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Diagnostics (Basel, Switzerland) Feb 2024oral alterations in Systemic Sclerosis (SSc) patients are widespread and include microstomia, periodontitis, telangiectasias, mandibular resorption, bone lesions, and...
BACKGROUND AND OBJECTIVES
oral alterations in Systemic Sclerosis (SSc) patients are widespread and include microstomia, periodontitis, telangiectasias, mandibular resorption, bone lesions, and xerostomia. This cross-sectional study aims to evaluate the differences between SSc patients (cases) and healthy subjects (controls) regarding oral manifestations, quality of life (QoL), and microcirculation alterations.
METHODS
plaque index (PCR), periodontal index (PSR), DMFT, salivary flow rate, and buccal opening were measured by expert clinicians. S-HAQ test, the Self-Rating Anxiety State (SAS), the Self-Rating Depression Scale (SDS), and the WHOQOL-BREF test were administered to patients to evaluate their QoL. Microvascular alterations were assessed by oral videocapillaroscopy, performed on gingival and labial mucosa. A statistical analysis was conducted to find significant differences between healthy people and SSc patients.
RESULTS
59 patients were enrolled in this study. Standard salivary flow is significantly more frequent in controls, while xerostomia, reduced flow, microstomia, lip retraction, and periodontitis are significantly more frequent in the cases. Gingival capillaroscopy showed differences concerning loop visibility, thickening of the gum, tortuosity of gingival loops, and reduced gingival density. Labial capillaroscopy demonstrates that visibility of the labial loops, the labial ectasias, and the tortuosity of the loops are significantly associated with the presence of scleroderma. Hand and facial deformities, hypomobility of the tongue, cheeks, lips, microstomia, and xerostomia significantly compromised the quality of life of SSc patients, which was significantly worse among them. Moreover, oral videocapillaroscopy could be a proper diagnostic method to detect oral microcirculation alterations. SSc patients often present ectasias, rarefaction of the reticulum, microhemorrhages, and megacapillaries, which negatively impact their oral health.
CONCLUSIONS
periodontitis, reduced salivary flow, and microstomia could be considered SSc oral manifestations. Joint deformities, facial appearance, and comorbidities significantly reduce the QoL of SSc patients compared to healthy subjects. Oral videocapillaroscopy could be an innovative and reliable technique to detect oral microcirculation anomalies.
PubMed: 38396477
DOI: 10.3390/diagnostics14040437 -
Journal of the American Academy of... Nov 2023Reduced oral aperture (ROA), resulting from systemic sclerosis (SSc), is a debilitating condition with limited treatment options. Improvement in oral function has been...
BACKGROUND
Reduced oral aperture (ROA), resulting from systemic sclerosis (SSc), is a debilitating condition with limited treatment options. Improvement in oral function has been reported with perioral administration of botulinum toxin type A.
OBJECTIVE
To prospectively evaluate the efficacy of onabotulinumtoxinA (onabotA) injection in improving oral opening and quality of life in SSc patients with ROA.
METHODS
Seventeen women with SSc and ROA were treated with 16 units of onabotA in 8 different sites around the cutaneous lips. Measurements of maximum mouth opening were taken before treatment, at 2 weeks posttreatment, and at 3 months posttreatment. Function and quality of life were also assessed via surveys.
RESULTS
Interincisor and interlabial distances were significantly increased 2 weeks after treatment with onabotA (P < .001) but not 3 months after. Subjective improvement in quality of life was noted.
LIMITATIONS
This single-institution study enrolled 17 patients and did not have a placebo control group.
CONCLUSION
OnabotA appears to have a strong short-term symptomatic benefit in patients with ROA due to SSc, with possible benefit to quality of life.
PubMed: 37301288
DOI: 10.1016/j.jaad.2023.04.069 -
Arthritis Research & Therapy Dec 2019Systemic sclerosis is a systemic connective tissue disease characterized by endothelium damage, fibrosis, and subsequent atrophy of the skin. Perioral fibrosis produces...
BACKGROUND
Systemic sclerosis is a systemic connective tissue disease characterized by endothelium damage, fibrosis, and subsequent atrophy of the skin. Perioral fibrosis produces a characteristic microstomia together with microcheilia, both of which cause severe difficulties and affects patients' daily life, such as eating and oral hygiene. Since there are no effective and specific therapies, we have aimed at evaluating the response to filler injections of hyaluronic acid together with platelet-rich plasma.
METHODS
Ten female patients aged between 18 and 70 were included in this study. Each patient was treated with three filler injections of hyaluronic acid and platelet-rich plasma at an interval of 15 to 20 days. Follow-up check-ups were recorded 1, 3, and 24 months after the end of the treatment. During the therapy and the subsequent follow-up, we evaluated the mouth's opening, freedom of movement of the lips, and skin elasticity.
