Michael W. Roberts

Non-Hodgkin's lymphoma of the hard palate mucosa and buccal gingiva associated with AIDS.

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Occurrence of multiple dentigerous cysts in a patient with the Maroteaux-Lamy syndrome (mucopolysaccharidosis, type VI)

The mucopolysaccharidoses (MPS) are a group of genetic lysosomal storage diseases. These diseases result from a defect in specific lysosomal enzymes required for the degradation of specific mucopolysaccharides. These incompletely degraded saccharides accumulate in tissues and are excreted in the urine. A general characteristic of these diseases is dysostosis multiplex. Dental complications can be severe and include unerupted dentition, dentigerous cystlike follicles, malocclusions, condylar defects, and gingival hyperplasia. This report examines multiple dentigerous cysts in a patient with a deficiency in N-acetylgalactosamine-4-sulfatase, Maroteaux-Lamy syndrome (MPS VI). The inability to hydrolyze the sulfate group from N-acetylgalactosamine-4-sulfate residue of dermatan sulfate due to a deficiency in this enzyme results in the accumulation of dermatan sulfate in tissues and its excretion in the urine. Examination of dentigerous cyst fluid revealed glycosaminoglycan content of 397 microgram per milliliter. Compositional analyses revealed 60% hyaluronic acid, 30% chondroitin 4- and -6-sulfate, and only 10% dermatan sulfate. This was consistent with dentigerous cyst fluid derived from persons without mucopolysaccharide-storage disorders but distinctly different from glycosaminoglycans assayed from other body fluids of this patient.

Tricho-dento-osseous syndrome: Features of the hair and teeth

Tricho-dento-osseous syndrome is a rare autosomal dominant hereditary disorder characterized by curly hair, enamel hypoplasia, taurodont teeth, and thickened cortical bone. A large kindred with this syndrome has been identified in North Carolina and characterized by pedigree analysis and clinical evaluation. Hair samples (N = 3) and an exfoliated primary tooth (N = 1) from persons with tricho-dento-osseous syndrome and hair and teeth from unaffected persons were examined. Scanning electron microscopy showed that the hair shaft morphology, diameter, and cuticular pattern and periodicity were similar for the tricho-dento-osseous syndrome and control samples. The affected tooth was examined using light microscopy and a scanning electron microscope that showed pitting hypoplasia of the enamel surface, an extremely large pulp chamber, and dentin that appeared structurally normal. The affected enamel was uniformly thin, only 10% the thickness of the control enamel. The bulk of this enamel showed no prism formation. This study indicates that although hair with tricho-dento-osseous syndrome often is clinically curly, it can be straight, and it does not appear to have unique ultrastructural features that are of diagnostic benefit. The dental characteristics of tricho-dento-osseous syndrome were the most consistent feature seen in this kindred.

Oral and maxillofacial complications associated with congenital sensory neuropathy with anhydrosis: Report of two cases

Pedigree data suggest that congenital peripheral sensory neuropathies are a group of disorders having an autosomal recessive mode of inheritance. They are manifested by an absence of pain sensation and are separated by clinical, laboratory, and neuropathological studies.’ Congenital sensory neuropathy with anhydrosis, first described by Swanson et al.,* has been referred to as type II familial dysautonomia and hereditary sensory neuropathy, type IV. To date, thirteen cases of this rare disorder have been reported. The disorder is characterized clinically by recurrent unexplained fevers, failure to thrive, absence of perspiration despite the presence of normal sweat glands, insensitivity to pain, self-mutilation, mild mental retardation and aplasia of the dental ename1.3*4 The autonomic dysfunction is apparently confined to the parasympathetic system since esophageal motility and serum catecholamine levels are within normal limits and neither erythematous skin blotching nor orthostatic hypotension occur. Histologic examination of the sural nerve shows a severe lack of nonmyelinated axons.

