Lingual Frenectomy in Joubert Syndrome.

AIM: Our aim is to present a case of a rare disorder, such as Joubert syndrome (JS) which required oral surgical intervention under general anesthesia at a very young age to help in achieving normal developmental milestones. BACKGROUND: Ciliopathies are an emerging class of diseases of which JS is a significant member. There have been very few cases of JS with its distinguishing features which have been reported in recent literature. CASE REPORT: We herewith present a case of JS who reported to our unit with a complaint of speech abnormality. Intraoral examination revealed a tongue-tie which was planned for surgical release. As any drugs that interfere with respiratory depression have to be used with utmost care, adequate precautions were taken in this case and the patient was treated under general anesthesia. The tongue-tie was released and associated ham-artomatous outgrowths from the floor of the mouth were also excised. Appropriate postoperative care was taken and the patient was discharged. CONCLUSION: The patient treated by us did not reveal any significant intraoperative or postoperative complications. Treatment of cases of JS required a team effort of multiple dental and medical specialists. Long-term follow-up of such patients is required so that they achieve normal chronological development. CLINICAL SIGNIFICANCE: Joubert syndrome being a rare disorder requires special anesthetic care during any surgical procedure, especially that involving the oral cavity. The need and timing of surgical intervention in such cases should be carefully assessed and appropriate precautions should be taken.

Glucose Levels and Hemodynamic Changes in Patients submitted to Routine Dental Extraction under Local Anesthesia with and without Adrenaline.

INTRODUCTION: In maxillofacial surgery, the simplest procedure that we perform is dental extraction. However, this simple procedure is challenged by the patient's poor medical condition. We generally use local anesthesia in combination with adrenaline; however, as we come across patients with diabetes mellitus and cardiovascular diseases who seek dental extraction, we need to be doubly cautious while using adrenaline. In this study, we intend to compare the effects of local anesthesia with adrenaline and local anesthesia without adrenaline on hemodynamic changes (blood pressure and pulse rate) and random blood sugar levels. The comparison is both within the group and between the two groups. MATERIALS AND METHODS: Healthy patients between the ages 20 and 60 years were included and randomized into two groups of 50 each. In one group, plain local anesthesia was used, whereas in the other group, local anesthetic solution containing adrenaline was used. Medically compromised patients were excluded from the study. Random blood sugar levels, blood pressures, and pulse rates were recorded in both groups before and 10 minutes after injecting the solutions. The findings were compared. RESULTS: When results are compared within the group, a modest increase in the blood sugar level was noted with the group receiving local anesthetic with adrenaline. However, blood pressure and pulse rate showed no significant difference. Similarly, when between-the-groups comparison was done, not a single variable showed any significant difference. CONCLUSION: The patients injected with local anesthesia containing adrenaline showed similar results to that observed in the patients injected with local anesthesia without adrenaline. However, there is a statistically significant rise in blood sugar levels when a local anesthetic is injected with adrenaline. CLINICAL SIGNIFICANCE: Dental extractions in healthy individuals can be safely performed with local anesthetic containing adrenaline. However, in diabetic patients, it should be cautiously used.

The Effect of Disinfectants and a Surface Wetting Agent on the Wettability of Elastomeric Impression Materials: An In Vitro Study.

BACKGROUND: This study was carried out to evaluate the effect of two commercially available chemical disinfectants namely sodium hypochlorite and glutaraldehyde and a surface wetting agent on the wettability of three high precision elastomeric impression materials, addition silicone, condensation silicone and polyether. MATERIALS AND METHODS: Three different types of elastomeric impression materials commonly used in prosthodontic practice were selected. The glutaraldehyde and sodium hypochloride solutions were employed to disinfect the impressions made with the above-mentioned elastomeric impression materials. True Blue surface wetting agent was selected. GBX contact angle analyzer was used to measure advancing and receding contact angle. RESULTS: The results of this study have demonstrated that the polyether impression material was the most hydrophilic of all the materials, followed by hydrophilic addition silicone. Condensation silicone was least hydrophilic. All materials showed improvement in the wettability when a topical surfactant was used. CONCLUSION: The short term disinfection of the three elastomeric impression materials does not affect the wettability of these impression materials.

Consanguineous Marital Union Resulting in a Progeny of Whistling-face Syndrome and Hemophilia: A Case Report.

Many different types of genetic disorders are noted to be prevalent among consanguineous progeny. Although the most common type of consanguineous union in all major societies is between first cousins, the importance of customary influences is apparent from variations in the specific types of first-cousin marriages contracted. Epidemiological data for the prevalence of whistling-face syndrome (WFS) are not available, but less than a hundred cases reported in the literature are noted. We are presenting a case where a consanguineous marriage resulted in two of their children presenting with WFS and one with hemophilia.

Freeman-sheldon syndrome presenting with microstomia: a case report and literature review.

Freeman-Sheldon syndrome (FSS), as first described by Freeman and Sheldon in 1938, is a morphologically well-defined syndrome that results in a dysmorphic status combining bone anomalies and joint contractures with characteristic facies. It is part of the nosologic group of pathologies currently known as distal arthrogryposis as reported by Hall et al. (Am J Med Genet 11:185-239, 1982 [1]). It is a rare disorder and its exact prevalence is unknown. Our objective is to report a case of FSS presenting with microstomia and add a brief review of the literature for similar cases.