Tissue-engineered Maxillofacial Skeletal Defect Reconstruction by 3D Printed Beta-tricalcium phosphate Scaffold Tethered with Growth Factors and Fibrin Glue Implanted Autologous Bone Marrow-Derived Mesenchymal Stem Cells.

The study aimed to investigate whether a 3D printed beta-tricalcium phosphate (ß-TCP) scaffold tethered with growth factors and fibrin glue implanted autologous bone marrow-derived mesenchymal stem cells would provide a 3D platform for bone regeneration resulting in new bone formation with plasticity. Twenty 3D printed ß-TCP scaffolds, ten scaffolds engrained with osteogenic mesenchymal stem cells with fibrin glue (group A), and ten scaffolds used as a control group with ß-TCP scaffold and fibrin glue inoculation only (group B) were included in the study. Cell infiltration, migration, and proliferation of human osteogenic stem cells on the scaffolds were executed under both static and dynamic culture conditions. Each scaffold was examined post culture after repeated changes in the nutrient medium at 2, 4 or 8 weeks and assessed for opacity and formation of any bone-like tissues macroscopic, radiographic, and microscopic evaluation. Significant changes in all the prerequisite parameters compiled with an evaluated difference of significance showing maxillofacial skeletal repair via tissue engineering by ß-TCP scaffold and MSCs remains will be the most promising alternative to autologous bone grafts and numerous modalities involving a variety of stem cells, growth factors from platelet-rich fibrin.

Adolescent and Young Adult Oral Maxillofacial Tumors: A Single-Institution Case Series and Literature Review.

Adolescent and young adult (AYA) oral maxillofacial tumors are rare and account for ∼12% of all AYA cancers. Due to the low incidence of these malignancies, diagnostic considerations, therapeutic approaches, and factors affecting prognosis have been difficult to characterize. Given the anatomic structures located within the head and neck, patients are at risk for treatment-related morbidity that may adversely impact their quality of life. We present a single-institution case series of AYA patients with oral maxillofacial tumors treated at the University of Illinois at Chicago. A multidisciplinary treatment approach, including collaboration with the Oral Maxillofacial Surgery, Dentistry, and the Ear, Nose, and Throat teams along with the utilization of Children's Oncology Group treatment protocols, can serve as a model to address the challenges in the management of these complex cases.

Comparative Study of Mandible Ramus Morphology Using 3-dimensional CT in Sagittal Split Ramus Osteotomy.

The purpose of the present study was to investigate the relevance of the external morphology of the mandibular ramus, internal bone tissue structure, and maxillofacial morphology at the site for sagittal split ramus osteotomy among different facial skeletal patterns. A total of 80 patients with jaw deformities who underwent sagittal split ramus osteotomy were included in the study. The patients were divided into two groups based on facial skeletal type (skeletal Class II or skeletal Class III). A further 7 patients with no skeletal abnormalities were established as the control group (skeletal Class I). Computed tomography images obtained from these patients were 3-dimensionally reconstructed and the morphology of the mandibular ramus determined. Thickness at the mandibular foramen in Class II was greater than that in Class III, and showed the lowest value at the midpoint of the mandibular foramen and mandibular notch in Class I. Mandibular morphology showed change according to facial skeletal type. Correlations were also observed between the cephalometric analysis values and mandibular morphology.

[Diagnostic and therapeutic interest of studying the nose in maxillo-dento-facial orthopedics]. / Intérêt diagnostique et thérapeutique de l'étude du nez en orthopédie dento-maxillo-faciale.

