Gorlin’s syndrome — Report of a case and management of cystic lesions

Gorlin’s syndrome associated with multiple cystic lesions is a rare and uncommon condition. It is characterized by cutaneous basal cell carcinomas, multiple keratocysts in the jaw bones and skeletal anomalies. A case is presented with multiple odontogenic keratocyst and dentigerous cysts occurring in all quadrants of oral cavity with features suggestive of Gorlin’s Syndrome. The clinical presentation and the management of these multiple cystic lesions and a two year follow up study is presented.     

Salivary electrophoresis in the diagnosis of Sjögren's syndrome

Objective. The purpose of this study was to investigate the potential use of salivary electrophoresis for the diagnosis of Sjögren's syndrome.Methods. Salivary protein profiles of 43 patients and 39 healthy control subjects were compared on three different gel electrophoresis systems: sodium dodecylsulfate-polyacrylamide gel electrophoresis, anionic polyacrylamide gel electrophoresis, and immobilized pH gradient gel electrophoresis (isoelectric point, 3.5−5.0).Results. Most of the patients with Sjögren's syndrome exhibited an electrophoretic profile that was different from that of the healthy control subjects. Among the three gel electrophoresis systems examined, the immobilized pH gradient system appeared to be the most reliable for Sjögren's syndrome. Tests of accuracy revealed that the immobilized pH gradient system exhibits high specificity (97%), sensitivity (95%), positive predictive value (97%), and negative predictive value (95%) in the diagnosis of Sjögren's syndrome.Conclusions. Our results suggest that salivary electrophoresis is a potentially useful test for the diagnosis of Sjögren's syndrome.     

Reliability of ultrasonography and sialography in the diagnosis of sjögren's syndrome

Objectives. The purpose of this study was to evaluate quantitatively observer performance with ultrasonography and sialography in the diagnosis of parotid gland involvement of Sjögren's syndrome.Study design. Sixty-four parotid gland sialograms and 65 ultrasonograms were prepared for observer performance experiments. They included both modalities in 24 Sjögren's syndrome and 19 nonspecific parotitis cases, 21 normal parotid gland sialograms, and 22 normal ultrasonograms in healthy volunteers. The images were randomly sequenced and presented to five observers who were asked to describe several findings and finally to determine the imaging diagnosis by ranking the abnormal features and the diagnosis on a five-point-rating scale. Observer performance was evaluated on the basis of the reliability of findings interpreted and the diagnostic accuracy of each modality from observers' rating scores.Results. The diagnostic accuracy of sialography was very high, nearly perfect. The diagnostic accuracy of ultrasonography was lower than that of sialography; resulting from the lower incidence of characteristic findings in the disease groups and lower sensitivity on ultrasonography. In the differentiation of Sjögren's syndrome from the normal, however, the diagnostic accuracy of ultrasonography increased to 80% for all cases, and up to nearly 90% in the advanced sialographic stages.Conclusion. Ultrasonography is useful for the diagnosis of Sjögren's syndrome in the advanced stages. Taking the noninvasiveness of this technique into account, we recommend first applying ultrasonography to the diagnosis of Sjögren's syndrome and performing sialography when no positive findings are detected on ultrasonography.     

The role of a labial salivary gland biopsy in the diagnostic procedure for Sj?gren’s syndrome; a study of 94 cases

Objectives: The purpose of the present study is to examine the role of the outcome of the labial salivary gland biopsy (LSGB) in the diagnostic procedure of patients suspected of suffering from Sjögren’s syndrome (SS).Material and Methods: In a retrospective study the result of histopathological assessment of 94 consecutively taken labial salivary gland biopsies has been examined. For the diagnosis of SS the American-European Consensus Group classification (AECG, 2002) have been used. The outcome of the assessment has been discussed in relation to a recently reported classification provided by the American College of Rheumatology (ACR, 2012). Results: In the 94 LSGBs support for a diagnosis of SS has been encountered in 24 out of 26 patients with SS. In the 68 patients with a negative diagnosis of SS only six positive LSGBs were observed. The sensitivity of the labial biopsy amounted 0.92; the specificity was 0.91, while the positive predictive value and the negative predictive value amounted 0.80 and 0.97 respectively. LSGBs taken by or on the request of the departments of Rheumatology or Internal Medicine had a significant higher yield compared to LSGBs taken in other clinical departments.Conclusions: The LSGB may play a role in the diagnostic procedure of Sjögren’s syndrome when using either the AECG classification or the ACR classification. A LSGB should preferably taken after counseling for the possible presence of SS by a department of Rheumatology or Internal Medicine since the yield of such biopsies is much higher than in patients who have not been counseled by these departments prior to the taking of a LSGB. When using the ACR classification, a positive serologic result and a positive ocular test make the taking of a LSGB redundant. Only in case of a negative serologic outcome or a negative result of the ocular test a LSGB is indicated. Since both the serologic test and the ocular test carry hardly any morbidity, these tests should, indeed, be performed first before considering to take a LSGB. Key words:Labial salivary gland biopsy, Sjögren’s syndrome.     

Semi-quantitative analysis of salivary gland scintigraphy in Sjögren’s syndrome diagnosis: a first-line tool

Clinical Oral Investigations - The aim of this study was the assessment of semi-quantified salivary gland dynamic scintigraphy (SGdS) parameters independently and in an integrated way in order to...     

