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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 -
British Dental Journal Oct 2018The ability to provide high-quality complete dentures has been a key skill for general dental practitioners throughout the history of dental care. The prevalence of...
The ability to provide high-quality complete dentures has been a key skill for general dental practitioners throughout the history of dental care. The prevalence of edentulism is becoming increasingly concentrated in an older patient cohort and general dental practitioners may more commonly face challenges associated with providing care for these patients. This two-part series explores various aspects of complete denture provision and is designed to act as a refresher on core aspects of managing these patients, while also covering common challenges associated with anatomical or patient factors. This first part will explore changes in the provision and teaching of complete denture care in the UK and will describe important aspects of patient examination. It will discuss the management of unstable lower dentures and fibrous replacement ridges. Part two will cover management of the gag reflex, tori, microstomia and copy dentures.
PubMed: 30337727
DOI: 10.1038/sj.bdj.2018.866 -
Journal of Obstetrics and Gynaecology... Aug 2022Otocephaly is a rare malformation characterized by agnathia (absence of the mandible), melotia (medially displaced ear pinna), aglossia (absence of the tongue) and...
INTRODUCTION
Otocephaly is a rare malformation characterized by agnathia (absence of the mandible), melotia (medially displaced ear pinna), aglossia (absence of the tongue) and microstomia (small oral aperture). This results due to failure of migration of the neural crest cells and is a defect of the first branchial arch. It is incompatible with life and early prenatal diagnosis is useful.
CASE REPORT
Our patient a primigravida with 19 weeks 6 days gestation was referred for micrognathia and polyhydramnios. On ultrasound examination, she had unilateral mild ventriculomegaly and posterior fossa cyst in the fetal brain. The fetus had agnathia and anophthalmia. There was an echogenic intracardiac focus and echogenic bowel. The stomach was not seen clearly. This could be due to agnathia and microstomia leading to swallowing difficulties. The patient was explained about the guarded prognosis. The pregnancy was terminated. A diagnosis of otocephaly was made.
DISCUSSION
Otocephaly is a rare disorder of development of the first branchial arch. The reported incidence is 1 in 70,000. It is mostly lethal due to respiratory difficulties and may be associated with cranial and extracranial malformations. Most case reports have found that it is sporadic and could be due to mutations in the PRRX1 gene. Other anomalies that may be associated with otocephaly are neural tube defects, cephalocele, dysgenesis of corpus callosum, atresia of the third ventricle, midline probocis, hypotelorism, renal ectopia, cyclopia, vertebral and rib abnormalities, tracheo esophageal fistula, cardiac anomalies and adrenal hypoplasia. Most of the cases reported so far were diagnosed in the second or the third trimester. Facial anomaly screening has undergone a huge evolution in the recent years. In addition to the usual facial screening, we recommend mandibular arch screening in the first and early second trimester. If there is a doubt the patient may be called back at 15 to 16 weeks of gestation considering the fact that these anomalies are usually lethal and medical termination is safer earlier in pregnancy than later. MRI may be a handy tool to confirm antenatal diagnosis as it can detect the abnormal ears. Agnathia and polyhydramnios occur together in the third trimester but in the first or second trimester polyhydramnios may not be observed.
CONCLUSION
Otocephaly, though rare, poses a clinical challenge for both patient and the reporting doctor. Considering the time limitation for termination of pregnancy in our country, early prenatal diagnosis is important. A detailed face evaluation in the first trimester can help detect this defect as early as 11-14 weeks. Early diagnosis of lethal anomalies helps in completing the fetal work up and offering a safer termination. Correct diagnosis and work up of fetal anomalies allows for documentation and awareness of the presence of these conditions in our population.
PubMed: 35923505
DOI: 10.1007/s13224-021-01494-x -
BioMed Research International 2022Epidermolysis bullosa (EB) is a group of skin disorders with skin fragility characterized by blistering from minimal mechanical trauma with rupture at the dermoepidermal... (Review)
Review
Epidermolysis bullosa (EB) is a group of skin disorders with skin fragility characterized by blistering from minimal mechanical trauma with rupture at the dermoepidermal junction. There are four major classical heritable EB types, due to mutations in as many as 20 distinct genes: EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB), and Kindler EB (KEB). This study is aimed at reporting case series on patients ( = 8; males, = 5 and females, = 3, age range 12-68 years) affected by EB and performs a review of the literature on this topic. This group of disorders can affect oral soft and hard tissues in various ways, resulting in various effects including enamel hypoplasia, dental caries, microstomia, ankyloglossia, oral blistering, and ulcerations early-onset periodontal disease. From the sample results, it can be concluded that the clinical manifestation of EB patients is highly variable and very different in prognosis. Oral health deeply influences the quality of life of EB patients. Dental management is essential to prevent the aggravation of soft tissue damage and tooth loss and to improve the quality of life through prosthetic and restorative therapies. Dentists should consider the oral alterations of EB subtypes to perform a personalized approach to the patients' needs in a preventive and therapeutic point of view.
