Sturge-Weber syndrome with cemento-ossifying fibroma in the maxilla and giant odontoma in the mandible: A case report.

Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome with vascular lesions of the cerebral meninges, port wine spots on the face, and glaucoma of the eyes; it is a congenital, non-genetic disease whose etiology and mechanisms are unknown. In this report, we describe a rare case of SWS with unilateral large odontogenic tumors in the maxilla and mandible. The histopathological diagnosis of the maxillary bone lesion on biopsy was juvenile psammomatoid ossifying fibroma, which is considered a type of ossifying fibroma of craniofacial bone origin. However, the final pathological diagnosis of the excision was cemento-ossifying fibroma derived from periodontal ligament cells, and we discuss the histopathology in detail. In addition, the mandibular lesion was one of the largest odontomas reported to date. Furthermore, in this case, we suggest the possibility that the maxillary and mandibular bone lesions are not separate lesions, but a series of lesions related to SWS.

Spindle Cell Variant of Ameloblastic Carcinoma: Another Example in a Japanese Male.

Spindle cell variant of ameloblastic carcinoma (SpCAC) is a rare subtype of ameloblastic carcinoma. Herein, we describe an additional case of SpCAC of the mandible of a 76-year-old Japanese male. We discuss diagnostic problems we encountered in this case, focusing on unusual expression of myogenic/myoepithelial markers, such as smooth muscle actin and calponin.

Treatment of Severe Open Bite Malocclusion with Four-Piece Segmental Horseshoe Le Fort I Osteotomy.

Appropriate operations in severe anterior open bite (AOB) cases are extremely complicated to perform because of the multiple surgical procedures involved, the difficulty of predicting posttreatment aesthetics, and the high relapse rate. We herein report a 16-year-old girl with skeletal Class II, severe AOB malocclusion, and crowding with short roots, and aesthetic and functional problems. Four-piece segmental Le Fort I osteotomy with horseshoe osteotomy was performed for maxillary intrusion, and bilateral sagittal split ramus osteotomy (SSRO) and genioplasty were performed for mandibular advancement. The malocclusion and skeletal deformity were significantly improved by the surgical orthodontic treatment. Functional and aesthetic occlusion with an improved facial profile was established, and no further root shortening was observed. Acceptable occlusion and dentition were maintained after a two-year retention period. This strategy of surgical orthodontic treatment with a complicated operative procedure might be effective for correcting certain severe AOB malocclusion cases.

Comparison of stone retropulsion between Moses mode and virtual basket mode: an in vitro study using artificial stones.

This study aimed to evaluate stone retropulsion in various laser pulse modes in both Moses mode (MM) and virtual basket mode (VBM). Experiments were performed using a channel-shaped rubber rail and artificial stones. We compared short pulse mode and long pulse mode in both MM and VBM with the laser tip positioned so that it was touching and at 1 and 2 mm distances from the stone surface. Stone retropulsion was measured after the laser fired for 10 s in three different laser settings: 0.5 J/8 Hz, 0.8 J/8 Hz and 1.0 J/8 Hz. When the laser tip was touching the artificial stone, stone retropulsion in MM was significantly shorter than that in VBM in all laser settings (P < 0.01, P = 0.02 and P = 0.02, respectively). At 1-mm distance, stone retropulsion in MM was significantly shorter than that in VBM in 0.8 J/8 Hz and 1.0 J/8 Hz settings (P < 0.01 and P = 0.01, respectively). At 2-mm distance, however, there were no differences between MM and VBM in stone retropulsion in any laser settings. Stone retropulsion was not significantly different between the laser settings at 1-mm distance in MM, or when touching in VBM. In conclusion, stone retropulsion distance in MM can be shorter than that in VBM. Stone retropulsion in MM and VBM may be differently influenced by laser settings and laser tip position.

Multicenter Retrospective Study of Weekly Cetuximab Plus Paclitaxel for Recurrent or Metastatic Oral Squamous Cell Carcinoma.

