Nevoid basal cell carcinoma syndrome: a review of the literature and a report of a case.

The purpose of this paper is to report the development of multiple odontogenic keratocysts (OKCs) in a 15-year-old female with nevoid basal cell carcinoma syndrome (NBCCS) and review the literature pertinent to NBCCS. Although more than 100 abnormalities have been reported in NBCCS, the development of OKCs is one of its principle features. In view of this, the patient was subjected to further medical, dermatological and radiographic investigation. Multiple basal cell naevi and skeletal anomalies associated with NBCCS were found. Because of the autosomal dominant inheritance of this syndrome, the patient's family was then investigated. The patient's father was found to have multiple OKCs. The report highlights the need for vigilance in considering the diagnosis of NBCCS in all cases of OKCs, particularly those affecting young patients.

Oral health behaviour of schoolchildren and parents in Jordan.

OBJECTIVES: The objectives of the study were: (i) to assess the level of dental knowledge and attitudes towards child dental care among parents in Jordan, (ii) to analyse the oral health care habits of schoolchildren and parents, and (iii) to evaluate the oral hygiene habits and use of professional dental services of children in relation to socio-demographic conditions. DESIGN: Cross-sectional, self-administered questionnaires for parents. SAMPLE AND METHODS: National representative sample of 6-16-year-olds living in urban areas (n = 1556, children and parents, response rate 92%, 48.7% boys, 51.3% girls). RESULTS: In all, 80% of the parents knew about the harmful effect of sugar and 79% thought that poor oral hygiene may induce dental caries. In addition to proper oral hygiene (79%) and restriction of sugar/sweets (42%), 36% of the parents emphasized regular dental visits for the prevention of dental disease in children. However, most children saw a dentist for symptomatic reasons only (86%), while 11% attended for dental check-ups. At their last visit to the dentist, 49% of the children had tooth extraction and only 8% had preventive services (fissure sealing). Toothbrushing at least twice a day was reported for 31% of the children; 14% of children aged 6-9 years had assistance from adults in brushing. Dental care habits of children were highly affected by dental visiting habits of parents, and variations by level of education of parents were also found. CONCLUSIONS: The discrepancy between dental knowledge and attitudes of parents and oral health care practices indicate the need for oral health education. School-based oral health promotion programmes should be established in Jordan to influence the oral health behaviour of children and parents and to avoid further deterioration in their oral health.

Congenital macroglossal angiodysplasia ("Lymphangioendotheliomatosis").

A case of congenital lingual angiodysplasia with macroglossia in a 5-year-old girl is presented. A diffusely enlarged tongue was present at birth and continued to grow as the child aged. It was accompanied by impaired speech, difficulty in eating and breathing, and sleep apnea, necessitating surgical intervention. The fundamental lesion represents a complex vascular malformation of the lymphangioma-hemangioma type, involving extensively the deep musculature of the tongue. Multifocal and multicentric cavernous lymphangioma-like and hemangioma-like areas merge with benign angioendotheliomatous-like foci in a background of variable muscle degeneration and marked fibrosis. Neither a borderline nor an overtly malignant vasoformative neoplasm was present. Because of its distinctively widespread, multicentric intramuscular distribution, this lesion may be construed as a diffuse variant of lingual lymphangioma-hemangioma malformation, closely resembling a previously described case of macroglossal lymphangioendotheliomatosis.

Desmoplastic fibroma of the mandible: report of a case.

This paper describes a case of desmoplastic fibroma in a 4-year-old patient with a history of a small slowly growing swelling at the right angle of the mandible over a 3-month period. Desmoplastic fibroma was diagnosed on histological and immunohistochemical bases. The lesion responded well to thorough curettage and has not shown signs of recurrence 3 years after the surgical intervention. The clinical picture, the pathology and the management of the case are described, and the differential diagnosis and treatment are discussed.

Hereditary gingival fibromatosis in a family with the Zimmermann-Laband syndrome.

Hereditary gingival fibromatosis is frequently an isolated condition of little consequence apart from a cosmetic problem and occasional associations with hypertrichosis and/or epilepsy. There are, however, several uncommon or rare eponymous syndromes described in which gingival fibromatosis can be a feature: these include the Zimmermann-Laband, Murray-Puretic-Drescher, Rutherfurd, Cowden and Cross syndromes. This paper describes two siblings with features of the rare Zimmermann-Laband syndrome and discusses the major aspects of this and other eponymous syndromes that may be associated with hereditary gingival fibromatosis.

Hepatitis B vaccination and infection control practices of Jordanian clinical dental students.

The knowledge of occupational hazards from blood-borne viruses, and practices related to cross-infection control were studied in Jordanian dental students. A survey in July 1990 showed that over 90% were aware of the existence of vaccines against hepatitis B virus and most appreciated the possible long-term hepatic sequelae of hepatitis B virus infection. Nearly 50% had already been immunized against hepatitis B and a further 4% intended to be vaccinated. Autoclaves were used by more than 80% for the sterilization of dental instruments.

Effects of a 1% chlorhexidine gel during the healing phase after inverse bevel mucogingival flap surgery.

A split-mouth double-blind trial was designed to compare the effects of chlorhexidine and placebo gels during the healing phase following mucogingival flap surgery. Fifteen patients were treated with 30 inverse bevel flap procedures, one on each side of the maxilla, with an interval of 4 weeks between each procedure. Gel was applied to the gums using a toothbrush once daily. Comparison of results revealed no significant differences in Plaque Index, Gingival Index, crevicular fluid or depth of pockets. More pain and swelling were recorded on the side treated with placebo gel, and more patients indicated that they preferred the chlorhexidine gel. The results were comparable to previous investigations using penicillin and suggest that, because of the reduced pain and swelling, chlorhexidine gel may be a suitable alternative to penicillin after periodontal flap surgery.