Topics: Adolescent; Adult; Aged; Child; Dental Caries; Epidermolysis Bullosa; Epidermolysis Bullosa Dystrophica; Epidermolysis Bullosa, Junctional; Female; Humans; Male; Middle Aged; Quality of Life; Young Adult
PubMed: 35686231
DOI: 10.1155/2022/6493156 -
Journal of Dental Hygiene : JDH Aug 2021Limited scleroderma is associated with significant risks to the orofacial complex. The purpose of this mixed methods study was to investigate the experiences of...
Limited scleroderma is associated with significant risks to the orofacial complex. The purpose of this mixed methods study was to investigate the experiences of participants with limited scleroderma related to oral health quality of life and oral health. A sequential mixed methods study was used in a population of individuals diagnosed with limited scleroderma. Participants were recruited through rheumatology specialists, referral, and social media; purposive sampling was used to recruit participants to be interviewed. The validated Oral Health Impact profile (OHIP) and Mouth Handicap in Systemic Sclerosis (MHISS) instruments and semi-structured interviews were used for data collection. Quantitative data were analyzed using descriptive statistics and the qualitative data were reported through thematic analysis. Forty-eight (n=48) qualifying participants participated in the quantitative phase, and 12 (n=12) participated in the qualitative phase. Based on a Likert Scale from 0-4, mean OHIP-14 scores ranged from 2.76 to 2.85, with anxiety over oral condition, embarrassed by oral problems, and oral self-consciousness having the greatest negative impact on quality of life. Mean MHISS scores ranged from 2.13 to 3.11. The highest OHRQoL scores were related to dry mouth symptoms. Factors influencing oral health-related quality of life were varied; dry mouth and microstomia were the prevalent complaints. Thematic analysis revealed challenges to oral health included self-care and professional dental care factors, both of which depended upon individual disease expression. Financial and emotional burdens also influenced participants' oral health. The oral health-related quality of life of those with limited scleroderma is impacted by multiple factors and oral health care professionals must seek out improved treatment modalities to address the needs of these vulnerable individuals. Future studies related to interprofessional collaborative care with scleroderma specialists are recommended.
Topics: Humans; Mouth; Oral Health; Quality of Life; Scleroderma, Limited; Surveys and Questionnaires
PubMed: 34376545
DOI: No ID Found -
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 -
Journal of the American Dental... Sep 2020Microstomia is a principal clinical symptom in patients with scleroderma. It is characterized by an abnormally small mouth opening, which makes traditional prosthetic...
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.
Topics: Computer-Aided Design; Dental Impression Technique; Denture Design; Denture, Partial, Removable; Humans; Microstomia
PubMed: 32711806
DOI: 10.1016/j.adaj.2020.05.016 -
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 -
GMS Interdisciplinary Plastic and... 2022The lip has functional and aesthetic importance. Lip defects occur due to the variety of etiology and the choice of their reconstruction has profound effect on...
The lip has functional and aesthetic importance. Lip defects occur due to the variety of etiology and the choice of their reconstruction has profound effect on functions and cosmesis. There are multiple options available for reconstruction according to defect size, but superiority of one method over another is still debated and hence the methods and their outcome were analyzed prospectively. Twenty-one patients with all sizes and locations of defects in upper and lower lip with acquired etiology were included in the evaluation. Reconstruction was performed according to defect size, availability of local/regional and distant donor tissue, defect location, patients' comorbid conditions and patients' preference. Patients were assessed at 1 month and 6 months postoperatively. Observers' and patients' input were also taken into account for outcome. Out of 21 patients, 5 free radial artery forearm flap reconstructions, 4 nasolabial flap reconstructions, 5 primary closures of defect, 4 Estlander flap reconstructions, 2 lip advancements, and one Karapandzic flap reconstruction were done. Free flap and nasolabial flap had hypoesthesia and incompetence if commissure is reconstructed and problem of bulk, restricted mobility and vermilion mismatch. Local and lip flaps were associated with decreased stoma size and some form of local scarring and asymmetry. However, all patients were satisfied with the functional and aesthetic outcome. Local flaps are better in terms of functional and aesthetic outcome but with some degree of microstomia which was well tolerated by most patients. Regional and distant flaps provide reconstruction where no other option is available and provide good functional support and acceptable cosmesis.
PubMed: 35465154
DOI: 10.3205/iprs000163 -
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