Long-Term Follow-Up of Hypoglossia-Hypodactylia Syndrome: A Case Report.

Hypoglossia-hypodactylia syndrome is an extremely rare congenital anomaly characterized by a hypoplastic mandible, absence of the lower incisors, hypoglossia, and a variable degree of absence of the digits and limbs, with a risk of dysarthria and dysphagia. We report the articulation function and the swallowing function of a patient with hypoglossia-hypodactylia syndrome who was followed up to eight years old. Our patient did not have feeding and swallowing disturbances. She did not have articulatory disturbance, including /t/ and /r/, of the sound articulated using a proglossis. In the future, it is necessary to have a plastic operation for abnormal adhesion of the lower lip and mandibular gingiva and depression on the lower lip, and distraction osteogenesis for micrognathia. Also, it will be necessary to continuously monitor for an articulatory disturbance until the child uses more words. Therefore, a long-term intervention with a multidisciplinary approach is necessary.

Cemented acetabular component with femoral neck autograft for acetabular reconstruction in Crowe type III dislocated hips.

AIMS: Various surgical techniques have been described for total hip arthroplasty (THA) in patients with Crowe type III dislocated hips, who have a large acetabular bone defect. The aim of this study was to evaluate the long-term clinical results of patients in whom anatomical reconstruction of the acetabulum was performed using a cemented acetabular component and autologous bone graft from the femoral neck. METHODS: A total of 22 patients with Crowe type III dislocated hips underwent 28 THAs using bone graft from the femoral neck between 1979 and 2000. A Charnley cemented acetabular component was placed at the level of the true acetabulum after preparation with bone grafting. All patients were female with a mean age at the time of surgery of 54 years (35 to 68). A total of 18 patients (21 THAs) were followed for a mean of 27.2 years (20 to 33) after the operation. RESULTS: Radiographs immediately after surgery showed a mean vertical distance from the centre of the hip to the teardrop line of 21.5 mm (SD 3.3; 14.5 to 30.7) and a mean cover of the acetabular component by bone graft of 46% (SD 6%; 32% to 60%). All bone grafts united without collapse, and only three acetabular components loosened. The rate of survival of the acetabular component with mechanical loosening or revision as the endpoint was 86.4% at 25 years after surgery. CONCLUSION: The technique of using autologous bone graft from the femoral neck and placing a cemented acetabular component in the true acetabulum can provide good long-term outcomes in patients with Crowe type III dislocated hips. Cite this article: Bone Joint J 2021;103-B(2):299-304.

Development of an Acoustic Simulation Method during Phonation of the Japanese Vowel /a/ by the Boundary Element Method.

OBJECTIVES: The purpose of the present study was to establish the method for an acoustic simulation of a vocal tract created from CT data during phonation of the Japanese vowel /a/ and to verify the validity of the simulation. MATERIAL AND METHODS: The subjects were 15 healthy adults (8 males, 7 females). The vocal tract model was created from CT data acquired during sustained phonation of the Japanese vowel /a/. After conversion to a mesh model for analysis, a wave acoustic analysis was performed with a boundary element method. The wall and the bottom of the vocal tract model were regarded as a rigid wall and a nonrigid wall, respectively. The acoustic medium was set to 37°C, and a point sound source was set in the place corresponding to the vocal cord as a sound source. The first and second formant frequencies (F1 and F2) were calculated. For 1 of the 15 subjects, the range from the upper end of the frontal sinus to the tracheal bifurcation was scanned, and 2 models were created: model 1 included the range from the frontal sinus to the tracheal bifurcation; and model 2 included the range from the frontal sinus to the glottis and added a virtually extended trachea by 12 cm cylindrically. F1 and F2 calculated from models 1 and 2 were compared. To evaluate the validity of the present simulation, F1 and F2 calculated from the simulation were compared with those of the actual voice and the sound generated using a solid model and a whistle-type artificial larynx. To judge the validity, the vowel formant frequency discrimination threshold reported in the past was used as a criterion. Namely, the relative discrimination thresholds (%), dividing ▵F by F, where F was the formant frequency calculated from the simulation, and ▵F was the difference between F and the formant frequency of the actual voice and the sound generated using the solid model and artificial larynx, were obtained. RESULTS: F1 and F2 calculated from models 1 and 2 were similar. Therefore, to reduce the exposure dose, the remaining 14 subjects were scanned from the upper end of the frontal sinus to the glottis, and model 2 with the trachea extended by 12 cm virtually was used for the simulation. The averages of the relative discrimination thresholds against F1 and F2 calculated from the actual voice were 5.9% and 4.6%, respectively. The averages of the relative discrimination thresholds against F1 and F2 calculated from the sound generated by using the solid model and the artificial larynx were 4.1% and 3.7%, respectively. CONCLUSIONS: The Japanese vowel /a/ could be simulated with high validity for the vocal tract models created from the CT data during phonation of /a/ using the boundary element method.

