Mucopolysaccharidosis: MPS VI and associated delayed tooth eruption.

Eight cases of mucopolysaccharidosis Type VI (Maroteaux-Lamy syndrome) are reviewed and two cases are presented in detail. Developmental dental anomalies including unerupted and impacted permanent teeth and associated hyperplastic tooth follicles are seen frequently in MPS patients. The surgical implications and management are discussed. All patients reviewed had significant cardiovalvular disease. It is essential for the primary medical provider to establish early basic dental care and evaluation for delayed eruption of primary and permanent dentition. This will probably minimize the possibility of infective endocarditis and allow for early treatment of impacted teeth.

Oral discomfort at menopause.

The relationship between oral discomfort and menopause was assessed in 149 women who were divided into three groups determined by response to a questionnaire. The groups consisted of 50 premenopausal women (30 to 43 years old), 47 menopausal women not receiving medical treatment for symptoms of menopause (37 to 66 years old), and 52 menopausal women attending a menopause clinic before and during treatment for menopausal symptoms (30 to 63 years old). The last group had a general medical assessment, including psychological and oral examinations with oral smears and cultures, and the following blood tests: full blood examination, follicle-stimulating hormone, oestradiol, folate, vitamin B12, iron, and total iron binding capacity. Of this last group, 33% reported oral discomfort but had no obvious organic abnormalities that could account for this symptom. The prevalence of oral discomfort was found to be significantly higher in perimenopausal and postmenopausal women (43%) than in premenopausal women (6%). The results also showed an association between oral discomfort and psychological symptoms in menopausal women. Approximately two thirds of the menopausal women with oral discomfort, but without oral clinical signs, found that this symptom was relieved after hormone replacement therapy. The results indicated that oral discomfort is a common symptom of menopause, that it often occurs without overt clinical signs, and that it frequently resolves during appropriate hormone replacement therapy.

Maxillary stability following downgraft and/or advancement procedures with stabilization using rigid fixation and porous block hydroxyapatite implants.

The stability of results using bone plates for skeletal stabilization and porous block hydroxyapatite implants as a bone graft substitute were evaluated in maxillary downgrafts, maxillary advancements, and modified LeFort III midface advancements. The records of 24 patients with greater than 5 mm of forward or downward movement were evaluated cephalometrically, analyzing the presurgical, immediate postsurgical, and longest follow-up radiographs. The 11 maxillary downgraft, 14 maxillary advancement, and three midface advancement procedures all showed less than 1 mm of relapse. Biopsies of the porous block hydroxyapatite grafts were taken on six patients in this study at 6 to 10 months postsurgery. All showed connective tissue ingrowth and 11.3% to 36.1% bone ingrowth throughout the pores of the implants, and no inflammatory response.

Hydroxylapatite as a bone graft substitute in orthognathic surgery: histologic and histometric findings.

The use of porous hydroxylapatite (HA) as a substitute for bone in grafting associated with orthognathic surgical procedures was studied histologically and histometrically. The surgical procedures included maxillary downgrafting, advancement, setback, superior repositioning with expansion, and mandibular advancement and chin augmentation. Seventeen biopsies were obtained from nine patients after successful healing from 4.7 to 16.4 months postoperatively. Anatomic sites of the biopsies included maxillary wall, interdental region, palatal midline, chin, and mandible. In addition, nine implants representing six planned and three unplanned exposures were retrieved from nine patients. One biopsy from a successful implant was decalcified and thin-sectioned to provide better cell detail of the antral lining of the implant. The remaining biopsies were sectioned undecalcified to permit backscattered electron imaging with a scanning electron microscope. Each of the 17 biopsy specimens contained bone ingrowth. The decalcified specimen showed an intact submucosa with loss of the mucosal epithelium due to prolonged acid exposure. The biopsies were composed of 48.5% HA matrix, 18.0% bone ingrowth, and 33.5% soft tissue or vascular space. The HA matrix surface area averaged 9.4 mm2/mm3 with 62.1% of the surface covered by appositional bone ingrowth. The nine exposed implants contained connective tissue ingrowth only at their margins, with little or no bone being present. This graft-like biologic response to a porous HA matrix confirmed its ability to serve as a bone graft substitute in clinical applications. The absence of any decrease over time confirmed the relative permanence of the HA matrix. The lack of inflammatory cells in the biopsies, along with the formation of a connective tissue protective barrier in the exposed specimens, suggests that host responses to contamination were not impaired by the porous HA matrix.

Coralline porous hydroxylapatite as a bone graft substitute in orthognathic surgery.

This prospective study was undertaken to evaluate the use of coralline porous hydroxylapatite as a bone graft substitute in orthognathic surgery. Ninety-two consecutive patients received a total of 355 block implants to the maxilla (294), mandible (41), and midface (20). There were 202 implants positioned directly adjacent to the maxillary sinus. Complications were minimal, the most common being exposure of the implant to the oral or nasal cavity. Histologic evaluation of implants that were biopsied in nine patients, four to 16 months postsurgery, revealed connective tissue ingrowth throughout the implants with approximately one-third being bone of variable maturity and two-thirds being soft tissue.