BACKGROUND/AIM: This study was conducted to compare the efficacy and safety of the weekly cetuximab plus paclitaxel (wCmab-PTX) regimen with those of the EXTREME regimen in patients with recurrent or metastatic oral squamous cell carcinoma (R/M OSCC). PATIENTS AND METHODS: This multicenter retrospective study involved a chart review of the clinical records of R/M OSCC patients treated with wCmab-PTX in each institution between January 2013 and December 2017. Data were collected, and the efficacy, safety, and treatment outcomes were analyzed. RESULTS: The best overall response and disease control rates were 48.4% and 61.3%, respectively. The median PFS and OS were 6 and 13 months, respectively. There was no significant difference in prognosis with or without previous platinum administration. The grade 3-4 adverse events were leukopenia (16.1%), followed by acne-like rash (12.9%), and neutropenia (9.7%). All adverse events, excluding more than grade 3 infusion reactions, were tolerable and manageable. CONCLUSION: wCmab-PTX may be considered as a treatment option for R/M patients with OSCC that is refractory to platinum-based chemotherapy, or progressive disease after receiving chemotherapy.

The influence of zoledronate and teriparatide on gamma delta T cells in mice.

BACKGROUND/PURPOSE: Few studies have investigated the possibility that bisphosphonate-related osteonecrosis of the jaw (BRONJ) might reflect an immune response; however, gamma delta T cells have been shown to significantly decline in the blood of BRONJ patients. Additionally, there have been some reports of teriparatide usage for the treatment of BRONJ. In this study, we compared the effects of zoledronate and teriparatide on lymphocyte populations and inflammatory cytokine production in mice. MATERIALS AND METHODS: Thirty female ICR mice were divided into three groups (n = 10 each): a vehicle, a zoledronate, and a teriparatide group. Drugs were administered for 8 weeks in each group. Lymphocytes in the blood and thymus were analyzed and femurs were used for histological observation and lymphocytes analysis of bone marrow. Cytokines were measured in separated serum using Milliplex® multiplex immunoassay analysis. RESULTS: Zoledronate decreased the T cell number in the bone marrow. Additionally, serum levels of interleukin (IL)-2, IL-7, IL-12, IL-15 and RANTES, which are cytokines that affect T cell activation, differentiation and/or proliferation, were significantly lower in zoledronate treated mice. Conversely, teriparatide treatment induced an increase in gamma delta T cells in peripheral blood. CONCLUSION: Gamma delta T cells in the bone marrow are expected to decrease with zoledronate treatment and increase with teriparatide treatment. If BRONJ involves a loss of gamma delta T cells in the circulation or bone marrow, then the increase in gamma delta T cells that is induced by teriparatide may account for its ability to resolve BRONJ.

A case of severe mandibular retrognathism with bilateral condylar deformities treated with Le Fort I osteotomy and two advancement genioplasty procedures.

We report a case involving a young female patient with severe mandibular retrognathism accompanied by mandibular condylar deformity that was effectively treated with Le Fort I osteotomy and two genioplasty procedures. At 9 years and 9 months of age, she was diagnosed with Angle Class III malocclusion, a skeletal Class II jaw relationship, an anterior crossbite, congenital absence of some teeth, and a left-sided cleft lip and palate. Although the anterior crossbite and narrow maxillary arch were corrected by interceptive orthodontic treatment, severe mandibular hypogrowth resulted in unexpectedly severe mandibular retrognathism after growth completion. Moreover, bilateral condylar deformities were observed, and we suspected progressive condylar resorption (PCR). There was a high risk of further condylar resorption with mandibular advancement surgery; therefore, Le Fort I osteotomy with two genioplasty procedures was performed to achieve counterclockwise rotation of the mandible and avoid ingravescence of the condylar deformities. The total duration of active treatment was 42 months. The maxilla was impacted by 7.0 mm and 5.0 mm in the incisor and molar regions, respectively, while the pogonion was advanced by 18.0 mm. This significantly resolved both skeletal disharmony and malocclusion. Furthermore, the hyoid bone was advanced, the pharyngeal airway space was increased, and the morphology of the mandibular condyle was maintained. At the 30-month follow-up examination, the patient exhibited a satisfactory facial profile. The findings from our case suggest that severe mandibular retrognathism with condylar deformities can be effectively treated without surgical mandibular advancement, thus decreasing the risk of PCR.