Bilateral Cleft Lip and Palate Accompanied by 13q- Syndrome with Deficiencies of FVII and FX: A Case Report.

The 13q deletion syndrome is a rare genetic disorder caused by structural and functional monosomy of chromosome 13. On 13q34, which is the terminal of the long arm, causative genes of coagulation factors VII and X (FVII and FX) are mapped. Patients with a combination of FVII and FX deficiencies are extremely rare and there have been few articles about perioperative coagulation support for such patients. Herein, we report on a case of bilateral cleft lip and palate accompanied by 13q deletion syndrome with deficiencies of FVII and FX. The chromosomal investigation indicated 46, XX, del(13)(q33) by G-banding. Prothrombin time and activated partial thromboplastin time were found to be 21.0 seconds (sec) (prothrombin time-international normalized ratio 1.76) and 41.6 sec (normal range; 23.9 - 39.7 sec), respectively. The activities of coagulation FVII and FX were 22% and 36%, respectively. A two-stage cheiloplasty was performed at 4 and 7 months of age followed by a palatoplasty at 1 year and 6 months. Tranexamic acid was given intravenously three times a day for three days after each surgery. There were no adverse events such as bleeding from the oral or nasal cavities and healing of the surgical wound was good without dehiscence.

The Elental® elemental diet for chemoradiotherapy-induced oral mucositis: A prospective study in patients with oral squamous cell carcinoma.

Oral mucositis is a common adverse effect of cancer treatment that can increase the risk for local and systemic infection. This prospective study was designed to evaluate the preventive effects of an amino-acid-rich elemental diet (ED), Elental®, on radiotherapy- or chemoradiotherapy-induced mucositis in oral squamous cell carcinoma (OSCC) patients. Fifty patients were enrolled in this prospective study, who had received radiation (60-70 Gy) with/without chemotherapy [S-1, UFT, cisplatin (CDDP), docetaxel (DOC) plus CDDP, or Cetuximab]. The Elental® group (25 patients) had received Elental® during treatment, and the control group (25 patients) had not. Multivariate logistic regression analysis was used to identify the factors related to abatement of oral mucositis. A comparison of the rates of completion of chemoradiation treatments as well as the nutritional or inflammatory status between Elental® and control groups was performed. Multivariate analysis indicated that most of the patients who received Elental® suffered from a lower degree of mucositis and showed significantly improved rate of completion of chemoradiation (no interruption) compared to the control group. There was a significant difference between the Elental® group and the control group in terms of the mean change of C-reactive protein (CRP) levels in blood serum; however, there was no significant difference in terms of a mean change of body weight and total protein level in blood serum before and after chemoradiation. Our study shows that the Elental® elemental diet could be useful for the treatment of oral mucositis induced by chemoradiation. Elental® might also promote improved completion rates of chemoradiotherapy in OSCC patients.

Russell-Silver syndrome with cleft palate: a case report.

