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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 -
Acta Otorhinolaryngologica Italica :... Dec 2021Reconstruction of the lower lip is complex. The Colmenero flap is an effective albeit rarely described method for the repair of medium- to large-sized defects of the... (Review)
Review
OBJECTIVE
Reconstruction of the lower lip is complex. The Colmenero flap is an effective albeit rarely described method for the repair of medium- to large-sized defects of the lower lip.
METHODS
A retrospective review was carried out using data gathered from patients who had undergone Colmenero flap reconstruction of the lower lip at our centre between 2015 and 2020. We analysed demographic, histologic and anatomic variables as well as surgical results. This review assessed flap functionality based on proper mouth closure, absence of microstomia and oral competence.
RESULTS
Thirteen Colmenero flaps were performed in 9 patients, with the flap being used bilaterally in four cases. All patients had squamous cell carcinoma of the lower lip. The mean length of the reconstructed defect was 4.1 cm (ranging between 3-7.5 cm). None of the flaps exhibited signs of necrosis. Five patients required minor surgical touch up during the second procedure: two for dehiscence, two for oral leakage and one for esthetic improvement. All patients had excellent functional and aesthetic final outcomes.
CONCLUSIONS
The Colmenero flap is a good resource for medium- and large-sized lower lip reconstructions due to its reliability, limited complications, and good aesthetic and functional results.
Topics: Humans; Lip; Reproducibility of Results; Retrospective Studies
PubMed: 34825668
DOI: 10.14639/0392-100X-N1458 -
Journal of the Korean Association of... Oct 2021Sodium hydroxide or caustic soda is a corrosive agent that can cause extensive damage to the oral mucosa, lips, and tongue when ingested either accidentally or...
Sodium hydroxide or caustic soda is a corrosive agent that can cause extensive damage to the oral mucosa, lips, and tongue when ingested either accidentally or intentionally. These injuries include microstomia, shallow vestibule, ankyloglossia, speech impairment, loss of teeth and impairment in facial expression. In the present article, we report a unique case of tongue adhesion to the mouth floor and its surgical management in a 66-year-old female patient, who had a history of caustic soda ingestion.
PubMed: 34713815
DOI: 10.5125/jkaoms.2021.47.5.394 -
Translational Pediatrics Aug 2021Agnathia-otocephaly complex (AOC) is a rare and complex craniofacial malformation characterized by mandibular hypoplasia or agnathia, auricular fusion (synotia), and...
Agnathia-otocephaly complex (AOC) is a rare and complex craniofacial malformation characterized by mandibular hypoplasia or agnathia, auricular fusion (synotia), and microstomia with oroglossal hypoplasia or aglossia. It can occur alone or in combination with forebrain anomalies and cardiac malformations and has an extremely poor prognosis. Here, we report a case of AOC diagnosed by systemic fetal screening at a gestational age of 25 weeks. Ultrasound revealed that the S-curve formed by the normal lower jaw and lower lip had disappeared, the lower jaw and mandible were invisible, the mouth was extremely small, and the oral fissure was "pinhole-shaped". There was a cone-shaped perioral bulge. Both ears were located in the front side of the neck, and the right foot was inverted. Excessive amniotic fluid was observed. The absence of a mandible was confirmed on X-ray examination after induced abortion. Specimen observation showed that the ear positions were extremely low, and both earlobes were connected in the front side of the neck. It was particularly challenging to identify the development of the mandible and locate auricles during prenatal ultrasound diagnosis, and the prenatal diagnosis of AOC was confirmed by combining two-dimensional and three-dimensional ultrasound in our current case.
PubMed: 34584884
DOI: 10.21037/tp-21-235 -
World Journal of Clinical Cases Aug 2021Moebius syndrome (MBS) is a nonprogressive and rare congenital neuromuscular disorder involving the facial nerve and abductor nerve; it mainly manifests as facial...
BACKGROUND
Moebius syndrome (MBS) is a nonprogressive and rare congenital neuromuscular disorder involving the facial nerve and abductor nerve; it mainly manifests as facial paralysis and eye strabismus paralytic symptoms. Tissues in the oral cavity are also compromised, characterized by microstomia, micrognathia, tongue malformation, cleft lip, high arched palate or cleft palate, bifid uvula, and dental malocclusion. Therefore, dentistry plays a fundamental and crucial role in caring for these individuals. However, there is limited available data on MBS treatment, particularly regarding dental management.