INTRODUCTION: Orthodontists have long tried to predict future growth. It is one of the most difficult goals to achieve precisely despite the different methods of growth forecasting. A simple technique based on clinical and radiological analyses of the nose and premaxilla makes it possible, using no measurements, to accurately predict future maxillary growth and to deduce the therapeutic indications. A morphologic study of the nose is also an important item in the diagnosis of cranio-facial syndromes. MATERIALS AND METHODS: Combining detailed semiologic and radiologic studies of the nasal and premaxillary structures, this article proposes a method for evaluating and predicting facial growth. RESULTS: Experience based on many observations and current embryological knowledg can detect growth abnormalities of the ethmoïdo-nasal-premaxillary unit and provide valuable therapeutic information. DISCUSSION: Combining clinical and radiologic analyses of nasal and premaxillary morphology is a good method to predict growth of the upper face. It is also an important feature in the diagnosis of cranio-facial syndromes. CONCLUSION: This technique should be included in the diagnosis of maxillo-dento-facial orthopedic cases.

Diagnosis and Treatment of Gorham-Stout Disease in Maxillofacial Regions.

PURPOSE: This study was aimed to investigate the clinical features, imaging examination, and treatment of the patients with Gorham-Stout disease (GSD) in maxillofacial region, so as to improve the understanding of GSD. METHODS: The medical records of the patients with GSD who were referred to Shanghai Ninth People's Hospital from January 2010 to May 2016 were reviewed. Their ages, lesion location, imaging results, laboratory examination results, treatment, and therapeutic effects were analyzed. RESULTS: A total of 4 cases were included (males 2, females 2). The average onset age was 40 years. GSD attacked the mandible in 2 cases; mandible and temporal bone in 1 case; and mandible, temporal bone, and zygoma in 1 case. All cases were examined by computed tomography (CT), which showed bone resorption and atrophy of soft tissue in involved region. Four patients were given alendronate for treatment. All of them had no significant signs of progress after treatment. CONCLUSIONS: GSD can affect one single bone or multiple bones in maxillofacial region. The diagnosis mainly depends on the imaging examinations. Enhanced CT or magnetic resonance imaging is advocated for differential diagnosis of this disease. Alendronate was used with apparent good effect in these patients.

Perfil epidemiológico dos pacientes portadores de fissuras labiopalatinas atendidos por equipe cirúrgica de referência no Estado do Amazonas / Epidemiological profile of patients with orofacial cleft treated by a reference surgical team in the State of Amazonas, Brazil

Introdução: As fissuras labiopalatinas são malformações congênitas e, no Brasil, estima-se a ocorrência de 1:650 nascimentos. A classificação adotada é a de Spina. A queiloplastia e a palatoplastia são as principais cirurgias executadas. Métodos: Estudo retrospectivo descritivo com obtenção de dados a partir do sistema Smile Train Express referente a pacientes com fissura labiopalatina atendidos por equipe cirúrgica de referência entre 1 de março de 2014 e 1 de dezembro de 2016. Resultados: Foram identificados 477 pacientes, predominando o sexo masculino e os dois primeiros anos de vida na admissão. A fissura mais prevalente foi transforame e unilateral esquerda. O tratamento cirúrgico mais frequente foi a queiloplastia. Conclusões: O padrão epidemiológico está em consonância com a literatura nacional.

Introduction: Orofacial clefts are congenital malformations with an estimated occurrence of 1:650 births in Brazil. The most widely adopted classification system in that country is the method developed by Spina, and cheiloplasty and palatoplasty are the main surgeries performed. Methods: This was a retrospective descriptive study using data collected from the Smile Train Express organization regarding patients with orofacial clefts treated by a reference surgical team between March 1, 2014 and December 1, 2016. Results: A total of 477 patients were identified, predominantly male and in the first two years of life at admission. The most prevalent type of malformation was left unilateral transforamen cleft. The most frequent surgical treatment was cheiloplasty. Conclusions: The epidemiological pattern is consistent with the findings described in the national literature.

Influence of low-level laser therapy on the healing of human bone maxillofacial defects: A systematic review.