A case of cricothyroidotomy for facial trauma in a patient taking antiplatelet agents after a simple ground-level fall.

INTRODUCTION: Cricothyroidotomy is an emergency procedure that can be used to secure the airway in situations in which intubation and ventilation are not possible. PRESENTATION OF CASE: We describe a case of 79-year-old male presenting with facial trauma combined with massive upper airway bleeding and swelling in which cricothyroidotomy was required to open the airway in an elderly male patient taking antiplatelet agents who suffered a simple ground-level fall. DISCUSSION: Although emergency airway management is often required in patients with Le Fort fractures, mandibular condyle fractures exhibit a significant relationship with ground-level falls, which are not usually associated with emergency airway management. Prophylactic intubation should be considered prior to transfer or deterioration in a trauma patient with dual antiplatelet drugs and fractures of bilateral mandibular condyle. CONCLUSION: Clinicians should be aware of the life-threatening injuries that can be caused by simple ground-level falls in patients taking antiplatelet agents.

Treatment of Osteonecrosis of the Jaw.

The definition of bisphosphonate-related osteonecrosis of the jaw (BRONJ) was recently broadened and it is now known as medication-related osteonecrosis of the jaw (MRONJ). To date, the management of MRONJ is controversial. Conservative treatment is recommended, but it is difficult to successfully treat stage 3 MRONJ. Administration of teriparatide for the MRONJ treatment has only been documented in independent case reports and there are few reports on men with MRONJ treated with teriparatide. An 81-year-old man was referred in May 2014 for treatment of an unhealed tooth extraction wound in the mandible. He took minodronic acid hydrate (1 mg/d orally) for 2 years because of osteoporosis cure. On clinical examination, soft tissue swelling in the left mandibular first molar region extended to the inferior border of the mandible with extraoral fistula. Computed tomography (CT) revealed osteolysis extending to the inferior border resulting in pathologic fracture of mandibular bone. Based on these findings, a diagnosis of stage 3 MRONJ was made. We performed conservative treatment, including amoxicillin, but his symptoms did not improve. He was then treated with once-weekly subcutaneous injection of teriparatide. Although teriparatide injections were started without antibiotics, after 1 week, swelling, erythema, and purulent discharge from the extraoral fistula increased rapidly. Therefore, we combined the once-weekly teriparatide injection with amoxicillin administration. Three months later, the osteonecrosis had healed and CT showed significant bone regeneration and healing of the mandibular pathologic fracture. In addition, the mandibular fistula showed healing and the intraoral fistula was covered with normal mucosa.

Solid-type primary intraosseous squamous cell carcinoma of the mandible: a case report with histopathological and imaging features.

Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare malignant odontogenic tumor arising from odontogenic epithelial remnants within the jawbones. PIOSCC is histopathologically divided into 3 types: solid-type carcinoma, carcinoma derived from a keratocystic odontogenic tumor, and carcinoma derived from an odontogenic cyst. In this article, we report a case of solid-type PIOSCC involving reactive bone formation in the mandible in a 60-year-old female patient together with its histopathological and imaging findings.

Nonlinear Dynamic Analysis of Vowels in Cleft Palate Children with or without Hypernasality.

