Joubert Plus Syndrome with Self-Mutilation: A Case report.

BACKGROUND: Joubert syndrome is a very rare condition with dismal prognosis. It is characterized by several abnormalities including molar tooth sign on MRI. When coupled with mega cisterna magna- a feature of the Dandy Walker syndrome- it is categorized as Joubert plus syndrome. CASE REPORT: A 16 month old male child with Joubert syndrome was referred to the Pediatric Dentistry Department Clinic, Faculty of Dentistry Alexandria University, complaining of severe tongue and lower lip injury due to self-mutilation. He required multiple teeth extractions under general anesthesia to prevent further tongue and lip mutilation. CONCLUSION: Joubert plus syndrome is a very rare occurring condition. Because self-mutilation is sometimes fatal, a treatment plan tailored to each patient's need is mandatory. A multidisciplinary approach is recommended.

Efficacy of buccal versus intranasal route of administration of midazolam spray in behavior management of preschool dental patients.

OBJECTIVE: To assess the efficacy of aerosolized midazolam, introduced through buccal versus intranasal mucosa in managing uncooperative children undergoing dental treatment. METHOD AND MATERIALS: A crossover randomized controlled clinical trial included 36 children aged 3 to 5 years, rated I or II according to the Frankl scale and ASA I or II. Each child fulfilled the requirement of having a dental condition that needed treatment in two dental settings. They were randomly assigned to one of two groups; either buccal or intranasal aerosolized midazolam was administered at the first visit. The alternate route was implemented with a 1-week washout period in the second visit. Drug acceptance and time until optimum sedation were measured. Crying, sleeping, head resistance, and child overall behavior were assessed using modified Houpt scale. RESULTS: In total, 34 patients (95 %) were drowsy on optimum sedation. There was a statistically higher acceptance of buccal midazolam (P < .001). Onset of optimum sedation was more rapid for the intranasal group, with a mean of 15.50 ± 4.226 minutes (P < .001), while in the buccal group the mean was 22.97 ± 4.582 minutes. No statistical differences were recorded between the two groups in all behavior rating scales, except for crying where the intranasal group was statistically higher (P = .010). Regarding the overall behavior, there was no significant difference recorded between the two groups (P = .204). CONCLUSION: Aerosolized buccal midazolam was more tolerated by the patients. However, intranasal aerosolized midazolam had a more rapid onset of sedation. Both buccal and intranasal administrations of aerosolized midazolam are safe and effective.