PURPOSE: This systematic review evaluates the effectiveness of low-level laser therapy (LLLT) to enhance maxillofacial area bone repair. METHODS: A comprehensive search of studies published up to February 2017 and listed in PubMed/MEDLINE, Scopus, and Cochrane Library databases was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. RESULTS: The 15 selected studies evaluated a total of 374 patients (mean age, 28.5years) who were treated with LLLT. Gallium-arsenide (GaAs) and gallium aluminium arsenide (GaAlAs) were the most commonly used devices, and LLLT parameters varied greatly. Wavelengths varied from 500 to 1000nm. Tooth extraction, distraction osteogenesis, maxillary expansion, periodontal defects, orthodontic movement and maxillary cystic defects were evaluated. From the 15 selected studies, six evaluated bone repair (primary outcomes). Of these, four studies showed improvement in bone formation after using LLLT, two demonstrated improved results for only one follow up period, and one showed no additional benefits. The other 9 studies evaluated secondary parameters related to healing (secondary outcomes) in the maxillofacial area after applying LLLT, including anti-inflammatory, analgesic, and healing accelerator effects, and quality of life related to oral health. There were no adverse or negative effects of LLLT reported. CONCLUSION: Within the limitation of this review, a possible improvement in bone density can be found when LLLT is applied postoperatively in maxillofacial bony defects. LLLT also seems to promote anti-inflammatory and analgesic effects and accelerate healing, as well as enhance quality of life related to oral health. However, LLLT use protocols need to be standardized before more specific conclusions can be drawn about this subject.

Characteristics of a novel treatment system for linear accelerator-based stereotactic radiosurgery.

The purpose of this study is to characterize the dosimetric properties and accuracy of a novel treatment platform (Edge radiosurgery system) for localizing and treating patients with frameless, image-guided stereotactic radiosurgery (SRS) and stereotactic body radiotherapy (SBRT). Initial measurements of various components of the system, such as a comprehensive assessment of the dosimetric properties of the flattening filter-free (FFF) beams for both high definition (HD120) MLC and conical cone-based treatment, positioning accuracy and beam attenuation of a six degree of freedom (6DoF) couch, treatment head leakage test, and integrated end-to-end accuracy tests, have been performed. The end-to-end test of the system was performed by CT imaging a phantom and registering hidden targets on the treatment couch to determine the localization accuracy of the optical surface monitoring system (OSMS), cone-beam CT (CBCT), and MV imaging systems, as well as the radiation isocenter targeting accuracy. The deviations between the percent depth-dose curves acquired on the new linac-based system (Edge), and the previously published machine with FFF beams (TrueBeam) beyond D(max) were within 1.0% for both energies. The maximum deviation of output factors between the Edge and TrueBeam was 1.6%. The optimized dosimetric leaf gap values, which were fitted using Eclipse dose calculations and measurements based on representative spine radiosurgery plans, were 0.700 mm and 1.000 mm, respectively. For the conical cones, 6X FFF has sharper penumbra ranging from 1.2-1.8 mm (80%-20%) and 1.9-3.8 mm (90%-10%) relative to 10X FFF, which has 1.2-2.2mm and 2.3-5.1mm, respectively. The relative attenuation measurements of the couch for PA, PA (rails-in), oblique, oblique (rails-out), oblique (rails-in) were: -2.0%, -2.5%, -15.6%, -2.5%, -5.0% for 6X FFF and -1.4%, -1.5%, -12.2%, -2.5%, -5.0% for 10X FFF, respectively, with a slight decrease in attenuation versus field size. The systematic deviation between the OSMS and CBCT was -0.4 ± 0.2 mm, 0.1± 0.3mm, and 0.0 ± 0.1 mm in the vertical, longitudinal, and lateral directions. The mean values and standard deviations of the average deviation and maximum deviation of the daily Winston-Lutz tests over three months are 0.20 ± 0.03 mm and 0.66 ± 0.18 mm, respectively. Initial testing of this novel system demonstrates the technology to be highly accurate and suitable for frameless, linac-based SRS and SBRT treatment.

[Upper eyelid congenital coloboma, aethiology pathogenesis and management]. / Colobome congénital de la paupière supérieure, étiologie physiopathologie et prise en charge.

Coloboma of the upper eyelid is a rare congenital disease. It is defined as an agenesis of the eyelid free edge. Multiple patho-physiological theories exist about its etiology. The surgical therapeutic management of this malformation is clearly codified. We illustrate the presentation by two cases treated in the Groupement Hospitalier Nord of Lyon University Hospital.

