Atlanto-occipital ligament calcification: a novel sign in nevoid basal cell carcinoma syndrome.

BACKGROUND: The nevoid basal cell carcinoma syndrome (NBCCS), first described by Gorlin and Goltz in 1960, is a hereditary autosomal dominant disease with high penetrance and variable expressivity. Almost 70% of patients with NBCCS have some degree of craniofacial anomaly. Among these, the presence of ectopic calcification have been reported but Atlanto-occipital ligament calcification has never been described. Therefore this investigation was carried out to determine the prevalence of atlanto-occipital ligament calcification on lateral x-ray of NBCCS patients aiming to assess the effectiveness of this sign in NBCCS diagnosis. PATIENTS AND METHODS: Lateral and frontal cephalometric radiographs of 18 patients (11 males and 7 females), aged 8-61 years, with the diagnosis of NBCCS were evaluated for the presence of intracranial calcifications (diaphragma sellae and falx cerebri) and or calcification of the atlanto-occipital ligament. RESULTS: A total of 11 patients presented calcification of atlanto-occipital ligament to various degrees and in three cases this represented the only sign of ectopic calcification. When compared to the other two sites of ossification, atlanto-occipital ligament calcification had a similar prevalence. CONCLUSION: The calcification of the atlanto-occipital ligament should be considered in addition to the other major criteria for NBCCS diagnosis.

Nevoid Basal cell carcinoma syndrome: a cephalometric study of patients and controls.

Craniofacial morphology of patients with nevoid basal cell carcinoma syndrome (NBCCS) has sometimes been reported at clinical examination, but any investigation has described it on the basis of cephalometric measurements.The purpose of this study was to conduct a cephalometric analysis of patients with NBCCS and to compare measurements with non-NBCCS subjects of similar ages, to elucidate if there is any relationship between NBCCS and craniofacial morphology.The study population consisted of 14 adult patients (9 men and 5 women), ranging in age from 18.2 to 56.8 years, with the diagnosis of NBCCS, with good-quality lateral cephalometric radiographs, and 14 adult healthy patients matched for age and sex to the NBCCS group. Cephalometric measurements were carried out on radiographs, and measurements of angles and distances were performed.Statistical differences between NBCCS subjects and controls were observed. Data analysis displayed that the measurements of the anterior cranial base (P

Reconstruction of the lower lip: rationale to preserve the aesthetic units of the face.

BACKGROUND: The boundaries of the aesthetic units of the face are often crossed after lower lip cancer surgery. The aim of this study was to compare the aesthetic and functional outcome after use of different operative techniques based on the concept of the aesthetic units of the face. MATERIALS: Sixty-three patients were evaluated after lower lip reconstruction. The aesthetic outcome was recorded by standard photography evaluating the disruption of the boundaries of the aesthetic units of the face, lip projection, and the resulting facial expression. The functional outcome consisted of the evaluation of mouth opening, pouting, and lips at rest for the evaluation of mouth continence. Three techniques were used: wedge excision, the Webster-Fries method, and the step technique. The step technique was combined with an Abbé or an Estlander flap in defects involving more than two-thirds of the lip. RESULTS: In defects involving up to one-third of the lip, the aesthetic outcome was better for the step technique than for wedge excision (a statistical trend was observed, p = 0.088). In defects involving two-thirds of the lip, the aesthetic and functional outcome was better using the step technique than the Webster-Fries method (p = 0.002), because the boundaries of the aesthetic units are respected. In defects involving more than two-thirds of the lip, the result was better using the step technique combined with the Abbé flap. CONCLUSION: The authors have shown that the step technique alone or combined with a flap of the opposite lip is a rational approach for preserving the aesthetic units of the face and its function.

Long-term recurrence rate of pleomorphic adenoma and postoperative facial nerve paresis (in parotid surgery).

BACKGROUND: The purpose of this study was to evaluate recurrence rates and comorbidity in patients with pleomorphic adenomas of patients after superficial and total conservative parotidectomy. PATIENTS AND METHODS: Localization of pleomorphic adenomas, age, sex distribution and facial nerve function of 73 patients were examined in this retrospective study. The recurrence rate could be determined in 43 of these patients. The interval between surgery and last recall varied between 2 and 20 years (median: 8.1 years). RESULTS: Most of the patients were female (67%) with a parotid pleomorphic adenoma. No recurrence was found regard less of whether a superficial or total conservative parotidectomy had been performed. Our data did show that the total conservative parotidectomy is associated with a higher incidence of temporary impaired facial nerve function, which was seen in 42% of this group. Temporary decreased nerve function after superficial parotidectomy was rare being apparent in only 16% of this group. CONCLUSION: A more radical procedure does not significantly lower the recurrence rate. We suggest that the indication for a total conservative parotidectomy in cases of superficial adenomas should be considered carefully.

One- or two-stage palate closure in patients with unilateral cleft lip and palate: comparing cephalometric and occlusal outcomes.

OBJECTIVE: To assess facial growth and dentoalveolar development in two groups of patients with complete unilateral cleft lip and palate. Primary surgical treatment differed in the timing of hard palate closure. DESIGN: Forty-three patients with unilateral cleft lip and palate were examined. Twenty-two patients underwent early one-stage closure of the hard and soft palate cleft (mean age 23.0 +/- 4.7 months); in 21 patients, the hard palate closure was delayed to 86.3 +/- 39.2 months of age. Lateral cephalograms and dental casts were consecutively analyzed at four stages between 6 and 18 years of age. RESULTS: Lateral cephalometric analysis revealed no significant intergroup differences in the sagittal and vertical craniofacial dimensions at any time. Dental cast analysis showed constriction of the upper anterior arch width at the ages of 6 and 10 years in patients with one-stage surgical palate closure, but a difference could no longer be verified at the ages of 15 and 18 years. CONCLUSIONS: The transverse distances in the upper jaw developed initially more positively in the group with delayed hard palate closure, but it became apparent later that the transverse deficiency after one-stage palate closure could be compensated for. When considering surgical treatment in general, the advantages of the delayed hard palate closure must be weighed against criteria favoring the early one-stage closure of the hard and soft palate.

Particulated bone grafts--effectiveness of bone cell supply.

OBJECTIVE: The objective of this study was to measure the amount of viable bone cells present in different types of bone graft. MATERIAL AND METHODS: Bone chips were harvested from the trabecular or cortical bone of the mandible or the iliac crest and either milled or not. The average size of unmilled bone particles was 5 x 5 x 5 mm and that of milled was 2 x 2 x 2 mm. Drill sludge was obtained using either a ball reamer, a diamond ball or an implant drill (the latter from mandibular bone and of average dimension 1 x 1 x 1 mm). A measure of 0.5 g of each category was cultured in Dulbecco's modified Eagle's medium with additives for four weeks. Cell counts were performed. An analysis of the osteocalcin synthesis, the alkaline phosphatase (ALP) activity, the collagen types and the concentration of bone-specific collagen cross-links in medium supernatants was performed. RESULTS: Cells stained positively for osteocalcin and ALP in all groups. Bone-specific collagen cross-links could be quantified and collagen of types I and V was present with no difference in all groups. Unmilled spongy bone chips revealed greater cell counts than milled (P<0.05). Spongy bone chips revealed greater cell counts than cortical bone chips (P<0.05). Drill sludge obtained by hard alloy ball reamer showed the least amount of viable cells (P<0.05). CONCLUSIONS: Bone milling reduces the quantity of osteoblasts. Bone obtained by the ball reamer supplies a smaller number of cells than bone obtained by other methods. Unmilled spongy bone chips appear to offer the greatest amount of viable osteoblasts.