Objectives. To clarify the difference between Lyapunov exponents (LEs) for cleft palate (CP) patients with hypernasality versus without hypernasality and to investigate the relationship between their LEs and nasalance scores (NSs). Material and Methods. Six CP patients with severe hypernasality (mean age 9.2 years) and six CP patients without hypernasality (mean age 8.0 years) were enrolled. Five Japanese vowels were recorded at 44.1 KHz, and the NSs were measured simultaneously. The mean first LE (mLE(1)) from all one-second intervals was computed. Results. The mLE(1) for /o/ in patients with hypernasality was significantly higher than that in patients without hypernasality. The correlation coefficients between the mLE(1) and NS for all vowels were not statistically different. Conclusion. The voice signal of /o/ for the patients with hypernasality was more instable than in those without hypernasality. The chaotic phenomenon was independent of nasal resonance in CP voice.

Bone quality and quantity of the anterior maxillary trabecular bone in dental implant sites.

OBJECTIVES: The aim of this study was to investigate the characteristics of implant sites on the edentulous alveolar ridge in the anterior maxilla. We studied the bone quantity and quality of implant sites at the anterior maxilla using CT images for the 33 implant sites on patients who underwent dental implant therapy in our Department since 2006. MATERIALS AND METHODS: Computed tomography (CT) images of 33 patients (20 women: 13 men) encompassing 33 implant sites were chosen and examined. The recipient sites for implant placement were determined based on CT data using an implant planning software (Simplant 11.0). The mean bone density values in Hounsfield unit (HU) were recorded using Simplant for both the simulated implant areas and the trabecular bone width. We classified the edentulous alveolar ridge and bone quality according to a classification based on Lekholm and Zarb (1985). RESULTS: Incisors had higher bone densities than canines. Women had lower bone densities than men. Canines displayed greater trabecular bone density and alveolar bone widths than incisors. No maxillary sites were judged to have a bone quality of 1 in this group. Quality 3 accounted for 69.7% of the total samples. CONCLUSIONS: An assessment of bone quality in the anterior alveolar ridge may well reflect age-related systemic pathological conditions and should be used in dental implant treatment planning to avoid associated risk factors.

Lyapunov exponents for Japanese vowels in normal adult and cleft palate speakers.

OBJECTIVE: The purpose of this study was to explore the possibility of using Lyapunov exponents (LE) to evaluate the voice and speech in cleft palate (CP) patients. SUBJECTS AND METHODS: Fifty healthy adults (25 males and 25 females) and 17 adult patients (10 males and 7 females) with repaired CP, who had no hypernasality, were enrolled. Five Japanese vowels were recorded at 44.1 kHz. Nasalance scores were measured simultaneously. The first LE was computed for each 1-second interval, and the mean for the first LE from all intervals (mLE(1)) was then calculated. The delay time and embedding dimension were estimated at 13, 40, 34, 27 and 22 points for the vowels /a/, /i/, /u/, /e/ and /o/, respectively, and at 8 points for all vowels. RESULTS: The mLE(1)s for all vowels did not differ between normal males and females. The mLE(1)s for /a/ were significantly higher both in males and females with CP than in normal individuals. The correlation coefficient between the mLE(1)s and nasalance scores for all vowels was not significant for both normal and CP subjects. CONCLUSION: The LE seemed to be related to pathological disorders of the vocal cords in CP patients, but was independent of resonance parameters.

Analysis of lip motion using principal component analyses.

OBJECTIVES: The aim of this study was to quantitatively determine lip movement characteristics during the phonation of Japanese vowels. MATERIALS AND METHODS: Lip motions of 14 normal individuals during the phonation of five Japanese vowels, /a/, /i/, /u/, /e/, and /o/ were analyzed. Using our developed motion analyzing system, range images were produced across the whole sequence during lip motions, and virtual grids were fitted onto surfaces. Principal component analyses were applied with three-dimensional coordinates of intersections for virtual grids regarded as variables. RESULTS AND CONCLUSION: The fourth and fifth principal component scores were shown to be parameters that control mouth opening and the retraction of the corners of the mouth, respectively. Discriminate analyses show it is possible to distinguish motion during the phonation of /u/ from that of other vowels.