Guilty as CHARGED: p53's expanding role in disease.

Unrestrained p53 activity during development, as occurs upon loss of the p53 negative regulators Mdm2 or Mdmx, causes early embryonic lethality. Surprisingly, co-expression of wild-type p53 and a transcriptionally-dead variant of p53, with mutations in both transactivation domains (p53(L25Q,W26S,F53Q,F54S)), also causes lethality, but later in gestation and in association with a host of very specific phenotypes reminiscent of a syndrome known as CHARGE. Molecular analyses revealed that wild-type p53 is inappropriately activated in p53(5,26,53,54/)(+) embryos, triggering cell-cycle arrest or apoptosis during development to cause CHARGE phenotypes. In addition, CHARGE syndrome is typically caused by mutations in the CHD7 chromatin remodeler, and we have shown that activated p53 contributes to phenotypes caused by CHD7-deficiency. Together, these studies provide new insight into CHARGE syndrome and expand our understanding of the role of p53 in diseases other than cancer.

Clinical and radiological presentation of hemimaxillofacial dysplasia/segmental odontomaxillary dysplasia: critical analysis and report of a case.

OBJECTIVES: The purpose of the present study was to critically evaluate the literature, describe the clinical and radiographic features of HD-SOD (hemimaxillofacial dysplasia/segmental odontomaxillary dysplasia), and describe one new case, involving a 12-year-old boy, with detailed radiological, clinical, and histologic characteristics. METHODS: Thirty-six cases published between 1987 and 2010, together with the present case, were evaluated according to criteria that included gender, age, location of the lesion, findings, and symptoms. RESULTS: We found that the lesion is discovered mainly in the first decade of life (71%), and has a male predilection (64%, 23/64). The maxillary alveolar process was affected unilaterally in all cases, with gingival and bone enlargement and facial asymmetry being constant findings. Missing premolars and skin manifestations were found to be common features. CONCLUSIONS: New case reports should include clinical, radiographic, and histologic findings; follow-up reports; and treatment protocols to improve dentist and parent information regarding HD.

Hereditary gingival fibromatosis with distinctive facies.

Hereditary gingival enlargement also known as gingivitis or familial elephantiasis is a rare type of gingival enlargement. It appears as an isolated autosomal dominant disorder or maybe associated with other conditions. Oral manifestations may vary from minimal involvement of only tuberosity area and the buccal gingiva around the lower molars to a generalized enlargement inhibiting eruption of the teeth. This paper discusses the case of a 13-year-old female patient with distinctive facial characteristics who presented to the department with a chief complaint of swollen gums since 1 year. She had severe diffuse gingival enlargement of the maxilla and mandible. Diagnosis was made based upon clinical examination and family history. Quadrant wise internal bevel gingivectomy procedure was done for the patient to restore her functional and esthetic needs.

Comparison of facial soft tissue measurements on three-dimensional images and models obtained with different methods.

AIM: The aim of this study was to compare the clinical facial soft tissue measurements with the measurements of facial plaster cast, three-dimensional scanned facial plaster cast, 3-dimensional digital photogrammetrical images, and three-dimensional laser scanner images. MATERIALS AND METHODS: Three-dimensional facial images of 15 adults were obtained with stereophotogrammetry and a three-dimensional laser scanner. Facial models of subjects were obtained using silicone impression and were scanned. Landmarks were marked on the subjects and plaster casts, digitized on three-dimensional models, and measured in Mimics 12.0 software (Materialise's Interactive Medical Image Control System, Leuven, Belgium). RESULTS: No statistically significant differences were found between all three-dimensional measurement methods in mouth width, philtrum median height, and nasal width. Comparison of clinical measurements with facial plaster cast measurements revealed that philtral width, nasal tip protrusion, and right lip and nostril heights were wider and longer in clinical measurements than in facial plaster cast measurements. Comparison of clinical measurements to the laser scanned and stereophotogrammetric model measurements revealed that philtrum lateral and lip heights and philtral width were significantly different between methods. When laser scanned and stereophotogrammetric measurements were compared, significant differences were observed in lip and nostril heights. CONCLUSIONS: Facial impression may be problematic owing to the depression caused by the impression material especially on the tip of the nose. Laser scanning is not sensitive enough to visualize the deeper indentations such as nostrils. Stereophotogrammetry is promising for three-dimensional facial measurements and even will be better when color identification between mucocutaneous junctions of the lip region is achieved.

