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Experimental and Therapeutic Medicine Dec 2022Microscopic polyangiitis (MPA) is a rare, idiopathic, autoimmune, systemic disease that most frequently involves the kidneys. The present study reports the case of a...
Microscopic polyangiitis (MPA) is a rare, idiopathic, autoimmune, systemic disease that most frequently involves the kidneys. The present study reports the case of a 48-year-old female patient who presented with diffuse myalgia, arthralgia of both hands and feet for 2 weeks before being admitted to the hospital. The patient exhibited involuntary loss of weight and occasional slight fever. Physical examination noted microstomia and perioral radial furrows, slight skin induration of the hands, discrete cyanotic skin areas on the dorsal side of both feet. The patient also presented bilateral crepitant rales. Laboratory findings at admission revealed non-specific biological inflammatory syndrome consisting of high erythrocyte sedimentation rate and high C-reactive protein. The patient was initially suspected of systemic sclerosis due to the appearance of microstomia and the slight skin induration of the hands with diffuse arthralgia and myalgia, although with negative immune tests (anti-SCL70 and anti-centromere B antibodies) and normal nailfold capillaroscopy. Instead, a high titer of MPO-ANCA was detected. The computerized tomography scan revealed early diffuse interstitial lung disease (ILD). Cases of MPA with pulmonary involvement, such as ILD before the onset of vasculitis or kidney involvement, are known. Therefore, the diagnosis of MPA was formulated considering the symptoms, the clinical examination and the high titer of MPO-ANCA. The particularity of the present case consists in the uncommon onset with atypical skin changes, positivity to MPO-ANCA, absent renal dysfunction and ILD involvement.
PubMed: 36420356
DOI: 10.3892/etm.2022.11668 -
Cureus Oct 2022During reconstructive interventions in patients presenting with severe post-burn mento-sternal scar contracture, securing the airway forms a critical part of...
During reconstructive interventions in patients presenting with severe post-burn mento-sternal scar contracture, securing the airway forms a critical part of management. Extreme contracture is more likely to develop in patients who have had thoracic burns with ascending involvement of the neck and mandibular region. When cervical hyperextension and elevation of the mandible are impeded, post-burn contracture of the neck might render endotracheal intubation difficult. The development of rigid scar tissue that distorts the laryngeal and mandibular anatomy, or the development of microstomia following scar tissue retraction in facial burns, may make alternative approaches to direct laryngoscopy challenging. In patients with healed neck burns, intubation difficulties should be anticipated, and equipment for aiding intubation should be kept ready. Furthermore, a surgeon must be present throughout anesthesia induction in case an emergency neck release is required. Although the role of awake fiberoptic intubation has been well established in the general population, it is yet to be assessed in patients with burns. In this report, we present a case of successfully managed post-burn contracture that was planned for awake fiberoptic intubation.
PubMed: 36348892
DOI: 10.7759/cureus.30011 -
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 -
Quintessence International (Berlin,... Jan 2023Epidermolysis bullosa (EB) is a rare genetic mucocutaneous disorder characterized by epithelial fragility leading to blister formation on skin and mucous membranes with...
OBJECTIVE
Epidermolysis bullosa (EB) is a rare genetic mucocutaneous disorder characterized by epithelial fragility leading to blister formation on skin and mucous membranes with even minor mechanical trauma. Most EB oral health publications give fragmented information, focusing on only one oral health aspect or one EB type. The aim of this study was to expand the knowledge of the overall oral health status of individuals with dystrophic, junctional, and simplex EB.
METHOD AND MATERIALS
A comparative multicenter study, including a control group, and based on questionnaires and clinical examinations, was undertaken in three EB expert centers.