Target genes of the largest human SWI/SNF complex subunit control cell growth.

The largest subunit of the mammalian SWI/SNF-A or BAF (BRG1-associated factor) chromatin-remodelling complex is encoded by two related cDNAs hOsa1/BAF250a and hOsa2/BAF250b that are unique to the BAF complex and absent in the related PBAF (Polybromo BAF). hOsa/BAF250 has been shown to interact with transcriptional activators and bind to DNA suggesting that it acts to target the remodelling complex to chromatin. To better understand the functions of hOsa2, we established inducible stable HeLa cell lines over-expressing FLAG-hOsa2 or a derivative lacking the ARID (AT-rich interactive domain) DNA-binding domain. Immunopurification of complexes containing hOsa2 that was followed by mass spectrometry and immunoblotting demonstrated the presence of BRG1 and known BAFs, but not hOsa1 or hBRM. Deletion of the ARID did not compromise the integrity of the complex. Induction of hOsa2 expression caused impaired cell growth and accumulation of cells in the G0/G1 cell cycle phase. Elevated levels of the p53 and p21 proteins were detected in these cells while c-Myc mRNA and protein levels were found to decrease. Chromatin immunoprecipitation and reporter assays suggested that hOsa2 had a direct effect on c-myc and p21 promoter activity. Thus hOsa2 plays an important role in controlling genes regulating the cell cycle.

Atypical lipomatous tumor of the tongue: report of a case.

The term atypical lipomatous tumor (ALT) is synonymous with well-differentiated liposarcoma (WDL). This tumor occurs very rarely in the tongue. Thus, it is difficult to predict its prognosis. Although recurrence of ALT/WDL is thought to be unlikely after complete excision, long-term follow-up is necessary when considering the pathologic conditions of this tumor at other sites. Here, we report a case of an ALT of the tongue, with a review of the literature. A 68-year-old man was referred to our hospital because of a tumor on the left side of his tongue. Upon palpation, the tumor was 12mm in diameter, circumscribed, elastic and hard, well demarcated, movable, and painless. We diagnosed the lesion as a lipoma and extirpated the tumor under local anesthesia. Because the specimen was histopathologically diagnosed as an ALT, as a precaution, we excised an additional 5mm from the area surrounding the original tumor under general anesthesia. Three years after the operation, the tongue demonstrated good healing without paresthesia or dysfunction, and to date there has been no evidence of recurrence.

Nasolabial morphologic changes after a Le Fort I osteotomy: a three-dimensional anthropometric study.

After a Le Fort I osteotomy, nasal and labial changes are sometimes undesirable. The aim of this study was to perform a three-dimensional evaluation of the morphologic changes of the nose and lips after a Le Fort I osteotomy with a three-dimensional laser scanner.Twelve female patients who underwent a Le Fort I osteotomy with bilateral sagittal split ramus osteotomy (mean age, 24.2 y) were selected. Three-dimensional facial morphology was measured immediately after surgery and 6 months after surgery with a three-dimensional laser scanner. Analysis of the three-dimensional data was performed with three-dimensional image analyzing software. The evaluation was performed by (1) linear and angular analyses of landmarks, (2) three-dimensional curvature, and (3) visual qualitative analysis using superimposing mapping images. The results were compared control with data taken from volunteers (mean age, 24.5 y).Lateral expansion of nasal alae was commonly observed after surgery. The labial changes were mainly due to movements of the jawbone, and obvious flattening was not detected in this study. There was no relationship between the nasal changes and maxillary advancement.The nasal morphologic changes after a Le Fort I osteotomy consist mainly of a widening of the nasal alae caused by the release of the muscle insertion and their retraction. This change was not influenced by the direction of maxillary movements.