BACKGROUND: Russell-Silver syndrome (RSS) is characterized by intrauterine growth retardation, short stature without postnatal catch-up growth, and an inverted triangular face with relative macrocephaly. There have been few case reports of RSS with cleft palate, in which perioperative problems such as difficult intubation due to trismus and impossibility to wear a mouth gag due to growth failure of the mandible were described. The case of a female RSS patient with cleft palate who underwent palatoplasty is reported. CASE PRESENTATION: Although her weight was particularly low (5920 g), palatoplasty was performed under general anesthesia at 3 years and 6 months of age. Despite limited mouth opening, intubation was relatively easy. Although her mandibular alveolar width was narrow, a Dingman mouth gag could be tightly fastened around her mouth. Postoperatively, the patient was transferred to the intensive care unit without extubation due to pharyngeal edema. On the following day, since the pharyngeal edema had improved, the endotracheal tube was extubated, and her respiratory status was subsequently stable. CONCLUSIONS: In RSS patients with cleft palate, there have been a few reports of pharyngeal edema. Thus, the risk of pharyngeal edema must be considered in such patients.

Appearance of cell-adhesion factor in osteoblast proliferation and differentiation of apatite coating titanium by blast coating method.

We have already reported that the apatite coating of titanium by the blast coating (BC) method could show a higher rate of bone contact from the early stages in vivo, when compared to the pure titanium (Ti) and the apatite coating of titanium by the flame spraying (FS) method. However, the detailed mechanism by which BC resulted in satisfactory bone contact is still unknown. In the present study, we investigated the importance of various factors including cell adhesion factor in osteoblast proliferation and differentiation that could affect the osteoconductivity of the BC disks. Cell proliferation assay revealed that Saos-2 could grow fastest on BC disks, and that a spectrophotometric method using a LabAssayTM ALP kit showed that ALP activity was increased in cells on BC disks compared to Ti disks and FS disks. In addition, higher expression of E-cadherin and Fibronectin was observed in cells on BC disks than Ti disks and FS disks by relative qPCR as well as Western blotting. These results suggested that the expression of cell-adhesion factors, proliferation and differentiation of osteoblast might be enhanced on BC disks, which might result higher osteoconductivity.

Quantitative Analysis of Velopharyngeal Movement by Applying Principal Component Analysis to Range Images Produced by a Three-Dimensional Endoscope.

Objectives: The purpose of this study was to develop a new technique for analyzing velopharyngeal movement and to investigate its utility. Materials and Methods: Velopharyngeal motion of 20 normal individuals was analyzed. A three-dimensional (3D) endoscope was inserted into the oral cavity, and the movement of the soft palate was measured using an exclusive fixation device. Range images of the soft palate were produced during phonation of the Japanese vowel /a/, and virtual grids were then overlaid on these images. Principal component analyses were applied to the 3D coordinates of the intersections of the virtual grids. The centers of gravity of the virtual grids were calculated, and the magnitude of the shift of the grid intersections during phonation was calculated. Results: The first and the second principal component scores were responsible for the upper posterior direction and the upper direction, respectively. The average magnitude of the shift of the center of gravity was 4.75 mm in males and 4.33 mm in females. Conclusions: Quantitative analysis of velopharyngeal movement was achieved by a method of applying principal component analysis (PCA) to the range images obtained from a 3D endoscope. There was no sex difference in velopharyngeal movement.

Characteristics of Posed Smiles for Class III Female Patients Before and After Osteotomy Using Principal Component Analysis.

The purpose of this study was to clarify characteristics of posed smiles for Class III female patients before and after orthognathic surgery. Just before retrusion surgery and the removal of fixation plates, 2 sets of posed smiles were recorded for 7 Class III female patients. As normal controls, 7 healthy female volunteers were also recorded. Using our video-based motion analyzing system, range images and 5 × 5 virtual grids projected onto the lips were recorded for all patients while making a posed smile. The gravity for each area in the lips was calculated from the intersections of the virtual grids. Principal component analysis was applied to the normalized virtual grids, that is, a homologous model of lip shape, for all frames of the posed smiles. While the sample size was too small to generalize from these results, we found that the upper vermilion shifted posteriorly and laterally in posed smiles for Class III female patients after retrusion surgery as compared with the preoperative posed smiles. In addition, the characteristic lip movements during postoperative posed smiles for Class III female patients did not resemble those of the normal controls.

