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Journal of Maxillofacial and Oral... Jun 2021Caustic soda ingestion causes strictures in the respiratory, gastrointestinal systems and even death. In the oral and perioral areas it causes contractures leading to...
Caustic soda ingestion causes strictures in the respiratory, gastrointestinal systems and even death. In the oral and perioral areas it causes contractures leading to microstomia that is difficult to manage to restore structure and function of the oral cavity. The present case is of a 42-year-old female who presented with microstomia, no endoscopic esophageal injury and ankyloglossia following ingestion of caustic soda in an attempted suicide following a dispute with her spouse. Satisfactory mouth opening and tongue movement were achieved by bilateral release of buccal contractures, commissuroplasty and release of the tongue that was tethered to the floor of the mouth.
PubMed: 33927490
DOI: 10.1007/s12663-019-01247-4 -
Korean Journal of Orthodontics Nov 2022Moebius syndrome (MBS) is a congenital neurologic disorder that causes cranio-facial abnormalities. It involves paralysis of the VI and VII cranial nerves and causes...
Moebius syndrome (MBS) is a congenital neurologic disorder that causes cranio-facial abnormalities. It involves paralysis of the VI and VII cranial nerves and causes bilateral or unilateral facial paralysis, eye movement disorder, and deformation of the upper and lower limbs. The orofacial dysfunctions include microstomia, micrognathia, hypotonic mimetic and lip muscles, dental enamel hypoplasia, tongue deformity, open bite or deep overbite, maxillary hypoplasia, high arched palate, mandibular hyperplasia or features indicating mandibular hypoplasia. This case report presents a 7-year-old male patient who was diagnosed with MBS at the age 2 years. The patient displayed typical clinical symptoms and was diagnosed with Class II malocclusion with a large overjet/overbite, tongue deformity and motion limitation, and lip closure incompetency. Treatment was initiated using a removable appliance for left scissor bite correction. After permanent tooth eruption, fixed appliance treatment was performed for correction of the arch width discrepancy and deep overbite. A self-ligation system and wide-width arch form wire were used during the treatment to expand the arch width. After 30 months of phase II treatment, the alignment of the dental arch and stable molar occlusion was achieved. Function and occlusion remained stable with a Class I canine and molar relationship, and a normal overjet/overbite was maintained after 9.4 years of retainer use. In MBS patients, it is important to achieve an accurate early diagnosis, and implement a multidisciplinary treatment approach and long-term retention and follow-up.
PubMed: 36070886
DOI: 10.4041/kjod22.012 -
Indian Journal of Plastic Surgery :... Dec 2023Management of post-electric burn microstomia is a challenging task, especially in children, as it causes difficulty in feeding and airway problems (secondary to nasal...
Management of post-electric burn microstomia is a challenging task, especially in children, as it causes difficulty in feeding and airway problems (secondary to nasal airway blockage). The recreated defect is often full thickness and requires full-thickness tissue for reconstruction. The free flap can provide adequate normal tissue for the restoration of functions and aesthesis of the perioral region. However, performing free flaps in children is equally demanding due to small-diameter vessel anastomosis and postoperative monitoring. We present a case of postburn microstomia that was managed by contracture release and reconstruction by free radial artery forearm flap in a 1-year-old child. Postoperatively, at 6 months of follow-up, the flap settled well and the child was able to open his mouth fully with good aesthetic outcome. The free flap can be considered a good and safe option for perioral contracture release and reconstruction for better functional and aesthetic outcomes.
PubMed: 38105870
DOI: 10.1055/s-0043-1776008 -
Anesthesia Progress 2020Systemic sclerosis (SSc) is an autoimmune disease that can cause fibrosis in vital organs, often resulting in damage to the skin, blood vessels, gastrointestinal system,...
