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.

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.

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.

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.

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.

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.

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.

A comparison between two educational methods in the rehabilitation of the microstomia in systemic sclerosis: a randomized controlled trial.

OBJECTIVE: To test the effectiveness of an educational intervention including "face to face" training, compared to a standard information program, to reduce microstomia in women with systemic sclerosis. DESIGN: Single-blind, two-arm, randomized controlled study with a 12-month follow-up period. SETTING: Hospital wards of a large Italian dermatological reference center. SUBJECTS: Female inpatients with diagnosis of systemic sclerosis. INTERVENTIONS: For both groups an information brochure and an audio-visual DVD were developed specifically for the study. The control group was assigned to educational materials alone (i.e. brochures and DVD), while the experimental group, in addition to the same educational materials, received specific "face-to-face" interventions, repeated at each follow-up visit. MAIN MEASURES: Primary outcome was measurement of the opening of the mouth. Secondary outcomes was the self-reported mouth disability. RESULTS: The intention-to-treat analysis included 63 patients. Compared to the baseline measurement, we observed an increase of the mouth opening of 0.31 cm (95% confidence interval: 0.13-0.49), P = 0.003; in the control group, the increase was 0.13 cm (95% confidence interval: 0.01-0.25), P = 0.06. The difference in improvement between the two groups was not statistically significant (P = 0.10); however, it reached statistical significance in the per-protocol analysis (39 patients, P = 0.02). CONCLUSION: Face-to-face nursing rehabilitation training seems to improve microstomia to a greater extent, when compared to a standard intervention based only on written and audio-visual materials.

Correction of Microstomia Reconstruction With the Use of Acellular Dermal Matrix for Buccal Reconstruction.

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

Prosthetic Rehabilitation of an Edentulous Patient with Microstomia Using Both Digital and Conventional Techniques: A Clinical Report.

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.

Prosthetic Rehabilitation of Microstomia Patients: A Systematic Review of Published Case Reports and Case Series.

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.

The Importance of Dental Appliances for Oral Commissure Reconstruction in Trauma-Induced Microstomia: A Clinical Report.

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

Impact of systemic sclerosis oral manifestations on patients' health-related quality of life: A systematic review.

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

Integrating hinge axis approximation and the virtual facial simulation of prosthetic outcomes for treatment with CAD-CAM immediate dentures: A clinical report of a patient with microstomia.

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

Restoring masticatory function in a patient with severe microstomia using rapid prototyped mesh and a custom-made hinge and swing-lock prosthesis.

This clinical report describes the use of rapid prototyped mesh in a complete swing-lock prosthesis to restore masticatory function in an edentulous patient with severe microstomia and perioral scar tissue after an industrial hot tar accident.

Management of postburn perioral contracture using a customized static commissural splint and intralesional injections of triamcinolone.

A perioral facial burn is usually accompanied by a reduction in size of the oral orifice because of the contraction of the healing wound. Perioral contracture leads to microstomia, restricting nutrition, speech, and jaw movements and impairing the esthetic appearance of the face. It is imperative to introduce splints as early as possible to prevent the postburn sequelae. A delay in splinting allows postburn contractures. Overcoming these contractures to regain the size of the stoma requires restructuring of the hypertrophic scar using mechanical force and the biomodification of the tissues. This article describes the treatment of microstomia and the hypertrophic scarring of the perioral tissue using a novel static commissural splint with customizable components in conjunction with intralesional injections of triamcinolone. Within 6 months, the splint together with the steroid injections had helped increase the vertical opening of the mouth by 15 mm and the intercommissural distance by 16 mm.

Effects of intense pulsed light in microstomia in patients with systemic sclerosis: A pilot study.

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

Effects of Fat Grafting Containing Stem Cells in Microstomia and Microcheilia Derived from Systemic Sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease with multisystem involvement, dominated by a general fibrosis. The early stage of the disease is associated with progressive damage to microcirculation, particularly in the respiratory tract, the gastrointestinal tract and skin. The face assumes a typical appearance characterized by microstomia (reduction of mouth opening) and microcheilia (thinning of the lips). These conditions cause a considerable reduction in performance status of patients. We treated them by fat grafting, rich in adipose stem cells, and we evaluated through time clinical, functional and aesthetic evaluation of oral pathology associated with SSc. MATERIALS AND METHOD: From September 2014 to May 2016, we enrolled and treated seven patients in the plastic, reconstructive and aesthetic surgery clinic. Through time, we evaluated the following parameters: evaluation of mouth opening (maximum opening in superior-inferior and lateral directions) and lip thicknesses, both measured by doctors of the aforementioned operating unit; variation in the quality of life as perceived by patients according to the MHISS scale (Mouth Handicap Systemic Sclerosis); variation in severity of labial fibrosis assessed by microscopic analysis of pre- and post-fat transfer samples in the pathology clinic; safety of the protocol, according to the management of side effects resulting from the procedure; aesthetic evaluation, made by external observers and non-experts in the field, on pre- and post-operative photographs. RESULTS AND CONCLUSIONS: We reported satisfying results, both functionally and aesthetically, for all parameters except one, for which the sample size might have proven critical. These data should be a starting point for further experimental research and clinical trials. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Glomerulopathy in patients with distal duplication of chromosome 6p.

BACKGROUND: Duplication of the distal part of chromosome 6p is a rare genetic syndrome. Renal involvement has been reported in the majority of patients, including a wide range of congenital abnormalities of kidney and urinary tract and, occasionally, a proteinuric glomerulopathy. CASE PRESENTATION: Here, we report a 13-year-old girl with 6p25.3p22.1 duplication who presented with proteinuria in infancy, was later diagnosed as focal segmental glomerulosclerosis, progressed to end-stage renal disease and was successfully transplanted. CONCLUSION: A systematic literature review suggests that 15-20 % of individuals with distal 6p duplication develop progressive proteinuric glomerulopathy. Monitoring of kidney function should be recommended in all cases.