CASE SUMMARY
This case report presents dental treatment of a 21-year-old man with MBS and discusses crucial interactions among oral complications of MBS. In this case, the patient was clinically characterized by congenital neuromuscular disorder, occlusal disorders, and tooth and gum problems. It is noteworthy that the patient presented early eruption of deciduous teeth 2 mo after birth, which has not been reported in other MBS cases and suggests a potentially new clinical manifestation of this syndrome. It is important to note that MBS cannot be cured, and oral manifestations of this syndrome can be managed by a multidisciplinary health care team that helps the patient maintain oral hygiene and dental health. After a series of oral treatments, no obvious poor oral hygiene, swollen gums, or abnormal imaging results were observed after 2 years of follow-up.
CONCLUSION
This case addressed the oral clinical manifestations of MBS and difficulties experienced during dental management, and suggested early tooth eruption as a potentially new clinical manifestation of this syndrome. Knowledge of the loop-mediated association among oral complications of this syndrome is essential to perfecting treatments.
PubMed: 34540988
DOI: 10.12998/wjcc.v9.i24.7269 -
Archives of Rheumatology Jun 2021This study aims to evaluate the effect of a home-based orofacial exercise program on the oral aperture (OA) of systemic sclerosis (SSc) patients.
OBJECTIVES
This study aims to evaluate the effect of a home-based orofacial exercise program on the oral aperture (OA) of systemic sclerosis (SSc) patients.
PATIENTS AND METHODS
This single-blind prospective randomized controlled study performed between March 2017 and January 2019 included 56 SSc patients (3 males, 53 females; mean age 52.9±10.6 years; range, 31 to 70 years) with an OA of <40 mm. Patients were divided into two groups. Group 1 (n=28, mean age 53.8±9.6 years) was given orofacial exercise program twice a day for one month in addition to oral hygiene care advices, followed by no activity for the next month. Group 2 (n=28, mean age 50.0±11 years) received oral hygiene care advices for the first month followed by the same exercise program for the next month. Patients' OA was measured at baseline, and at first and second months.
RESULTS
After the first month, OA increased in Group 1 (p<0.001), whereas no change was observed in Group 2 (p=0.579). At the end of two months, there was no additional increase in Group 1 (p=0.352), while there was a significant increase in Group 2 (p<0.001). There was no difference between OAs of the groups at the end of the trial (p=0.564).
CONCLUSION
Our results suggest that home-based orofacial exercise program improves OA of SSc patients.
PubMed: 34527921
DOI: 10.46497/ArchRheumatol.2021.8295 -
World Journal of Gastroenterology Aug 2021Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical... (Review)
Review
Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical manifestation of systemic sclerosis and is strongly related to esophageal dysmotility. However, there are multiple other contributing factors in each step in the physiology of swallowing that may contribute to development of severe dysphagia. The oral phase of swallowing may be disrupted by poor mastication due to microstomia and poor dentition, as well as by xerostomia. In the pharyngeal phase of swallowing, pharyngeal muscle weakness due to concurrent myositis or cricopharyngeal muscle tightening due to acid reflux can cause disturbance. The esophageal phase of swallowing is most commonly disturbed by decreased peristalsis and esophageal dysmotility. However, it can also be affected by obstruction from chronic reflux changes, pill-induced esophagitis, or Candida esophagitis. Other contributing factors to dysphagia include difficulties in food preparation and gastroparesis. Understanding the anatomy and physiology of swallowing and evaluating systemic sclerosis patients presenting with dysphagia for disturbances in each step can allow for development of better treatment plans to improve dysphagia and overall quality of life.
Topics: Deglutition Disorders; Esophagitis, Peptic; Humans; Manometry; Quality of Life; Scleroderma, Systemic
PubMed: 34497445
DOI: 10.3748/wjg.v27.i31.5201 -
Autopsy & Case Reports 2021Scleroderma is a rare autoimmune disease characterized by excessive collagen production. The oral manifestations of the patient with scleroderma can include microstomia,...