RESULTS
Most EB (90.2%) participants brushed their teeth at least once a day despite the pain. The prevalence of enamel defects and caries experience did not differ between the 42 EB participants and the 42 age-/sex-matched healthy controls. Gingival inflammation unrelated to dental plaque accumulation was found in EB participants. Blisters, erythema, and erosion/ulceration mainly involved gingiva, buccal mucosa, lips, and palate, with different topographic patterns according to EB type. EB patients whatever the age showed a similar lesion distribution. Simplex and dystrophic EB patients under 12 years old displayed higher lesion severity than junctional EB ones. Only dystrophic type exhibited microstomia and ankyloglossia.
CONCLUSION
Oral health status seemed to benefit from a close collaboration between dental practitioner and dermatologist, and from regular dental examination, starting at a young age and with a focus on prevention. The new appreciation of oral health involvement highlighted by this study is essential for EB patients care, regarding comorbidities and quality of life.
Topics: Humans; Child; Oral Health; Quality of Life; Dentists; Professional Role; Epidermolysis Bullosa; Blister
PubMed: 36268944
DOI: 10.3290/j.qi.b3479975 -
The Journal of Craniofacial SurgeryAgnathia-otocephaly complex (AOC), a first branchial arch defect, is characterized by mandibular hypoplasia or aplasia, ear abnormalities, microstomia, and macroglossia...
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.
Topics: Male; Humans; Child; Microstomia; Fibula; Craniofacial Abnormalities; Free Tissue Flaps; Mandible; Jaw Abnormalities; Mandibular Reconstruction
PubMed: 36217223
DOI: 10.1097/SCS.0000000000009017 -
Zhonghua Shao Shang Yu Chuang Mian Xiu... Sep 2022To investigate the clinical effects of ultra-pulsed fractional carbon dioxide laser (UFCL) in the treatment of mild to moderate microstomia after burns. A... (Observational Study)
Observational Study
To investigate the clinical effects of ultra-pulsed fractional carbon dioxide laser (UFCL) in the treatment of mild to moderate microstomia after burns. 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 test. 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 (=4.68, 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 values of 10.73 and 18.97, respectively, <0.01). The VSS score after the last treatment was 4.1±1.6, which was significantly better than that before treatment (=22.96, <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. 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.
Topics: Blister; Burns; Cicatrix; Female; Humans; Lasers, Gas; Male; Microstomia; Treatment Outcome
PubMed: 36177585
DOI: 10.3760/cma.j.cn501225-20220620-00252 -
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 -
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 -
National Journal of Maxillofacial... 2022We present a new technique of reconstruction of a partial defect of vermilion in a case of post traumatic defect of lower lip. The vermilion defect was reconstructed by...
We present a new technique of reconstruction of a partial defect of vermilion in a case of post traumatic defect of lower lip. The vermilion defect was reconstructed by the myomucosal flap taken from the labial side of lip posterior and below the anterior lip defect. The lower rounded part of this 3 cm × 2 cm rectangular flap was brought on the outer anterior lip defect through a hole made by splitting the orbicularis oris muscle below the vermilion and its lower end was rotated upward to reconstruct the defect in the anterior and inferior part of vermilion. The secondary defect on the inner lip was closed by mobilization of mucosa. The triangular-shaped skin loss of anterior lower lip and its underlying muscle was closed in anatomic layers meticulously after mobilization of muscle and skin on both sides. The orocutaneous communication created was closed after 3 weeks by a minor operation under local anesthesia. The mouth opening remained normal between the period of lip repair and closure of iatrogenic orocutaneous communication. Good shape with normal color of vermilion and length having proper lip fullness was achieved without any microstomia. The surface of vermilion was smooth without any irregularity. The single scar of lower lip was supple and mobile over the underlying muscle. There was no deglutition, chewing, labial phonation, or any drooling saliva problems. The procedure provided functionally and esthetically satisfying result. We authors have not found a similar technique of lip reconstruction in the literature.
PubMed: 35911806
DOI: 10.4103/njms.NJMS_197_20 -
Dermatologic Surgery : Official... Sep 2022
Topics: Humans; Hyaluronoglucosaminidase; Microstomia; Scleroderma, Localized; Scleroderma, Systemic
PubMed: 35834642
DOI: 10.1097/DSS.0000000000003543