RESULTS
After the treatment, patients had achieved good results already after the first injection and the improvement was maintained in the following months, up to 2 years. In particular, 8 (80%) patients showed a greater mouth's opening and increased upper lip's thickness during 1-month follow-up and maintained these results after 2 years (maximum mouth's opening T0 47.61; T3 49.23; T4 48.60 p < 0.0001. Upper lip's thickness T0 4.20; T3 4.75; T4 4.45 p < 0.0001). Moreover, distance between upper and lower incisors (T0 27.05; T3 29.03; T4 28.14 p < 0.0001), inter-commissural distance (T0 49.12; T3 51.44; T4 50.31: p < 0.0001), and lower lip's thickness (T0 3.80; T3 4.85, 5.10; T4 4.25; p < 0.0001) were increased in all of patients in 1-month follow-up, keeping these benefits after 24 months and having a significant increase of skin elasticity 1 month after the end of therapy.
CONCLUSIONS
Our study demonstrates that filler injections of hyaluronic acid and platelet-rich plasma represent an efficient local therapeutic alternative for patients affected by scleroderma. The treatment has significantly improved patients' quality of living.
Topics: Adult; Elasticity; Female; Humans; Hyaluronic Acid; Lip; Middle Aged; Platelet-Rich Plasma; Prospective Studies; Quality of Life; Scleroderma, Systemic; Skin; Treatment Outcome; Viscosupplements
PubMed: 31836018
DOI: 10.1186/s13075-019-2062-0 -
BMC Oral Health Feb 2024Moebius syndrome (MS) is a rare, non-progressive, neuromuscular, congenic disease involving the oral maxillofacial region. The present study aimed to describe the oral...
BACKGROUND
Moebius syndrome (MS) is a rare, non-progressive, neuromuscular, congenic disease involving the oral maxillofacial region. The present study aimed to describe the oral and extraoral findings in MS patients and their comprehensive dental management.
METHODS
A digital search was carried out in PubMed/MEDLINE, Scopus, Web of Science, and Google Scholar, restricted to articles in English from Jan 01, 2000, to Apr 02, 2023, following PRISMA guidelines. The methodological quality of the studies was evaluated following the JBI guidelines. Qualitative analysis was carried out on the overall result, extraoral and intraoral manifestations, considering dental management as appropriate.
RESULTS
Twenty-three studies were included, and a total of 124 cases of patients with MS were analyzed. The 82% of patients with MS were younger than 15 years of age. The most frequent extraoral manifestations were blinking and visual problems (78,22%), malformations of the upper and lower limbs (58,22%), bilateral facial paralysis (12,90%), lack of facial expression (12.09%), and unilateral facial paralysis (6,45%). On the other hand, the most frequent oral manifestations were tongue deformities (78,22%), micrognathia (37,90%), labial incompetence (36,29%), cleft palate (22,87%), gothic palate (16,12%), microstomia (15,32%), anterior open bite (15,32%), dental caries (8,87%), and periodontal disease (8,06%). The majority of MS patients were treated by pediatric dentistry (60,86%), using a surgical approach (56,52%), and orthodontic and orthopedic maxillary (43,47%) followed by restorative (39,13%), and periodontal treatments (21,73%).
CONCLUSIONS
This systematic review demonstrates that patients with MS present a wide variety of oral and extraoral manifestations, for which dental treatments are planned and tailored to each patient in accordance with oral manifestations. These treatments encompass problem resolution and oral health maintenance, incorporating recent techniques in managing and treating patients with MS.
Topics: Child; Humans; Mobius Syndrome; Facial Paralysis; Dental Caries; Cleft Palate; Dental Care
PubMed: 38321523
DOI: 10.1186/s12903-024-03968-6 -
JA Clinical Reports Jul 2023Freeman-Sheldon syndrome (FSS) is a rare disorder characterized by specific deformities of the extremities and face. There have been no reports of open-heart surgery in...
BACKGROUND
Freeman-Sheldon syndrome (FSS) is a rare disorder characterized by specific deformities of the extremities and face. There have been no reports of open-heart surgery in pediatric patients with FSS.
CASE PRESENTATION
We present the case of an 8-year-old girl with FSS who underwent atrial septal defect closure. Tracheal intubation was uncomplicated, although the patient had microstomia. Inhalational anesthetics and dopamine antagonists were avoided intraoperatively and perioperatively. We chose dexmedetomidine as an adjuvant for postoperative pain management contributing to adequate analgesia and early extubation without causing respiratory depression.
CONCLUSIONS
Anesthetic management of FSS requires consideration for airway management and prevention of malignant hyperthermia and respiratory complications. We successfully managed the case avoiding the use of malignant hyperthermia-triggering drugs.
