A case of Beckwith-Wiedemann syndrome with peculiar dental findings.

BACKGROUND: Beckwith-Wiedemann syndrome (BWS, OMIM 130650) is a rare genetic disorder characterised by overgrowth, tumor predisposition and congenital malformations. Few systemic manifestations and oral features have been reported so far. CASE REPORT: We report on a case of BWS, describing all features expanding the knowledge on oro-dento-facial phenotypes, along with a review of the literature.

[A global approach to the dental care of children and young adults with trisomy 21. I. The dental and orofacial characteristics of Down's syndrome]. / Un approccio globale all'assistenza odontostomatologica nei bambini e nei giovani adulti con trisomia 21. Parte I. Caratteristiche dentali ed orofacciali della sindrome di Down.

On the basis of a full analysis of recently reported data, it is shown that manifold and quite characteristic problems of dental and orofacial nature requiring global consideration coexist in Down's syndrome. These are addressed one by one in order to stress the need for dental treatment and to provide for correct planning of interventions right from the early months of life in the case of a patient with Down's syndrome.

[A global approach to the dental care of children and young adults with trisomy 21. II. The planning of the preventive and interceptive care of Down's syndrome patients]. / Un approccio globale all'assistenza odontostomatologica nei bambini e nei giovani adulti con trisomia 21. Parte II. Pianificazione di un'assistenza preventiva ed intercettiva nei pazienti con sindrome di Down.

In this second part, the principles and modalities of a personal contribution to the realisation of a specific protocol that uses preventive and interceptive criteria to plan odontostomatological treatment in patients with Down's syndrome are presented. A special type of treatment is proposed. This is distinguished chronologically into five stages with periodic visits to specialist hospital structures, implying the start of treatment in Down's children at the age of 2-3 months and programmed continuation of such treatment right up to the age of at least 18 years.

[The evaluation of anxiety and the analysis of pain perception in Down's syndrome patients undergoing dental procedures]. / Valutazione dell'ansia e analisi della percezione dolorosa in pazienti con sindrome di Down sottoposti a procedure odontoiatriche.

Twenty Down's syndrome dental patients between 9 and 20 years of age have been identified in order to evaluate anxiety and pain induced by various kinds of dental treatment such as some "moderately painful" dental out-patient procedures (10 subjects), general anaesthesia dental treatment (5 subjects) and painless out-patient visit or routine orthodontic services (5 subjects). Each patient received a previous psychological evaluation to assess his intellectual and cognitive developmental degree. Anxiety was assessed twice, before and after intervention by means of a questionnaire; pain was tested in a subjective way during the procedure (or the control oral visit 12 hours after the g.a. treatment) and by a questionnaire in the post-treatment phase. This investigation indicates that anxiety increased in Down's syndrome subjects receiving out-patient interventions, remained at the original level in the general anaesthesia hospitalized patients, while it showed a clear reduction in the out-patient painless control group. Anxiety, however, proves to be influenced also by the patient's effective mental age so it my be lessened after painful dental treatment when the intellectual development is about or above that of a 7 year old boy. Our study could prove to be important since it underlines the need to assess, at least approximately, the Down's syndrome patient's cognitive development before giving dental treatment, especially if it is an out-patient intervention. This may not be necessary for routine free of pain dental visits.

[The incidence of malocclusion in the school population of the the Province of Trieste]. / Prevalenza delle malocclusioni nella popolazione scolastica della Provincia di Trieste.

The results of an epidemiological investigation on the prevalence of malocclusions in the school population of the Province of Trieste carried out on a sample of 2099 subjects aged 10 and 12 are reported. Subjects were classified according to Angle classes. Other malocclusion parameters such as overjet, overbite, median line deviation, crossbite and upper and/or lower crowding have also been investigated.

[The incidence of dental caries in the school population of the Province of Trieste]. / Prevalenza della carie dentaria nella popolazione scolastica della provincia di Trieste.

The results of an epidemiological investigation on the prevalence of dental caries in the school population of the province of Trieste carried out on a sample of 2954 children aged 6, 10 and 12 years are reported. The results were evaluated using the dmf and DMF epidemiological indices suggested by the WHO. Certain "intervention indices" were also calculated.

[Hypertrophy of the concha of the auricle. Details of the technic]. / Ipertrofia della conca del padiglione auricolare. Un dettaglio di tecnica.

Ear concha hypertrophy corrected by elliptical conchal excision often resulted in deformity due to lateral deviation of the anterior part of the concha. A modification of the method allows to avoid that deviation: the conchal ellipse is left connected to the conchal floor, is weakened by half thickness incisions parallel to the edge; the conchal floor thus flattened is sutured to the lateral side of the head by non absorbable suture.

[A simple method for proposing several profiles to patients undergoing rhinoplasty or profileplasty]. / Semplice metodo per proporre vari profili ai pazienti da sottoporre a rinoplastica o profiloplastica.

The authors describe a simple mean to visualize the several profiles which is possible to perform in the patient who is undergoing rhinoplasty or profiloplasty procedure. Failure in giving a clear opportunity of choice of a profile may lead to misunderstanding between surgeon and patient.

[Modified technique of Waters for retrograde intubation. Apropos of a case of difficult intubation]. / Technique d'intubation rétrograde de Waters modifiée. A propos d'un cas d'intubation difficile.

The authors describe a case of impossible intubation by classical means in an obese 37-yr old woman, affected with encephalopathy, hydrocephalus and severe psychomotor retardation. After failure of intubation by classical methods using a flexible introducer, the patient was intubated by Waters retrograde technique using an epidural catheter passed through the thyrocricoid membrane and recovered in the mouth. Following this guide the endotracheal tube passed the glottis fairly easily with the help of a flexible "mouse tail-ended" introducer (C-Mettro, Cook, 19.0.80) inserted into the same tube to facilitate alignment of distal tube and trachea. The authors conclude that the combination of epidural catheter and flexible introducer may facilitate the retrograde technique performance; they consider this technique to be an interesting alternative when endotracheal intubation is difficult or impossible by usual methods.