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Oral manifestation of Rett's syndrome.

Severe dental attrition has several causes but may be the result of bruxism in neurological or psychological disturbances. Rett's syndrome is an uncommon but increasingly recognised neurological disorder, characterised by acquired microcephaly, progressive dementia, bruxism, and loss of purposeful movements of the hands. A patient with Rett's syndrome who demonstrated masseteric hypertrophy, bruxism, and severe attrition is reported.

Bruxism↗

Oral manifestations and dental management of patients with hereditary angioedema.

Hereditary angioedema (HAE) is a genetic disorder in which affected individuals develop extensive, spontaneous angioedema of the extremities, gastrointestinal tract, and oropharynx. Dental treatment of unmedicated patients with HAE can trigger life-threatening pharyngeal edema. Previously, it was demonstrated that the administration of fresh frozen plasma (FFP) before surgery prevented angioedema attacks in 6 patients undergoing dental extractions. The present study examines the long term effectiveness of FFP in preventing angioedema from developing in 53 patients with HAE undergoing all types of dental treatment over a ten-year period. Only 3 of 45 patients (6.7%) covered with FFP had a minor angioedema attack after dental therapy in 10 yr. No attacks of moderate or severe swelling were seen. Attacks occurred independently of the disease activity of the patient and the trauma of the dental procedure. The use of fresh frozen plasma is effective in preventing attacks of angioedema in HAE patients undergoing all types of dental procedures.

Adolescent↗

Oral manifestations of Beckwith-Wiedemann syndrome.

A report of case of a patient with Beckwith-Wiedemann syndrome is presented. The macroglossia associated with the syndrome necessitated long-term dental follow-up to prevent and treat problems with occlusion, speech, and esthetic appearance that might develop. Early intervention might be required if feeding or respiratory difficulties, resulting from the macroglossia, arise. The diagnosis of the syndrome should alert the dentist to the increased incidence of intra-abdominal malignancies and the need for periodic observation.

Beckwith-Wiedemann Syndrome↗