A classification and construction of congenital lateral facial clefts.

BACKGROUND: The repair of the lateral or transverse facial cleft is a surgical challenge on the account of the abnormal positioning and appearance of the cleft. MATERIALS AND METHODS: Over a twenty-seven year period, 22 lateral facial cleft cases were evaluated at a cleft lip (CL) and palate clinic and seven children underwent reconstruction of the lateral CL. RESULTS: Twenty-two of 3187 (0.69%) cases presented with a lateral CL. Five of these 22 cases (23%) had a bilateral, eight (36%) had a right-sided and nine (41%) had a left-sided cleft. The evaluation of these cases resulted in a new classification (namely an extension of the Tessier 7 cleft) classification for the cutaneous and muscle involvement: a superior (T7.1), middle (T7.2), inferior (T7.3) and agenetic (T7.4) lateral CL. The altered surgical construction: an internal mucosal straight-line closure, a curved cutaneous-mucosal red-lip/vermilion-lined flap for the lip commissure, muscle reconstruction at the modiolus and a positional cutaneous z-plasty for the rare lateral cutaneous cleft. CONCLUSION: The paper introduced a new classification for the lateral CL, as well as an altered surgical reconstructive technique for the most natural functioning of the lateral part of the face.

Maxillofacial mass as the first presentation of acute lymphoblastic leukemia in a nine-year-old girl.

A nine-year-old girl complained of a maxillofacial mass and mild fever lower than 37.5 degrees C. A solid mass was detected in the right maxillary fossa by CT and sonographic examination, and the tumor extended backward into the infratemporal fossa without bony destruction. Laboratory studies showed a white blood cell (WBC) count of 31.6x10(9)/L, which increased progressively to 50.0x10(9)/L over the next several days. Peripheral blood smear revealed 92% lymphoblast with uneven bulk. A biopsy through the right labiogingival groove disclosed a submucosal homogeneous mass. Histologic examination under the microscope revealed atypical mononuclear cells with obvious atypia and nuclear divisions. Bone marrow puncture showed atypical lymphocytes with active proliferation. Immune phenotypic analysis by flow cytometry showed that malignant cells were positive for CD19, CD34, HLA-DR, CD38, CD22, CD58, CD45 and CD56. Real time RT-PCR revealed positive BCR/ABL arrangement. High-risk ALL was diagnosed and four stages of chemotherapies were given for nearly three years. After the induction of the chemotherapy, the facial mass disappeared. A bone marrow biopsy revealed that CR had been achieved since a month after chemotherapy's onset. The initial presentation of ALL as a maxillofacial mass is exceedingly rare. To the best of the authors' knowledge, this is the first reported case of ALL presenting as a maxillofacial lesion.

Impact of nonhybrid 99mTc-MDP-SPECT/CT image fusion in diagnostic and treatment of oromaxillofacial malignancies.

PURPOSE: The aim of this study was to prospectively investigate the clinical impact of image fusion of computed tomography (CT) and single photon emission computed tomography (SPECT) diagnostics in head and neck cancer adjacent or fixed to bony maxillofacial structures. PROCEDURES: Computer-based manual image fusion has been applied in 74 patients with suspected malignancies in the oromaxillofacial region following CT and SPECT. Afterward, image fusion was compared to separate evaluation of CT and SPECT and visual coregistration with histopathological results serving as control. RESULTS: In nine out of 74 patients, image fusion achieved more precise anatomical findings regarding tumor dimension than simultaneous evaluation of CT and SPECT, but there was no improvement of identification of bone infiltration. CONCLUSION: Manual image fusion of CT and SPECT does not improve identification of bone infiltration compared to simultaneous evaluation. Though particularly in complex anatomical regions, a fixed preoperative diagnostic algorithm in image registration lead to a more precise evaluation and treatment of head and neck malignancies.