PubMed: 37438578
DOI: 10.1186/s40981-023-00633-9 -
Dental Research Journal 2020Microstomic patients may experience a significant limitation in the mandibular opening. The prosthetic rehabilitation of microstomic patients presents difficulties at...
Microstomic patients may experience a significant limitation in the mandibular opening. The prosthetic rehabilitation of microstomic patients presents difficulties at all stages, from preliminary impressions to prosthetic fabrication. This clinical report described the method of fabrication of sectional denture for patients with microstomia caused by burns. The denture was fabricated in two pieces, and patients inserted and removed the denture easily.
PubMed: 32435440
DOI: No ID Found -
Limited cutaneous systemic sclerosis: Total rehabilitation with fixed prosthesis on dental implants.Journal of Scleroderma and Related... Oct 2021Limited cutaneous systemic sclerosis with special manifestations (calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is...
INTRODUCTION
Limited cutaneous systemic sclerosis with special manifestations (calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is part of the group of connective tissue diseases, these rare autoimmune systemic pathologies cause thickening and hardening of tissues in different parts of the body and can lead to complex disorders. Oral manifestations of systemic sclerosis may include limited ability to open the mouth, xerostomia, periodontal disease, enlarged periodontal ligament, and bone resorption of the jaw.
CASE DESCRIPTION
A 54-year-old Caucasian patient presented with oral pain, swallowing, phonation and chewing difficulties associated with dental instability, hygiene/handling difficulties and her main problem with microstomia, which prevented her from removing the skeletal prosthesis for 4 years, depriving her of social life. Gradual treatment with dental implants was diagnosed and planned to support a fixed total denture adapted to the ridge with self-cleaning characteristics. After implant insertion, panoramic radiographs with standardized parameters were taken to compare crestal bone levels at the time of prosthesis placement and with 10 years of follow-up.
CONCLUSION
The average crestal bone loss of the 12 implants after the 10 years of follow-up was 1.26 mm for the maxilla and 1.17 mm for the mandible. The survival of the 12 support implants of two total fixed prostheses in a clinical/radiographic follow-up of 10 years was 100%. After 10 years of follow-up, the 12 implants inserted had a bone loss similar to that of healthy patients and no pathologies were registered, recovering function, aesthetics, and self-esteem. This therapy must be implemented before the interincisal distance decreases to 30 mm to allow intraoral surgical/prosthetic access. Implant-supported total fixed rehabilitation is a viable, predictable, and recommended therapy in patients with limited cutaneous systemic sclerosis.
PubMed: 35387213
DOI: 10.1177/23971983211004362 -
The EPMA Journal Mar 2023This study assessed sleep quality in patients with burn scars and investigated risk factors of sleep disorders to guide clinical therapy. From the strategy of...
PURPOSE
This study assessed sleep quality in patients with burn scars and investigated risk factors of sleep disorders to guide clinical therapy. From the strategy of predictive, preventive, and personalized medicine (PPPM/3PM), we proposed that risk assessment based on clinical indicators could prompt primary prediction, targeted prevention, and personalized interventions to improve the management of sleep disorders present in patients with burn scars.
METHODS
This retrospective study recruited patients with burn scars and healthy volunteers from the Shanghai Burn Treatment Center between 2017 and 2022. Relevant information and data, including demographic characteristics, scar evaluation, and sleep quality, were obtained through the hospital information system, classical scar scale, and self-report questionnaires. Sleep quality was assessed using the Pittsburgh Sleep Quality Index (PSQI) and monitored using a cardiopulmonary-coupled electrocardiograph. Pain and pruritus were assessed using the visual analog scale (VAS). Scar appearance was assessed using the modified Vancouver scar scale (mVSS).
RESULTS
The sample was comprised of 128 hypertrophic scar (HS) patients, with 61.7% males, a mean age of 41.1 ± 11.6 years, and burn area of 46.2 ± 27.9% total body surface area (TBSA). Patients with PSQI ≥ 7 accounted for 76.6%, and the global PSQI score was 9.4 ± 4.1. Objective sleep data showed that initial enter deep sleep time, light sleep time, awakening time, light sleep efficiency, and sleep apnea index were higher but deep sleep time, sleep efficiency, and deep sleep efficiency were lower in HS patients than that in healthy controls. Preliminary univariate analysis showed that age, hyperplasia time of scar, narrow airway, microstomia, VAS for pain and pruritus, and mVSS total (comprised of pigmentation, vascularity, height and pliability) were associated with the PSQI score ( < 0.1). Multivariable linear regression showed narrow airway, VAS for pain and pruritus, and mVSS specifically height, were the risk factors for PSQI score ( < 0.1).