Burkitt’s lymphoma of the jaws in the Amazon region of Brazil

Objectives: To describe the clinicopathologic and immunohistochemical features of Burkitt’s lymphoma of the jaws in 7 patients of Northern Brazil. Study Design: Clinical data concerning gender, age, affected site, clinical presentation, symptomatology and follow-up were collected from the clinical files. Histopathology was complemented with a broad immunohistochemical panel and in situ hybridization for Epstein-Barr virus (EBV).Results: Most of the patients were infants and 5 out of 7 were males. The mandible was affected in 5 cases and all patients also presented abdominal involvement. All cases were positive for CD45, CD20, CD79a, CD10, Bcl-6 and EBV. Ki-67 proliferative index was approximately 100%. Six patients were treated with R-CHOP (Rituximab + Cyclophosphamide, Doxorubicin, Vincristine and Prednisolone) chemotherapy, and 2 of these died of the disease. One young adult patient refused treatment and died 3 months after initial diagnosis. Conclusions: Burkitt’s lymphoma of the jaws diagnosed in the Amazon region of Brazil present similar clinicopathologic features to those described in endemic areas of Africa, including EBV positivity. Key words:Burkitt’s lymphoma, EBV, Brazil, Amazon region.     

An intraoral surgical approach to the styloid process in Eagle’s syndrome

Purpose

The purpose of this study is to present an alternative method to the extraoral surgical approach to remove the elongated styloid process, the intraoral surgical approach, and discuss their advantages and disadvantages. A literature review is also presented.

Patients and methods

A casuistic of intraoral surgical approach to remove the elongated styloid process is presented in five patients.

Results

Four patients experienced postoperative moderate pain and trismus for 1 week. Bilateral surgery in one patient caused severe trismus, great discomfort, and moderate difficulty in breathing. All were followed up for 6 months and showed complete relief of the oral pharyngeal symptoms and complete improvement in functional ability.

Discussion

The advantages of the external approach are good visualization and reduced possibility of deep neck space infection. The disadvantages are an external scar, longer duration of surgery, and risk of injury to the facial nerve. The advantages of the intraoral approach are that the method is safe, simple, and less time consuming and an external scar is avoided. The disadvantages are possible infection of deep neck spaces, risk of injury to major vessels, and poor visualization.

Conclusions

Intraoral resection of the styloid process is a safe treatment technique of Eagle’s syndrome. It is not recommended the bilateral intervention at the same surgery, because of possible great discomfort at postoperative time.     

Clinical relevance of the utilization of vital Lugol’s iodine staining in detection and diagnosis of oral cancer and dysplasia

Clinical Oral Investigations - The aim of this study is to assess the clinical relevance of the utilization of vital Lugol’s iodine staining in detection of oral cancer and dysplastic lesions...     

The association between oral dryness and use of dry-mouth interventions in Sjögren’s syndrome patients

Clinical Oral Investigations - Sjögren’s syndrome patients use different dry-mouth interventions for the relieve of their oral dryness. Recently, it was shown that patients with...     

A preliminary study of the oral microbiota in Chinese patients with Sjögren’s syndrome

ObjectiveTo investigate the oral microbiota in Sjögren’s syndrome (SS) as opposed to that of healthy subjects.Study designTen patients with primary SS, [6 patients daily taking stable dosage of hydroxychloroquine (HC) and 4 patients taking hydroxychloroquine combined with Prednisone acetas (HC + PA)], along with 10 age-matched healthy controls were examined in regard of number of teeth, stimulated/unstimulated saliva secretion rate. Microflora on bilateral buccal mucosa was analyzed by high throughput sequencing. Statistical analyses were performed using the chi-square test, t test and Mann-Whitney U test. The Venn diagrams and Redundancy Analysis (RDA) were also used to evaluate effects of the disease and treatment on the bacterial community composition.ResultsThe relative abundance of Proteobacteria in SS group was lower compared to controls (P = 0.002). The total richness of genera for all groups was 339. The numbers of genera in SS group and in control group were 248 and 270, respectively. Some taxa with different prevalence and/or relative abundance were found between two groups.ConclusionsSS affects the oral microbiota and SS patients carry a different and less diverse microorganism community compared with healthy subjects. Prednisone acetas is an influence on the oral microbiome. This study provides a basic data on the oral flora in SS patients.     

Eagle’s syndrome – A case report and review of the literature

Eagle’s syndrome (ES) occurs when an elongated styloid process or calcified stylohyoid ligament causes recurrent throat pain or foreign body sensation, dysphagia, or facial pain. Additional symptoms may include neck or throat pain with radiation to the ipsilateral ear. The symptoms related to this condition can be confused with those attributed to a wide variety of facial neuralgias. ES can be diagnosed radiologically and by physical examination. The treatment of ES is primarily surgical. The styloid process can be shortened through an intraoral or external approach. In this paper a case of ES exhibiting unilateral symptoms with bilateral elongation of styloid process is reported and the literature is reviewed.     

Supra-gingival microbiota in Sjögren’s syndrome

The aim of this study was to investigate the microbiota of noncaries associated supra-gingival plaque (SGP) microbiology in Sjögren’s syndrome (SS). Stimulated whole saliva (SWS) and full-mouth SGP on intact tooth surfaces were collected from 26 primary (p) SS, 27 secondary (s) SS, and 29 control subjects for selective culture of lactobacilli, mutans streptococci, and aerobic and facultatively anaerobic gram-negative rods (AGNR). Predominant cultivable anaerobes from SGP of 11 randomly selected subjects from each group were investigated. Clinical and sialometric data were also collected. SS sufferers had significantly lower SWS flow rate and higher mean DMFT (decayed, missing, filled teeth), while pSS subjects also had lower SWS pH and fewer standing teeth. Lactobacillus acidophilus levels in SWS (P?=?0.012) and SGP (P?P?=?0.047). Non-oral species were isolated in greater proportions from pSS SGP (P?=?0.007). Subjects with pSS harbored increased levels of L. acidophilus and non-oral species, while SS sufferers generally had lower proportions of gram-negative species. The microbial composition of noncaries associated SGP indicates a potential source of increased caries risk in SS.