Facial metrics in children with corticotrophin-producing pituitary adenomas suggest abnormalities in midface development.

BACKGROUND: Tumors of the hypothalamic-pituitary unit have been linked to genetic syndromes that are associated with midfacial abnormalities. AIM: We hypothesized that mutations of genes that affect the development of the face (and consequently of the anterior pituitary) may be present in children with ACTH-producing pituitary adenomas, and if this is true then facial measurements would be different from those predicted by parental features. METHODS: We studied 20 children with corticotropinomas and a control group and their parents. All facial measurements were expressed according to standard deviation scores. RESULTS: Significant differences were seen between the children with pituitary adenomas and their parents for vertical facial height measures: nasal length (p < 0.001), lower facial height (p < 0.03) and overall facial height (p < 0.01). CONCLUSION: We conclude that some of the indices of midline craniofacial development, in particular those affecting the vertical axis, are different in children with corticotroph adenomas producing ACTH.

The role of computer-aided 3D surgery and stereolithographic modelling for vector orientation in premaxillary and trans-sinusoidal maxillary distraction osteogenesis.

BACKGROUND: Maxillary distraction osteogenesis is a challenging procedure when it is performed with internal submerged distractors due to obligation of setting accurate distraction vectors. METHODS: Five patients with severe maxillary retrognathy were planned with Mimics 10.01 CMF and Simplant 10.01 software. Distraction vectors and rods of distractors were arranged in 3D environment and on STL models. All patients were operated under general anaesthesia and complete Le Fort I downfracture was performed. RESULTS: All distractions were performed according to orientated vectors. All patients achieved stable occlusion and satisfactory aesthetic outcome at the end of the treatment period. CONCLUSION: Preoperative bending of internal maxillary distractors prevents significant loss of operation time. 3D computer-aided surgical simulation and model surgery provide accurate orientation of distraction vectors for premaxillary and internal trans-sinusoidal maxillary distraction. Combination of virtual surgical simulation and stereolithographic models surgery can be validated as an effective method of preoperative planning for complicated maxillofacial surgery cases.

Hutchinson-Gilford progeria syndrome: oral and craniofacial phenotypes.

OBJECTIVE: Hutchinson-Gilford progeria syndrome (HGPS) is a rare early-onset accelerated senescence syndrome. In HGPS, a recently identified de novo dominant mutation of the lamin A gene (LMNA) produces abnormal lamin A, resulting in compromised nuclear membrane integrity. Clinical features include sclerotic skin, cardiovascular and bone abnormalities, and marked growth retardation. Craniofacial features include 'bird-like' facies, alopecia, craniofacial disproportion, and dental crowding. Our prospective study describes dental, oral soft tissue, and craniofacial bone features in HGPS. METHODS: Fifteen patients with confirmed p.G608G LMNA mutation (1-17 years, seven males, eight females) received comprehensive oral evaluations. Anomalies of oral soft tissue, gnathic bones, and dentition were identified. RESULTS: Radiographic findings included hypodontia (n = 7), dysmorphic teeth (n = 5), steep mandibular angles (n = 11), and thin basal bone (n = 11). Soft tissue findings included ogival palatal arch (n = 8), median sagittal palatal fissure (n = 7), and ankyloglossia (n = 7). Calculated dental ages (9 months to 11 years 2 months) were significantly lower than chronological ages (1 year 6 months to 17 years 8 months) (P = 0.002). Eleven children manifested a shorter mandibular body, anterior/posterior cranial base and ramus, but a larger gonial angle, compared to age/gender/race norms. CONCLUSION: Novel oral-craniofacial phenotypes and quantification of previously reported features are presented. Our findings expand the HGPS phenotype and provide additional insight into the complex pathogenesis of HGPS.