Systemic sclerosis (SSc) is an autoimmune disease that can cause fibrosis in vital organs, often resulting in damage to the skin, blood vessels, gastrointestinal system, lungs, heart, and/or kidneys. Patients with SSc are also likely to develop microstomia, which can render dental treatment difficult and painful, thereby necessitating advanced anesthetic management. This is a case report of a 61-year-old woman with a history of SSc with microstomia, interstitial pneumonia, and gastroesophageal reflux disease in whom intravenous moderate sedation was performed using a combination of dexmedetomidine and ketamine for dental extractions. Both anesthetic agents are known to have analgesic effects while minimizing respiratory depression. Consequently, the increased discomfort caused by opening the patient's mouth and stretching the buccal mucosa was sufficiently managed, permitting an increase in maximum interincisal opening and completion of treatment without complications. Patients with SSc present with serious comorbidities that can negatively impact anesthetic management, so the implementation of an anesthetic plan that takes such risks into account is required. Furthermore, emergency airway management is likely to be difficult in patients with microstomia. For intravenous moderate sedation, combined use of dexmedetomidine and ketamine, which have analgesic effects while minimizing respiratory depression, may be particularly effective in patients with SSc and microstomia.
Topics: Anesthetics; Female; Humans; Ketamine; Microstomia; Middle Aged; Scleroderma, Systemic
PubMed: 32191504
DOI: 10.2344/anpr-66-03-07 -
American Journal of Medical Genetics.... May 2020Craniofacial morphogenesis is regulated in part by signaling from the Endothelin receptor type A (EDNRA). Pathogenic variants in EDNRA signaling pathway components...
Craniofacial morphogenesis is regulated in part by signaling from the Endothelin receptor type A (EDNRA). Pathogenic variants in EDNRA signaling pathway components EDNRA, GNAI3, PCLB4, and EDN1 cause Mandibulofacial Dysostosis with Alopecia (MFDA), Auriculocondylar syndrome (ARCND) 1, 2, and 3, respectively. However, cardiovascular development is normal in MFDA and ARCND individuals, unlike Ednra knockout mice. One explanation may be that partial EDNRA signaling remains in MFDA and ARCND, as mice with reduced, but not absent, EDNRA signaling also lack a cardiovascular phenotype. Here we report an individual with craniofacial and cardiovascular malformations mimicking the Ednra mouse phenotype, including a distinctive micrognathia with microstomia and a hypoplastic aortic arch. Exome sequencing found a novel homozygous missense variant in EDNRA (c.1142A>C; p.Q381P). Bioluminescence resonance energy transfer assays revealed that this amino acid substitution in helix 8 of EDNRA prevents recruitment of G proteins to the receptor, abrogating subsequent receptor activation by its ligand, Endothelin-1. This homozygous variant is thus the first reported loss-of-function EDNRA allele, resulting in a syndrome we have named Oro-Oto-Cardiac Syndrome. Further, our results illustrate that EDNRA signaling is required for both normal human craniofacial and cardiovascular development, and that limited EDNRA signaling is likely retained in ARCND and MFDA individuals. This work illustrates a straightforward approach to identifying the functional consequence of novel genetic variants in signaling molecules associated with malformation syndromes.
Topics: Animals; Craniofacial Abnormalities; Ear; Ear Diseases; GTP-Binding Protein alpha Subunits, Gi-Go; Gene Expression Regulation, Developmental; Genetic Predisposition to Disease; Humans; Loss of Function Mutation; Mandibulofacial Dysostosis; Mice; Mice, Knockout; Morphogenesis; Neural Crest; Phenotype; Receptor, Endothelin A; Signal Transduction
PubMed: 32133772
DOI: 10.1002/ajmg.a.61531 -
Experimental and Therapeutic Medicine Jul 2020Systemic sclerosis (SSc) is a collagenosis with insufficiently known etiopathogenesis, characterized by microvasculopathy and excessive fibrosis in the context of an...
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.
PubMed: 32508995
DOI: 10.3892/etm.2020.8572 -
Medicina (Kaunas, Lithuania) Jun 2023Epidermolysis bullosa (EB) is a genetically inherited disease characterized by recurrent bullae and erosions on the skin with numerous signs of dental caries and poor...