Nonnutritive, Low Caloric Substitutes for Food Sugars: Clinical Implications for Addressing the Incidence of Dental Caries and Overweight/Obesity

Caries and obesity are two common conditions affecting children in the United States and other developed countries. Caries in the teeth of susceptible children have often been associated with frequent ingestion of fermentable sugars such as sucrose, fructose, glucose, and maltose. Increased calorie intake associated with sugars and carbohydrates, especially when associated with physical inactivity, has been implicated in childhood obesity. Fortunately, nonnutritive artificial alternatives and non-/low-caloric natural sugars have been developed as alternatives to fermentable sugars and have shown promise in partially addressing these health issues. Diet counseling is an important adjunct to oral health instruction. Although there are only five artificial sweeteners that have been approved as food additives by the Food and Drug Administration (FDA), there are additional five non-/low caloric sweeteners that have FDA GRAS (Generally Recognized as Safe) designation. Given the health impact of sugars and other carbohydrates, dental professionals should be aware of the nonnutritive non-/low caloric sweeteners available on the market and both their benefits and potential risks. Dental health professionals should also be proactive in helping identify patients at risk for obesity and provide counseling and referral when appropriate.

Bulimia Nervosa Its Effect on Salivary Chemistry

Erosion of the dental hard tissues and enlarged parotid and submandibular saliva glands are commonly associated with bulimia nervosa. In 15 patients and controls, no significant difference was detected in the concentrations of potassium, chloride, calcium, urea nitrogen or albumin. There was also no evidence of olfactory dysfunction.

Dysphagia in bulimia nervosa

The findings on 13 patients with bulimia nervosa referred for evaluation of salivary glands and swallowing patterns are presented. Each patient completed a medical, oral, and social history questionnaire. A complete oral examination supported by appropriate dental radiographs and photographs was conducted. Unstimulated and stimulated parotid and submandibular saliva was collected. The presence or absence of pharyngeal and velar gag reflexes was ascertained. Real-time ultrasound scanning and barium swallow studies were used to evaluate the oral-motor functions while swallowing on 6 of the subjects. Activity of the pharynx, larynx, and esophagus was recorded during the videofluorographic studies. Saliva concentrations of amylase were determined in the referred subjects as well as 13 age-matched healthy controls. No significant difference was detected between the salivary gland flow rates and amylase concentrations of the two groups, whether stimulated or unstimulated. The pharyngeal gag reflex was absent in 9 of the 13 bulimic patients and a velar gag reflex could be elicited in only 1. All of the normal controls had both gag reflexes. All of the patients with bulimia were found to have abnormal oropharyngeal swallow patterns and an increased duration of dry swallow.

Dental root agenesis secondary to irradiation therapy in a case of rhabdomyosarcoma of the middle ear.

There have been only a few published reports on the dental-facial effects of radiation therapy and the subsequent oral management of these patients. A case involving a 13-year-old black male patient with a history of rhabdomyosarcoma is presented. The patient received 4,050 rads of radiation to the right middle ear when he was 2 years of age. His residual medical and dental difficulties are apparently complications from the initial therapy. Examination of the oral cavity revealed bimaxillary micrognathia and marked loss of vertical dimension. A Class II facial profile with Class I molar relationship was observed. The mandible was thin and hypoplastic, with a small knife-edge alveolar ridge. The remaining eighteen erupted permanent teeth were very mobile, and root development had ceased after only initial formation. All of the teeth except the first permanent molars were extracted, and immediate partial dentures were inserted at the time of surgery. A discussion of therapeutic considerations follows the case report.

Clinical evaluation of a composite resin system with a dentin bonding agent for restoration of permanent posterior teeth: A 3-year study

This study evaluated the clinical performance of a visible light-cured small particle bimodally filled hybrid condensable composite resin system that included a dentin bonding agent compared with an amalgam alloy in class II restorations of permanent teeth. A total of 108 restorations were placed in 34 patients. Fifty-three composite resin and 55 amalgam restorations were inserted. Each restoration was evaluated immediately after placement and then on an annual basis for a 3-year period using the Public Health Service (PHS) criteria. In addition, the Moffa-Lugassy scale was used to measure the loss of material on the occlusal surface of these materials. One hundred percent of the resin and amalgam restorations were evaluated, measured, and reasons for replacement were recorded over the 3-year period. There was no significant difference (p greater than 0.05) in the clinical performance of the composite resin and the amalgam when evaluated by the PHS criteria. Analyses of wear at each of the three annual recall periods did not reveal any significant difference (p greater than 0.05) between the two restorative materials when measured by the Moffa-Lugassy scale.

Enlarged dental follicles, a follicular cyst, and enamel hypoplasia in a patient with Lowe syndrome

Abstract An 11 ½-year-old body with Lowe syndrome presented with various dental abnormalities including delayed eruption, enamel hypoplasia, and enlarged follicles surrounding unerupted teeth.