Brian M. Smith

Lesch-Nyhan syndrome : a case report

Lesch-Nyhan syndrome is a rare anomaly consisting of a deficiency in the production of hypoxanthine phosphoribosyltransferase that leads to the overproduction of purine and the accumulation of uric acid. Major manifestations include mental retardation and self-destructive behavior resulting in self-mutilation through biting and scratching. Because no medical treatment exists to alleviate the symptoms of self-mutilation, direct dental intervention is the only way these behaviors can be affected. A unique case of this type involving two male identical twins is reported.

Use of auricular cartilage in the repair of orbital floor defects

Various materials have been used to repair orbital defects. This article advocates the use of autogenous conchal cartilage, especially in the repair of large posttraumatic defects, where contour, biocompatibility, and strength are most important. The technique for harvesting the cartilage is reviewed, and a case demonstrating its successful use is reported.

Reconstruction of a severely atrophic maxilla using a Le Fort I downgraft and dental implants: clinical report.

A 55-year-old woman developed chronic infections of the maxillary sinuses with osteomyelitis resulting in extensive bone destruction and atrophy from a failed subperiosteal implant. After surgical removal of the subperiosteal implant, extensive debridement of the maxilla, and long-term antibiotic therapy, maxillary reconstruction was initiated. A Le Fort I osteotomy and downgraft was performed, and a posterior iliac bone graft was harvested and grafted to reconstruct the severely atrophic maxilla. In a later surgical procedure, maxillary and mandibular implants were placed. Implant-retained overdentures were fabricated to restore function and esthetics. (Implant Dent 1997;6:105–108)

A sublingual-submandibular calcific mass associated with tumoral calcinosis in a patient with suspected milk-alkali syndrome.

The milk-alkali syndrome is a disorder with diverse clinical and biochemical manifestations. It includes three known clinical syndromes. A case of milk-alkali syndrome with associated tumoral calcinosis is presented. The patient developed an unusual oral mass as part of the disease complex. Treatment consisted of excision of the intraoral mass, diet management, and surgical correction of the associated ulcer disease.