Treatment of macroglossia in a child with Weaver syndrome.

Weaver syndrome is a rare disorder, characterized by accelerated growth, advanced osseous maturation and distinct craniofacial features. Macroglossia and hypothyroidism are seldom mentioned in the literature as clinical findings associated with the syndrome. This paper describes a patient with Weaver syndrome, referred for consultation and treatment of macroglossia, who also suffered from congenital hypothyroidism. This is the first reported case of Weaver syndrome treated with partial glossectomy (tongue reduction). The paper describes the clinical findings of the syndrome, emphasizing the difficulty in identifying it, the indications for partial glossectomy and the authors' recommended operative technique.

Case report: desmoplastic fibroma of the mandible in a child presenting with TMJ dysfunction.

BACKGROUND: Desmoplastic fibroma of bone is a rare intraosseous benign but locally aggressive tumor of connective tissue origin. The lesion may affect the metaphyses of the long bones but it may also involve the skull bones and more specifically the mandible, with most lesions appearing in the ramus-angle area. Treatment is surgical although additional chemotherapy or radiation has been applied. CASE REPORT: A 10-year-old boy initially presented with restriction and deviation of mouth opening. Clinical and radiological examination revealed a tumor-like lesion of the mandible extending into the soft tissues, which on biopsy proved to be a desmoplastic fibroma. TREATMENT: The surgical treatment included peripheral ostectomy of the mandible, via an intraoral approach, for the removal of the lesion and restoration of the bone defect with an iliac bone autograft. FOLLOW-UP: The high recurrence rate of this type of lesion, demands a strict follow-up schedule. In the case presented, 5 years post-operatively, there are no signs of local recurrence. CONCLUSION: Changes in mouth opening when not attributed to obvious reasons, such as trauma, should make a dentist suspicious and lead to further investigation. In the case presented, a central lesion was revealed in the radiograph and the patient was referred and treated early. Extended surgical removal of the tumor, with wide margins, proved to be the appropriate treatment.

Anaerobic bacteria in jaw cysts.

The bacterial flora of the fluid content of 54 cysts of the jaws with a history suggestive of infection were studied. Forty-seven of these cysts were subsequently considered to be infected and from 78.6% of these, positive bacterial cultures were developed. Of the bacterial strains 89.2% were anaerobes and only 10.8% were pure aerobes or facultative anaerobes. Microorganisms isolated from each specimen ranged from 1 to 4 bacterial species. Gram positive anaerobic cocci revealed to be the most frequent bacterial group (36.9%), followed by Gram negative anaerobic rods (29.8%), Gram positive aerobic cocci being the third most common group of the isolates (19.0%). Antibiotic sensitivity tests of the isolated anaerobic cocci to a group of nine antibiotics revealed chloramphenicol and minocycline as the most effective. All anaerobic rods tested, were sensitive to metronidazole.