Scleroderma is a rare autoimmune disease characterized by excessive collagen production. The oral manifestations of the patient with scleroderma can include microstomia, xerostomia, and changes in the resorption teeth. We report the case of a 7-year-old female patient diagnosed with systemic scleroderma where photobiomodulation therapy was used to treat xerostomia associated with hyposalivation. She attended a pediatric clinic and presented with dry and rigid facial skin, trismus, xerostomia, malocclusion, and difficulty swallowing. Stimulated salivary flow was assessed before, during, and after treatment. Photobiomodulation therapy was conducted at four points at the sublingual glands with 660 nm, 100 mW, and 0.8 J/cm to each point; eight points at the parotid glands; and six points at the submandibular glands with 808 nm, 100 mW, and 0.8 J/cm for 8 seconds at each point. After this therapy, an increase in salivary flow, remission of the xerostomia, and an improvement in mastication and swallowing were observed. Photobiomodulation therapy was effective in controlling xerostomia in this pediatric patient, resulting in increased salivary flow and an improvement in her quality of life.
PubMed: 34277488
DOI: 10.4322/acr.2020.220 -
Faculty Reviews 2021Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs and has significant clinical sequelae.... (Review)
Review
Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs and has significant clinical sequelae. Management of SSc cutaneous disease remains challenging and often is driven by extracutaneous manifestations. Methotrexate is the typical first-line therapy for patients with early progressive cutaneous disease. However, in patients with diffuse progressive skin disease and inflammatory arthritis, methotrexate or rituximab monotherapy should be considered. First-line therapy for patients with concomitant myositis includes methotrexate or intravenous immunoglobulin (IVIG). For patients with both cutaneous findings and interstitial lung disease, studies have suggested the efficacy of mycophenolate mofetil or rituximab. Second-line therapies, including UVA-1 phototherapy, IVIG, or rituximab, can be considered in patients with disease refractory to first-line treatments. Clinical trials investigating the utility of emerging therapies such as abatacept and tocilizumab in the treatment of SSc are under way, and preliminary results are promising. Nonetheless, all patients with SSc benefit from a gentle skin-care regimen to alleviate pruritis, which is a commonly reported symptom. Additional cutaneous manifestations of SSc include telangiectasias, calcinosis cutis, microstomia, and Raynaud's phenomenon. Telangiectasia may be managed with camouflage techniques, pulse dye laser, and intense pulse light. Calcinosis cutis therapy is guided by the size of the calcium deposits, although treatment options are limited. Mouth augmentation and oral stretching exercises are recommended for patients with reduced oral aperture. Raynaud's phenomenon is treated with a combination of lifestyle modification and calcium channel blockers, such as amlodipine. Overall, SSc is a clinically heterogenous disease that affects multiple organ systems. Providers should assess extracutaneous involvement and use evidence-based recommendations to select the most appropriate therapy for patients with SSc.
PubMed: 34131653
DOI: 10.12703/r/10-43 -
Rheumatology Advances in Practice 2021SSc often leads to fibrotic cutaneous involvement of the face and reduced oral aperture, with impaired food intake and oral hygiene. Oral exercises can increase oral...
OBJECTIVE
SSc often leads to fibrotic cutaneous involvement of the face and reduced oral aperture, with impaired food intake and oral hygiene. Oral exercises can increase oral aperture but are often hampered by low adherence rates. The aim of this mixed method study was to explore the feasibility, patient satisfaction and effectiveness of two exercise programmes in SSc-associated microstomia.
METHODS
Adult patients suffering from SSc and microstomia (maximal oral aperture <40 mm) were randomized to two groups. Group A exercised with a jaw motion device (Therabite), whereas group B performed mouth-stretching exercises. Patients were expected to exercise for 10 min, three times per day for 3 months. Patients were evaluated at baseline, 3 months (period without intervention), 6 months (after 3 months of intervention) and 9 months (post-intervention). At month 6, semi-structured one-to-one interviews were conducted.
RESULTS
We included six women and three men, median age 60 years and median disease duration 8 years. At 6 months, all patients in group A ( = 4) and four in group B ( = 5) improved, with a median of 9 and 7 mm, respectively. The adherence ranged between 63.7 and 98.9% in group A and between 48.5 and 97.4% in group B. The interview revealed three themes: drivers, challenges and perceived improvement.
CONCLUSION
Both interventions improved maximal oral aperture. The adherence to therapy was high, but none of the patients considered it feasible to continue practising three times per day. Future studies are needed in order to define feasible long-term exercise programmes.
PubMed: 34013159
DOI: 10.1093/rap/rkab017