Gender differences in posed smiles using principal component analysis.

OBJECTIVES: The purpose of this study was to clarify gender differences in posed smiles using principal component analysis (PCA). MATERIALS AND METHODS: Fourteen adult volunteers, 7 males and 7 females, were enrolled. Using the motion analyzing system we developed, range images and 5 × 5 virtual grids were produced across the whole sequence while the volunteers were asked to smile. Two sets of all intersections of the virtual grids captured while the subject was smiling were regarded as PCA variables. Discriminate analysis was then applied to compare the males and females. RESULTS: The first and second principal component scores (PCSs) were plotted on the x-axis and y-axis, respectively. The center of gravity of the PCSs is shown by the plus on the x-axis and minus on the y-axis for the males and by the minus on the x-axis and the plus on the y-axis for the females. Discriminate analyses of the PCSs revealed a correct gender classification rate of 74.4% for posed smiles. CONCLUSIONS: While the sample size is too small to extrapolate from these results, we can conclude that PCA can be used to identify gender differences while smiling.

Three-dimensional intra-rater and inter-rater reliability during a posed smile using a video-based motion analyzing system.

OBJECTIVES: The purpose of this study was to determine the three-dimensional reproducibility of lip movement during a posed smile using a video-based motion analyzing system. MATERIALS AND METHODS: In six adult volunteers (4 males and 2 females), the lip motions during a posed smile were recorded six times. Using our recently-developed motion analyzing system, range images were produced across the whole sequence during the posed smile. Virtual grids of 5 × 5 were fitted onto the surfaces, and the three-dimensional coordinates of the intersections of these grids were then computed. The magnitude of the shift of the intersections during smiling was calculated and summed in each area. Intraclass correlation coefficients (ICC), ICC (1,1) for intra-rater reliability and ICC (2,1) for inter-rater reliability were calculated. The number of repeated measurements necessary for an ICC level beyond 0.8 was determined using the formula of Spearman-Brown. RESULTS: The ICC (1,1) and ICC (2,1) ranged from 0.71 to 0.83 and from 0.77 to 0.99, respectively. The number of repeated measurements necessary for an ICC beyond 0.8 was 2. CONCLUSIONS: From the present study, both the three-dimensional intra-rater and inter-rater reliabilities during a posed smile were considered to be relatively high, and enough reliability could be expected by calculating the average of the values measured two times. However, the sample size was very small, this could not be generalized simplistically.

Comparison of apatite-coated titanium prepared by blast coating and flame spray methods--evaluation using simulated body fluid and initial histological study.

It has previously been demonstrated that apatite may be coated on the surface of titanium (Ti) at room temperature when the titanium is blasted with apatite powder. This method is known as the blast coating (BC) method. In this study, the osteoconductivity and tissue response to Ti implants blast-coated with apatite (BC implants) were evaluated using apatite-coated Ti implants produced using the flame spraying (FS) method (FS implants) and pure Ti implants as a control. Initial evaluation using simulated body fluid demonstrated higher osteoconductivity in BC implants than in FS implants. Therefore, specimens were implanted in rat tibias for 1, 3 and 6 weeks. At one week after implantation, BC implants showed much higher bone contact ratio when compared with FS implants; the bone contact ratio of BC implants was 75.7%, while the FS and pure Ti implants had ratios of 30.8% and 5.5%, respectively. The difference in bone contact ratio between BC and FS implants decreased with implantation time and the ratios were equal after 6 weeks. In conclusion, BC implants show higher osteoconductivity than FS implants, and thus BC implants are beneficial for early fixation of implants to bone tissue.