoral scanning technique and the primary diagnostic dentures were fabricated using 3-dimensional printing (3DP). The definitive impression and jaw relation were obtained and digitally scanned. The second diagnostic denture was also made to confirm the esthetic and occlusal effects. Next, the adjusted dentition parts were scanned and milled from a cross-linked polymethyl methacrylate disk. In addition, the titanium alloy frameworks were also fabricated using 3DP. With flasking, the milled dentition parts were inserted and the definitive dentures were finally fabricated and delivered to the patient. RESULTS: The authors constructed the definitive removable partial dentures successfully using the digital methods of intraoral scanning, computer-aided design, and 3DP. CONCLUSIONS AND PRACTICAL IMPLICATIONS: These digital methods provide an alternative option for prosthetic rehabilitation in scleroderma patients with severe microstomia.

Prosthetic Management of Microstomia with Customized Dynamic Splint.

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.

Assessment of Cortical Auditory Function Using Electrophysiological and Neuropsychological Measurements in Children with Bone-Anchored Hearing Aids.

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.