Epidermolysis bullosa (EB) is a genetically inherited disease characterized by recurrent bullae and erosions on the skin with numerous signs of dental caries and poor oral hygiene. The aim of this study was to investigate the general clinical and oral findings of patients with EB. In this prospective study, the clinical and oral findings and family history of 26 cases with EB were evaluated. The type of EB, gender, age, parental consanguinity, dental caries, oral findings, distribution of lesions and presence of associated anomalies, clinical and oral findings correlated with gender were recorded. All 26 patients with EB had a history of consanguinity and siblings with EB to varying degrees. In our study, malnutrition, anemia and growth retardation, gastrointestinal system complications, hair thinning, hand and nail deformity, ocular problems and renal disease (in one case) were observed with variable frequencies. When the intraoral findings of the patients were investigated, extensive dental caries in all EB types, enamel hypoplasia in junctional EB (JEB) and the presence of tooth-root to be extracted in dystrophic EB (DEB), intraoral bullae and lesions, ankyloglossia, vestibular sulcus insufficiency, microstomia and maxillary atrophy were observed. Three cases had restorative treatment and one case had prosthetic rehabilitation. Oral involvement can be seen with varying frequencies depending on the type of EB and the severity of the disease. It may result from delayed oral and dental rehabilitation due to physical disabilities, limitations and more pressing medical problems. Microstomy, pain from mucosal lesions, and restricted access to the mouth can be caused by poor oral hygiene. Oral complications and caloric needs of individuals with EB should be determined, and individual prophylaxis should be applied to prevent caries formation and protect teeth.
Topics: Humans; Blister; Prospective Studies; Dental Caries; Epidermolysis Bullosa; Mouth
PubMed: 37511997
DOI: 10.3390/medicina59071185 -
Indian Journal of Dental Research :... 2007Systemic sclerosis (scleroderma) is a rare generalized disorder of connective tissue origin. This condition is predominantly a clinical diagnosis, based on the clinical... (Review)
Review
Systemic sclerosis (scleroderma) is a rare generalized disorder of connective tissue origin. This condition is predominantly a clinical diagnosis, based on the clinical signs and symptoms. Here is a case report of 26-year-old female patient with the classical features of this disease. This case is reported for its rarity and variable expressivity. This article also reviews the literature of this uncommon condition.
Topics: Adult; Female; Hand Deformities; Humans; Microstomia; Mouth Mucosa; Periodontitis; Radiography; Scleroderma, Systemic
PubMed: 17347542
DOI: 10.4103/0970-9290.30919 -
Cureus Sep 2022Microstomia presents a unique challenge to the dentist as well as the patient. Microstomia patients often face difficulty in inserting or removing removable dental...
Microstomia presents a unique challenge to the dentist as well as the patient. Microstomia patients often face difficulty in inserting or removing removable dental prostheses due to constricted opening of the oral cavity. Dentists often face difficulty in inserting impression trays in these patients because of the limited mouth opening. Standard impression procedures need to be modified in such patients. This article describes a simple design for the fabrication of a sectional handle for a definitive impression in microstomia patients. The sectional handle could be sterilized and reused.
PubMed: 36299972
DOI: 10.7759/cureus.29433 -
Clinical, Cosmetic and Investigational... 2022Mucormycosis, a rare fungal infection seen in diabetes, is now very frequent owing to the deadly triad of COVID-19 infection, diabetes, and rampant use of...
Mucormycosis, a rare fungal infection seen in diabetes, is now very frequent owing to the deadly triad of COVID-19 infection, diabetes, and rampant use of corticosteroids. Immediate management revolves around therapeutic drugs like antifungals, antibiotics, and aggressive surgical debridement. The cases described in the article explain prosthetic rehabilitation of maxillectomy defects. The findings focus on prosthetic rehabilitation of patients with acquired maxillectomy defects after mucormycotic necrosis post-COVID-19 infection and the techniques to overcome the complications like lack of supporting tissues and post-surgical microstomia. The maxillectomies were performed on patients who suffered a superinfection of mucormycosis after COVID-19 contraction and uncontrolled blood sugar levels. Case 1 elaborates a technique to overcome the complications like lack of supporting structures and microstomia by fabrication of sectional and hollow obturator prostheses using sectional impression technique and lost salt technique. Case 2 explains the management of an extensive defect with a mobile soft tissue flap and lone standing tooth by using a functional impression technique to gain retention and support from the remaining soft and hard tissues. Both the techniques overcome the clinical complications and give predictable outcomes. Prosthetic rehabilitation of such challenging cases needs modifications depending upon the clinical challenges encountered.
PubMed: 35023975
DOI: 10.2147/CCIDE.S346315