A case of ATLL (adult T-cell leukemia/lymphoma) mimicking odontogenic infection.

A case of adult T-cell leukemia/lymphoma (ATLL) in which cheek swelling was the initial symptom is presented. A 44-year-old man referred to our hospital with swelling in his right cheek, and a dental infection was suspected by a previous physician. Computerized tomographic scans and magnetic resonance imaging revealed tumorous lesions from the right alveolus of the maxilla to the anterior and posterior walls of the maxillary sinus. Anti-HTLV-1 antibodies were positive, and the level of sIL-2R was high; other laboratory test results were normal., based on immunohistochemical results on a specimen biopsy, the patient was diagnosed with a lymphoma-type ATLL. Immediately, the patient was treated in the department of hematologic medicine. After several courses of multiagent chemotherapy, 27 Gy radiation therapy was directed to the maxilla. However, remission was not achieved. Cord blood transplantation was subsequently performed, but his general condition gradually worsened until he died 7 months after his initial visit.

Mammalian SWI/SNF--a subunit BAF250/ARID1 is an E3 ubiquitin ligase that targets histone H2B.

The mammalian SWI/SNF chromatin-remodeling complex facilitates DNA access by transcription factors and the transcription machinery. The characteristic member of human SWI/SNF-A is BAF250/ARID1, of which there are two isoforms, BAF250a/ARID1a and BAF250b/ARID1b. Here we report that BAF250b complexes purified from mammalian cells contain elongin C (Elo C), a BC box binding component of an E3 ubiquitin ligase. BAF250b was found to have a BC box motif, associate with Elo C in a BC box-dependent manner, and, together with cullin 2 and Roc1, assemble into an E3 ubiquitin ligase. The BAF250b BC box mutant protein was unstable in vivo and was autoubiquitinated in a manner similar to that for the VHL BC box mutants. The discovery that BAF250 is part of an E3 ubiquitin ligase adds an enzymatic function to the chromatin-remodeling complex SWI/SNF-A. The immunopurified BAF250b E3 ubiquitin ligase was found to target histone H2B at lysine 120 for monoubiquitination in vitro. To date, all H2B monoubiquitination was attributed to the human homolog of yeast Bre1 (RNF20/40). Mutation of Drosophila osa, the homolog of BAF250, or depletion of BAF250 by RNA interference (RNAi) in cultured human cells resulted in global decreases in monoubiquitinated H2B, implicating BAF250 in the cross talk of histone modifications.

Bone formation in a rat calvarial defect model after transplanting autogenous bone marrow with beta-tricalcium phosphate.

In the present study, we evaluated the osteogenic potential of an autogenous bone marrow graft combined with beta-tricalcium phosphate (beta-TCP) in a rat calvarial bone defect model. The bone marrow harvested from the tibia of 7-week-old rats was grafted autogenously in a calvarial defect together with beta-TCP (=BTG group, n=16) or without beta-TCP (=BG group, n=16). Groups of animals were also treated with beta-TCP alone (=TG group, n=16) and control animals (n=8) received no graft implanted into the defect. We then observed the process of bone formation by histology, enzyme histochemistry and immunohistochemistry. Five days after grafting, in the BTG and BG groups, cell proliferation and osteogenic differentiation were observed. From 5 to 10 days after surgery, active Runx2, osteopontin (OPN), and TRAP- positive cells appeared in the BTG and BG groups. New bone formation started in the defect in both the BTG and BG groups. At 30 days after grafting, the BTG group showed new bone development and replacement of beta-TCP to fill the bone defect. New bone formation in the BTG group was significantly greater than in the BG group (P<0.01). The TG group showed no marked bone formation in the defect. The combination graft of bone marrow with beta-TCP showed marked bone formation in rat calvarial defects. Our results indicate that the combination grafts of bone marrow with beta-TCP may be an effective technique for repairing bone defects Beta-TCPgraft (TG) group.