CONCLUSIONS
This study model identified that narrow airway, pain, pruritus and scar appearance specifically height may provide excellent predictors for sleep disorders in HS patients. Our results provided a basis for the predictive diagnostics, targeted prevention, and individualized therapy of somnipathy predisposition and progression of HS patients in the setting of PPPM/3PM health care system, which contributed to a paradigm shift from reactive cure to advanced therapy.
PubMed: 36684850
DOI: 10.1007/s13167-022-00309-x -
Medical Research Archives Oct 2023Systemic sclerosis (SSc) is a rare autoimmune disorder with pathological manifestations affecting multiple organ systems. Few studies have examined perioperative...
BACKGROUND
Systemic sclerosis (SSc) is a rare autoimmune disorder with pathological manifestations affecting multiple organ systems. Few studies have examined perioperative outcomes in patients with this disorder. The primary aim of this retrospective single-center comparative cohort analysis was to estimate the incidence of select perioperative complications in a population of SSc patients. In an exploratory analysis, we analyzed the relationship between SSc and susceptibility to select perioperative complications when treated at a large quaternary-care institution.
METHODS
We conducted a single-center retrospective, comparative cohort study to compare perioperative outcomes in a SSc (n=258) and a frequency matched control cohort (n=632). We analyzed for the presence of major composite infection (MCI), major adverse cardiac events (MACE), 30-day readmission, 30-day mortality, in-hospital complications, length of stay and airway management outcomes.
RESULTS
MCI was higher in the SSc compared to the control cohort [adjusted odds ratio (ORadj)=5.02 (95%CI: 2.47-10.20) p<0.001]. Surgical site infection (3.5% vs. 0%, p<0.001), and other infection types (5% vs. 0%, p<0.001) were higher in the SSc cohort. MACE was not significantly different between SSc vs. Control groups [6.2% vs. 7.9%, ORadj=1.33 (95%CI: 0.61-2.91) p=0.48]. Higher rates of limited cervical range of motion (13.6% vs. 3.5%, p<0.001), microstomia (11.5% vs. 1.3%, p<0.001) and preoperative difficult airway designation (8.7% vs. 0.5%, p<0.001) were observed in the SSc cohort. Bag mask ventilation grade was similar between groups (p=0.44). After adjustment, there was no between-group difference in Cormack-Lehane grade 3 and 4 view on direct laryngoscopy in SSc patients [ORadj = 1.86 (95%CI: 0.612 -5.66) p=0.18] but evidence of higher rates of video laryngoscopy [ORadj= 1.87 (95%CI:1.07 - 3.27) p=0.03]. Length of stay [median: 0.2 vs. 0.3 days, p=0.08], 30-day mortality [1.2% vs. 0.6%, ORadj=2.79 (95%CI: 0.50-15.6) p=0.24] and readmission [11.5% vs. 8.1%, ORadj=1.64 (95%CI: 0.96 - 2.82) p=0.07] were not statistically significant.
CONCLUSIONS
SSc patients demonstrate mostly similar rates of MACE, 30-day mortality, length of stay intraoperative and airway complications. There is evidence of increased risk of overall 30-day MCI risk and readmission after endoscopic procedures.
PubMed: 38037571
DOI: 10.18103/mra.v11i10.4606 -
The Journal of International Medical... Nov 2021Diffuse cutaneous systemic sclerosis may occur in women of childbearing age. Pregnancies in this population are associated with a markedly increased risk of adverse... (Review)
Review
Diffuse cutaneous systemic sclerosis may occur in women of childbearing age. Pregnancies in this population are associated with a markedly increased risk of adverse obstetric and maternal outcomes even before the onset of symptoms related to sclerosis. We report a case involving the management and outcome of pregnancy in a 30-year-old woman with diffuse cutaneous systemic sclerosis. The course of her pregnancy was good and was assisted by a group consultation including obstetricians and rheumatologists. Vaginal delivery was the patient's preferred choice because she had irregular skin tightness in her lower abdominal skin. She underwent induction of labor and combined spinal-epidural analgesia, and successfully delivered. Importantly, these pregnancies need to be planned, where possible, to allow the opportunity to counsel women and their partners in advance and to decrease any risks. These pregnancies should be considered high risk, and they require close antenatal monitoring and good supervision from an expert multidisciplinary team experienced in high-risk pregnancies. The management of delivery for patients with cutaneous systemic sclerosis is challenging, and vaginal delivery with labor analgesia is an alternative option to cesarean section.
Topics: Adult; Analgesia, Epidural; Cesarean Section; Delivery, Obstetric; Female; Humans; Labor, Obstetric; Pregnancy; Scleroderma, Diffuse
PubMed: 34851779
DOI: 10.1177/03000605211060160